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Als thar

  1. 1. “AMYOTROPHIC LATERAL SCLEROSIS AND IT’S PHYSICAL THERAPY MANAGEMENT” BY Lalruatfela Sailo A PROJECT SUBMITTED TO Hemwati Nandan Bahuguna Garhwal University, Srinagar (U.K) In partial fulfillment of The Requirement For The Degree Of Bachelor Of Physiotherapy Under The Guidance of Dr. Archana Chauhan, PT MPT (Neurology) Department Of Physiotherapy Shri Guru Ram Rai Institute Of Medical & Health Science College Of Paramedical Sciences Patel Nagar, Dehradun – (2009-2013)
  2. 2. DECLARATION BY THE CANDIDATE I hereby declared that the project work entitled “AMYOTROPHIC LATERAL SCLEROSIS AND IT’S PHYSICAL THERAPY MANAGEMENT” embodies the work done by me at Shri Guru Ram Rai Institute Of Medical And Health Sciences, Patel Nagar, Dehradun”. This project work in part or full has not been submitted to any other university. Lalruatfela Sailo Signature of Candidate (BPT IV Year) Place: Date: (i)
  3. 3. ENDORSEMENT BY THE HEAD OF THE DEPARTMENT This is to certify that the project work entitled “AMYOTROPHIC LATERAL SCLEROSIS AND IT’S PHYSICAL THERAPY MANAGEMENT” is a bonafied project work done by Lalruatfela Sailo under the guidance of Dr Archana Chauhan PT, MPT (Neurology) in partial fulfillment of the requirement for the degree of Bachelor of Physiotherapy. Place: Seal and Signature Date: Dr. Tarang Srivastava, PT MPT (Ortho), MIAP Head of department of Physiotherapy SGRRIMHS & SMI Hospital Patel Nagar, Dehradun (U.K) (ii)
  4. 4. CERTIFICATE BY THE GUIDE This is to certify that the project work entitled “AMYOTROPHIC LATERAL SCLEROSIS AND IT’S PHYSICAL THERAPY MANAGEMENT” submitted by Lalruatfela Sailo in partial fulfillment of the requirement for the award of the Bachelor of Physiotherapy of Hemwati Nandan Bahuguna Garhwal University, Srinagar, is bonafied word carried out by him under my supervision and guidance during the academic year 2009-2013. Neither this dissertation nor the part of it has been submitted for any degree or diploma. Place: Signature of Guide Date: Dr. Archana Chauhan, PT MPT (Neurology) (iii)
  5. 5. CERTIFICATE BY THE EXAMINER This is to certify that the project work entitled “AMYOTROPHIC LATERAL SCLEROSIS AND IT’S PHYSICAL THERAPY MANAGEMENT” submitted by Lalruatfela Sailo in partial fulfillment of the requirement for the award of the Bachelor of Physiotherapy of Hemwati Nandan Bahuguna Garhwal University, Srinagar, has been thoroughly examined and approved by us. Sign. of Internal Examiner Sign. of External Examiner Accepted/ not accepted Place: Date: (iv)
  6. 6. DEDICATED TO The Almighty, My Family, My Friends & My seniors Who have acted as a source of Inspiration, Encouragement And strength. (v)
  7. 7. ACKNOWLEDGEMENT It is my extreme pleasure to present the project and I, wholehearted thanks to each and every one who helped me in completing this task. I express my deep sense of gratitude to my esteemed teacher and guide Dr. Archana Chauhan, Lecturer, Department of Physiotherapy, SGRR Institute Of Medical And Health Sciences, Dehradun (U.K), who immensely helped and rendered her valuable advice, precious time, knowledge and relevant information regarding the collection of material and whose suggestion and guidance has enlightened on this subject. On this opportunity, I would like to thanks honorable Chairman Shri Devendra Das Ji Maharaj for providing all the facilities to carry out my project work with pride and pleasure. I humbly thank all the staff members of Physiotherapy Department for their co-operation and invaluable guidance and all the members of SGRRIMHS and SMI, who helped me throughout this project. I will be failing in my efforts if I do not extend my gratitude towards my family who has always inspiring and act as my strength throughout these years. I would always be indebted to my friends and my seniors who have been acted as a source of inspiration Above all, I thank God who have given me strength, health and blessings through all these years and guided me to complete my project work. (vi)
  8. 8. LIST OF ABBREVIATIONS 1) ALS - Amyotrophic Lateral Sclerosis 2) UMN – Upper motor neurone 3) LMN - Lower motor neurone; 4) FALS - Familial ALS 5) SALS - Sporadic ALS 6) SOD1 - Superoxide dismutase 1 7) TDP-43 - TAR DNA-binding protein 43 8) FUS - Fused in Sarcoma 9) CuZnSOD - Copper-Zinc superoxide dismutase 10) MnSOD - Manganese superoxide dismutase 11) ECSOD – Extracellular superoxide dismutase 12) CSF – Cerebrospinal Fluid 13) EAAT2 - Excitatory amino acid transporter-2. 14) EMG – Electromyography 15) PLS – Primary Lateral Sclerosis 16) PBP – Progressive Bulbar Palsy 17) PMA – Progressive Muscular Atrophy 18) DPS – Diaphragm Pacing System (vii)
  9. 9. 19) ROM – Range of Motion 20) FVC- Forced vital capacity 21) VC – Vital Capacity 22) Dx – Diagnosis 23) AFO – Ankle Foot Orthotic 24) MMSE – Mini Mental Status Examination 25) MMT – Manual Muscle Testing (viii)
  10. 10. INDEX CHAPTER PAGE NO. 1. Introduction……………………………………………………………1-3 2. Epidemiology …………………………………………………………4-5 3. Eitiology ……………………………………………………………....6-8 4. Anatomy……………………………………………………………….9-15 5. Pathophysiology……………………………………………………….16-19 6. Classification…………………………………………………………..27-59 7. Clinical Presentation..............................................................................28-31 8. Management……………………………………………………….......27-59 ·Medical Management.................................................................28-31 ·Surgical Management.................................................................32 ·Physiotherapy Management.......................................................33-59 9. Complications……………………………………………………….....60-61 10. Prognosis……………………………………………………………....62-63 11. Prevention...............................................................................................64-65 12. Conclusion..............................................................................................66-69 13. References …………………………………………………………….71-75 (ix)
  11. 11. LIST OF FIGURES FIGURE NO. Fig 4.1 TITLE PAGE NO. The difference between ALS-nerve cells and...........................10 muscle and normal nerve cells and muscle. Fig 4.2 Coricospinal tract......................................................................12 Fig 4.3 Spinothalamic tract...................................................................13 Fig 4.4 Spinocerebellar tract.................................................................14 Fig 4.5 Fasciculus gracilis and fasciculus cuneatus..............................15 Fig 5.1 A normal spinal cord is shown compared to the cord..............19 of a patient with ALS to highlight the difference in size of the nerve roots. Fig 8.1 Algorithm for respiratory management....................................42 Fig 8.2 Dysarthria in ALS. Medical, speech therapy.............................45 and other interventions. (x)
  12. 12. FIGURE NO. TITLE PAGE NO. Fig 8.3 Posture Check...........................................................................48 Fig 8.4 Standing extension....................................................................49 Fig 8.5 Press-ups...................................................................................50 Fig 8.6 Single knee to chest..................................................................50 Fig 8.7 Partial sit-up.............................................................................51 Fig 8.8 Shoulder exercise 1..................................................................52 Fig 8.9 Shoulder exercise 2..................................................................52 Fig 8.10 Shoulder exercise 3..................................................................53 Fig 8.11 Shoulder exercise 4..................................................................54 Fig 8.12 Shoulder ROM exercise 1........................................................55 Fig 8.13 Shoulder ROM exercise 2........................................................56 Fig 8.14 Elbow ROM exercise ..............................................................56 Fig 8.15 Hand and wrist ROM exercise 1..............................................57 Fig 8.16 Hand and wrist ROM exercise 2..............................................57 Fig 8.17 Hand and wrist ROM exercise 3..............................................58 Fig 8.18 Hand and wrist ROM exercise 4..............................................58 (xi)
