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It has been well established that stress is one of the leading factors of chemical changes in the body. Persons who suffer from high anxiety are more prone to depression, disrupted sleep, heart disease and a kaleidoscope of other health disorders. In the case of those persons suffering from a plethora of health conditions, aromatherapy massage and massage have been shown to alleviate stress and anxiety. While these natural healing techniques may not always cure disease, they can have a remarkable effect on the physical body.What many individuals do not realize is that stress is the leading proponent in raising blood sugar levels and blood pressure, red blood cell over activity, constricted blood vessels, racing pulse, and digestive problems.[4] These facts prove that stress can have a very negative impact on overall health.In a report by the Research Institute of Nursing Science (College of Nursing Seoul National University, Seoul, South Korea)[1], aromatherapy massage was studied on elderly women in Korea to determine its efficacy on anxiety and self-esteem issues. In the study, aromatherapy massage was administered using aromatic essential oils, such as chamomile, lavender, lemon and rosemary. Overall, the study lasted seven (7) weeks in duration. During that time frame, aromatherapy massage sessions were set three (3) times per week, with each session being 20 minutes. After the initial three-week period, a one-week intermission ensued, and then continued the remaining three weeks. While blood pressure and pulse rate were not significantly affected, remarkable differences were clearly established in regard to anxiety and self-esteem. In another study, aromatherapy massage was administered to older patients suffering from constipation. In the 10-day study, the experimental group was given abdominal aromatherapy massage; using the essential oils lemon, peppermint and rosemary. The control group, too, was given massage; but without the aid of aromatherapy. Upon commencement of the study, levels of constipation were measured by the constipation assessment scale (CAS). While the results found that the average number of bowel movements had improved in both groups (being treated with both aromatherapy massage and placebo massage); the experimental group who had received the aromatherapy massage showed a significant improvement. Purportedly, the aromatherapy massage demonstrated that its effects lasted for two (2) weeks after treatment; whereas non-aromatherapy massage lasted approximately seven to ten days (7-10). In conclusion, the study found that aromatherapy, coupled with massage does help ease geriatric constipation.[2] Other research suggests that both aromatherapy massage and massage are beneficial to persons suffering from cancer. In a study performed by Princess Alice Hospice in the UK, patients were administered both aromatherapy massage and massage alone during a four-week course. To assess its efficacy on physical and psychological symptoms, both techniques were randomly administered to patients with advanced stages of cancer. The findings were promising, as significant sleep patterns were enhanced in both the aromatherapy massage group and the massage-alone group. In addition, depression levels were statistically significant in the massage group. Overall, the study showed that patients who had heightened levels of distress were more likely to respond to both aromatherapy massage and massage alone.[3]In conclusion, we have learned that stress-related disorders and health complications can be significantly reduced and/or alleviated by natural healing techniques such as aromatherapy massage, massage and other noninvasive health treatments. References:Effects of aromatherapy massage on anxiety and self-esteem in Korean elderly women: a pilot study. - Int J Neurosci. 2006 Dec; 116(12):1447-55.Effect of aromatherapy massage for the relief of constipation in the elderly - Taehan Kanho Hakhoe Chi. 2005 Feb;35(1):56-64A randomized controlled trial of aromatherapy massage in a hospice setting. - Palliat Med. 2004 Mar;18(2):87-92Chronic Stress Affects both Mind and Body: The Health Effects of Stress and Increased Cortisol (http://www.stress-and-health.com/)

The prostate is a walnut sized gland that sits just below the bladder in men and is an integral part of the male reproductive system. Made up of two lobes and enclosed by a layer of tissue, the prostate goes through two main periods of growth. The first occurs early in puberty, when the prostate doubles in size. At around the age of 25, the gland begins to grow again. This second growth phase often results in what is identified as an enlarged prostate. As the prostate gets larger, the layer of tissue surrounding it stops it from expanding, causing the gland to press against the urethra. While the data varies, it is believed that most men over the age of 45 experience some amount of prostate enlargement, but may live symptom free. This enlargement is usually harmless, but it often results in problems urinating later on in life. By 60, it is believed that 80% of all men experience some sort of urinary interference due to prostate enlargement. Enlargement of the prostate is not a malignant condition, but it does put pressure on the urethra and can create a number of urinary complaints such as frequent urination, urinary urgency, the need to get up at night to urinate, difficulty starting, a reduction to the force of the urine stream, terminal dribbling, incomplete emptying of the bladder and even the inability to urinate at all. If left unchecked, benign prostatic hypertrophy can cause serious problems over time including urinary tract infections, bladder or kidney damage, bladder stones or incontinence. It is important to take care of your prostate and address any prostate, be it an enlarged prostate, prostetitis (inflammation of the prostate) or prostate cancer early on. Take an active role and protect yourself by having your prostate checked regularly. Traditional treatments for prostate issues include the surgical removal of all or part of the prostate. While most people experience a relief of symptoms, it may leave them impotent. For the health conscious, this should only be used as a last resort. Recommendations For WellnessIf you are over 40, have your doctor perform a prostate exam during your regular checkup. Incorporate a healthful diet filled with lots of fruits and vegetables and low on unsaturated fats, sugars and processed foods. Kegel exercises can be done to improve circulation to these tissues. A kegel exercise is performed by pulling up all the muscles around the scrotum and anus. Repeat this movement 10 times. Try doing this “invisible” exercise 5-6 times daily. For men suffering from prostate enlargement, saw palmetto is the place to start. Saw palmetto has a long history of relieving testicular inflammation and inhibiting the hormone responsible for prostate enlargement. Zinc has also been used for decades to support a healthy prostate. The herbs nettle and pygeum have also been show to be effective for relieving symptoms associated with an enlarged prostate. They can be used alone or in conjunction with saw palmetto. Perhaps look for an herbal combination that contains pygeum, saw palmetto, gotu kola, stinging nettle and zinc – this would an ideal mix for anyone with prostate issues. Incorporate the herb damiana into your wellness program. Damiana has a long history of helping balance the body’s hormones. Hydrangea is another herb that supports the urinary system, where it is know to promote the overall health of the prostate gland and help to reduce inflammation and swelling. Take antioxidants. Supplementation with antioxidants (vitamins A,C, E and selenium), such as those found in
Defense Maintenance or super Antioxidant will help to protect these sensitive tissues from oxidative damage.

Is drugging our children the answer or could a natural supplement help? Simple diet changes and vitamin supplementation can help reduce Attention Deficit Hyperactivity Disorder (ADHD) in children.
Attention Deficit Hyperactivity Disorder (ADHD) has been a commonly diagnosed illness occurring in children since the 1980s. Many people argue that ADHD should not be categorized as a disorder, but rather a set of problems that are normal to childhood. Either way, ADHD’s symptoms consist of wandering attention, nervousness, and hyperactivity. These symptoms can be extremely troubling for the parents and teachers that have to work with children suffering from ADHD. Drugs like Ritalin have been promoted ADHD treatments for many years, but have been found to have highly dangerous side effects and not be a solution to everything. Meanwhile, many nutritionists have discovered that many of the answers to ADHD can be found in a child’s diet in the form of food additives, sugar, and the missing essential nutrients. One child who was horribly hyperactive, out of control, and diagnosed with ADHD by a school psychologist was taken off any foods that contained a particular red dye. Almost immediately, the child’s hyperactivity ceased and he began paying attention and living the life of a normal child. A study recently completed at Yale University School of Medicine supported this result, showing a direct connection between food additives and hyperactivity. This study consisted of 297 children who were given drinks that contained common artificial food colorings and additives that are usually found in food and candy. Meanwhile, a control group was given drinks without additives. The children who drank the additive-enhanced drinks showed a significantly greater amount of hyperactivity and had shorter attention spans. Another common cause of ADHD symptoms in children is overdosing of sugar. With all the snack-like breakfast cereals available and the high sugar-constant treats, hyperactivity should be expected. Once a child consumes a large quantity of sugar, he will hit a high of out-of-control hyperactivity and then quickly become grouchy and prone to tantrums. This is often called a “sugar rush” and can easily be solved by taking children off sugar. After removing chemical additives and processed sugar from the diet, ADHD symptoms have also been traced to a lack of mineral magnesium. This nutrient is essential and often missing from most diets today. Kids who drink a lot of soda or fruit juices are getting high amounts of sugar but not magnesium. Those children without magnesium often suffer from irritability, insomnia, and constipation.
These symptoms, which are commonly diagnosed as ADHD, can be cleared up quickly by putting your child on a magnesium supplement. Most nutritionists agree, believing that many ADHD symptoms come from a high sugar diet and a magnesium deficiency. Sugar and over-stimulation, such as stress, actually exhausts a child’s nervous system. This can be cured with a magnesium supplement which allows the nervous system to rebuild. In addition, this supplement will assist the child in relaxing. Since children are the future for our society, helping them to survive in the best way possible, naturally, is something each and every one of us should take much more responsibility for. Magnesium and other natural supplements can be found at your local pharmacy or department store.
While most in the scientific community continues to believe that medication alone, or medication in combination with intensive behavioral therapy is superior to other types of treatment, growing concerns about the drugs, addictive and zombie-like qualities have parents searching for alternative approaches. In an attempt to embrace these concerns, nutritionists and naturopaths, having gathered an abundance of anecdotal evidence, are pointing toward dietary changes and natural supplementation as an alternative option to medication. “We have seen a number of hyperactive children dramatically change behavior when their parents instituted a comprehensive program that included herbs, a change in diet, avoiding potential allergic substances and counseling, usually for the whole family,” said Kathi Keville and Peter Korn in Herbs for Health and Healing.

Habits: so easy to develop but so hard to break. As a hypnotherapist, I work with many people trying to break a habit that they’ve had for years and some, even decades. As I tell my clients during the first session - in order to change a habit, you first must understand it. I’ve come to believe that all habits start with a basic desire to emulate somebody else. As a teenager, most of us want to be a “carbon copy” of the person we idolize. Then as we become older, advertisers with a false belief system bombard us through print and electronic media on a daily basis, subjecting us to a way of life as they see it (or would like us to see it). As an example consider smokers, before smoking advertisements on T.V. and in magazines were banned (Canada); we were subjected to various images of peace and serenity with cigarettes - the ultimate relaxation. How many of us still remember the lone cowboy sitting on his horse in the wild country smoking his cigarette? That advertisement hasn’t been around since the 70’s, but the majority of us still sees and remembers the image. If you ask smokers today why they smoke, they’d most probably tell you “it relaxes me”, which is anything but the truth. Even if you show them studies proving nicotine stimulates rather than relaxes the body, the false belief that advertisers have given us will inevitably win out, its so imbedded into our subconscious. Even dieters are seduced with images of looking younger and being idolized if they buy and use a certain piece of athletic equipment or eating a certain diet product: all false beliefs. And don’t forget the kids. Turn to any children’s program on a Saturday morning and you’ll see images of happy, ideal families playing together, all by just from eating certain fast foods. The truth is, advertisers feed on the idea that the two things every living creature wants are to be loved and to fit in, which according to the advertisers can all be obtained through the purchase of their product. Yeah right, as if that was only true. The first step for permanent change for a habit is to examine your own core beliefs. This can easily be accomplished by the following method. On a sheet of paper, put down the heading WHY. Now, being honest with yourself, why do you do those things (habits) that you want to change….why do you smoke?…why do you overeat? Do you smoke to relax? Do you overeat to feel “good” about yourself? List the reasons and when finished, examine them. You’ll likely see a false belief imposed upon you by some advertiser from the past. The second step for permanent change is to understand that habits are nothing more than the linking up of two or more actions, together. As science has shown us, doing the same actions for 21 days or more in a row, establishes that pattern into our subconscious as a habit. The secret for change is not to eliminate that habit (and leaving a void), but to change those actions that are bad for us and to replace them with actions that are better for us. When smokers take a break and smoke, those two actions establish themselves as a habit, i.e. taking a break and smoking. You certainly wouldn’t want to eliminate the break but you would want to eliminate the action, smoking. To determine your actions, I advise my clients to label a second sheet WHERE. Put down the places where you do those actions (the habits you want to change). As examples…do you smoke/overeat after work, do you smoke/overeat in the car, do you smoke/overeat in front of the T.V?….just list those places where you perform that habit that you want changed. Along the same line, start another sheet, labeling it WHEN. Now on this sheet, list the times when you do that habit. Do you smoke/overeat when feeling stressed? Do you smoke/overeat when you have nothing better to do? Again, listing as many whens as possible. Now in those short steps you’ve examined your WHY, WHERE and WHENs for your habit. You’re now starting to understand your habit. Now comes the time to create a concrete action plan to change those “bad” actions you’ve identified. I tell people that you wouldn’t plan a car trip without first planning which correct routes to take. It’s the same for changing habits, mapping out actions which will help you reach your goal. As stated earlier, you can’t eliminate habits or actions by will power alone. The void you create will last only for a few days, then you’re back to the same problem you had (yo-yo dieters will tell you this!). Will power just doesn’t work for the majority of us.
Go back to the three sheets you started (WHY, WHERE and WHEN) and under the list you created for each one, put down a sub-title labeled OPTIONS. Create new actions (options) that are doable for you. As an example, lets say you said on the WHY sheet you smoked to relax. Find an alternative action to replace that action, smoking, but still get the benefit. Maybe you’ll go for a walk instead. But it’s important to make the actions doable. Don’t say you’re going to run a marathon if you’ve never ever run before. Otherwise you’ll become disillusioned and defeated before you start. But it is OK to say that each night you’re going to go for a walk in order for you to build strength and stamina so you can work up to running and then doing a marathon in the future, if that action is of interest to you. Do the same for your WHERE and WHEN sheets. Create new options of interest to replace that “bad” action (smoking, overeating etc) with an action that will benefit you. Now I’ve used smoking and overeating as examples only but the same plan will work for any habit that you want to change. Examine it, understand it and then create an action plan. By doing all the above, you’re more than halfway to creating a new you.

