Analysis of large patient samples drawn from clinic based populations or population registries consistently show that the

overall median survival from onset of symptoms for ALS ranges between 23 years for bulbar onset cases and 35 years for limb onset ALS cases [12,58,261]. Large clinic cohort studies have shown 3 year and 5 year survival tares to be around 48% and 24% respectively, with approximately 4% surviving longer than 10 years [262,263], whereas 5 year survival reported in population based studies is much lower and ranges from 430% [12]. Important prognostic indicators of survival consistently arising from population based studies and clinic cohort studies include clinical phenotype (PMA and Flail arm have better prognosis than typical forms) [264], site of onset (bulbar vs. limb onset) [17,261,264-266], age of symptom onset [58,261,264-267], shorter time from symptom onset to diagnosis [266], baseline FVC decline [261,265,268], El Escorial category at presentation [17,267] and riluzole use [265,269]. Orphanet Journal of Rare Diseases Amyotrophic lateral sclerosis is usually considered a disease that will have a fatal termination in 1 to 3 years. A prospective study of 100 patients with this disorder revealed that 20 of them were living 5 years after the onset of their disorder. Review of other published series reveals that patients have been reported who lived for longer than 5 years and have then usually been reported as atypical cases, although the only way in which they are described as atypical is the duration of the disease. It is our assumption, based on these data as well as on additional clinical observations, that many patients with amyotrophic lateral sclerosis live for longer than 5 years and, rarely, they may have remissions of their illness. The possible significance of these observations is discussed. http://ukpmc.ac.uk/abstract/MED/957788

Team Effort for Treatment of ALS Other ALS treatments are designed to relieve symptoms and improve a person's quality of life. Multidisciplinary teams of healthcare professionals are available to provide supportive care, including:

Physicians Pharmacists Physical, occupational, and speech therapists Nutritionists Social workers Home care and hospice nurses.

These teams can design an individualized ALS treatment plan of medical and physical therapy, and provide special equipment aimed at keeping people as mobile and comfortable as possible.

Medications Used in ALS Treatment Physicians can prescribe medications for the treatment of ALS symptoms. These medications help to:

Reduce fatigue Ease muscle cramps Control spasticity Reduce excess saliva and phlegm.

Medications are also available to help people with pain, depression, sleep disturbances, and constipation. Pharmacists can give advice on the proper use of medications and monitor a person's prescriptions to avoid the risk of drug interactions.

Physical Therapy as a Treatment for ALS Treating ALS will often involve physical therapy. Physical therapy and special equipment can enhance a person's independence and safety throughout the course of ALS. Gentle, low-impact aerobic exercise, such as walking, swimming, and stationary bicycling, can help people:

Strengthen unaffected muscles Improve cardiovascular health 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. Occupational therapists can suggest devices such as ramps, braces, walkers, and wheelchairs that help people conserve energy and remain mobile.

Speech Therapy as a Treatment for ALS People with ALS who have difficulty speaking may benefit from working with a speech therapist. A speech therapist can teach people adaptive strategies to help them speak louder and more clearly.

As the disease progresses, speech therapists can help people develop ways for responding to yes-or-no questions with their eyes or by other nonverbal means. They can also recommend aids such as speech synthesizers and computer-based communication systems. These methods and devices help people communicate when they can no longer speak or produce vocal sounds.

ALS Treatment Through Nutrition Nutritionists can help people with ALS and their caregivers plan and prepare numerous small meals throughout the day that provide enough calories, fiber, and fluid. Nutritionists can also teach how to avoid foods that are difficult to swallow. People may begin using suction devices to remove excess fluids or saliva and prevent choking. When they can no longer get enough nourishment from eating, doctors may advise inserting a feeding tube into the stomach, which reduces the risk of choking and pneumonia that can result from inhaling liquids into the lungs. The tube is not painful and does not prevent people from eating food orally.

Breathing Aids as a Treatment of ALS When the muscles that assist in breathing weaken, nocturnal ventilator assistance may be used to aid breathing during sleep. Such devices artificially inflate the lungs from various external sources that are applied directly to the face or body. When muscles are no longer able to maintain oxygen and carbon dioxide levels, these devices may be used full-time. People may eventually consider forms of mechanical ventilation (respirators) during later treatment of ALS. This type of machine inflates and deflates the

lungs. To be effective, this may require a tube that passes from the nose or mouth to the windpipe (trachea). For long-term use, an operation such as a tracheostomy, in which a plastic breathing tube is inserted directly into the windpipe through an opening in the neck, may be needed. People living with ALS and their families should consider several factors when deciding whether and when to use one of these options. Ventilation devices differ in their effect on quality of life and in cost. Although ventilation support can ease problems with breathing and prolong survival, it does not affect the progression of ALS. People need to be fully informed about these considerations and the long-term effects of life without movement before they make decisions about ventilation support.