  14. 14. Amyotrophic lateral sclerosis (ALS), sometimes known as Lou Gehrig’s disease is a condition that causes a person to become gradually and progressively weaker. This progressive weakness is due to degeneration of nerves in the anterior horn of the spinal cord, which transmits information from the brain to the muscles of the body. The patient experiences multiple losses such as that of mobility, speech, the ability to eat and drink, and independence. Most patients are not able to walk, get out of bed on their own, or use their hands and arms. The patient may ultimately lose the ability to initiate and control all the voluntary movements. 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. The bladder and bowel sphincters and the muscles responsible for eye movement are usually (but not always) spared The patient may also experience loss of some relationships and personal fulfillment. The frustration and loss of control may lead to anxiety, anger, depression and controlling behavior. Up to 50% of ALS patients have pseudo bulbar effect, pathological uncontrolled bouts of laughing or crying not concordant with their mood. It is the most common and devastatingly fatal motor neuron disease among adults. In some cases, ALS affects only one leg at first. Patients notice that they are awkward when they walk or run or that they stumble more often. Some ALS patients notice the first signs of ALS in their hand or arm. They may find that simple tasks such as buttoning a shirt, writing, or turning a key in a lock are difficult. Other patients notice speech problems. Disease progression tends to be slower in patients who are younger than 40 at onset, have disease restricted primarily to one limb, and those with primarily upper motor neuron symptoms. Conversely, progression is faster and prognosis poorer in patients with bulbar-onset disease, respiratory-onset disease, and frontotemporal dementia. ALS is more often found in the 40 to 70 year age group, though it can affect anyone. Once thought rare, it is in fact quite common. According to International Alliance of ALS there are nearly 120,000 cases diagnosed worldwide each year. That is 328 new cases every day! 2
  15. 15. The impact on the community of ALS is usually measured by the incidence and prevalence of the disease. Incidence is the number of new cases added in a defined period, usually a year. Prevalence is the number of cases existing at any point in time. The incidence of ALS is 2 per 100,000 of total population, while prevalence is 6 per 100,000 of total population. Research has found that the incidence is higher in people aged over 50 years. Only 10% of cases are familial (inherited) with the remaining 90% sporadic.In India Male to female ratio is 3:1 The defining feature of ALS is the death of both upper and lower motor neurons in the motor cortex of the brain, the brain stem, and the spinal cord. Prior to their destruction, motor neurons develop proteinaceous inclusions in their cell bodies and axons. This may be partly due to defects in protein degradation. These inclusions often contain ubiquitin, and generally incorporate one of the ALSassociated proteins: SOD1, TAR DNA binding protein (TDP-43, or TARDBP), or FUS. Physiotherapy aims to promote independence, by improving normal movement patterns. This means helping the patients to move with purpose to carry out daily activities, with the best possible coordination and the most efficient use of energy. It helps the individuals to achieve their maximum functional mobility, to promote independence, opportunity, control and dignity within the limits of their condition. It helps maintain maximum range of movement (ROM) and maintain comfort and reduce problems associated with muscle weakness. Physiotherapist should provide guidance on breathing management and how to conserve energy. Chest physiotherapy can also be beneficial during a chest infection, providing techniques to help clearing chest to aid breathing and speed up recovery. Emotional, psychological, social, and spiritual dimensions of care must always be considered by the therapist, along with the physical care. 3
  17. 17. It is estimated that 30,000 individuals in the United States have ALS at any one time and 15 cases of the disease are diagnosed per day. Except in a very few areas, such as Guam and the Kii Peninsula of Japan, more recent studies report the overall incidence of ALS to be in the range of 0.4 to 2.4 cases per 100,000, with the incidence increasing with each decade of life, until at least the seventh decade. The prevalence of ALS has been reported to be higher than the incidence, 4 to 10 cases per 100,000 In India, the male female ratio was 3.7:1. Six patients (5%) had familial ALS. The mean age of onset for all patients was 42.8±15.9 years, and duration of disease was 19.5±16.2 months. Men were younger at onset of disease than women (41.3±16.6 years vs 48.3±11.7 years, p =0.018), but had delayed presentation to hospital (20.9±17.4 months vs 14.3±9.3 months, p value =0.01). Fiftysix patients (70%) out of 80 patients (age of onset >40 years) had bulbar onset ALS whereas it was involved in only 14 patients (35.9%) with age <40 years. Upper limb onset ALS was commonest presentation (53.8%) followed by lower limb and bulbar onset (26.1% and 20% respectively). Clinical and/or electrophysiological evidence of cervical spinal segment, lumbo-sacral, bulbar and thoracic involvement were present in 95.8%, 78.2%, 56.3% and 55.5% patients respectively. Definite, probable and possible ALS was 62.2%, 16.8% and 14.3% respectively. Although ALS can occur at any age, the average age at onset is the mid-to-late 50s. Most studies have found that the disease affects men slightly more than women, with an approximate ratio 1.7:1, however, after the age of 65, this gender related incidence is less pronounced. In 5 percent to 10 percent of individuals with ALS, the disease is inherited as an autosomal dominant trait (familial ALS [FLAS]), although rare cases of juvenile onset ALS are inherited in an autosomal recessive pattern. Of the hereditary ALS cases, approximately 20 percent are a result of more than 90 mutations in SOD1, a gene that encodes the copper-zinc superoxide dismutase enzyme (CuZnSOD). The very large majority of adult individuals with ALS have no family history of the disease (sporadic ALS), and a very small percentage of individuals with sporadic ALS also have a mutation n SOD1. Approximately 70 to 80 percent of individuals develop limb onset ALS, with initial involvement in the bulbar muscles. Bulbar-onset ALS is more common in middle-aged and initial symptoms may include difficulty speaking, chewing, or swallowing. 5
  18. 18. CHAPTER 3 ETIOLOGY 6