Hi readers ,

i hope you’ve learnt  alot about the topic Amyotrophic lateral sclerosis, before i finally close up on this topic i will like to make some conclusion and recommendation .

The previous article might have x-rayed vividly the general aspect of amyothrophic lateral sclerosis including its aetiology, its pathology, its epidemiology, the assessment as well as its management.

Physiotherapy treatment is of utmost importance for the person with amyothrophic lateral sclerosis, especially exercise. Physical exercise depends on patients’ physiological tolerance and response to exercise. Lateral sclerosis patients can exhibit dysfunction of cardiovascular adjustment accompanied by respiratory involvement, which alter aerobic capacity. These abnormalities tend to increase with the neurological impairment. Muscle weakness is the consequence of not only altered central motor drive but also disuse. This work has shown the benefits of physical therapy, with improvements in aerobic capacity, gait parameters and fatigue, and an influence on quality of life. Regular aerobic physical activity which does not induce fatigue is necessary to maintain the benefit of physical training, and delay the eventualities of lateral sclerosis.

Certain observations have been made as to the particular problem confronting the physiotherapist together with guidelines to treatment and general management. The use of certain techniques has been recommended though it is emphasized that its suitability and application should be the responsibility of the physiotherapist directly concerned.

Perhaps it should also be mentioned that the relationship which develops between the therapist and the patient in the treatment situation, may be a very powerful stimulant to for motivation, quite apart from techniques employed.

Finally, continuing support of various kinds is needed from the relatives so that they do not develop preconceived ideas about the limited abilities of the person living with amyothrophic lateral sclerosis which could lead to little being expected of him and, as a result, he will receive adequate stimulation at this rather demanding state.

RECOMMENDATION

More research needs to be done as per revealing the real cause of this disease. Also, physicians should know that physiotherapy is highly indicated in the management of amyothrophic lateral sclerosis especially in the tropics where the disease is rare. It is hoped that this presentation will stimulate physiotherapist to early and proper diagnosis and management of lateral sclerosis.

The key to exercise testing and prescription is determination of the stage of the disease. Regardless of the stage, care must be taken to prevent overuse fatigue and disuse atrophy, (Dal Bellow-Haas et al., 1998).

Engaging the patient

Engaging patients with amyotrophic lateral sclerosis in an exercise program can be difficult, as many fear that exercise will exacerbate their symptoms. Patients should therefore be informed that all studies that used an exercise intervention in amyotrophic lateral sclerosis reported improved physiological and psychological function, (Wallman, 2004). Importantly, this exercise protocol is based on individual capabilities and is increased only if the patient is coping. A structured exercise protocol may also help prevent amyotrophic lateral sclerosis patients overdoing physical activity and consequently exacerbating symptoms.

Patients should also be informed that exercise has been associated with improvement in physical function, fatigue and mood disorder in other chronic illnesses, such as cancer, cardiac heart failure, multiple sclerosis and fibromyalgia, (Richards, 2002 ) which are both associated with debilitating fatigue, and in which exercise was once considered contraindicated. Finally, aerobic exercise can prolong deconditioning, which would typically further reduce physical capacity and worsen psychological symptoms, (Wessely, 1993).

Individualise the Exercise Prescription

Classification of function is helpful in designing an individualised exercise programme where exercises are selected to coincide with a given functional level. In designing the programme, muscular fitness and physical activity can be considered separately, although the two are not necessarily mutually exclusive.

When muscular fitness is considered, patients with considerable disability may be limited to passive range of motion exercises whereas those with little motor deficit can participate in an integrated strength training programme. Similarly for physical activity, performance of activities of daily living may be adequate exercise for patients with limited function. At the other end of the scale, highly functioning patients may be able to undergo structured aerobic training, (Wallman, 2004).

Preparing for the program

Before beginning any exercise program, patients should be screened. Patients should also be informed that the exercise sessions are in addition to their normal activities, and that some initial aches and pains are usual when beginning exercise for the first time.

Patients should purchase or hire a heart rate monitor, as this will assist in keeping heart rate (beats per minute, [bpm]) constant during exercise sessions. Alternatively, heart rate can be determined by assessing pulse rate.

Patients should also be taught how to determine their ratings of perceived exertion (RPE) using the Borg scale (Borg, 1982). Patients must record their RPE on completion of each exercise session and then average these values each fortnight. The averaged RPE value forms the basis for determining the duration of future exercise sessions. An exercise diary is also important . This allows patients to monitor progress over time and also assists in linking poor performance with a possible emotional or physiological event.

The exercise program

Exercise should be attempted once every second day and should be in a form that uses the major muscles of the body, such as walking, jogging, swimming or cycling. The duration of each exercise session during the first fortnight should be negotiated with the patient, and may range from 1 to 10 minutes, depending on individual physical capabilities. For those already exercising, the duration should be one that the individual is currently coping with consistently.

The intensity of the exercise should represent a pace that the individual can perform comfortably. Importantly, this intensity should be determined on a day when symptom severity is typical, rather than either better or worse than usual. The average peak heart rate when exercising at a comfortable pace on a typical day should be recorded, with this intensity representing the patient’s target heart rate (±3 bpm) for future sessions. The “warm-up” time that it takes for heart rate to reach this target is included in the overall exercise duration.

Program monitoring and modification

Patients should discuss how they coped with the previous session before the next one. If the patient feels that the initial session was too easy (i.e. an overall RPE score of 9 or lower), a slight increase in duration could be considered. Conversely, if the RPE score was greater than 14, then the duration of subsequent sessions for that fortnight should be reduced to a time period that elicits an RPE score of 11–14. It is important that the patient be eased gently into the exercise program.

. If patients coped with the exercise regimen, did not experience a major relapse, and reported averaged fortnightly RPE values of 14 or less, then the exercise duration for the following fortnight should be increased by 2–5 minutes. If the average RPE score was 15 or higher, then the exercise duration should be reduced to a time period that elicits an averaged fortnightly RPE score of 11–14.

The same procedure and recommendations for the first fortnight apply to the next and subsequent fortnights, in that individual target heart rate is kept constant, and RPE scores are recorded after each exercise session and averaged at the end of each fortnight.

Importantly, many CFS sufferers describe fluctuations in their symptoms and capabilities. However, on days that patients feel comparatively well, they must adhere to their current exercise regimen and must not perform any extra exercise above this level. This rule also applies to normal everyday physical tasks, such as housework and gardening.

In addition, on days when symptoms are worse, patients should either shorten the session to a time they consider manageable or, if feeling particularly unwell, abandon the session altogether. They should always endeavour to commence the exercise program again when symptoms subside to a tolerable level. When recommencing exercise, the pace should be comfortable, while the duration should be reduced to a time that the individual feels is manageable and elicits an RPE score of 11–14. Patients should then continue at this modified duration for a fortnight and increase this time period for the subsequent fortnight only if the averaged fortnightly RPE score was 14 or lower.

Finally, if the duration of exercise reaches 30 minutes, patients could consider increasing the intensity of sections of the exercise session.

1 Borg’s Ratings of Perceived Exertion Scale

PROGNOSIS OF AMYOTHROPHIC LATERAL SCLEROSIS

Regardless of the part of the body first affected by the disease, muscle weakness and atrophy spread to other parts of the body as the disease progresses. Individuals have increasing problems such as Delusions/ and or paranoia, swallowing, and speaking or forming words. Eventually people with amyothrophic lateral sclerosis will not be able to stand or walk, get in or out of bed on their own, or use their hands and arms. In later stages of the disease, individuals have difficulty breathing as the muscles of the respiratory system weaken. Although ventilation support can ease problems with breathing and prolong survival, it does not affect the progression of amyothrophic lateral sclerosis. Most people with amyothrophic lateral sclerosis die from respiratory failure, usually within 3 to 5 years from the onset of symptoms. However, about 10 percent of those individuals with amyothrophic lateral sclerosis survive for 10 or more years, (Wikipedia, 2007).

OTHER THERAPIES

An occupational therapist can help design solutions to movement and coordination problems, and provide advice on adaptive devices and home modifications.

Speech and swallowing difficulties can be minimized or delayed through training provided by a speech-language pathologist. This specialist can also provide advice on communication aids, including computer-assisted devices and simpler word boards, (Wikipedia, 2007).

Nutritional advice can be provided by a nutritionist. A person with ALS often needs softer foods to prevent jaw exhaustion or choking. Later in the disease, nutrition may be provided by a gastrostomy tube inserted into the stomach, (Wikipedia, 2007).

amyotrophic lateral sclerosis is a devastating neurodegenerative condition that typically begins with focal muscle weakness and eventually progresses to death from respiratory failure. Although there is no cure for ALS, treatment can improve both the quality and length of life. Care of amyothrophic lateral sclerosis patients is best provided by multidisciplinary amyothrophic lateral sclerosis clinics, (AFP, 2007).

PRINCIPLES OF GENERAL MANAGEMENT

A coordinated multidisciplinary approach is required to meet the rapidly changing physical and psychosocial needs of patients and careers throughout the course of the disease. This should be underpinned by the following principles, (NCPAD, 2007):

• Care encompassing the whole person and those that matter to them.

• Prompt provision of treatments to secure symptom control and quality of life.

• Respect for patient autonomy.

• Open and sensitive communication,

• Planning for the future and timely liaison e.g. with the palliative care team.

MEDICAL MANAGEMENT OF AMYOTROPHIC LATERAL SCLEROSIS

The management of amyothrophic lateral sclerosis is a complex and demanding team effort requiring individualized therapy and continual adaptation of medications and therapies, (AFP, 1999).

Managing amyotrophic lateral sclerosis involves typically focusing on effort to relieve symptoms of and maintain quality of life in the years after diagnosis, and preventing or reducing the rate of complications and maintenance of maximal optimal functions (Caline and Eisen, 2000).Presently no cure has been found for the disease, (ALSA, 2004).