Last updated/reviewed: May 07, 2010 Written by/reviewed by: Arthur Schoenstadt, MD Last reviewed by: Arthur Schoenstadt, MD Copyright 2006-2012 Clinaero, Inc. eMedTV serves only as an informational resource. This site does not dispense medical advice or advice of any kind. Site users seeking medical advice about their specific situation should consult with their own physician. Click Terms of Use for more information.

FISIOTERAPI 1. Treatmen dan atau nasehat nasehat akan tergantung pada masalah masalah

pada pasien saat itu. 2. Jika terdapat spastisitas akan mengakibatkan ketidaknyamanan pada pasien pada saat melakukan fungsi yang normal. 3. Relaksasi otot dapat membantu untuk ekstermitas atas dan latihan aktif atau latihan aktif assisted diberikan untuk mempertahankan ROM. 4. Spastisitas pada tungkai memungkinkan pasien untuk berdiri sehingga tidak dianjurkan untuk memberikan relaksasi otot. Saat otot menjadi lemah pasien dianjurkan untuk mempertahankan fungsi selama mungkin. 5. Jika pasien tidak mampu melakukan gerakan, latihan ( gerakan ) aktif assisted atau pasif diberikan untuk ROM 6. Hal ini sangat penting karena apabila sudah terdapat kekakuan sendi akan menjadi sangat sulit bagi yang merawat untuk menggerakkan pasien juga akan menimbulkan nyeri yang mengganggu, oleh karena itu mempertahankan ROM akan membantu mencegah masalah masalah tersebut. 7. Jika bagi pasien sangat mengunjungi klinik fisioterapi dan waktu treatmen harus tetap diberikan walaupun ini minim maka fisioterapis dapat memberitahu pasien dan yang merawatnya apa yang harus dilakukan di rumah. 8. Pada kondisi seperti ini fisioterapi harus yakin adanya pengawasan yang teratur terhadap kondisi fisik pasien dan pencatatan yang lengkap. 9. Pasien harus tahu bahwa ada bantuan yang tersedia pada saat dia membutuhkannya. Pasien dengan masalah pernafasan membutuhkan latihan latihan pernafasan dan pengeluaran slem. Akan sangat sulit bagi pasien untuk batuk karena adanya kelemahan otot dan akan sangat membantu memberikan support pada dada pasien pada saat pasien akan batuk dengan tambahan fibrasi untuk melepaskan sekresi bila memang dibutuhkan. 10. Alih baring posisi dapat membantu pasien tapi posisi postural drainage khususnya untuk lobus paling bawah biasanya sangat membuat pasien tertekan. 11. Pasien dapat mengalami hal hal tidak baik dan tidak nyaman dari posisi yang jelek, oleh karena itu fisioterapi harus membantu pasien untuk mencari posisi yang nyaman dan tersanggah baik sehingga mencegah deformitas. 12. Fisioterapi harus memberikan semangat dan simpati sehingga pasien dapat

mempertahankan kemandirian selama mungkin. Hal ini penting sehingga pasien tidak mempunyai harapan kosong.

IMPLIKASI KHUSUS BAGI FISIOTERAPI Kekuatan otot menurun sepanjang perjalanan penyakit secara progresif rata rata kehilangan adalah stabil setelah tahun pertama, tetapi selama tahun pertama terdapat fluktuasi kekuatan yang merupakan potensial adaptasi dari system saraf pusat. Pada titik ini tujuan terapi adalah memelihara aktifitas fisik umum dan tonus otot. Latihan latihan regular sampai sedang dapat membantu mengurangi kelelahan dan keuntungan lainnya adalah baik untuk kesehatan pasien secara umum. Kontribusi spastisitas yang menyebabkan keluhan kelemahan dengan memberikan stretching yang perlahan sehingga mengurangi tonus otot juga sangat baik diberikan. Kram yang menyebabkan sumber rasa nyeri juga berdampak baik bila diberikan stretching setiap hari secara teratur.

