Amyotrophic Lateral Sclerosis in Physical Medicine and Rehabilitation

Amyotrophic lateral sclerosis (ALS) is the most common type of adult-onset motor neuron disease (MND). Adult-onset motor neuron diseases are a group of neurologic disorders that present in adult life and are characterized primarily by progressive degeneration and loss of motor neurons. ALS involves upper and lower motor neurons and presents as an idiopathic, progressive degeneration of anterior horn cells and their associated neurons, resulting in progressive muscle weakness, atrophy, and fasciculations. ^{[1, 2, 3, 4]}

The disease was first described in 1869 and is commonly called Lou Gehrig disease, after the professional baseball player who died of ALS in 1941.

Other diseases classified as adult-onset motor neuron diseases have more restricted presentations and can evolve into idiopathic ALS if the patient is tracked for a long period. These diseases include the following:

• Progressive bulbar palsy - Pure bulbar involvement

• Progressive muscular atrophy - Pure lower motor neuron degeneration

• Primary lateral sclerosis - Pure upper motor neuron degeneration

• Adult-onset spinal muscular atrophy - Includes a broad range of primary motor neuron diseases classified by pattern of inheritance, distribution of weakness, or age of onset

According to a review by Angelini and Siciliano of exercise’s role in the management of ALS, most clinical studies suggest that in patients with the disease, quality of life, functionality, and, in some instances, cardiorespiratory function and/or muscle strength are improved with resistance, endurance, and concurrent training. The investigators state, however, that several such studies were only preliminary, because sample size and control of confounding variables were inadequate, control populations were nonrepresentative, and disease stages varied. ^[5]

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).

Comprehensive rehabilitation

In a review paper, Paganoni et al reported that patient-centered, multidisciplinary, comprehensive rehabilitation care significantly impacts people with ALS, serving as the current standard of care for these patients. ^[6]

Special concerns

An attorney should be consulted regarding a "living will," and power of attorney should be established prior to the terminal phase of this ultimately fatal disease.

A study by Ng et al reported that Cochrane systematic reviews on symptomatic treatments for ALS/MND have not found robust evidence regarding management of ALS/MND symptoms. They also conclude, however, that rather than an actual lack of efficacy, clinical trials may not show benefit from an intervention owing to other factors, such as insufficient statistical power, wrong dose choice, insensitive outcome measures, or inappropriate eligibility of participants. Moreover, the generally small size of the trials meant that adverse treatment effects could not be reliably assessed. ^[7]

Also see the following:

• Emergent Treatment of Amyotrophic Lateral Sclerosis (ALS)

• Amyotrophic Lateral Sclerosis

• Primary Lateral Sclerosis

Physical therapists should instruct proper stretching and daily range-of-motion (ROM) exercises to the patient with amyotrophic lateral sclerosis (ALS) and his/her caregivers. Therapists should try to anticipate the patient's future needs and introduce assistive devices in a timely manner. Lightweight ankle-foot orthoses can be provided to minimize foot drop and stabilize weak quadriceps muscles to prevent falls. ^[8]

The physical therapist should also emphasize energy conservation and teach patients and caregivers methods for performing safe, efficient transfers. Therapists also can provide instruction for strengthening exercise programs, but exercises should be performed at submaximal levels in muscles without marked weakness and should be prescribed only for patients with slowly progressive disease. ^[9]

In one study, individualized, moderate-intensity, endurance-type exercises for the trunk and limbs performed 15 minutes twice daily was shown to significantly reduce spasticity as measured by the Ashworth scale. The effects of exercise in people with ALS are not well understood. A Cochrane review concluded that studies were too small to determine to what extent strengthening exercises for people with ALS are beneficial or whether exercise is harmful. There is a complete lack of randomized or quasi-randomized clinical trials examining aerobic exercise in this population. ^[10]

However, a systematic review and meta-analysis by Meng et al of randomized, controlled studies evaluating the effects of exercise in patients with ALS concluded that the functional ability and pulmonary function in these individuals can safely be improved with exercise. ^[11]

A prospective study by Meyer et al found that despite the fact that patients with ALS who underwent physical therapy experienced, as anticipated with the progressive disorder, a 3-point decline in the ALS Functional Rating Scale-Revised (ALSFRS-R) and a composite-score reduction of 0.8 in the Measure Yourself Medical Outcome Profile (MYMOP), individuals evaluated via an online assessment perceived the therapy to be beneficial. By the end of the study, the Net Promoter Score, used to assess patient recommendation levels for physical therapy, had risen from its baseline value of 20 points to 50 points. ^[12]