Disease-Modifying drugs include, (AFP, 1999):

The only agent currently labeled for the treatment of ALS is riluzole (Rilutek). At least one other drug (mecasermin) is under consideration by the U.S. Food and Drug Administration. Clinical trials of other drugs are in progress, (AFP, 1999).

Riluzole is believed to decrease glutamate release.(AFP, 1999) One large study, (Lacomblez, 1996) reported that 56.8 percent of patients treated with 100 mg of riluzole daily were alive without tracheostomy after 18 months, compared with 50.4 percent of patients who received a placebo, a clinically small but statistically significant difference. Previously, a smaller study, (Bensimon, 1994) reported a significant improvement in survival, but most of those patients had the bulbar form of amyothrophic lateral sclerosis. Any positive effect on functional abilities from the use of riluzole is unclear, and no studies have reported that the drug halts the disease process, (Wokke, 1996). Adverse effects include asthenia, nausea, dizziness, elevation of liver enzymes and granlocytopenia, (Rowland, 1994).

Symptomatic treatment:

Various symptomatic treatments may be helpful. Frequent, close contact with the patient and the family helps the physician gauge the significance of individual symptoms and the possible benefit of treatment compared with the risk of adverse side effects, (Oliver, 1994)

Spasticity may be relieved by use of baclofen (Lioresal), in a dosage of 10 to 25 mg three times daily, diazepam (Valium), in a dosage of 2 to 15 mg three times daily, or dantrolene (Dantrium), gradually titrated to a dosage of 50 to 100 mg four times daily. Unfortunately, these drugs can increase weakness and cause sedation, dizziness and other adverse effects, (AFP, 1999)

Pain may result from muscle contractures and secondary effects on joints. Muscle cramps occur in almost all patients and may cause severe pain and sleep disturbance. Nonsteroidal anti-inflammatory agents and anticonvulsive medications such as carbamazepine (Tegretol), in a dosage of 200 mg three times daily, or phenytoin (Dilantin), in a dosage of 300 mg at bedtime, may be useful. Early use of amitriptyline (Elavil), in a dosage of 50 to 150 mg at bedtime, or nortriptyline (Pamelor), in a dosage of 50 to 75 mg at bedtime, may potentiate analgesic medications. The traditional treatment of cramps with quinine is no longer recommended because of the risk of reactions, (Hogan,1995) Fasciculations may be reduced by decreasing caffeine and nicotine intake. Lorazepam (Ativan) may relieve severe fasciculations.

Drooling may be one of the most distressing symptoms for patients with bulbar ALS. When excess saliva spills into the airway, bronchospasm can result. Mechanical suction devices are useful in preventing aspiration. Medications that suppress sialorrhea include anticholinergic drugs such as atropine, in a dosage of 0.4 mg four times daily, or scopolamine (Transderm-Scop), one 0.5-mg transdermal patch applied every three days. Some antihistamines, such as diphenhydramine (Benadryl), in a dosage of 25 to 50 mg three times daily, may also be helpful in suppressing sialorrhea, (AFP,1999) Tricyclic antidepressants are widely used in the treatment of amyotophic lateral sclerosis because of their multiple effects. Amitriptyline, in a dosage of 5 to 100 mg at bedtime, can provide antidepressant and antisialorrheic actions as well as nocturnal sedation, potentiation of analgesia and possible weight gain. Doxepin (Sinequan) and imipramine (Tofranil) have similar actions. Tricyclic agents have potential hypotensive, cardiac, sedative and anticholinergic side effects. The selection of agent and dosage requires balancing the desired effects and potential adverse effects.

Depression and anxiety are common in ALS and require individualized therapy. Supportive counseling is appropriate for all patients and their families, and antidepressant medication may also be helpful. An adequate dosage of a tricyclic agent may relieve the patient’s depression, with the advantage that the side effects (dry mouth, sedation and weight gain) actually help to counter symptoms of ALS. Selective serotonin reuptake inhibitors such as fluoxetine (Prozac), in a dosage of 20 mg once or twice daily, are effective but have the potential adverse effects of insomnia and agitation. Benzodiazepines may relieve anxiety and insomnia but can cause daytime sedation, (AFP, 1999).

PHYSICAL THERAPY EVALUATION AND ASSESSMENT

According to Piemonte and Ramirez (2001) assessment allows for classification into three stages for functional dependence (independent, semi-independent and dependent):

Independent stage: Motor ability is preserved, with the patient walking and performing normal daily activities. There is a slight reduction in muscle strength and susceptibility to fatigue. The main aims are to keep motor functioning stable for as long as possible, to avoid muscle retractions and joint deformities, to reeducate about posture and to give guidance on the use of orthoses.

Semi-independent stage: Individuals present difficulty in performing daily activities and the use of wheelchairs is necessary. This is the start of respiratory system involvement, with dyspnea during moderate effort. Stretching, muscle strengthening, torso posture exercises and respiratory kinesiotherapy exercises are recommended. These procedures increase flexibility, reduce cramp, strengthen the musculature and improve the posture.

Dependent stage: Patients require caregivers to assist them in performing day-to-day activities because of the evolution of the symptomatology. Preservation of joint mobility with emphasis on the pelvic and scapular regions, preservation or improvement in control over the torso and neck, respiratory training and postural changes are recommended.

Likewise, given that physiotherapy is so commonly used in amyotrophic lateral sclerosis, understanding, assessing, and managing the unique set of symptoms in each person with amyothrophic lateral sclerosis necessitates careful evaluation which involves the use of SOAP format described by Balogun 1989

S-Subjective data

O-Objective data

A-Assessment

P-Plan.

The subjective procedure involves history taking, the physiotherapist is interested in the cause of the patient’s problem and how the problem affects the patient’s sleep, occupation, social life as applicable.

Objective data are the data that are quantifiable and measurable. Assessment is usually incorporated here.

Examination and Evaluation for Patients With amyothrophic lateral sclerosis

Physical therapist examination and evaluation for patients with ALS throughout the stages of the disease is necessary to plan appropriate treatment programs. The tests include:

Functional status:

The Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS) 32 is used to assess functional status and changes in patients. The evaluator asks the patient (or the caregiver if the patient cannot speak) to rate his or her function for each of the 10 items of the ALSFRS on a scale ranging from 0 (unable to attempt the task) to 4 (normal function). The evaluator questions the patient by asking “How are you doing at …” and prompts the patient by using one of the available choices. Each scale item has criteria related to patient functioning, and the evaluator must have a thorough understanding of each item’s criteria. The Schwab and England Rating Scale (SERS) is an 1 l-point global measure of function that asks the patient (or caregiver) to report amyothrophic lateral sclerosis function from 0% (vegetative functions only) to 100% (normal) by circling the number that best corresponds to the phrase that describes the patient at the moment, this can also be used.

Range of movement:

A full assessment of the passive range of motion in each joint is recorded, noting reasons for any limitation, the goniometer is widely used.

Tonicity:

Alteration of tone may present as hypotonic or hypertonic. The therapist notes the distribution of the abnormal tone and the factors which influence its increase or decrease, such as specific posture or a particular movement.

Reflexes:

Assessment of the reflexes of both the upper and lower motor neurons is also done.

Motor function:

Assessment of motor function in individuals with amyothrophic lateral sclerosis should include impairment and disability measures that can detect both upper motoneuron (UMN) and LMN loss. Quantitative muscle testing for these patients consists of measuring maximal voluntary isometric contractions (MVICs) of the shoulder extensors, elbow flexors, ankle dorsiflexors, knee extensors, and hip flexors using a strain gauge tensiometer system.34 This method eliminates muscle length and contraction speed as factors in testing.”-” Measurement of MVlCs is currently considered by some experts to be the most direct technique for investigating motor unit loss.34 Timed 4.6m (15-ft) walking tests assess the impact of physical impairments, such as reflex abnormalities, lower-extremity muscle weakness, and diminished motor control, on ambulation speed.” The “PaTa” test, a test of bulbar function (oral-labial dexterity), requires the patient to repeat the syllables “PaTa” as many times as possible for 5 seco n d ~In. ~pa~tie nts with ALS, the ability to perform a task may not change until a critical level of motoneuron loss is reached. Thus, the timing of motor tasks may be a less sensitive measure of disease progression than isometric muscle force testing using a tensiometer.

Spasticity

The modified Ashworth Spasticity Scale is a clinical measure of resistance to passive stretch that has been shown to produce reliable data. Scores range from 0 (no increase in muscle tone) to 4 (affected parts rigid in flexion or extension).

The purdue pegboard test:

Involves placing pins in a pegboard to assess right-hand and left-hand prehension, manual dexterity, and gross movement of the hands, fingers, and arms. Normative values are available, and the test has been used in various patient population, (Ashwort, 1964 and Bohannon, 1987).

Pulmonary function

Has a marked impact on an individual’s comfort, ability to communicate, and quality of life. Thus, forced vital capacity (FVC) and maximum inspiratory pressure (MIP), which are sensitive measures of respiratory functional, re-evaluated using a desktop spirometer, (Bohannon, 1987).

CLINICAL OUTCOME MEASUREMENT

Amyotrophic Lateral Sclerosis Functional Rating Scale32

A. Speech

4 Normal speech processes

3 Detectable speech disturbances

2 Intelligible with repeating

1 Speech combined with nonvocal communication

0 Loss of useful speech

B. Salivation

4 Normal

3 Slight but definite excess of saliva in mouth; may have nighttime drooling

2 Moderately excessive saliva; may have minimal drooling

1 Marked excess of saliva with some drooling

0 Marked drooling; requires constant tissue or handkerchief

C. Swallowing

4 Normal eating habits

3 Early eating problems; occasional choking

2 Dietary consistency changes

1 Needs supplemental tube feeding

0 Nothing by mouth (exclusively parenteral or enteral feeding]

D. Handwriting

4 Normal

3 Slow or sloppy; all words are legible

2 Not all words are legible

1 Able to grip pen, but unable to write

0 Unable to grip pen

E1.Cutting food and handling utensils (patients without gastrostomy)

4 Normal

3 Somewhat slow and clumsy, but no help needed

2 Can cut most foods, although clumsy and slow; some help needed

1 Food must be cut by someone, but can still feed self slowly

0 Needs to be fed

E2.Cutting food and handling utensils (alternate scale for patients with gastrostomy)

4 Normal

3 Clumsy, but able to perform all manipulations independently

2 Some help needed with closures and fasteners

1 Provides minimal assistance to caregiver

0 Unable to perform any aspect of task

F. Dressing and hygiene

4 Normal function

3 lndependent and complete selfcare with effort or decreased efficiency

2 lntermittent assistance or substitute methods

1 Needs attendant for selfcare

0 Total dependence

G. Turning in bed and adjusting bed clothes

4 Normal

3 Somewhat slow and clumsy, but no help needed

2 Can turn alone or adjust sheets, but with great difficulty

1 Can initiate, but not turn or adjust, sheets alone

0 Helpless

H. Walking

4 Normal

3 Early ambulation difficulties

2 Walks with assistance

1 Nonambulatory functional movement only

0 No purposeful leg movement

I. Climbing stairs

4 Normal

3 Slow

2 Mild unsteadiness or fatigue

1 Needs assistance

0 Cannot do

J. Breathing

4 Normal

3 Shortness of breath with minimal exertion (eg, walking, talking)

2 Shortness of breath at rest

1 lntermittent (eg, nocturnal) ventilatory assistance

0 Ventilatordeoendent (Dal, 1998).

PHYSICAL THERAPY MANAGEMENT OF AMYOTHROPHIC LATERAL SCLEROSIS

It’s well known that regular physical activity is beneficial to physical and emotional health. As population ages, physical activity is increasingly seen as an integral component for improving health and well-being and delaying or averting many chronic conditions associated with inactivity, (White and Petajan, 2001)

Physical therapy and special equipment can enhance patients’ independence and safety throughout the course of amyothrophic lateral sclerosis. Gentle, low-impact aerobic exercise such as walking, swimming, and stationary bicycling can strengthen unaffected muscles, improve cardiovascular health, and help patients fight fatigue and depression. Range of motion and stretching exercises can help prevent painful spasticity and shortening (contracture) of muscles. Physical therapists can recommend exercises that provide these benefits without overworking muscles, (ALSA, 2007).