  19. 19. Other than a small percentage of cases, etiology for the most part is unknown. It is hypothesized that no one single mechanism, but rather multiple mechanisms, may be responsible for neuron degeneration in ALS. Possible pathological mechanisms include: 1. Superoxide dismutase (SODs) are a group of enzymes that eliminate oxygen free radicals, that, although products of small cell metabolism, have been implicated in neurodegenaration. There are three isoforms of SOD in humans: cytosolic copper-zinc superoxide dismutase (CuZnSOD), mitochondrial manganese superoxide dismutase (MnSOD), and extracellular superoxide dismutase (ECSOD). SOD1, a gene on chromosome 21, encodes CuZnSOD. Genetic studies of individuals with adult-onset FALS have determine that about 20 percent of these individuals have mutations in SOD1: however, the primary gene defect is unknown. When the SOD enzyme activity is decreased, as has been observed in individuals with FALS with SOD1 mutations, free radicals may accumulate causing damage. Most mutations identified in FALS show modest loss in enzyme activity, suggesting the mutant SOD-1 protein may have toxic properties that cause motor neurons to die, the mechanism of which has yet to be determined. 2. Glutamate, an excitatory neurotransmitter, has also been implicated in neurodegeneration. Excess glutamate triggers a cascade of events leading to cell death. Increased levels if glutamate in the cerebrospinal fluid (CSF), plasma, and in post-mortem issue of individuals with ALS has been reported. In 1995, a deficiency in EAAT2, a specific glutamate transporter protein, in the motor cortex and spinal cord of post-mortem ALS tissue was reported and lends support to the theory of excitotoxicity causing neurodegeneration 3. Clumping of neurofilament proteins into spheroids in the cell body and proximal axon is one of the histopathological characteristics of ALS. Whether or not abnormal accumulation is secondary to the pathology or if it contributes to motor neuron degeneration has yet to be determined. 4. Several studies have implicated an autoimmune reaction the etiology of ASL. For example, serum factors toxic to anterior horn motor neurons in individuals with ASL have been reported, and antibodies in calcium channels have been identified in individuals with ASL. 7
  20. 20. 5. It has been hypothesized that a lack of neutrophic factors could contribute to the development of ALS and other neurodegenerative disorders. In vivo experiments and experiments with isolated motor neurons in cell culture have shown neutrophic factors are important in motor neuron survival. However, factor deficits in ASL have not been conclusive. 6. Other potential theories that have been thought to contribute to neurodegenation in ALS, which have limited or indirect evidence, include exogenous or environmental factors, apoptosis, programmed cell death, and viral infections 8
  21. 21. CHAPTER 4 ANATOMY 9
  22. 22. Fig 4.1 The difference between ALS-nerve cells and muscle and normal nerve cells and muscle. 10
  23. 23. SPINAL TRACTS Decending Tracts: Corticospinal tracts: The corticospinal tract originates in the cerebral cortex where voluntary motor control is localized. There are two branches, the lateral and the anterior. The lateral crosses in the medulla in an area known due to its appearance as the pyramids. The anterior does not cross. These fibers are called “upper motor neurons” and they synapse with “lower” motor neurons in the cord which lead to the skeletal muscles. Ascending Tracts: Spinothalamic tracts: These tracts carry conscious pain, temperature, crude touch, and pressure. There is a lateral and an anterior tract. They carry this information first to the thalamus of the brain which receives all conscious sensations, and then terminate in the areas of the cerebral cortex which perceive these sensations. Spinocerebellar tract: This tract carries unconscious proprioception (muscle sense) to the cerebellum which is responsible for muscle coordination. The fibers either do not cross, or cross and recross so that they innervate the cerebellum on the same side. The Fasciculus Gracilis and Fasciculus Cuneatus: These tracts carry discriminative touch (the gracilis) and conscious proprioception (the cuneatus). Discriminative touch allows you to specifically localize the location whereas crude touch does not. The fibers of these tracts go first to the medulla where they synapse and cross. Then through a pathway called the medial lemniscus they pass to the thalamus. Then on to the cerebral cortex for perception. 11
  24. 24. Fig 4.2 Coricospinal tract 12
  25. 25. Fig 4.3 Spinothalamic tract 13
  26. 26. Fig 4.4 Spinocerebellar tract 14
  27. 27. Fig 4.5 Fasciculus gracilis and fasciculus cuneatus 15
  29. 29. Amyotrophic lateral Sclerosis is characterized by a progressive degeneration and loss of motor neuron in the spinal cord, brain stem, and motor cortex. (fig. 5.1). UMNs in the cortex are affected, as are the corticospinal tracts. Brainstem nuclei for cranial nerves V (trigeminal), VII (facial), IX (glossopharyngeal), X (vagus), and XII (hypoglossal) and anterior horn cells in the spinal cord are also involved. Brainstem nuclei for cranial nerves controlling external ocular muscles (III: occulomotor, IV: trochlear, and VI: abducens) are usually spared, and if degeneration occurs, it does so late in the course of the disease. Motor neurons of the Onufrowicz nucleus (Onuf’s nucleus), located in the ventral margin of the anterior horn in the second sacral spinal level, are also generally spared; if they are affected, it is to a very limited extent. These neurons control striated muscles in the pelvic floor, including anal and external urethral sphincters. The sensory system and spinocerebellar tracts are also generally spared in ALS. Some studies suggest that sensory neurons may be involved in ASL, but to a much lesser extent than the motor neurons. Morphological studies have found peripheral sensory nerves exhibit axonal atrophy, demyelination, and degeneration, dorsal root ganglia cells at autopsy reveal loss if large ganglion cells. Degeneration of Clarke’s neurons and of the spinocerebellar tracts has also been reported. Degeneration of the spinocerebellar tracts is a well-recognized pathological feature of FALS and has been described in sporadic ALS, although it is rare. Posterior column degeneration is more common in FALS, but rare in sporadic ALS. As motor neurons degenerate, they can no longer control the muscle fibres they innervate. Healthy, intact surrounding axons can sprout and reinnervate the partially denervated muscle. (fig), in essence assuming the role of the degenerated motor neuron and preserving strength and function early in the disease: however, the surviving motor units undergo enlargement. Reinnervation can compensate for the progressive degeneration until motor unit loss is about 50 percent and electromyography (EMG) studies have found evidence of motor unit reinnervation in individuals with ALS. As the disease progress, reinnervation cannot compensate for the rate of degeneration and a variety of impairments develop. 17
  30. 30. The progression of ALS is thought to spread in a contiguous manner, within spinal cord segments (eg, cervical segments to cervical segments), before developing rostral or caudal symptoms. Thus, signs and symptoms spread locally within a region (eg, bulbar, cervical, thoracic, lumbosacral) before moving to other regions. Caudal-to-rostral spread within the spinal cord and spread from the cervical to bulbar region appears to occur faster than rostral-to-caudal spread within the spinal cord. 18
  31. 31. Fig 5.1 A normal spinal cord is shown compared to the cord of a patient with ALS to highlight the difference in size of the nerve roots. The atrophy is apparent in the ALS cord. 19