Perubahan pola jalan, jelas terlihat dan analisa jalan sangat dibutuhkan untuk memberikan alat bantu jalan yang sesuai untuk pasien. Jatuh yang mendadak dan berat disebabkan oleh kelemahan otot adalah masalah utama yang sering terjadi. Kemampuan dorsi fleksi ankle hilang sebelum hilangnya kekuatan plantar fleksi ankle. Kekuatan hamstring yang berhubungan dengan fungsi berjalan menjadi isometric otot bawah persentase normal akan secara dramatis menurun dan perlahan pada saat ditemukan hilangnya kemampuan fungsional yang hebat. Jumlah otot aktif yang sedikit, yang melewati sendi dibutuhkan untuk menjalankan fungsi normal suatu sendi. Beberapa gerakan dan sendi yang stabil dipelihara sampai terjadinya degerasi yang menyebabkan atropi sehingga aktifitas kekuatan otot berkurang, kurang dari 20 % dari normal. Kelemahan dan pembuangan sering kali nyeri menyebabkan nyeri karena subluksasi sendi scapulohumeral dan lengan lurus tersanggah dengan bantuan dari luar. Kontraktur harus secara teratur di ulur ( stretched ) untuk menjaga agar sendi tetap dalam kondisi yang baik walaupun hanya terhadap kontrol yang minimal dari aktifitas otot pada tahap lanjut. Keluhan nyeri dapat diawali saat pasien tidak dapat

memindahkan berat badan kondisi yang baik walaupun hanya terdapat kontrol yang minimal dari aktifitas otot pada tahap lanjut. Keluhan nyeri dapat di awali saat klien tidak dapat memindahkan berat badan dalam kondisi tidur atau duduk. Merubah sudut ketinggian tempat tidur atau kursi roda ataupun posisi tungkai dapat mengurangi rasa nyeri. Perawatan pasien atau yang merawat pasien harus diajarkan tehnik perawatan tersebut yang di atas.

Braces atau alat bantu yang lain, skooter atau kursi roda dapat membantu memelihara mobilitas dan kebebasan pasien. Beberapa pasien dapat diajarkan untuk meningkatkan inspirasi dan ekspirasi pada otot diagfragma dan otot intercostal melemah. Pada tahap teminal kenyaman pasien adalah tujuan terapi. Seperti table di bawah ini menjelaskan intervensi yang berhubungan dengan fase fase penyakit ALS. Copyright 2010 - Fisio's Blog

Treatment of ALS with stem cells Indications for ALS treatment with stem cells include confirmed ALS diagnosis, disease progression, and lack of response to existing treatment methods. Prior to year 2000, contraindications to treatment included respiratory failure, grade III, artificial lung ventilation, dysphagia, and presence of gastric stoma. However, nowadays the clinic has experience of treating patients with the above-mentioned complications and disorders that allows for prolonging their life by more than five years. After transplantation of fetal stem cells, 67% of ALS patients note diminishing weakness, improvement of appetite and mood, decrease in intensity of fasciculations and spasticity. Within two months after transplantation, increase of motion volume in extremities, decrease in muscular rigidity, normalization of reflexes, decrease in the number of fasciculation zones, better tolerability of daily chores, reduction in intensity of dysphagia and dysarthria (improvement of swallowing, articulation, and clarity in

pronunciation of words) have been observed in 34% of patients. Stable positive clinical effect after treatment persists for three to six months. About 48% of patients need to repeat the course of treatment during the year for retaining the attained positive results. In 25% of cases, patients need periodic transplantations of stem cells every 1.52 years. Duration of the ALS treatment course at EmCell clinic, as a rule, totals 2 days. Transplantation of fetal stem cells does not combat the immediate cause of the disease (to date, the exact cause of disease has not been found); however, it exerts substantial influence on links of the pathological process and slows down disease progression. Transplantation of fetal stem cells improves the quality of life of ALS patients and their ability to work, and prolongs their life. 1999-2012 EmCell.com

Scientists have not found a cure for ALS (amyotrophic lateral sclerosis). However, the U.S. Food and Drug Administration (FDA) has approved the first drug treatment for ALS, which is called riluzole. Riluzole is believed to:

Reduce damage to motor neurons by decreasing the release of glutamate

Prolong survival by several months (mainly in those with difficulty swallowing)

Extend the time before a person needs ventilation support.

Riluzole does not reverse the damage that has already occurred to the motor neurons, and people taking it must be monitored for liver damage and other possible side effects.