The primary roles of physiotherapy include, (Piemonte and Ramirez, 2001):

To keep motor functioning stable for as long as possible: Exercise is needed to prevent muscle wasting from disuse yet not to induce fatigue which hinders strengthening, (Petajan, 2006).Active exercise to maintain muscle tone and strength, (Robineau et al, 2007). Resistance training may be an effective intervention strategy for improving functional abilities in moderately disabled persons with amyothrophic lateral sclerosis, (Gutierrez et al, 2005).

To prevent joint deformities, improve mobility and give guidance on the use of orthoses: Specialist neurological physiotherapists helps to improve mobility in people with amyothrophic lateral sclerosis, (Freeman and Thompson, 2001).Ambulatory patients may use a cane, one or two lightweight forearm crutches, or a four-wheel rolling walker (preferably with large swivel wheels, a flip-up seat, backrest and hand brakes) at the first stage of the condition. An ankle foot orthosis (AFO), preferably custom-made, can effectively correct foot-drop secondary to weakness or fatigue. A motorized mobility aid, such as a scooter or a power wheelchair, is often appropriate for the ambulatory person with fatigue. A power wheelchair would be more appropriate for individuals who are non-ambulatory and require additional seat and support, (Patricia and Provance, 2004).

Reduce spasticity: Mild spasticity may be managed by stretching and exercise programs such as water therapy (described below) and physical therapy, (Peters, 2004).Also, slow stretching, cold packs and controlled position changes do reduce spasticity, (Patricia and Provance, 2004).Stretching and range of motion exercises are very important where spasticity is a problem and joint motion is compromised. Stretches of the affected muscles need to be done slowly and held for three-five minutes, allowing the muscle fibres to stretch without tearing, (Petajan, 2006).Avoidance of positions and activities which increases tone and reinforces abnormal movement pattern, (Todd, 1990).Relaxation techniques, mat activities for trunk rotation, and peripheral neuromuscular facilitation,(PNF) to encourage trunk flexion. Neurorehabilitation has been shown to ease the burden of these symptoms by improving self-performance and independence at the semi-independent phase, (Kesselring, 2007).

To improve cardiovascular endurance: Gentle, low-impact aerobic exercise such as walking, swimming, and stationary bicycling can strengthen unaffected muscles, improve cardiovascular health, and help patients fight fatigue and depression, (ALSA,200)

To clear airway: Breathing exercises should be incorporated into the program at all stages. Postural drainage and coughing techniques to clear airways. In severe cases the use of suction to remove secretions may be more effective, (Physiocare, 2006).

To prevent muscle atrophy of the unaffected side: Exercise is needed to prevent muscle wasting from disuse yet not to induce fatigue which hinders strengthening, (Petajan, 2006).Active exercise to maintain muscle tone and strength, (Robineau et al, 2007). Resistance training may be an effective intervention strategy for improving functional ability in moderately disabled persons with amyothrophic lateral sclerosis, (Gutierrez et al, 2005).

To improve balance and coordination: Patients with lateral sclerosis at the independent state showed improvement in walking abilities using virtual reality visual-feedback cues, (Baram and Miller, 2006).weight bearing activities and weight shifting. Ataxic limb movement can be decrease by using cuff weights to increase proprioceptive feedback.For individuals who are relatively unsteady for these activities, balance and coordination drills may be done in a pool, (Petajan and White, 1999).In this environment, the patient will not fall or be injured if balance is lost and the support of the water will allow the accomplishment of movements that would be difficult on land. The benefits of water exercise include improved flexibility, posture, muscles tone and coordination, (Tourtellotte, 1983).

To teach energy conservation: this involves carefully spacing work schedules, proactive resting, avoiding over exertion and substitution for less demanding activities.

To encourage community physiotherapy: The community physiotherapy can be of particular help to the patient by:

• Advising and teaching about the need for personal daily assessment so that treatment can be adapted to the need of each day.
• Teaching routine physiotherapy treatment for lateral sclerosis, e.g. stretching, walking practice, prone lying and weight bearing as required.
• Reinforce the need rest periods and relaxation especially on days when fatigue is present.
• Develop a daily exercise program with variations to cope with daily changes.
• Give emotional support during the different stages.
• Provide suitable aids or wheelchairs as necessary.
• Over an extended period of visits considerable confidence can be developed and the person with lateral sclerosis can often confide in the community Physiotherapist in the security of his or her home. This can give considerable emotional relief.

• Encourage home programs and education: The key components to successful home programs are that it’s enjoyable, varied, goal oriented, and realistic. Consideration includes the person’s endurance, supports from friends and family, level of understanding, and time constrains. Emphasis must be placed on the performance of the above procedures, (Patricia and Provance, 2004).

To give psychotherapy: No one chooses to live with amyotrophic lateral sclerosis. Yet many have managed to do it with daring, dignity, humor and a hopeful attitude, (MFMER, 2007).

1. Take time to accept your diagnosis and the changes it will bring. Hearing that you have a fatal condition that will rob you of your mobility and independence is often nothing less than shocking. If you are newly diagnosed, you and your family will likely experience a period of mourning and grief that is similar to the period after the death of a loved one. Although the grieving process is different for everyone, it’s common to experience denial or disbelief, then sadness, anger, bargaining and, finally, acceptance.

2. Be hopeful. Some people with amyotrophic lateral sclerosis live much longer than the three to five years typically associated with this condition. Some live 10 years or more. Keeping hope alive is vital for people with amyothrophic lateral sclerosis.

Think beyond the physical changes. Amyothrophic lateral sclerosis doesn’t typically affect the intellect or spirit. Many people with amyotrophic lateral sclerosis lead rich, rewarding lives. Try to think of amyothrophic lateral sclerosis as only one part of your life, not your entire identity.

3. Join a support group. Having amyotrophic lateral sclerosis greatly changes your lifestyle and that of your family and may create a high financial burden. You may find comfort in sharing your concerns in a support group with others who have amyothrophic lateral sclerosis. Your caregivers may also benefit from a support group of others who care for people with amyotrophic lateral sclerosis. Find support groups in your area by talking to your doctor or by contacting the amyothrophic lateral sclerosis Association.

4. Make decisions now about your future medical care. Planning for the future allows you to be in control of decisions about your life and your care. With the help of your doctor or hospice nurse, you can decide whether or not you want certain life-extending procedures. And you can determine where you want to spend your final days. Talking about these issues isn’t easy. But facing your anxieties about the future may help you better enjoy life today, (MFMER, 2007).

Given the highly variable presentations of this disease, however, the work of Sinaki and Mulder’s (1978) approach to staging is a useful framework for designing treatment programs because the majority of people with amyothrophic lateral sclerosis referred for physical therapy have lower-extremity weakness and gait problems. Use of this framework does not dictate the needs of the individual; rather, staging provides the therapist with knowledge of the natural course of the disease, especially the progression of trunk and lower-extremity impairments and resultant disabilities, allowing physical therapists to anticipate the potential needs of their patients, even in the end stage,

(Iezzoni, 2000).

Sinaki and Mulder described the natural course of amyothrophic lateral sclerosis as consisting of 6 stages. The stages are based on the progressive loss of function in the trunk and extremity muscles. Identification of a patient’s stage can assist physical therapists in designing intervention throughout the disease process, (Dal, 1998).

The general goals of physical therapy in amyothrophic lateral sclerosis at the different stages are:

STAGE1: encourage functional activities by prescribing exercises

STAGE2: provide appropriate equipment and assistive devices to support weak muscles

STAGE3: keep patient physically independent

STGAE4: encourage activities of daily living and prevent complications

STAGE5: manage pain and maintain a clear airway

STAGE6: similar to that of hospice, address patients need and maximize each day.

Stage 1

The patient is in the early stages of the disease and is independent in mobility and ADL. A specific group of muscles are mildly weak, which may be manifested as limitations in performance or endurance, or both, (Sinaki, 1978). Therapy consists:

1. Education of patient and caregiver on energy conservation training,

2. Modification of the home and workplace, and psychological support

3. The patient is advised to continue normal physical activities.

4.General active range of motion (AROM) and stretching of affected joints, resistive strengthening exercises of unaffected muscles with low to moderate weights, and 5.Aerobic activities (e.g. swimming, walking, bicycling) at sub maximal levels may be prescribed. Moderate-intensity aerobic walking three to four days per week safely produced improvements in work capacity, aerobic capacity, and cardiovascular variables such as decreased heart rate and blood pressure (Wright et al., 1996). Improvement in physical fitness can potentially reduce the strains of daily living by increasing reserve capacities. This would allow individuals to meet the physiological demands required for activities of daily living (ADL) more effectively, (Wright et al., 1996).

Stage I1

The patient has moderate weakness in groups of muscles. The patient, for example, may have a foot drop on one or both sides or may have intrinsic muscle weakness in one hand that interferes with fine motor activities, (Sinaki, 1978). Assessing the need for and providing appropriate equipment and assistive devices to support weak muscles is the

primary goal of intervention.

1. The patient is encouraged to continue stretching and AROM exercises, strengthening exercises of unaffected muscles, and aerobic activities, as he or she is able.

2. In addition, the patient and caregivers are instructed in performing active-assisted range of motion (AAROM) and passive range of motion (PROM) exercises of affected joints to prevent contractures.

3. When designing a strengthening exercise program for a patient in amyotrophic lateral sclerosis stages I and II, the therapist should consider prevention of overuse fatigue and disuse atrophy. Evidence from patients with some other neuromuscular diseases indicates that highly repetitive or heavy resistance exercise can cause permanent loss of force in weakened, denervated muscle. A marked reduction in activity level secondary to amyotrophic lateral sclerosis, however, can lead to cardiovascular deconditioning and disuse weakness beyond the amount caused by the disease. Sinaki did not advocate any vigorous exercise for individuals with amyotrophic lateral sclerosis, stating that “in most patients, no exercise other than that inherent in everyday ambulatory activities is indicated, (Sinaki, 1980). Other authors, however, have reported beneficial effects of specific muscle strengthening and endurance exercise programs on patients with other neuromuscular disease. Exercise programs have physiologically and psychologically beneficial for patients with amyotrophic lateral sclerosis, especially when implemented before there is a great deal of muscle atrophy. Therefore, modified Sinaki and Mulder’s framework now include muscle strengthening and endurance exercises when tolerated, particularly during the early stages of the disease. Continuously adjust the intensity of exercise to prevent excessive fatigue, while at the same time promoting use of intact muscle groups to perform functional activities. Patients are advised not to carry out any activities to the point of extreme fatigue (i.e. inability to perform daily activities following exercise (Appe, 1986 and Bensimon, 1994). Some individuals have cramping or fasciculations because of ALS; an increase in these lower motoneuron (LMN) symptoms may indicate overuse. Patients are also advised to exercise for several brief periods throughout the day, with sufficient rest between exercise sessions. The total daily exercise time is 30 to 45 minutes. This total daily exercise time would be divided into 2 or 3 sessions depending on the patient’s tolerance, response to exercise, and daily schedule. The exercises may include resistive exercises, active exercises, and aerobic conditioning exercises (e.g. cycling, walking, and swimming). Swimming may be a good choice for people with ALS, as it provides a low-impact workout to most muscle groups, (Fleming, 2007).

Stage 111

The patient remains ambulatory but has severe weakness in certain muscle groups that may result in severe foot drop or a markedly weak hand. The patient may be unable to stand up from a chair without help. Overall, the patient may exhibit mild to moderate limitation of function. In this stage, as with all other stages, the goal is to keep the patient physically independent.