  33. 33. ALS Medical Classification Making a proper diagnosis in ALS is complicated because symptoms can vary in each patient. For greater accuracy, physicians have classified every known form. Classical ALS A progressive neurological disease characterized by a deterioration of upper (nerve cells leading from the brain to the spinal cord) and lower motor neurons (nerve cells from the spinal cord to muscles). Classical ALS affects more than two-thirds of those with the disease. Primary Lateral Sclerosis (PLS) A progressive neurological disease in which the upper motor neurons (nerve cells) deteriorate. If the lower motor neurons are not affected within two years, the disease usually remains a pure upper motor neuron disease. This is the rarest form of ALS. Progressive Bulbar Palsy (PBP) An ongoing condition that begins with difficulties in speaking, chewing and swallowing due to lower motor neuron (nerve cells from the spinal cord to muscles) deterioration. This disorder affects about 25 percent of those with ALS. Progressive Muscular Atrophy (PMA) A progressive neurological disease in which the lower motor neurons deteriorate. If the upper motor neurons are unaffected within two years, the disease usually remains a pure lower motor neuron disease. Familial ALS A progressive neurological disease that affects more than one member of the same family and that’s due to a known gene mutation. This type of ALS accounts for a very small number of people with ALS in the United States (between 5 and 10 percent). 21
  34. 34. STAGES OF ALS Early Stages Physical effects · Muscles may be weak and soft or stiff, tight and spastic. Muscle cramping and twitching (fasciculations) occur, as does loss of muscle bulk (atrophy). · Symptoms may be limited to a single body region or mild symptoms may affect more than one region. · The person may experience fatigue, poor balance, slurred words, a weak grip, tripping when walking or other minor symptoms. · Sometimes this stage occurs before a diagnosis is made. Middle Stages Physical effects · Symptoms become more widespread. · Some muscles are paralyzed, while others are weakened or unaffected. Fasciculations may continue. · Unused muscles may cause contractures, in which the joints become rigid, painful and sometimes deformed. · If a fall occurs, the person may not be able to stand back up alone. · Driving is discontinued. · Weakness in swallowing muscles may cause choking and difficulty eating and managing saliva. 22
  35. 35. · Weakness in breathing muscles can cause respiratory insufficiency, especially when lying down. · Some people experience bouts of uncontrolled and inappropriate laughing or crying (pseudobulbar affect). Late Stages Physical effects · Most voluntary muscles are paralyzed. · The ability to move air in and out of the lungs is severely compromised. · Extremely limited mobility; unable to care for own needs. · Poor respiration may cause fatigue, fuzzy thinking, headaches and susceptibility to pneumonia. · Speech may be no longer possible. · Eating and drinking by mouth aren’t possible. 23
  37. 37. 1. At onset: a) Asymmetrical weakness and wasting of extremities – 75% b) Bulbar or pseudobulbar features – 25% - dysphagia or dysarthria In both limb-onset and bulbar-onset disease the key feature is the mixture of upper and lower motor neuron involvement with normal sensation. 2. Frontal lobe involvement: a) Frontal dementia occurs in 3-5% of all patients, but is more prevalent in familial cases. b) Emotional lability – unprovoked outbursts of laughing or crying occur. 3. Limb on-set disease: a) Limb-onset ALS results from involvement of corticospinal tracts and anterior horn cells. Signs of corticospinal tract degeneration lead to: -Increased tone -Brisk reflexes -Exterior plantar responses -Distinctive distribution of weakness (extensors in upper limb; flexors in lower limbs). b) Spasticity is rarely severe (intact extrapyramidal inhibition). c) Anterior horn cell involvement leads to muscle atrophy, weakness and fasciculation. Muscle cramps are common. Weakness is not severe as the degree of wasting suggests. d) In the hand: wasting is evident. 1st dorsal interosseous muscle and tendons become prominent as hand muscle waste, giving ‘guttered’ appearance – ‘skeleton hand’ 25
  38. 38. 4. Bulbar-onset disease = Progressive bulbar palsy: a) Progressive bulbar palsy presents with a combination of corticobulbar degeneration and lower cranial nerve motor nuclei involvement. b) Degeneration of corticobulbar pathways to V, VII, X, XI and XII cranial nerve motor nuclei (with sparing of III, IV and VI) leads to an apparent weakness of the muscles of mastication and expression, the patient has difficulty in chewing and the face is expressionless. As the disease progresses, all levels of the motor system become involved. Patients with limb-onset develop bulbar symptoms and vice versa. Respiratory muscle weakness ultimately occurs and is the usual cause of death. 5) Less common clinical presentations: -Breathlessness from respiratory muscle failure -Repeated chest infections from occult aspiration -Weight loss. 6) Uncommon clinical variants: ‘Flail arm’ variant is when there is marked weakness and wasting of the arms with only modest weakness in the legs. This generally progresses more slowly. 26
  40. 40. Medical Management: RILUZOLE (Rilutek) There is no cure for ALS. But Riluzole is the first approve drug treatment for ALS by Food and Drug Administration, or the FDA. Riluzole works by decreasing body’s levels of glutamate, an amino acid that affects nerves that send messages from brain to the muscles. People with amyotrophic lateral sclerosis may have very high levels of glutamate, which can damage these nerve cells. Riluzole is not a cure for ALS, but it may delay progression of the disease and prolong your life. Riluzole (Rilutek) may prolong survival by about 2 months. It may slow the release of certain brain chemicals (neurotransmitters) that are believed to play a role in ALS Adult Dosage: -50 mg orally every 12 hours. -Riluzole should be taken on an empty stomach at least 1 hour before or 2 hours after a meal to avoid a food-related decrease in bioavailability. -Riluzole is usually taken twice a day, every 12 hours. It is important to take your medicine at the same times each day. Side Effects: Emergency medical help is necessary if the patient have any of these signs of an allergic reaction: hives; difficult breathing; swelling of face, lips, tongue, or throat. Stop using riluzole if a patient have a serious side effect such as: · Nausea, stomach pain, low fever, loss of appetite, dark urine, clay-colored stools, jaundice (yellowing of the skin or eyes); · Fever, chills, body aches, flu symptoms; 28
  41. 41. · Chest discomfort, dry cough, trouble breathing; · Cough with yellow or green mucus; or · Stabbing chest pain, feeling short of breath. Less serious side effects may include: · Dizziness, spinning sensation; · Muscle stiffness; · Weakness, drowsiness; · Numbness or tingly feeling in or around your mouth; · Mild nausea, diarrhea, stomach pain, constipation; · Headache; or · Runny nose. 29
  42. 42. TOPIRAMATE Topiramate is also an FDA-approved anti-seizure medication. One mechanism of its action is to antagonize the activation of glutamatergic excitatory amino acid receptors. It has protected motor neurons in both in vitro and in vivo models of motor neuron death. A12-month, double-blind, randomized, placebo-controlled trial enrolled 296 subjects with ALS in 2000. Dosage : 200 to 400 mg/day in two divided doses, and 400 mg/day in two divided doses as adjunctive treatment in adults with primary generalized tonic-clonic seizures. It is recommended that therapy be initiated at 25 to 50 mg/day followed by titration to an effective dose in increments of 25 to 50 mg/day every week. Patients should maintain an adequate fluid intake in order to minimize the risk of kidney stones. Symptom management (sialorrhea and pseudobulbar affect). GOAL: The goal of symptom management is to improve the quality of life of the patient, family, and healthcare provider. Prominent symptoms include sialorrhea, pseudobulbar affect, speech impairment, sleep disorders and fatigue, depression, difficulties with activities of daily living, and ambulation Pharmacologic interventions reducing sialorrhea: Glycopyrrolate (Robinul) for control of sialorrhea in patients with cerebral palsy or developmental disabilities. Amitriptyline (Elavil) has been used widely in ALS but not studied in controlled trials. Decreased drooling in patients with cerebral palsy was also reported with benztropine (Cogentin), trihexyphenidyl hydrochloride (Artane), and transdermal hyoscine (Scopolamine). Transdermal hyoscine decreased drooling in developmentally delayed children, patients with mental retardation, and patients with oropharyngeal carcinoma.Atropine reduced sialorrhea in a crossover study of a patient with closed head injury. For thick mucus production associated with sialorrhea, the addition of a beta blocker, such as propranolol (Inderal) or metoprolol (Toprol), appeared to confer clinical benefit for patients with ALS in an uncontrolled trial. 30