No cure has yet been found for ALS. However, the Food and Drug Administration (FDA) has approved the first drug treatment for the diseaseriluzole (Rilutek). Riluzole is believed to reduce damage to motor neurons by decreasing the release of glutamate. Clinical trials with ALS patients showed that riluzole prolongs survival by several months, mainly in those with difficulty swallowing. The drug also extends the time before a patient needs ventilation support. Riluzole does not reverse the damage already done to motor neurons, and patients taking the drug must be monitored for liver damage and other possible side effects. However, this first disease-specific therapy offers hope that the progression of ALS may one day be slowed by new medications or combinations of drugs. Other treatments for ALS are designed to relieve symptoms and improve the quality of life for patients. This supportive care is best provided by multidisciplinary teams of health care professionals such as physicians; pharmacists; physical, occupational, and speech therapists; nutritionists; social workers; and home care and hospice nurses. Working with patients and caregivers, these teams can design an individualized plan of medical and physical therapy and provide special equipment aimed at keeping patients as mobile and comfortable as possible. Physicians can prescribe medications to help reduce fatigue, ease muscle cramps, control spasticity, and reduce excess saliva and phlegm. Drugs also are available to help patients with pain, depression, sleep disturbances, and constipation. Pharmacists can give advice on the proper use of medications and monitor a patient's prescriptions to avoid risks of drug interactions. Physical therapy and special equipment can enhance patients' independence and safety throughout the course of ALS. 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. Occupational therapists can suggest devices such as ramps, braces, walkers, and wheelchairs that help patients conserve energy and remain mobile. ALS patients who have difficulty speaking may benefit from working with a speech therapist. These health professionals can teach patients adaptive strategies such as techniques to help them speak louder and more clearly. As ALS progresses, speech therapists can help patients develop ways for responding to yes-or-no questions with their eyes or by other nonverbal means and can recommend aids such as speech synthesizers and computer-based communication systems. These methods and devices help patients communicate when they can no longer speak or produce vocal sounds. Patients and caregivers can learn from speech therapists and nutritionists how to plan and prepare numerous small meals throughout the day that provide enough calories, fiber, and fluid and how to avoid foods that are difficult to swallow. Patients may begin using suction devices to remove excess fluids or saliva and prevent choking. When patients can no longer get enough nourishment from eating, doctors may advise inserting a feeding tube into the stomach. The use of a feeding tube also reduces the risk of choking and pneumonia that can result from inhaling liquids into the lungs. The tube is not painful and does not prevent patients from eating food orally if they wish. When the muscles that assist in breathing weaken, use of nocturnal ventilatory assistance (intermittent positive pressure ventilation [IPPV] or bilevel positive airway pressure [BIPAP]) may be used to aid breathing during sleep. Such devices artificially inflate the patient's lungs from various external sources that are applied directly to the face or body. When muscles are no longer able to maintain oxygen and carbon dioxide levels, these devices may be used full-time. Patients may eventually consider forms of mechanical ventilation (respirators) in which a machine inflates and deflates the lungs. To be effective, this may require a tube that passes from the nose or mouth to the windpipe (trachea) and for long-term use, an operation such as a tracheostomy, in which a plastic breathing tube is inserted directly in the patient's windpipe through an opening in the neck. Patients and their families should consider several factors when

deciding whether and when to use one of these options. Ventilation devices differ in their effect on the patient's quality of life and in cost. Although ventilation support can ease problems with breathing and prolong survival, it does not affect the progression of ALS. Patients need to be fully informed about these considerations and the long-term effects of life without movement before they make decisions about ventilation support. Social workers and home care and hospice nurses help patients, families, and caregivers with the medical, emotional, and financial challenges of coping with ALS, particularly during the final stages of the disease. Social workers provide support such as assistance in obtaining financial aid, arranging durable power of attorney, preparing a living will, and finding support groups for patients and caregivers. Respiratory therapists can help caregivers with tasks such as operating and maintaining respirators, and home care nurses are available not only to provide medical care but also to teach caregivers about giving tube feedings and moving patients to avoid painful skin problems and contractures. Home hospice nurses work in consultation with physicians to ensure proper medication, pain control, and other care affecting the quality of life of patients who wish to remain at home. The home hospice team can also counsel patients and caregivers about endof-life issues. What research is being done?