1. Adaptive equipment (e.g. ankle-foot orthoses [AFOs], splints, electrically powered height-adjustable chairs) may be needed to support weak muscles, decrease energy expenditure, and improve the patient’s safety and mobility. Patients may begin to report heaviness and fatigue while holding their head up in this stage, and they may benefit from a soft collar.

2. In addition, to avoid exhaustion, a wheelchair may become necessary when traveling long distances.

Stage IV

The patient in this stage has severe weakness of the legs and mild involvement of the arms. Thus, the patient uses a wheelchair and may be able to perform ADL, (Saniki, 1980).

1. PROM and AAROM exercises are recommended to prevent contractures.

2. Strengthening exercises and AROM of any noninvolved muscles should be continued. 3. As general mobility decreases, the need for instruction to inspect the skin for pressure areas increases, and sleeping and sitting systems that allow position changes and pressure relief surfaces (e.g. egg-crate bed cushion, alternating pressure bed pad) may be recommended.

Stage V

This stage is characterized by progressive weakness and deterioration of mobility and endurance. The patient uses a wheelchair when out of bed, and arm muscles may exhibit moderate or severe weakness. Transferring the patient to and from a wheelchair becomes a major effort, and a lift may be necessary, (Saniki, 1978) Patients become unable to move themselves in bed; thus, frequent repositioning and skin care by the caregiver are necessary. Pain may become a major problem in immobilized joints and needs to be addressed in the overall treatment plan.

1. Pain is addressed according to the pathophysiology of the problem causing the pain. For example, pain due to spasticity or muscle cramping may be addressed by stretching and massage; pain due to contractures may be addressed by the use of thermal modalities, stretching, splinting, and soft tissue mobilization; pain to due joint hypomobility or acute injuries (e.g. trauma to a shoulder resulting from a fall) may be addressed by joint mobilization, the use of thermal modalities, and electrical stimulation; pain due to joint instability may be addressed by the use of assistive devices, orthoses, slings, and positioning; and so on.

2. Patients may be unable to hold their head up for extended periods. Thus, a semirigid collar (e.g. Philadelphia collar, Newport collar) is appropriate in this stage of the disease.

3. If the patient has a tracheostomy, a Miami collar, a similar collar that allows for anterior neck access, is prescribed. By maintaining the head in a neutral position, breathing, eating, and seeing may be facilitated.’

Stage VI

The patient must remain in bed and requires maximal assistance with ADL. A hospital bed should be prescribed.

1. Frequent repositioning of the body, padding to prevent uneven pressure, and prevention of venous stasis in the legs are crucial.

2. Pain management continues to be important. “Head drop” from weak neck extensor muscles may become a major problem.

3. Progressive respiratory distress develops in this stage, and a suction machine should be available.

4. Cardiopulmonary physical therapy techniques may be required, such as body positioning to optimize ventilation-perfusion matching and prevent atelectasis; modified postural drainage positioning to decrease retention of secretions and aid in mobilization of secretions; self-assisted (if the patient is able) or manually assisted coughing techniques to compensate for a weak, ineffective cough and to aid in mobilization of secretions; and airway clearance techniques (i.e. vibrations, shaking, percussions) to mobilize secretions. Goals in this stage are similar to those of hospice care: to address the patient’s and caregivers’ needs and to maximize the quality of each day. Nurses, aides, and caregivers are instructed in home programs depending on the patient’s problems and needs (e.g. PROM, stretching, transfers, massage, airway clearance techniques).

Although many authors consider amyotrophic lateral sclerosis to be caused by a combination of genetic and environmental risk factors, so far the latter have not been firmly identified, (Wikipedia, 2007).Scientists have not found a definitive cause for amyotrophic lateral sclerosis and the onset of the disease can be linked to a variety of risk factors. It is believed that one or more of the following factors are responsible for the majority of amyothrophic lateral sclerosis cases, (ALSA, 2003):

1. Prolonged exposure to a dietary neurotoxin is the suspected risk factor in Guamanian amyothrophic lateral sclerosis. The neurotoxin is a compound (a sterol beta-D-glucoside) found in the seed of the cycad Cycas circinalis, a tropical plant found in Guam, which was used in the human food supply during the 1950s and early 1960s. An inherited genetic defect linked to a defect on chromosome 21 is believed to cause approximately 40% of familial amyotrophic lateral sclerosis cases. This mutation is believed to be autosomal dominant, (Khabazian, 2002)

2. The children of those diagnosed with familial amyotrophic lateral sclerosis have a higher risk factor for developing the disease, however those who have close family members diagnosed with sporadic amyothrophic lateral sclerosis have no greater a risk factor than the general population, (Rachele, 1998)

3. According to the amyothrophic lateral sclerosis association, military veterans are at an increased risk of contracting amyotrophic lateral sclerosis. In its report amyothrophic lateral sclerosis in the military, the group pointed to an almost 60% greater chance of the disease in military veterans than the general population.

CLASSIFICATION OF AMYOTHROPHIC LATERAL SCLEROSIS

According to MFMER (2007) amyotrophic lateral sclerosis is classified into three general groups, familial, sporadic and Guamanian.

1. Familial amyothrophic lateral sclerosis accounts for approximately 5%-10% of all amyothrophic lateral sclerosis cases and is caused by genetic factors. Of these approximately 10% are linked to a mutation in Superoxide dismutase (SOD1), a copper/zinc dependant dismutase that is responsible for scavenging free radicals. As the name suggests, familial amyotrophic lateral sclerosis is believed to be caused by the inheritance of one or more faulty genes. About 15% of families with this type of amyothrophic lateral sclerosis have mutations in the gene for SOD-1. SOD-1 gene defects are dominant, meaning only one gene copy is needed to develop the disease. Therefore, a parent with the faulty gene has a 50% chance of passing the gene along to a child, (Fleming, 2007)

2. Most of the remaining 90-95% of cases are classified as “sporadic amyotrophic lateral sclerosis “and have no known hereditary component. While many environmental toxins have been suggested as causes, to date no research has confirmed any of the candidates investigated, including aluminum and mercury and lead from dental fillings. As research progresses, it is likely that many cases of sporadic amyotrophic lateral sclerosis will be shown to have a genetic basis as well, (Fleming, 2007).

3. A third type, called “Guamanian amyotrophic lateral sclerosis “, represents a small cluster of cases concentrated on the Pacific island of Guam.

A third type, called Western Pacific amyothrophic lateral sclerosis, occurs in Guam and other Pacific islands. This form combines symptoms of both amyothrophic lateral sclerosis and Parkinson’s disease, (Fleiming, 2007).

ETIOLOGY

The cause of amyotrophic lateral sclerosis is not known. An important step toward answering that question came in 1993 when scientists discovered that mutations in the gene that produces the Cu/Zn superoxide dismutase (SOD1) enzyme were associated with some cases (approximately 20%) of familial amyothrophic lateral sclerosis. This enzyme is a powerful antioxidant that protects the body from damage caused by superoxide, a toxic free radical. Free radicals are highly reactive molecules produced by cells during normal metabolism, (Reaume, 1996). Free radicals can accumulate and cause damage to DNA and proteins within cells. Although it is not yet clear how the SOD1 gene mutation leads to motor neuron degeneration, researchers have theorized that an accumulation of free radicals may result from the faulty functioning of this gene. Current research, however, indicates that motor neuron death is not likely a result of lost or compromised dismutase activity, suggesting mutant SOD1 induces toxicity in some other way (a gain of function), (Bruijn, 1998).

It is speculated that aggregate accumulation of mutant SOD1 plays a role in disrupting cellular functions by damaging mitochondria, proteasomes, protein folding chaperones, or other proteins, (Bruijn, 1998). Any such disruption, if proven, would lend significant credibility to the theory that aggregates are involved in mutant SOD1 toxicity. However, it is important to remember that SOD1 mutations cause only 10% or so of overall cases and the etiological mechanisms may be distinct from those responsible for the sporadic form of the disease, (Boillée, 2006).

Studies also have focused on the role of glutamate in motor neuron degeneration. Glutamate is one of the chemical messengers or neurotransmitters in the brain. Scientists have found that, compared to healthy people, amyotrophic lateral sclerosis patients have higher levels of glutamate in the serum and spinal fluid. Laboratory studies have demonstrated that neurons begin to die off when they are exposed over long periods to excessive amounts of glutamate (excitotoxicity). Now, scientists are trying to understand what mechanisms lead to a buildup of unneeded glutamate in the spinal fluid and how this imbalance could contribute to the development of amyotrophic lateral sclerosis. Failure of astrocytes to sequester glutamate from the extracellular fluid surrounding the neurones has been proposed as a possible cause of this glutamate-mediated neurodegeneration, (Wikipedia, 2007).

Autoimmune responses which occur when the body’s immune system attacks normal cells have been suggested as one possible cause for motor neuron degeneration in amyothrophic lateral sclerosis. Some scientists theorize that antibodies may directly or indirectly impair the function of motor neurons, interfering with the transmission of signals between the brain and muscles. More recent evidence indicates that the nervous system’s immune cells, Microglia, are heavily involved in the later stages of the disease, (Wikipedia, 2007).

In searching for the cause of ALS, researchers have also studied environmental factors such as exposure to toxic or infectious agents. Other research has examined the possible role of dietary deficiency or trauma. However, as of yet, there is insufficient evidence to implicate these factors as causes of amyothrophic lateral sclerosis, (Wikipedia, 2007).

EPIDEMIOLOGY OF AMYOTHROPHIC LATERAL SCLEROSIS

Amyotrophic lateral sclerosis is one of the most common neuromuscular diseases worldwide, and people of all races and ethnic backgrounds are affected. Between 1 to 2 people per 100,000 develop amyothrophic lateral sclerosis each year, (Wikipedia, 2007). Amyotrophic lateral sclerosis most commonly strikes people between 40 and 60 years of age, but younger and older people can also develop the disease. Men are affected slightly more often than women, (eMedicine, 2007).

The incidence of the disease is reported to be from 0.4 to 2.4 cases per 100,000 inhabitants, with a prevalence of 2.5 to 7.0 cases per 100,000 inhabitants, (Dal, 1998). In Brazil, the characteristics are similar to those found in Europe and North America, with a higher prevalence in men (gender ratio of 3:2) and an average onset age of 57 years, (Dietrich, 1998)

As many as 20,000 Americans have amyothrophic lateral sclerosis, and an estimated 5,000 people in the United States are diagnosed with the disease each year. The estimated prevalence is up to six cases per 100,000 of the adult population, with an annual incidence of one to two cases per 100,000. The peak age of onset is between 55 and 75 years, with a male preponderance of 1.5 to 2.0:1, (Belsh, 1996). Recently, a modest increase in incidence has been noted, along with a tendency for the condition to present at younger ages, (Brooks, 1996).

MORTALITY

Amyotrophic lateral sclerosis usually progresses slowly. In about half of all patients, it causes death within three years. About 80 percent of all patients die in less than five years, and a small number (about 10 percent) survive more than eight years, (Fleming, 2007). The prognosis tends to be worse for older patients and those with the bulbar form of the disease, (Swash, 1992).

PATHOPHYSIOLOGY

Skeletal muscles are innervated by a group of neurones (lower motor neurones) located in the ventral horns of the spinal cord which project out the ventral roots to the muscle cells. These nerve cells are themselves innervated by the corticospinal tract or upper motor neurones that project from the motor cortex of the brain. On macroscopic pathology, there is a degeneration of the ventral horns of the spinal cord, as well as atrophy of the ventral roots. In the brain, atrophy may be present in the frontal and temporal lobes. On microscopic examination, neurones may show spongiosis, the presence of astrocytes, and a number of inclusions including characteristic “skein-like” inclusions, bunina bodies, and vacuolisation, (Reginald, 1998).

There is a role in excitotoxicity and oxidative stress, presumably secondary to mitochondrial dysfunction.