  43. 43. Other Drugs which may be used to relieve symptoms and make patient more comfortable include: · 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. 31
  44. 44. Surgical Management: Diaphragm Pacing System (DPS): It is done by invasive laparoscopic surgery. The Food and Drug Administration has approved the NeuRx Diaphragm Pacing System for treatment of hypoventilation (inadequate breathing) in amyotrophic lateral sclerosis (ALS). In people with ALS who have chronic breathing problems and whose primary breathing muscle, the diaphragm still is able to respond to electrical stimulation, the pacing system may forestall or negate the need for invasive ventilation (ventilation via tracheostomy, or an opening in the neck) The device does not treat the underlying molecular mechanisms that cause ALS, so it is unable to slow, stop or reverse the disease course. However, the treatment may improve quality of life. DPS requires implantation of electrodes, via laparoscopic surgery, to stimulate the phrenic nerve – the motor innervation to the diaphragm, which is responsible for the act of breathing. The diaphragm pacing device is a unit – about the size of a large remote control – that is worn externally by the patient and connected to wires that exit the chest. The implanted electrodes act as a pacing device, causing the diaphragm to contract, just as it would in people with normal connections between the brain and muscles. The hope is that this implantable system can provide part-time or full-time respiratory support to persons who normally need a mechanical ventilator. Benefits to the Patient · Improved speech, mobility and more normal breathing · Increased olfactory sensation · Greater social interaction · Expanded participation in rehabilitation activities · Improved occupational opportunities 32
  45. 45. Physiotherapy Management: GOALS OF THE TREATMENT Goal setting is a continuously evolving process, due to the changing condition of the person with ALS. Short term goals may be revised weekly or monthly. Long term goals are more reflective of the eventual disabling nature of the disease, increased care needs, and end of life concerns which transcend the physical body. Short Term Goals - Increase Strength by ½ grade - Increase Range of Motion 10 degrees - Decrease Pain by 2 on 10 scale - Improved productive cough, vital capacity - Independence with home exercise program Long Term Goals - Maximize Strength - Maintain adequate Range of Motion for function, mobility, and care - Minimize discomfort and pain - Safe Bed Mobility with assistance as needed - Safe Transfers with appropriate equipment and assistance as able - Safe Ambulation with appropriate equipment and assistance as able - Maximize respiratory function - Adequate pressure relief in sitting and lying positions - Knowledge of an appropriate level home exercise program as desired - Caregivers are knowledgeable and safe assisting with functional mobility 33
  46. 46. Physiotherapy for ALS may include: · Exercises and stretches to keep joints and muscles supple · Pain management advice · Advice and provision of equipment to maintain your independence as long as possible e.g. mobility aids · Advising those who care for you on appropriate manual handling techniques e.g. how you assist in and out of bed · Education on minimizing secondary complications · Liaising with other professionals as appropriate e.g. occupational therapists, speech and language therapists · Providing advice re: chest care (breathing exercises and chest clearance techniques) Benefits of physiotherapy include: · Improved quality of life · Improved confidence · Improved independence · Maintenance of range of movement · Reduced muscle stiffness · Reduced joint stiffness · Reduced risk of developing a chest infection · Reduced pain levels · Reduced anxiety 34
  47. 47. PHYSICAL THERAPY INTERVENTION WITH ALS Amyotrophic Lateral Sclerosis (ALS) provides a challenge to those physical therapists charged with the care and treatment of patients affected by this condition. Though the disease course may vary in its progression, body areas affected, and symptoms (spasticity vs. flaccidity), the trend is an inevitably downward one with the likelihood of severe disability preceding death. There are significant therapy interventions to benefit mobility, independence, safety, comfort, and quality of life throughout the course of care. Special attention must be made by the physical therapist to identify the skilled intervention provided and appropriate goals with awareness of the treatment setting from acute care, rehab, outpatient, home health or hospice. It is helpful to begin with frank recognition of the terminal nature of ALS. Each person with this disease will die. Having that clearly in mind, we can then think in terms of what are the end goals that will be considered in the last weeks and months. Hospice care is appropriate at such a time and sets goals of: - safe and comfortable dying - self determined life closure - effective grieving - patient and family self-care and independence - maximize quality of life - aggressive symptom management despite lack of cure Understanding these goals must be the foundation on which all care is ultimately based. 35
  48. 48. TREATMENT ISSUES A number of unique treatment issues arise in the course of care for a person with ALS. This section will outline specific interventions that may be applicable. 1) Maximizing surface heights for transfers becomes essential so as to allow the use of efficient body mechanics as strength wanes. Inversely, carefully lowering the height of assistive devices for ambulation can conserve energy and prolong mobility through the use of more extension in the upper extremities, and thus less energy costs. 2) Primary upper extremity involvement affecting one or both limbs may severely limit the use of assistive devices for ambulation. Positioning for comfort becomes extremely difficult with a person who lacks any significant motor function yet retains full sensation. All of those small motions we make as we settle into bed at night, allow us to relax and decrease stimulus to our bodies as we try to fall asleep. 3) The ALS person cannot move in such ways and is likely also limited in communication which compounds this task for a caregiver. The eyes, knowledge, and hands of the physical therapist can be essential tools in training family caregivers in the nuances of positioning. 4) Positioning to assist breathing is also essential. Relieving the resistance to diaphragmatic contraction can be achieved by elevating the head of the bed. Along with elevation, the patient must be positioned such that the hips are aligned with angle of the bed. If the person has slid down in bed, the flexion of the trunk will obstruct the respiratory pattern. Elevation of the lower end of the bed as well will minimize this sliding from occurring. 5) Other respiratory care issues include maximizing breathing capacity and strength, use of various breath techniques, coughing, and clearing of secretions. Pressure relief both in sitting and in bed become significant issues. Again, the knowledge and experience of the therapist can provide caregivers the best seating system or mattress surfaces (foam vs. alternating pressure vs. low air loss) for comfort and mobility. 6) Massage is often useful for inhibition of spasticity, and to provide pain relief and comfort. Caregivers can be taught appropriate massage techniques to perform to ease discomfort by providing a normal stimulus and enhance quality of life. 36