The National Institute of Neurological Disorders and Stroke, part of the National Institutes of Health, is the Federal Government's leading supporter of biomedical research on ALS. The goals of this research are to find the cause or causes of ALS, understand the mechanisms involved in the progression of the disease, and develop effective treatment. Scientists are seeking to understand the mechanisms that trigger selective motor neurons to degenerate in ALS and to find effective approaches to halt the processes leading to cell death. This work includes studies in animals to identify the means by which SOD1 mutations lead to the destruction of neurons. The excessive accumulation of free radicals, which has been implicated in a number of neurodegenerative diseases including ALS, is also being closely studied. In addition, researchers are examining how the loss of neurotrophic factors may be

involved in ALS. Neurotrophic factors are chemicals found in the brain and spinal cord that play a vital role in the development, specification, maintenance, and protection of neurons. Studying how these factors may be lost and how such a loss may contribute to motor neuron degeneration may lead to a greater understanding of ALS and the development of neuroprotective strategies. By exploring these and other possible factors, researchers hope to find the cause or causes of motor neuron degeneration in ALS and develop therapies to slow the progression of the disease. Researchers are also conducting investigations to increase their understanding of the role of programmed cell death or apoptosis in ALS. In normal physiological processes, apoptosis acts as a means to rid the body of cells that are no longer needed by prompting the cells to commit "cell suicide." The critical balance between necessary cell death and the maintenance of essential cells is thought to be controlled by trophic factors. In addition to ALS, apoptosis is pervasive in other chronic neurodegenerative conditions such as Parkinson's disease and Alzheimer's disease and is thought to be a major cause of the secondary brain damage seen after stroke and trauma. Discovering what triggers apoptosis may eventually lead to therapeutic interventions for ALS and other neurological diseases. Scientists have not yet identified a reliable biological marker for ALSa biochemical abnormality shared by all patients with the disease. Once such a biomarker is discovered and tests are developed to detect the marker in patients, allowing early detection and diagnosis of ALS, physicians will have a valuable tool to help them follow the effects of new therapies and monitor disease progression. NINDS-supported researchers are studying families with ALS who lack the SOD1 mutation to locate additional genes that cause the disease. Identification of additional ALS genes will allow genetic testing useful for diagnostic confirmation of ALS and prenatal screening for the disease. This work with familial ALS could lead to a greater understanding of sporadic ALS as well. Because familial ALS is virtually indistinguishable from sporadic ALS clinically, some researchers believe that familial ALS genes may also be involved in the manifestations of the more common sporadic form of ALS. Scientists also hope to identify genetic risk factors that predispose people to sporadic ALS.

Potential therapies for ALS are being investigated in animal models. Some of this work involves experimental treatments with normal SOD1 and other antioxidants. In addition, neurotrophic factors are being studied for their potential to protect motor neurons from pathological degeneration. Investigators are optimistic that these and other basic research studies will eventually lead to treatments for ALS. Results of an NINDS-sponsored phase III randomized, placebo-controlled trial of the drug minocycline to treat ALS were reported in 2007. This study showed that people with ALS who received minocycline had a 25 percent greater rate of decline than those who received the placebo, according to the ALS functional rating scale (ALSFRS-R). Last updated April 3, 2012 "ALS (Amyotrophic Lateral Sclerosis) Fact Sheet," NINDS. Publication date February 2010.

NIH Publication No. 10-916

Because the exact causes of the disease haven't been pinpointed, ALS can neither be prevented nor cured. Medications are available, however, that may slow the progress of the disease and that deal with some of the symptoms. Riluzole* is a medication that appears to prolong the life of some people with ALS by at least a few months. Riluzole seems to do two things: slow the release of glutamate from neurons and block its effects on glutamate receptors. Muscle spasms - and sometimes cramps - can be controlled with the medications baclofen or diazepam. Medications can also be used to lower the amount of saliva that's made to help people who've lost the ability to swallow it. To avoid choking or inhaling food and saliva into the lungs, a feeding tube might need to be placed into the stomach. People with difficulty breathing will eventually have to go on a respirator. Physical therapy can help people maintain strength in their muscles and

control muscle contractions. Speech therapy can help people with speech and swallowing difficulties. 1996 - 2012 MediResource Inc.

There is no known cure for ALS. The first drug treatment for the disease is a medicine called riluzole. Riluzole slows the disease progression and prolongs life. Treatments to control symptoms are also helpful:

Baclofen or diazepam may be used to control spasticity that interferes with daily activities.

Trihexyphenidyl or amitriptyline may be prescribed for people with problems swallowing their own saliva.