Five to 10% of patients with amyothrophic lateral sclerosis have a family history following an autosomal dominant pattern of inheritance. About 20% of these patients have a mutation of the superoxide dismutase 1 (SOD1) enzyme. This enzyme functions as an antioxidant, (Kathleen, 2006).

PATHOLOGY

No single cause for amyotrophic lateral sclerosis explains its entire pathology; indeed, there may be multiple causes resulting in phenotypic similarity. While amyotrophic lateral sclerosis is ultimately a diffuse disease, onset is often focal and asymmetric. About 90% of cases of amyotrophic lateral sclerosis are “sporadic”, meaning that the patient has no family history of amyotrophic lateral sclerosis and the case appears to have occurred with no known cause. Genetic factors are suspected to be important in determining an individual’s susceptibility to disease, and there is some weak evidence to suggest that onset can be “triggered” by as yet unknown environmental factors, (ALSOD, 2004).

Approximately 10% of cases are “familial amyotrophic lateral sclerosis “, defined either by a family history, or by testing positive for a known genetic mutation associated with the disease. The following genes are known to be linked to ALS: Cu/Zn superoxide dismutase SOD1, ALS2, NEFH (a small number of cases), senataxin (SETX) and vesicle associated protein B (VAPB), (ALSOD, 2004).

Of these, SOD1 mutations account for some 20% of familial cases. The SOD1 gene codes for the enzyme superoxide dismutase, a free radical scavenger that reduces the oxidative stress of cells throughout the body. So far over 100 different mutations in the SOD1 gene have been found, all of which cause some form of ALS (ALSOD, 2004).

In North America, the most commonly occurring mutation is known as A4V and occurs in up to 50% of SOD1 cases. In people of Scandinavian extraction there is a relatively benign mutation called D90A which is associated with a slow progression. Future research is concentrating on identifying new genetic mutations and the clinical syndrome associated with themutive dysfunction, (Reginald, 1998).

It is thought that SOD1 mutations confer a toxic gain, rather than a loss, of function to the enzyme. SOD1 mutations may increase the propensity for the enzyme to form protein aggregates which are toxic to nerve cells, (Reginald, 1998).

Amyotrophic lateral sclerosis is one of a group of diseases known as motor neuron diseases. Neurons are nerve cells, and motor neurons are neurons that control movement, (Wikipedia, 2007).

Movement occurs when neurons in the brain (upper motor neurons) send messages to neurons in the spinal cord (lower motor neurons). The lower motor neurons relay these messages to the specific muscles that carry out the movement.

In some motor neuron diseases, only the upper motor neurons are affected, or only the lower motor neurons. In amyothrophic lateral sclerosis, however, both upper and lower motor neurons are affected. Atrophy of the anterior horn cells and replacement of the large motor neurons by fibrous astrocytes (gliosis) causes the affected anterior and lateral columns of the spinal cord to become hard, hence the term “lateral sclerosis”, (Williams, 1991). Large neurons tend to be affected before small ones, (Adams, 1997) but the general distribution of pathologic findings within the spinal cord should correlate with the clinical findings. In the brain, atrophic changes may be found in the motor and premotor cortex, (Adams, 1997). Peripheral nerves show secondary degeneration of axons and myelin, (Walton, 1993). Surviving motor axons develop collateral branches to attempt reinnervation of muscles. The denervated muscles display various stages of atrophy, (Walton, 1993).

The neurons die and stop sending messages to muscles. The muscles are unable to function without these messages and gradually become weaker. They waste away (atrophy) and twitch (fasciculate). Eventually all voluntary movement is lost, and muscles become paralyzed. The loss of strength and control follows different patterns in different people. Some people lose control of one or both legs first. They may have trouble walking or running or become clumsy, tripping or falling. Others notice a problem with a hand or arm first, having problems with simple tasks such as writing or buttoning a shirt. In some, speech or swallowing is the first thing affected, (Wikipedia, 2007).

In every case, the weakness and disability spread to almost every part of the body.

The diaphragm is a muscle under the lungs that partly controls their expansion as they take in air. When the chest muscles and diaphragm become too weak, the person is no longer able to breathe on his own. This is called respiratory failure and is by far the leading cause of death for people with amyothrophic lateral sclerosis.

Amyotrophic lateral sclerosis does not affect a person’s mental abilities or senses. It does not impair intelligence, reasoning, memory, or personality. Senses such as vision and touch are not lost. Most people retain their ability to move their eyes. Bowel and bladder control are not impaired, (Wikipedia, 2007).

STAGES OF AMYOTHROPHIC LATERAL SCLEROSIS

Dal Bello-Hass et al (1998) classified amyotrophic lateral sclerosis into stages of functional dependence, with the aims of simplifying the guidelines for clinical attendance, maintaining maximum functional mobility for patients and improving their quality of life. These stages are:

Stage 1: Preserved functional independence (that is independent in mobility and ADL), muscle weakness with changes in resistance. Physiological support and continuity of normal physical activities are recommended.

Stage 2: Involvement of the distal musculature with the use of orthoses indicated in some cases. The same conduct as in Stage 1 is maintained, but cardiopulmonary and neuromuscular conditioning and physical-functional training are added.

Stage 3: Moderate functional limitations and more susceptibility to fatigue. Patients require wheelchairs and may use orthoses for the arms.

Stage 4: This is the start of the severe phase, with changes in the lower limbs. Conti-nuation of the exercises from Stage 2 is necessary, with the addition of exercises aimed at preventing muscle contracture and guidance about positioning when lying down.

Stage 5: Functional dependence requiring stretching exercises and manual therapy performed to reduce the muscle and joint pains, spasticity and muscle fasciculation. Electrical stimulation and hydrotherapy help with the muscle instability, as well as the use of orthoses.

Stage 6: This is the maximum state of dependence. Patients are sometimes immobile, with involvement of the respiratory system. Cardiopulmonary physiotherapy is of great importance at this stage, as are changes in posture and a homecare program. The physiotherapeutic techniques of Stages 1 and 2 should be continued.

SIGNS AND SYMPTOMS AMYOTHROPHIC LATERAL SCLEROSIS

The onset of amyothrophic lateral sclerosis may be so subtle that the symptoms are frequently overlooked. The earliest symptoms may include twitching, cramping, or stiffness of muscles; muscle weakness affecting an arm or a leg; and/or slurred and nasal speech. These general complaints then develop into more obvious weakness or atrophy that may cause a physician to suspect amyothrophic lateral sclerosis, (Wikipedia, 2007).

The parts of the body affected by early symptoms of amyothrophic lateral sclerosis depend on which muscles in the body are damaged first. About 75% of people experience “limb onset” amyothrophic lateral sclerosis. In some of these cases, symptoms initially affect one of the legs, and patients experience awkwardness when walking or running or they notice that they are tripping or stumbling more often. Other limb onset patients first see the effects of the disease on a hand or arm as they experience difficulty with simple tasks requiring manual dexterity such as buttoning a shirt, writing, or turning a key in a lock, (Wikipedia, 2007).

About 25% of cases are “bulbar onset” amyothrophic lateral sclerosis. These patients first notice difficulty speaking clearly. Speech becomes garbled and slurred. Nasality and loss of volume are frequently the first symptoms. Difficulty swallowing, and loss of tongue mobility follow. Eventually total loss of speech and the inability to protect the airway when swallowing are experienced.

Regardless of the part of the body first affected by the disease, muscle weakness and atrophy spread to other parts of the body as the disease progresses. Patients experience increasing difficulty moving, swallowing (dysphagia), and speaking or forming words (dysarthria). Symptoms of upper motor neuron involvement include tight and stiff muscles (spasticity) and exaggerated reflexes (hyperreflexia) including an overactive gag reflex. An abnormal reflex commonly called Babinski’s sign (the large toe extends upward as the sole of the foot is stimulated) also indicates upper motor neuron damage. Symptoms of lower motor neuron degeneration include muscle weakness and atrophy, muscle cramps, and fleeting twitches of muscles that can be seen under the skin (fasciculations). Around 15–45% of patients experience pseudobulbar affect, also known as “emotional lability”, this consists of uncontrollable laughter or crying, (Reginald, 1998).

To be diagnosed with amyothrophic lateral sclerosis, patients must have signs and symptoms of both upper and lower motor neuron damage that cannot be attributed to other causes, (ALSOD, 2004).

Although the sequence of emerging symptoms and the rate of disease progression vary from person to person, eventually patients will not be able to stand or walk, get in or out of bed on their own, or use their hands and arms. Difficulty swallowing and chewing impair the patient’s ability to eat normally and increase the risk of choking. Maintaining weight will then become a problem. Because the disease usually does not affect cognitive abilities, patients are aware of their progressive loss of function and may become anxious and depressed. A small percentage of patients go on to develop frontotemporal dementia characterized by profound personality changes; this is more common amongst those with a family history of dementia. A larger proportion of patients experience mild problems with word-generation, attention, or decision-making. Cognitive function may be affected as part of the disease process or could be related to poor breathing at night (nocturnal hypoventilation). Health care professionals need to explain the course of the disease and describe available treatment options so that patients can make informed decisions in advance, (ALSOD, 2004).

As the diaphragm and intercostal muscles weaken, forced vital capacity and inspiratory pressure diminish. In bulbar onset amyothrophic lateral sclerosis, this may occur before significant limb weakness is apparent. Bilevel positive pressure ventilation (frequently referred to by the tradename BiPAP) is frequently used to support breathing, first at night, and later during the daytime as well. It is recommended that long before BiPAP becomes insufficient, patients (with the eventual help of his/her family) must decide whether to have a tracheostomy and long term mechanical ventilation. Most patients do not elect this route, and instead choose palliative hospice care at this point. Most people with amyothrophic lateral sclerosis die of respiratory failure or pneumonia, not the disease itself, (Wikipedia, 2007).

Amyotrophic lateral sclerosis predominantly affects the motor neurons, and in the majority of cases the disease does not impair a patient’s mind, personality, intelligence, or memory. Nor does it affect a person’s ability to see, smell, taste, hear, or feel touch. Control of eye muscles is the most preserved function, although some patients with an extremely long duration of disease (20+ years) may lose eye control too. Unlike multiple sclerosis, bladder and bowel control are usually preserved in amyotrophic lateral sclerosis, although as a result of immobility and diet changes, intestinal problems such as constipation can require intensive management, (Flemings, 2007).

COMPLICATIONS OF AMYOTHROPHIC LATERAL SCLEROSIS

Spasticity

Atrophy

Muscle weakness

Respiratory complications

Loss of ability to care for self

Pneumonia

Skin breakdown

Pressure sore

Weight loss (NINDS, 2003)

DIAGNOSIS OF AMYOTHROPHIC LATERAL SCLEROSIS

ALS is difficult to diagnose early because it may appear similar to other neurological diseases, (MFMER, 2003)

According to Brooks (1994), the diagnostic criteria of the World Federation of Neurology (the “El Escorial criteria”) for the diagnoses of amyotrophic lateral sclerosis include:

Diagnostic Criteria for amyothrophic lateral sclerosis

Positive features • Definite ALS –LMN and UMN signs in three to four regions – Evidence of progression • Probable ALS LMN and UMN signs in at least two regions with UMN above LMN signs and evidence of progression • Possible ALS –LMN and UMN in one region –UMN in two regions –LMN above UMN signs –LMN and UMN signs but no evidence of progression • Suspected ALS –LMN signs in two to three regions Negative features • Findings inconsistent with diagnosis of ALS • Neuroimaging, EMG, clinical or other evidence of an alternative disease explaining signs or symptoms • Lack of progression to other body regions • Cognitive decline • Sphincter abnormalities • Sensory dysfunction • Visual decline

Notes: ALS=amyotrophic lateral sclerosis; LMN=lower motor neuron; UMN=upper motor neuron; EMG=electromyographic.

Diagnosing amyothrophic lateral sclerosis first involves:

Ø Reviewing family’s medical history and signs and symptoms.