  49. 49. PRACTICE PATTERNS The progressive decline in physical and functional status requires unique approaches to the practice patterns used for physical therapy. 1. ‘Rehabilitation in Reverse’ This is recognized as an appropriate treatment approach. If one can visualize the progressive impairment of motor strength which occurs with ALS, there are many opportunities when a physical therapist could intervene for : a) Gait and balance training with a cane and using AFOs b) Fitting and gait training with a walker, on levels, steps and a variety of indoor and outdoor surfaces. c) A caregiver may also be instructed and trained to assist for safe ambulation. All of this patient and caregiver training is skilled physical therapy intervention, but it is occurring in the reverse order for a traditional rehabilitation situation. New goals of safety, independence, and equipment use can be written at each new level of function. Depending on the rate of physical loss, the visit or treatment frequency for ‘Rehabilitation in Reverse’ will vary from several times in a week, to once a week, or even less often. 2. ‘Case Management’ This is the second model of practice that becomes evident and necessary which is covered and recognized under the ‘Medicare Home Health Benefit’. This model is applicable for cases with co-morbidities and/or secondary diagnoses, where care is complex, and there may be multiple caregivers involved. Ongoing re-evaluation with instruction to the caregivers, modifying the care plan, and updating a home exercise and activity program are the skilled care provided by the therapist, even when direct patient treatment is not the focus of intervention. 37
  50. 50. This model provides for optimal prevention of complications which might require hospitalization, knowledge and safety for the caregivers and quality of life for the patient. Case Management visits might be scheduled once per 30 or 60 days, and are also opportunities to identify needs for short term direct intervention to solve new care issues. 3. ‘Skilled Maintenance’ Some functional activities such as walking and transferring become impaired to the point where it is no longer safe for a caregiver to provide adequate assistance. At times the skill of a physical therapist may be sufficient to provide enough facilitation, assistance with balance and weight shift, and cuing to allow completion of the transfer or walking. While these efforts do not resolve the underlying condition and would otherwise be considered maintenance activities, the requisite skill required allows for intermittent performance as one part of the physical therapy program. 4. ‘Supportive Care’ This includes manual care such as massage, range of motion, joint mobilization, modalities and instruction of caregivers in performance and follow through with these activities. Also part of Supportive care is the psycho-social-spiritual support that is ongoing throughout the entire course of care for people with ALS. 38
  51. 51. Management according to Stages of ALS: 1. Early Stage: Physical Interventions: Restorative/Preventive: -Strengthening exercises -Endurance exercise -Active ROM, Active-assisted ROM, stretching exercises Compensatory: -Determine potential need for adaptive or assistive devices -Determine potential need for ergonomic modifications of home/workplace -Energy conservation -Educate the patient about the disease process, energy conservation, and support groups. 2. Middle stage: Physical therapy interventions Compensatory: -Support weak muscles (assistive and supportive devices, adaptive equipment, slings, orthoses) -Modification to workplace/home (e.g, install ramp, move bedroom to first floor) -Wheelchair prescription -Education of caregivers regarding functional training 39
  52. 52. Preventative: -Active-assistive, passive ROM and stretching exercises -Strengthening exercise (early middle) -Endurance exercise (early middle) -Determine need for pressure-relieving devices (e,g, pressure distributing mattress) 3. Late Stage Physical therapy interventions: Preventative: -Passive ROM -Pulmonary care -Hospital bed and pressure-relieving device -Skin care, hygiene Compensatory: -Caregiver education regarding transfers, positioning, turning, skin-care -Mechanical lift 40
  53. 53. Respiratory management. Respiratory impairments place the patient at risk for respiratory tract infections. Important management considerations include: · Pneumococcal and yearly influenza vacations · Prevention of aspiration · Effective oral and pulmonary secretion management Supplemental oxygen must be used with caution because it can suppress respiratory drive, exacerbate hypoventilation, and ultimately lead to hypercarbia and respiratory arrest. Typically, supplemental oxygen is recommended only for individuals with concomitant pulmonary disease or as a comfort measure for patients who decline ventilator support. 41
  54. 54. Fig 8.1. Algorithm for respiratory management. 1Forced vital capacity (FVC) or vital capacity (VC) can be used. VC may be more accurate in patients with bulbar dysfunction. 2Agreement needed for conditions of withdrawal prior to or concurrent with instituting invasive ventilation (e.g., locked in state, coma, etc.). Dx = diagnosis. 42
  55. 55. Management of Dysarthria and Dysphagia in Patients with Amyotrophic Lateral Sclerosis Treatments for dysphagia and dysarthria include: · To optimize the intelligibility of speech for as long as possible and to concentrate not only on the disabled person, but also on partner-to-partner communication. · Speech and language therapy to learn new swallowing techniques · Changing the consistency of food and drinks to make them safer to swallow · Alternative forms of feeding, such as tube feeding through the nose or stomach · Treating the narrowing of the esophagus with surgery, by stretching or inserting a metal tube Measures to reduce spasticity. Sometimes patients with spastic dysarthria are temporarily helped by ice placed over the larynx or sucked, or antispastic drugs such as baclofen Strategies for coping. In just detectable speech impairment (speech is made worse by fatigue or stress), simple things such as minimizing the noise in the environment, reducing the distance from the listener, are helpful. In mild dysarthria, ALS patients may compensate by a number of speaking strategies slowing the speech rate, speaking face to face, substituting articulation manoeuvres such as: alternative words, spelling, repetition, overarticulating consonants, or even using key words or monosyllabic speech. Concentration on speaking only and energy conservation may prolong the time of successful communication. Speech therapy (logopedic) training. Speech therapy training may be useful in patients with relatively slow progression of dysarthria but there is no such evidence in ALS cases. Lip and tongue exercises may sometimes help the patient to enunciate words more clearly. There are no credible data on strengthening exercises of the orofacial 43