Physical therapy, rehabilitation, use of braces or a wheelchair, or other orthopedic measures may be needed to maximize muscle function and general health. Choking is common. Patients may decide to have a tube placed into their stomach for feeding. This is called a gastrostomy. A nutritionist is very important. Patients with ALS tend to lose weight. The illness itself increases the need for food and calories. At the same time, problems with swallowing make it hard to eat enough. Breathing devices include machines that are used only at night, and constant mechanical ventilation. Patients should discuss their wishes regarding artificial ventilation with their families and doctors. MedlinePlus

Therapy Pharmacotherapy Riluzole, a glutamate antagonist, is the only drug approved by the Food and Drug Administration for the treatment of ALS (Table 3Table 3 Therapy for ALS.). In two therapeutic trials, riluzole prolonged survival by three to six months.124,125 In one of these trials,124 treatment slightly slowed the decline in the strength of limb muscle; there was no benefit with respect to many measures of function in either trial. In one retrospective analysis,126 patients who received riluzole remained in a milder stage of disease longer than did controls. For patients, the effects are invisible. The efficacy of riluzole has been taken as evidence in support of the excitotoxicglutamate theory of the pathogenesis of ALS. But other glutamate antagonists, including branched-chain amino acids, lamotrigine, and dextromethorphan, had no beneficial effects in clinical trials.127,128 When tested in transgenic mice with mutant SOD1, gabapentin, like riluzole, extended survival but did not significantly affect the onset of clinical disease.129 In contrast, vitamin E delayed the onset and the progression of the disease but failed to extend survival. Despite the moderate benefits of these agents in mice, gabapentin and vitamin E were of no benefit in trials of patients with ALS.130,131 More than 60 years ago, Wechsler touted the benefits of vitamin E in a series of patients with ALS.132 Although Wechsler reported an improvement in the condition of Patient 4, identified on the basis of his initials and age as Lou Gehrig himself, Gehrig nevertheless died within a year. Other treatments have also failed in clinical trials (Table 3). Agents that are currently being evaluated include xaliproden (which may foster the release of neurotrophic factors), creatine,133 coenzyme Q10, intrathecally administered (by lumbar puncture) brain-derived neurotrophic factor, and orally administered brain-derived neurotrophic factor.134 Inhibitors of cyclooxygenase-2135 and caspase inhibitors are being considered, and high-throughput drug development is on the horizon.136

Reliable cell-based or other in vitro assays are needed to expedite the process of identifying potential therapies. Mechanical Ventilatory Support The central problem of treatment is the decision ultimately faced by all patients: whether to elect to undergo a tracheostomy for long-term mechanical ventilation. That choice can be postponed by the use of noninvasive positive-pressure ventilation, which relieves symptoms and prolongs life. Few patients actually agree to the use of mechanical ventilation, because it invokes the prospect of years of total immobility and limited communication and places a heavy burden on their families. Treatment for Depression Because it is widely believed that everyone who is given a diagnosis of ALS becomes depressed, antidepressant drugs are often prescribed, but there have been no trials to evaluate the effects of this practice. In two studies involving 100 patients with ALS, clinical depression was found in only 11 percent.137,138 Psychological and spiritual considerations are also determinants of the quality of life.139,140 In addition, health care workers are treating physical symptoms more actively.141 Proposed Treatments Therapeutic trials have become increasingly well organized, and most have been funded by pharmaceutical companies. The lack of effective treatment has caused many patients and their families to become activists, raising money for research and bypassing traditional granting agencies.142 This guerrilla science approach has led to proposals for gene therapy. Such approaches must first be attempted in animals to evaluate their safety and efficacy. One approach is to use a viral vector to deliver the gene for EAAT2 into the spinal cord by an intraparenchymal injection in an attempt to lower circulating glutamate levels.143 The aim of another project is to restore motor function by introducing human stem cells into the spinal cord to replace degenerating motor neurons. Stem-cell therapy for ALS was propelled by four 1999 reports that described how stem cells made their way to the proper location, settled, and replaced dysfunctional cells.144

In the case of ALS, this approach will be particularly difficult because of the complex pathways involved in motor function. Precise connections have to be restored between motor neurons, target muscle, and descending motor systems. Nevertheless, stem-cell therapy may be of protective value, slowing or preventing further neuronal degeneration. NEJM.org Copyright 2012

Treatments and drugs By Mayo Clinic staff Because there's no reversing the course of amyotrophic lateral sclerosis, treatments mainly involve efforts to slow the progression of symptoms and make you more comfortable and independent. Medications The drug riluzole (Rilutek) is the first and only medication approved by the Food and Drug Administration for slowing ALS. The drug appears to slow the disease's progression in some people, perhaps by reducing levels of glutamate a chemical messenger in the brain that's often present in higher levels in people with ALS. Your doctor may also prescribe medications to provide relief from:

Muscle cramps Constipation Fatigue Excessive salivation Excessive phlegm Pain Depression

Therapy

Physical therapy. A physical therapist can recommend low-impact exercises to maintain your cardiovascular fitness, muscle strength and range of motion as long as possible, helping you preserve a sense of independence. Regular exercise can also help improve your sense of well-being.