Ø Physical examination that may include some basic in-office testing of the nerves and muscles.

Ø Additionally, the patient undergoes a variety of tests, including tests to rule out other conditions.

These diagnostic tests may include:

• Electromyogram (EMG). Electromyography measures the tiny electrical discharges produced in muscles. A thin-needle electrode is inserted into the muscles that the doctor wants to study. An instrument records the electrical activity in the muscle at rest and contract the muscle.

• Nerve conduction study (NCS). In a variation of electromyography, two electrodes are taped to the skin above a nerve to be studied. A small shock is passed through the nerve

to measure the size and speed of nerve signals.

• Imaging. The doctor may suggest magnetic resonance imaging (MRI) or a computerized tomography (CT) scan to produce images of the brain and spinal cord. These tests can help the doctor determine if something other than lateral sclerosis.

• Spinal tap (lumbar puncture). This test analyzes the fluid surrounding the brain and spinal cord (cerebrospinal fluid). The patient typically lies on the side with the knees drawn up to the chest. A local anesthetic is injected in an area over the lower spine to reduce any discomfort from the procedure. Then a needle is inserted into the spinal canal, and fluid is collected. Tests on this fluid indicate the number and types of white blood

cells, the level of sugar, and the levels and types of proteins. Additional tests on this fluid can indicate evidence of bleeding, infection and abnormal cells.

• Blood and urine tests. Analyzing samples of the blood and urine in the laboratory may help the doctor eliminate other possible causes of the signs and symptoms.

• Muscle biopsy. If the doctor believes that the patient may have a muscle disease rather than ALS, then a muscle biopsy is recommended. Under local anesthesia, the doctor removes a small portion of tissue to be analyzed in the laboratory, (MFMER, 2007)}

DIFFERENTIAL DIAGNOSIS OF AMYOTHROPHIC LATERAL SCLEROSIS

Targeted laboratory, radiographic and special investigations may be necessary to enable exclusion of other causes of symptoms, (Oliver, 1994).The diagnostic strategy requires balancing the need for certainty against the discomfort and potential adverse effects of excessive testing. Patients and families may request referral to specialty centers or additional testing to eliminate uncertainty, (Oliver, 1994).

Spinal cord lesion: Examples: tumors, lymphoma, syringomyelia, vascular malformations Spinal bone lesions: Examples: spondylosis, cervical rib, metastatic tumors Infections: Examples: HIV, syphilis, myelitis, poliomyelitis, Lyme disease Endocrine disorders: Examples: hyperthyroidism, hyperparathyroidism, diabetic radiculopathy Toxins: Examples: lead, mercury Other: Examples: postpolio syndrome, Huntington’s disease, Friedreich’s ataxia, sarcoidosis, multiple sclerosis, polymyositis, myasthenia gravis, muscular dystrophies

(Moss,1993) and (Walton ,1993) ALS=amyotrophic lateral sclerosis; HIV=human immunodeficiency virus.

The central nervous system consists of the brain and spinal cord:

The brain plays a central role in the control of most bodily functions, including awareness, movements, sensations, thoughts, speech, and memory. Some reflex movements can occur via spinal cord pathways without the participation of brain structures, (Moore, 1999).

The spinal cord is connected to a section of the brain called the brainstem and runs through the spinal canal. Cranial nerves exit the brainstem. Nerve roots exit the spinal cord to both sides of the body. The spinal cord carries signals (messages) back and forth between the brain and the peripheral nerves, (Moore, 1999).

Cerebrospinal fluid surrounds the brain and the spinal cord and also circulates within the cavities (called ventricles) of the central nervous system. The leptomeninges surround the brain and the spinal cord. The cerebrospinal fluid circulates between 2 meningeal layers called the pia matter and the arachnoids (or pia-arachnoids membranes). The outer, thicker layer serves the role of a protective shield and is called the dura matter.

The basic unit of the central nervous system is the neuron (nerve cell). Billions of neurons allow the different parts of the body to communicate with each other via the brain and the spinal cord. A fatty material called myelin coats nerve cells to insulate them and to allow nerves to communicate quickly, (Moore, 1999).

The BRAIN

The Cerebrum

The cerebrum is the largest part of the brain and controls voluntary actions, speech, thought, and memory. The surface of the cerebral cortex has grooves or infoldings (called sulci), the largest of which are termed fissures. Some fissures separate lobes. The convolutions of the cortex give it a wormy appearance, (Moore, 1999).

Each convolution is delimited by 2 sulci and is also called a gyrus (gyri in plural). The cerebrum is divided into 2 halves, known as the right and left hemispheres. A mass of fibers called the corpus callosum links the hemispheres. The right hemisphere controls voluntary limb movements on the left side of the body, and the left hemisphere controls voluntary limb movements on the right side of the body. Almost every person has one dominant hemisphere. Each hemisphere is divided into 4 lobes, or areas, which are interconnected, (Moore, 1999).

The frontal lobes are located in the front of the brain and are responsible for voluntary movement and, via their connections with other lobes, participate in the execution of sequential tasks; speech output; organizational skills; and certain aspects of behavior, mood, and memory, (Moore, 1999).

The parietal lobes are located behind the frontal lobes and in front of the occipital lobes. They process sensory information such as temperature, pain, taste, and touch. In addition, the processing includes information about numbers, attentiveness to the position of one’s body parts, the space around one’s body, and one’s relationship to this space.

The temporal lobes are located on each side of the brain. They process memory and auditory (hearing) information and speech and language functions, (Moore, 1999).

The occipital lobes are located at the back of the brain. They receive and process visual information.

The cortex, also called gray matter, is the most external layer of the brain and predominantly contains neuronal bodies (the part of the neurons where the DNA-containing cell nucleus is located). The gray matter participates actively in the storage and processing of information. An isolated clump of nerve cell bodies in the gray matter is termed a nucleus (to be differentiated from a cell nucleus). The cells in the gray matter extend their projections, called axons, to other areas of the brain.

Fibers that leave the cortex to conduct impulses toward other areas are termed efferent, and fibers that approach the cortex from other areas of the nervous system are termed afferent (nerves or pathways). Fibers that go from the motor cortex to the brainstem (for example, pons) or the spinal cord receive a name that generally reflects the connections (that is, corticopontine tract for the former and corticospinal tract for the latter). Axons are surrounded in their course outside the gray matter by myelin, which has a glistening whitish appearance and thus gives rise to the term white matter.
Cortical areas receive their names according to their general function or lobe name. If in charge of motor function, the area is called motor cortex. If in charge of sensory function, the area is called a sensory or somesthetic cortex. The calcarine or visual cortex is located in the occipital lobe (also termed occipital cortex) and receives visual input. The auditory cortex, localized in the temporal lobe, processes sounds or verbal input. Knowledge of the anatomical projection of fibers of the different tracts and the relative representation of body regions in the cortex often enables doctors to correctly locate an injury and its relative size, sometimes with great precision, (Moore, 1999).

Central Structures of the Brain:

The central structures of the brain include the thalamus, hypothalamus, and pituitary gland. The hippocampus is located in the temporal lobe but participates in memory and emotions and is interconnected with central structures. Other structures are the basal ganglia, which are made up of gray matter and include the amygdala (localized in the temporal lobe), the caudate nucleus, and the lenticular nucleus (putamen and globus pallidus). Because the caudate and putamen are structurally similar, neuropathologists have coined for them the collective term striatum, (Wikipedia, 2007).

The thalamus integrates and relays sensory information to the cortex of the parietal, temporal, and occipital lobes. The thalamus is located in the lower central part of the brain (that is, upper part of the brainstem) and is located medially to the basal ganglia. The brain hemispheres lie on the thalamus. Other roles of the thalamus include motor and memory control.

The hypothalamus, located below the thalamus, regulates automatic functions such as appetite, thirst, and body temperature. It also secretes hormones that stimulate or suppress the release of hormones (for example, growth hormones) in the pituitary gland.

The pituitary gland is located at the base of the brain. The pituitary gland produces hormones that control many functions of other endocrine glands. It regulates the production of many hormones that have a role in growth, metabolism, sexual response, fluid and mineral balance, and stress response, (Wikipedia, 2007).

The ventricles are cerebrospinal-fluid–filled cavities in the interior of the cerebral hemispheres.

The Base of the Brain

The base of the brain contains the cerebellum and the brainstem. These structures serve complex functions. Below is a simplified version of these roles, (UVHSND, 2004):

Traditionally, the cerebellum has been known to control equilibrium and coordination and contributes to the generation of muscle tone. It has recently become evident, however, that the cerebellum plays more diverse roles such as participating in some types of memory and exerting a complex influence on musical and mathematical skills.

The brainstem connects the brain with the spinal cord. It includes the midbrain, the pons, and the medulla oblongata. It is a compact structure in which multiple pathways traverse from the brain to the spinal cord and vice versa. For instance, nerves that arise from cranial nerve nuclei are involved with eye movements and exit the brainstem at several levels. Damage to the brainstem can therefore affect a number of bodily functions. For instance, if the corticospinal tract is injured, a loss of motor function (paralysis) occurs, and it may be accompanied by other neurologic deficits, such as eye movement abnormalities, which are reflective of injury to cranial nerves or their pathways in the brainstem, (UVHSND, 2004).

The midbrain is located below the hypothalamus. Some cranial nerves that are also responsible for eye muscle control exit the midbrain.

The pons serves as a bridge between the midbrain and the medulla oblongata. The pons also contains the nuclei and fibers of nerves that serve eye muscle control, facial muscle strength, and other functions.

The medulla oblongata is the lowest part of the brainstem and is interconnected with the cervical spinal cord. The medulla oblongata also helps control involuntary actions, including vital processes, such as heart rate, blood pressure, and respiration, and it carries the corticospinal (that is, motor function) tract toward the spinal cord, (UVHSND, 2004).

THE SPINAL CORD

The spinal cord is an extension of the brain and is surrounded by the vertebral bodies that form the spinal column. The central structures of the spinal cord are made up of gray matter (nerve cell bodies), and the external or surrounding tissues are made up of white matter, (Moore, 1999).

Within the spinal cord are 30 segments that belong to 4 sections (cervical, thoracic, lumbar, and sacral), based on their location, (Moore, 1999):

Eight cervical segments: These control signals from or to areas of the head, neck, shoulders, arms, and hands.

Twelve thoracic segments: These control signals from or to part of the arms and the anterior and posterior chest and abdominal areas.

Five lumbar segments: These control signals from or to the legs and feet and some pelvic organs.

Five sacral segments: These control signals from or to the lower back and buttocks, pelvic organs and genital areas, and some areas in the legs and feet.

The spinal cord is a long cylindrical structure, invested by meninges that lie in the vertebral canal. It extend from the foramen magnum where it continues with the medulla to the lower border of the first lumber vertebrae, (Carpenter, 1999). The spinal cord proper ends at the level of the lumber vertebrae, (Inderbir, 2002). It terminates at a conical point known as the conus medullaris. A condensation of pia mater,extending caudally from the conus medullaris,forms the filum terminale which penetrates the dural tube at the level of the second sacral vertebral, becomes invested by dura and continues as the coccygeal ligament to the posterior surface of the coccyx, (Carpenter, 1999).Ronald et al (2006) describes the anatomical component of the spinal cord. These are:

Dorsal root: the dorsal carries both myelinated and unmyelinated afferent fibres to the spinal cord. Each fibre is the central process of a dorsal root ganglia cell.

Ventral root: bundle of somatic motor fibres (axon of somatic motor neurons) and preganglionic fibres (axons of autonomic motor neurons).They constitute the efferent outflow of the spinal cord.

Posterior grey column: long and narrow column of grey matter reaching almost to the surface of the spinal cord. Primarily it’s concerned with sensory input due to the presence of sensory cell bodies.

Anterior grey column: short and broad column of grey matter. It’s concerned with motor function due to the presence of motor cell bodies.