  56. 56. muscles in ALS patients and a number of neurologists discourage this practice. Energy conservation is a key component in managing bulbar function in ALS Palatal lift and palatal augmentation prostheses A palatal lift may temporarily improve resonance by displacing the weak soft palate to the level of normal palatal elevation and reduce hypernasality and hypophonia. Palatal augmentation prosthesis may temporarily improve articulation by lowering the palate, improving the production of the lingual consonant sounds. There is no good evidence as to the effectiveness of these prostheses, or of their period of usefulness if effective. Palatal lift would not be contemplated in patients with spastic dysarthria 44
  57. 57. Fig 8.2. Dysarthria in ALS. Medical, speech therapy and other interventions 45
  58. 58. EQUIPMENT Adaptive equipment and aids are essential to be considered. A partial list of possible equipment includes: Cane: Standard, Small or Large Based Quad, Hemi Walker: Pick-up, Front Wheeled, or 4 Wheeled Crutches: Axillary or Lofstrand Ankle Foot Orthoses Sliding Board Floor to Ceiling Pole Commode: Standard, Drop Arm Wheelchair: Manual, Transport, Recliner, Power Cushion/Seating System Lap board Bath Seat: Bench/Chair, Tub Transfer, Rolling Shower Chair Electric Lift Chair Electric Hospital Bed Mattress: Foam, Alternating Pressure, Low Air Loss Mechanical Lift Head/Neck supports/collars Slings, Splints 46
  59. 59. EXERCISE The role of exercise is important to consider. Recent studies have shown that limited and targeted exercises can help maximize and preserve strength during the decline and loss of strength, although some consider just the strenuous nature of normal activity to be sufficient. Ultimately the person with ALS can choose which provides the best sense of control and quality of life. Neither excessive repetition nor heavy resistance is recommended so as not to cause further motor impairment. A modest home exercise program can be designed for the person who desires one and can thus maximize their strength through a declining course. In later stages a passive range of motion program is essential to prevent contractures that might limit the caregiver’s ability to provide assistance (plantar flexion for weight bearing during transfers) or personal care (hip or shoulder adduction to allow bathing access). More specific range of motion along with joint mobilization techniques can help manage the painful joints that can occur as a secondary condition from immobility. Caregivers can be instructed in these specific activities depending on their capability and availability. 47
  60. 60. STRETCHING EXERCISES: The following exercises are intended to stretch your muscles or move the joints through their full range-of motion. A stretching program performed daily may help to modulate muscle tightness. SPECIAL NOTE: If tightness is present in the arms or legs, you’ll feel resistance to the movement. Overcome this resistance by continuing slow, ste Fig 8.3 Posture Check Stand with your back to a wall. Press your heels, buttocks, shoulders and head against the wall. Move the feet forward, and bend your knees so your back slides a few inches down the wall. Tighten your abdominal muscles so you can flatten your lower back against the wall. Relax. Repeat. 48
  61. 61. Back Exercises Fig 8.4 Standing extension Stand with your hands in the small of your back. Bend backwards with knees straight. Hold for 5 seconds. Return to starting position. 49
  62. 62. Fig 8.5 Press-ups Lie face down, palms at shoulder level. Press the top half of your body up, using your arms. Hold for 5 seconds. Return to starting position. Fig 8.6 Single knee to chest Lie on your back with your knees bent. Grasp your left knee with your hands and pull back toward your left shoulder until you feel a good stretch. Slowly lower the leg back to the floor and repeat the same procedure with the right leg. 50
  63. 63. Fig 8.7 Partial sit-up Lie on your back with your knees bent and your arms crossed over your chest. Roll your chin toward your chest and raise your head and shoulders off the floor. Relax. 51
  64. 64. Shoulder The “shoulder” consists of many muscles that extend from the neck, upper back and chest to the upper arm. Stretching can be achieved with the following simple exercises: Fig 8.8 Shoulder exercise 1 Starting with your hands on your knees and palms down, raise both arms as high as possible over your head. Fig 8.9 Shoulder exercise 2 With your arms straight out in front of your body at shoulder height and palms down, move your arms out to your sides. Hold for 5 seconds. Return your arms to the front. 52
  65. 65. Fig 8.10 Shoulder exercise 3 Starting with your arms down at your sides raise your arms out to the sides with palms down. Bring both arms over your head as high as possible 53
  66. 66. Fig 8.11 Shoulder exercise 4 Lie on your back, arm out to your side at shoulder height, elbow bent to 90 degrees, fist pointing toward the ceiling. Bring your hand forward to the bed, palm down. Return to the starting position, then bring your hand backward to the bed, palm up. Do exercise with other arm. 54
  67. 67. RANGE-OF-MOTION EXERCISE Arm Exercises You can do these exercises when you have one arm that’s significantly stronger than the other. You can perform them in a sitting position or lying on your back. Shoulder Fig 8.12 Shoulder ROM exercise 1 Grasp your weaker wrist with your stronger hand. Raise both arms over your head. 55
  68. 68. Fig 8.13 Shoulder ROM exercise 2 Cradle your weaker arm with your stronger arm, with your stronger hand under your weaker elbow. Raise both arms to chest height. Move your arms from side to side as far as possible. Elbow Fig 8.14 Elbow ROM exercise Grasp your weak wrist with your strong hand. Bring your hands to your right shoulder, then back to your knee, then to your left shoulder. 56
  69. 69. Hand and wrist Fig :8.15 Hand and Wrist ROM exercise 1 Grasp the palm of your weaker hand with your strong hand. Move your weak hand slowly up and down as if waving. Fig :8.16 Hand and Wrist ROM exercise 2 Sit with the palm of your weaker hand facing upward. Grasp the fingertips with your stronger hand. Bend the fingers inward to touch the palm. Straighten them out fully. 57
  70. 70. Fig :8.17 Hand and Wrist ROM exercise 3 With the palm of your weaker hand facing upward, grasp the tip of the thumb with your stronger hand. Move the tip of your thumb over to the base of the little finger, then straighten it again. Fig :8.18 Hand and Wrist ROM exercise 4 With the palm of the weaker hand facing down, grasp the thumb with the stronger hand and move the thumb out to the side as far as possible. Return to your original position. 58
  71. 71. PSYCHOLOGICAL, SOCIAL, SPIRITUAL ISSUES The psychological, social, and spiritual dimensions of care must always be considered by the therapist, along with the physical care. Having the support of an interdisciplinary team common to palliative care and hospice programs can assist in addressing these issues. Because the physical losses are often recognized by the physical therapist (changing strength levels which preclude an activity or no further adaptation being possible) the questions around loss and meaning of life are often first confided and heard during a therapy treatment session. Being able to listen and offer support with sensitivity becomes paramount in the person’s ability to continue the struggle of both living and letting go of life at the same time. At times the therapist can be helpful in ‘giving permission’ to let go of activity that is no longer physically possible. Education about the disease process in relation to function and physical changes from ALS, a comorbid, or a secondary condition is often helpful as the knowledge allows for an increased sense of control. Re-framing activity around maximal effort and performance, as well as exploring the dimensions of self beyond the physical body are also useful in coping with these changes. The physical therapist, often seen by the patient and family as a source of hope, can gently broach these issues when appropriate during the course of care. THE ESSENTIALS: - Physical therapy has an important role in management of ALS at any stage. - The physical therapist needs to understand the various stages of decline and different practice patterns to provide and document appropriate care. - Communication with the patient and family caregivers provides direction and choices about the interventions they want. - Identify unique treatment and adaptive equipment issues. - Education of patient and caregiver are essential through the course of care. - Psychological, social and spiritual issues must be considered and addressed. 59