Occupational therapy. An occupational therapist can help you become accustomed to a brace, walker or wheelchair and may be able to suggest devices such as ramps that make it easier for you to get around.

Speech therapy. Because ALS affects the muscles you use to speak, communication becomes an issue as the disease progresses. A speech therapist can help teach adaptive techniques to make your speech more clearly understood or help you explore alternative methods of communication, such as an alphabet board or simple pen and paper. Later in the disease, a speech therapist can recommend devices such as speech synthesizers and computer-based equipment that may help you communicate. Ask your therapist about the possibility of borrowing or renting these devices. 1998-2012 Mayo Foundation for Medical Education and Research

There is no cure for ALS, but treatment can help you stay strong and independent for as long as possible. It can also help you avoid other problems from ALS. For example:

Physical and occupational therapy can help you stay strong and make the most of the abilities you still have.

Speech therapy can help you keep your ability to talk after problems with speech begin.

Supportive devices and equipment can help you stay mobile, communicate, and do daily tasks like bathing, eating, and dressing. Some examples are canes, walkers, wheelchairs, ramps, handrails, raised toilet seats, and shower seats. You can also get braces to support your feet, ankles, or neck.

Medicines can help relieve your symptoms and keep you comfortable. There are medicines that can help with many of the symptoms you might have, such as muscle problems (stiffness, cramps, twitching), drooling and extra saliva, depression and mood swings, and pain.

Breathing devices can help you breathe more easily as your chest muscles weaken. A medicine called riluzole (Rilutek) may prolong survival by about 2 months.2 But it doesn't improve symptoms or quality of life in ways that people with ALS, their caregivers, or their doctors have been able to notice. Most people tolerate riluzole very well, but it can cause side effects, including nausea, vomiting, weakness, dizziness, and coughing. Treatment with riluzole is also expensive, and it may not help some people.

2005-2012 WebMD, LLC

Amyotrophic Lateral Sclerosis (ALS) - Treatment Overview Although there is no cure for amyotrophic lateral sclerosis (ALS), treatment can help you maintain strength and independence, manage symptoms, and avoid complications for as long as possible. Treatment also focuses on providing emotional support as your disability increases. Physical therapists and occupational therapists can help you maintain strength and function and make the most of your remaining abilities. Speech therapists can help you maintain your ability to communicate after speaking problems begin. Recommended Related to Brain & Nervous System General Information About Pituitary Tumors A pituitary tumor is a growth of abnormal cells in the tissues of the pituitary gland. Pituitary tumors form in the pituitary gland, a pea-sized organ in the center of the brain, just above the back of the nose. The pituitary gland is sometimes called the "master endocrinegland" because it makes hormones that affect the way many parts of the body

work. It also controls hormones made by many other glands in the body. Anatomy of the inside of the brain, showing the pineal and pituitary glands, optic... Read the General Information About Pituitary Tumors article > > Medicines may be used to relieve symptoms and make you more comfortable. These include:

Glycopyrrolate (Robinul), benztropine, transdermal hyoscine (Scopolamine), atropine, trihexyphenidyl hydrochloride, or amitriptyline for extra saliva or drooling. These medicines may be helpful if you have trouble with the muscles that control swallowing.

Amitriptyline or fluvoxamine (Luvox) to help with uncontrollable emotions and mood swings (emotional lability).

Baclofen (Lioresal), tizanidine (Zanaflex), dantrolene (Dantrium), or benzodiazepines such as diazepam (Valium), to relieve muscle stiffness, spasms, and twitching.

Quinine, phenytoin sodium (Dilantin), benzodiazepines, or gabapentin to relieve muscle cramps.

Antidepressant medicines, to help with depression, sleeplessness, poor appetite, or fatigue.

Pain relievers, to reduce muscle pain, which sometimes develops late in the course of the disease.