Anterior median fissure: it is about 3mm deep. It contains blood vessels (anterior spinal artery) supplying the anterior two thirds of the cord.

Anterior lateral sulcus: site of exit of ventral root.

Posterior lateral sulcus: site of entry of dorsal root.

Posterior intermediate septum: found only in the cervical and upper thoracic segments.

Posterior median sulcus: about 5mm deep, reaches the deep lying grey matter.

Ascending and descending fibres occupying particular regions of the white matter are organized into more or less distinct bundles, (Carpenter, 1999).According to Ronald et al (2006) the white matter of the spinal cord is divide into 3 paired funiculi.

Anterior funiculus: this is between the anterior median fissures and anteriorlateral sulcus (ventral root).it merges with the lateral funiculus and contains ascending and descending tracts.

Lateral funiculus: this is between the dorsal and the ventral roots. It merges with the anterior feniculus and contains ascending and descending tracts.

Posterior feniculus: this is between posterior median sulcus and dorsal root. It contains ascending tracts.

Tracts in the lateral and anterior funiculi are both ascending and descending. Ascending in these funiculi arise from cells cell within the spinal grey matter while long descending tract arise in the nucleus in the brainstem and cerebral cortex.

Tracts of the spinal cord

According to Inderbir (2002), a tract may be defined as a collection of nerve fibres having the same origin, course and termination. And tract of the spinal cord can be grouped as ascending and descending tracts as shown in

Descending tracts ending in the spinal cord:

Corticospinal tracts; they are made up predominantly of axons of neurons lying in the motor area of the cerebral cortex (area 4). Some fibres also arise from the premotor area (area 6), some from the somatosensory area (area 3,2,1) and few fibres arise in the parietal cortex (area 5).From these origins,fibres pass through the corona radiate to enter the internal capsule. After passing through the internal capsule, the fibres enter the crus cerebri (of the midbrain).Near the lower end of the medulla, about 80 percent of the fibres cross to the opposite side (the crossing fibre of the two sides constitute the decussation of pyramids).The fibres that cross in the medulla enter the lateral funiculus of the spinal cord and descend as the lateral corticopsinal tract. The fibres of this tract terminate in the grey matter at various levels of the spinal cord. Most of them end by synapsing with the internuncial neurons in the base of the dorsal and ventral grey columns (Lamina IV to VII).The internuncial neurons carries the impulses brought by fibres of the tract to ventral horn cells. Some fibres of the tract terminate directly on ventral horn cells.

The corticospinal fibres that do not cross in the pyramid decussation enter the anterior funiculus of the spinal cord to form the anterior corticospinal tract. On reaching the appropriate level of the spinal cord, the fibres of this tract cross the midline (through the anterior white commisure) to reach grey matter on the opposite side of the cord. The corticospinal fibre of both lateral and anterior tracts ultimately connects the cerebral cortex of one side with the ventral horn cells in the opposite half of the spinal cord. The cerebral cortex controls voluntary movement through the corticospinal tract.

Rubrospinal tract; this is made up of axons of neuron lying in the red nucleus 9in the upper part of the midbrain).The fibres of the tract cross to the opposite side in the lower part of the tegmentum of midbrain (ventral tegmental decussation).The tract descend through the pons and medulla to enter the lateral funiculus of the spinal cord. The tract lies just in front of the lateral corticospinal tract .The fibre of the rubrospinal tract ends by synapsing with ventral horn cells through internuncial neurons located in lamina V to VII of the spinal gray matter.

Tectospinal tract; the fibres of this tract arise from neurons in the superior colliculus (mid brain).The cross to the opposite side in upper part of the tegmentum of the midbrain(dorsal tegmental decusation).The tract descends through the pons and medulla into the anterior funiculus of the spinal cord.

Olivospinal tract; this tract arises from the inferior olivary nucleus located in the medulla and terminate in relation to ventral horn cells of the spinal cord.

Reticulospinal tract; this has both the medial and lateral tracts. Medial reticulospinal tract fibre arises from the medial part of the reticular formation of both pons and medulla. The fibres (crossed and uncrossed) descend in the anterior funiculus.The fibres reach all levels of the spinal cord. They end directly or through the interneurons, on alpha and gamma motor neurons. The tract is concerned with postural adjustments of the head, trunk and limbs. Lateral reticulospinal tract is constituted by fibres arising in the ventrolateral part of the reticular formation of the pons.The fibres cross to the opposite side in the medulla and runs down the lateral funiculus.

Vestibulospinal tracts; these are grouped into two:

Lateral vestibulospinal tract; its neuron originate from the lateral vestibular nucleus. This tract is uncrossed and lays in the anterior feniculus of the spinal cord. Its fibres end in relation to the neurons in the ventral grey column. This tract is an important efferent part for equilibrium.

Medial vestibulospinal tract; this arise mainly from the medial vestibular nucleus (with some fibres from the inferior and lateral nuclei).The tract descend through the anterior funiculus.

Ascending Tracts connecting the spinal cord to the cerebral cortex

The posterior column (medial lemniscus pathway): fasiculus gracilus and fasciculus cuneatus are referred to as posterior column tracts because these tracts occupy the posterior funiculus of the spinal cord. They are formed predominantly by central processes of neurons located in the dorsal nerve root ganglia. The fibre of these feniculi extends upwards as far as the lower part of the medulla. The fibres if the gracilus and cunneate fasciculi terminate by synapsing with neurons in the nucleus gracilius and cunneate nuclei respectively. The neurons of the gracilus and cunneate nuclei are second order neurons. Their axons run forwards and medially (as internal arcuate fibres) to cross the middle line.

The crossing fibres of the two sides constitute the sensory decussation (or lemniscal decussation) having crossed the middle line, the fibres turn upwards to form a prominent bundle called the medial lemniscus.

The posterior-column (medial lemniscus pathway) is responsible for tactile localization, tactile discrimination, stereognosis, proprioception and the sense of vibration.

Spinothalamic pathways (Anterior and Lateral spinothalamic tracts): the cells of origin of these tracts arise from lamina IV, V, VI and VII.The axons of these neurons constitute the anterior and lateral spinothalamic tracts. They cross obliquely to the opposite side of the spinal cord in the white commisure.The fibres for the lateral spinothalamic tracts cross within the same segment of the cord to enter the lateral funiculus and runs through the brainstem as a separate bundle called the spinal lemniscus which ends in the thalamus while the anterior spinothalamic fibres may ascend for one or more segment before they cross to the opposite side and enter the anterior funiculus.It also join the medial lemniscus and travels through it to the thalamus. The two tracts form one continuous band that runs up the spinal cord, which later separate at the medulla end in the ventral posterolateral nucleus.

The anterior spinothalamic tract carries sensations of crude touch and pressure while the lateral spinothalamic tracts carry sensation of pain and temperature.

Amyotrophic lateral sclerosis (ALS) was first described by Charcot in 1869 (Wikipedia, 2007) and is known as Lou Gehrig’s disease, as a tribute to an American baseball player who died of amyotrophic lateral sclerosis in 1941, (Andreza et al, 2006).

The word amyotrophic is present Greek in origin. “A” means no or negative, “myo” refers to muscle, and “trophic” means nourishment. When put together it means “no-muscle-nourishment. “Lateral” identifies the areas of the spinal cord where portions of the nerve cells that signal and control the muscles are located. As this area degenerates it leads to scarring or hardening (sclerosis) in the region, (Wikipedia, 2007)

It’s (amyotrophic lateral sclerosis .ie.ALS) sometimes called, Maladie de Charcot or motor neurone disease. It is a progressive, fatal, neurodegenerative disease caused by the degeneration of motor neurons, the nerve cells in the central nervous system that control voluntary muscle movement, (ALSA, 2007). Motor neurons are nerve cells located in the brain, brainstem, and spinal cord that serve as controlling units and vital communication links between the nervous system and the voluntary muscles of the body. Messages from motor neurons in the brain (called upper motor neurons) are transmitted to motor neurons in the spinal cord (called lower motor neurons) and from them to particular muscles. In amyotrophic lateral sclerosis, both the upper motor neurons and the lower motor neurons degenerate or die, ceasing to send messages to muscles. Unable to function, the muscles gradually weaken, waste away (atrophy), and twitch (fasciculations). Eventually, the ability of the brain to start and control voluntary movement is lost, (NINDS, 2003). The disease does not necessarily debilitate the patient’s mental function, those suffering advanced stages of the disease may retain the same memories, personality, and intelligence they had before its onset, (ALSA, 2007). It does not affect a person’s ability to see, smell, taste, hear, or recognize touch. Patients usually maintain control of eye muscles and bladder and bowel functions, although in the late stages of the disease most patients will need help getting to and from the bathroom, (NINDS, 2003).

The resultant clinical features of amyotrophic lateral sclerosis include weakness of the arms, legs, and face and difficulties with speech, swallowing, and breathing. Usually, amyotrophic lateral sclerosis comes on slowly, starting out as weakness in one or more muscles. The first symptoms of amyotrophic lateral sclerosis can include weakness in the extremities, head drop, dysarthria, and dysphagia. About 75% of patients present with onset in the limbs; about 21% present with onset in the bulbar area. Weakness usually progresses slowly, but can progress rapidly, (Christen, 2007). Only one leg or arm may be affected. People notice that they stumble, having trouble lifting things, or have trouble with using their hands. As the disease progresses, the person with this condition will not be able to stand or walk, have trouble moving around, and trouble talking and swallowing, (Miller, 2001).

The exact cause of amyotrophic lateral sclerosis is unknown. Although about 10% of cases of amyotrophic lateral sclerosis are hereditary (run in families), the other 90% are not. A mutation of a gene called SOD1 has been identified in some of the hereditary cases, but the role mutation plays in the disease is unknown, (eMedicine, 2007)

The incidence is approximately 2 per 100,000 persons per year, (Richard, 2007). Amyotrophic lateral sclerosis affects women and men, regardless of ancestry, and the risk of disease increases with age, (Richard, 2007). Amyotrophic lateral sclerosis affects about 60,000 people worldwide and there is an unexplained increased incidence of amyotrophic lateral sclerosis in Guam and certain areas of Japan. This form of amyotrophic lateral sclerosis usually occurs at the rate of about one new case per 50,000 people per year. In the United States there are roughly 30,000 afflicted individuals. More than 3,000 Canadians have amyothrophic lateral sclerosis, (Encarta, 2005).

The onset, or first appearance, of amyotrophic lateral sclerosis usually occurs between the ages of 40 and 70, although it may affect people anywhere from 20 to 90 years of age. The onset and course of ALS varies from person to person (Encarta, 2005)

Average survival time ranges from 3 to 5 years after onset of symptoms, although some patients survive much longer, (Christen, 2007).

The diagnosis of amyotrophic lateral sclerosis is based on the symptoms and signs the physician observes, as well as tests eliminating all the other possibilities, such as multiple sclerosis, post-polio syndrome, or infectious diseases, (Miller, 2001). People are most commonly diagnosed with amyotrophic lateral sclerosis between the ages of 40 and 70, but younger people can also develop it. Amyotrophic lateral sclerosis is diagnosed in about 5000 people each year in the United States, where about 20,000 people are believed to have the condition. It affects all races and ethnic groups, (eMedicine, 2007)

Care of ALS patients is provided collaboratively by multidisciplinary ALS clinics and patients’ family physicians. After referring patients to neurologists, family physicians’ role is to help with treatment of symptoms, to monitor pulmonary status and provide early treatment for pneumonia, to provide emotional support, and to assist with end-of-life care, (Christen, 2007.).

Physical therapy and special equipment can enhance patients’ independence and safety throughout the course of the disease, (NINDS, 2003). Physical therapists can recommend exercises that provide these benefits without overworking muscles. Occupational therapists can suggest devices such as ramps, braces, walkers, and wheelchairs that help patients conserve energy and remain mobile, (NINDS, 2003)