  73. 73. As the disease progresses, people with ALS experience one or more of the following complications: Breathing problems: ALS eventually paralyzes the muscles needed to breathe. Some devices to assist your breathing are worn only at night and are similar to devices used by people who have sleep apnea (for example, continuous positive airway pressure, or CPAP, masks). In the latter stages of ALS, some people choose to have a tracheostomy — a surgically-created hole at the front of the neck leading to the windpipe (trachea) — to enable the full-time use of a respirator that inflates and deflates their lungs. The most common cause of death for people with ALS is respiratory failure, usually within three to five years after symptoms begin. Eating problems: When the muscles that control swallowing are affected, people with ALS can develop malnutrition and dehydration. They are also at higher risk of aspirating food, liquids or secretions into the lungs, which can cause pneumonia. A feeding tube can reduce these risks. Dementia: Some people with ALS experience problems with memory and making decisions, and some are eventually diagnosed with a form of dementia called frontotemporal dementia. 61
  74. 74. CHAPTER 10 PROGNOSIS 62
  75. 75. Age at time of onset has the strongest relationship to prognosis. Studies have found that patients less than 35 to 40 years of age at onset had better 5-year survival rates than older individuals. Individuals with limb-onset ALS have a better prognosis than those with bulbar-onset; 5-year survival rates were reports to be 37 percent and 44 percent, compared to survival rates of 9 percent and 16 percent for patients with bulbar-onset ALS. Less severe involvement at the time of diagnosis, a longer interval between onset and diagnosis, and no symptoms of dyspnea at onset are other factors associated with a better prognosis. A study of 144 individuals with ALS found that those individuals with psychological well-being had significantly longer survival times compared to those with psychological distress. Mortality rates were found to be 6.8 times greater in those experience psychological distress, and the relationship was independent of age, disease severity, and length of time from diagnosis. These findings were confirmed in later study that found degree of physical disability, disease progression, and survival could be predicted by the patient’s psychological status. 63
  76. 76. CHAPTER 11 PREVENTION 64
  77. 77. While there is no cure for ALS, there are some biomedical and nutritional weapons to combat it. From a nutritional standpoint, it has been found that patients with ALS have an elevated level of sulfur in their bodies. Sulfur affects the central nervous system by causing an inflammatory response. Unfortunately, though, cutting out exposure to sulfur will not necessarily help. The presence of sulfur is related to the disease, not to a certain diet. However, lowering sulfur levels of patients with ALS by using sulfur antagonists seems to provide some short-term relief. ALS may be prevented by eating colorful fruits and vegetables. Eating bright colored foods, especially those that are yellow, orange, and red, may prevent or slow the onset of amyotrophic lateral sclerosis (ALS). According to the study which was published in the Annals of Neurology, confirmed that colorful carotenoids prevented the onset of ALS, Carotenoids are what make fruits and vegetables a bright red, orange, or yellow color, and are dietary sources of Vitamin A. Earlier research reported that oxidative stress contributes to the development of ALS. Other research has shown that people with high antioxidant intake, such as vitamin E have a decreased risk of ALS. Carotenoids and vitamin C are antioxidants, therefore the researchers decided to analyze their link to ALS risk. The investigators analyzed more than one million participants. In total, 1,093 cases of ALS were found after excluding participants with unlikely food consumption. Researchers revealed a higher total carotenoid intake was associated with decreased risk of ALS. People with extra carotenoids in their diets were found more likely to exercise, have elevated vitamin C intake, take vitamin C and E supplements, and have an advanced degree. They also found that participants with diets high in carotene and lutein - commonly present in dark green vegetables - had a decreased risk of ALS. 65
  78. 78. CHAPTER 12 CONCLUSION 66
  79. 79. Amyotrophic Lateral Sclerosis, the most common and devastatingly fatal motor neuron disease among adults, causes a progressive increase in the number and severity of impairments, functional limitations and disabilities. Other than a small percentage of cases, etiology for the most part is unknown, and it is hypothesized that multiple mechanism may be responsible for the disease. Although there is no cure for ALS and its course cannot be altered, it should be considered a “treatable disease”. Medical management is primarily symptomatic, and a team approach to care is considered optimal. Rehabilitation management is focused on maximizing function and promoting independence to the highest level possible and ensuring optimal quality of life throughout the course of the disease and across health care settings. The physiotherapist plays an integral role in designing and implementing therapeutic interventions for individuals with ALS that will allow them to maintain independence and function for as long as possible. The selection of interventions, grounded in evidence-based research whenever possible, is based on the stage and progression of the disease may be restorative, compensatory, or preventative. This intervention should take into consideration the individual’s goals; psychosocial factors that may impact decision making such as the individual’s acceptance of the diagnosis and the individual’s social and financial resources. Because of the progressive nature of the ALS, the physiotherapist must not only address the patient’s current problem, but also plan for the patient’s future needs 67
  80. 80. APPENDIX 68
  81. 81. ASSESSMENT FORM Demographic data Name: Age: Sex: Occupation: Address: Dominance of Hand History of present illness: History of past illness: Personal History: Medical History: Socio economy History: On observation: Build: Posture: Attitude of Limbs: Any visual deformity: Any assisted device: Other findings: 69
  82. 82. On Examination: Vital Signs: Temperature: Blood Pressure: Pulse: Respiratory rate: Cardio thoracic examination: Breathing pattern: Breath sounds: a) Normal b) Abnormal Neurological Examination: a) MMSE (Mini Mental Status Examination) b) Cranial Nerve Testing c) Speech testing d) ROM e) MMT f) Muscle girth g) Cerebellar Signs h) Reflex: i) Cortical reflex ii) Superficial reflex iii) Deep reflex i) Muscle tone j) Gait Assessment 70
  83. 83. CHAPTER 12 REFERENCES 71
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