Morphine, to help with breathing problems (dyspnea). Antianxiety medicines such as diazepam or lorazepam may be helpful in relieving anxiety caused by breathing problems. The American Academy of Neurology also considers acupuncture a useful option for breathing problems when used in addition to medicine or breathing devices. 3 If you are having problems getting enough food or taking medicine, a tube can be inserted through the skin into the stomach (percutaneous endoscopic gastrostomy, or PEG). The tube provides an easier way to get adequate nutrition and take medicines. Breathing devices may be needed as the muscles of the chest become affected. Initially, extra oxygen can be given through a nasal cannula, a flexible plastic tube that is placed in the nostrils. For people who have mild breathing problems, nonsurgical forms of ventilation may be used. These include bilevel positive airway pressure

(BiPAP) and noninvasive positive pressure ventilation (NPPV), which deliver air or extra oxygen through a face mask. But in some cases, an opening in the neck leading to the windpipe (tracheostomy) may have to be created and oxygen delivered through a tube in the opening. At first, breathing devices may be needed only part of the time, such as when you are sleeping. As the disease and breathing problems get worse, you may need to be on a breathing machine all the time. Riluzole A medicine called riluzole (Rilutek) may prolong survival by about 2 months.4 It is not known exactly how the medicine works, but it may slow the release of certain brain chemicals (neurotransmitters) that are believed to play a role in ALS. Riluzole is the only medicine approved for the treatment of ALS. 1998-2012 Mayo Foundation for Medical Education and Research

KOMPLIKASI

Breathing in food or fluid (aspiration) Loss of ability to care for self Lung failure (See: Adult respiratory distress syndrome) Pneumonia Pressure sores Weight loss

MedlinePlus

Respiratory complications All deaths directly caused by ALS result from respiratory complications. This occurs primarily from the patient's inability to ventilate as respiratory muscle weakness progresses. In patients

with bulbar weakness, aspiration of secretions or food may occur and precipitate pneumonia, resulting in further respiratory compromise; therefore, aggressive respiratory management is necessary in the comprehensive care of patients with ALS. Routinely measure vital capacity in the sitting and recumbent positions. Most often, the recumbent measurement declines prior to the sitting measurement. Gravity assists in lowering the diaphragm as the patient's angle of inclination is increased. As respiratory weakness progresses, patients have increasing difficulty with diaphragmatic movement when supine because of the elimination of this effect from gravity. This results in alveolar hypoventilation and ultimate oxyhemoglobin desaturation. Difficulty sleeping may be the first symptom of hypoventilation. Patients should be questioned routinely regarding sleep habits, and if a sleep disturbance develops, measure vital capacities sitting and supine. In addition, perform overnight oxygen saturation monitoring to assess for nocturnal hypoxemia and the need for nocturnal noninvasive intermittent positive pressure ventilation (IPPV). WebMD

PROGNOSIS Over time, people with ALS progressively lose the ability to function and care for themselves. Death often occurs within 3 - 5 years of diagnosis. About 25% of patients survive for more than 5 years after diagnosis. MedlinePlus Analysis of large patient samples drawn from clinic based populations or population registries consistently show that the overall median survival from onset of symptoms for ALS ranges between 23 years for bulbar onset cases and 35 years for limb onset ALS cases [12,58,261]. Large clinic cohort studies have shown 3 year and 5 year survival tares to be around 48% and

24% respectively, with approximately 4% surviving longer than 10 years [262,263], whereas 5 year survival reported in population based studies is much lower and ranges from 430% [12]. Important prognostic indicators of survival consistently arising from population based studies and clinic cohort studies include clinical phenotype (PMA and Flail arm have better prognosis than typical forms) [264], site of onset (bulbar vs. limb onset) [17,261,264-266], age of symptom onset [58,261,264-267], shorter time from symptom onset to diagnosis [266], baseline FVC decline [261,265,268], El Escorial category at presentation [17,267] and riluzole use [265,269]. Orphanet Journal of Rare Diseases Amyotrophic lateral sclerosis is usually considered a disease that will have a fatal termination in 1 to 3 years. A prospective study of 100 patients with this disorder revealed that 20 of them were living 5 years after the onset of their disorder. Review of other published series reveals that patients have been reported who lived for longer than 5 years and have then usually been reported as atypical cases, although the only way in which they are described as atypical is the duration of the disease. It is our assumption, based on these data as well as on additional clinical observations, that many patients with amyotrophic lateral sclerosis live for longer than 5 years and, rarely, they may have remissions of their illness. The possible significance of these observations is discussed. http://ukpmc.ac.uk/abstract/MED/957788