A literature review by Ortega-Hombrados et al indicated that therapeutic physical exercise can help to inhibit muscle deterioration in patients with ALS, promoting the maintenance of score levels in the ALSFRS-R, particularly in the medium and long-term (3 and 6 months, respectively). This was in contrast to patients in whom treatment was mostly passive. Although exercise programs did not improve actual survival, better results were obtained from exercise patients with regard to forced vital capacity levels. ^[13]

An observational study by Sanjak et al concluded that in ambulatory patients with ALS, the 6-minute walk test is a valid means of measuring walking capacity. The report also determined that an association exists between the test and assessments of lower extremity muscle strength and function in patients who ambulate with or without an assistive device. ^[14]

A literature review by Macpherson and Bassile indicated that pulmonary physical therapy interventions—specifically, inspiratory muscle training (IMT), lung volume recruitment training (LVRT), and manually assisted coughing (MAC)—can have a beneficial effect on respiratory outcome measures and length of survival in ALS. The investigators reported that respiratory muscle strength could be prolonged through IMT, with the mean length of survival being extended by a year. Immediate cough efficacy benefited from LVRT, while peak cough expiratory flow showed just a small amount of improvement in association with MAC. ^[15]

A prospective, single-center, double-blind, randomized, controlled study by Plowman et al indicated that in patients with ALS, respiratory and bulbar function can be improved through respiratory training. Patients who underwent 8 weeks of in-home expiratory muscle strength training displayed differences in scores for maximum expiratory pressure and Dynamic Imaging Grade of Swallowing Toxicity compared with those who underwent sham training. ^[16]

However, a literature review by Silva et al suggested that in patients with ALS, respiratory muscle training has no significant impact on physical functioning or quality of life. The investigators found that after an 8-week period, in patients with ALS who underwent expiratory muscle training (EMT), the percent predicted forced vital capacity (FVC%) demonstrated no clear difference from that of patients treated with sham EMT. Although after 4 months’ therapy, patients who underwent IMT showed improvement in FVC%, the evidence was of low certainty, and outcomes derived from the Amyotrophic Lateral Sclerosis Functional Rating Scale did not clearly differ between IMT and sham-IMT patients. ^[17]

A study by Kitano et al indicated that a structured, unsupervised, home-based exercise program can prove effective in patients with early stage ALS. The investigators found that individuals who participated in the 6-month program, which included muscle stretching, as well as muscle and functional training, achieved a significantly higher total score and respiratory function subscore on the ALSFRS-R than did patients who underwent a 6-month program supervised by a physical therapist. ^[18]

However, a Canadian survey by Jones et al found that among physicians in academic ALS clinics, 31% cited lack of confidence or competence as a barrier to prescribing exercise for patients with ALS, while 22% cited lack of evidence regarding the benefit of exercise, and another 22% cited lack of infrastructure. ^[19]

When making wheelchair recommendations, the therapist should try to anticipate the patient's future needs. Wheelchairs should be introduced while the patient is still ambulatory in order to enhance energy conservation. Initially, a lightweight manual or propulsion-assist wheelchair should be rented, with plans to purchase a heavier, more expensive power chair when the patient is no longer able to ambulate. Recommend modifications to the patient's wheelchair in a timely manner and in accordance with the patient's tolerance for gadgets.

Occupational therapists should focus on teaching energy conservation techniques to the patient with amyotrophic lateral sclerosis (ALS) and his/her caregivers. Adaptive equipment should be introduced early and in synchronization with the patient's progressing needs. ^[9]

Upper extremity bracing can be used in cases where weakness may alter joint biomechanics. Patients with poor grasp can be provided with a universal cuff, and patients with hand weakness can be braced in 20-25° of extension to improve grip strength. For patients with proximal upper extremity weakness, a balanced forearm orthosis (ie, deltoid aid) may be beneficial to enhance upper extremity movement by eliminating the effects of gravity. When shoulder girdle weakness progresses to advanced stages, slings can be used to decrease pain by limiting traction on the associated ligaments, nerves, and vessels.

For patients with severe limb involvement, introduce environmental control units (ECUs) that utilize oral-motor movements. In patients with severe bulbar involvement, extraocular movements are usually preserved so ECUs incorporating eye gaze technology can be used. ^[20]

A literature review by Arbesman and Sheard of studies evaluating the effects of occupational therapy on patients with ALS found limited-to-moderate evidence that multidisciplinary programs are associated with longer survival than are general care programs. The review, which encompassed 14 studies, also found limited evidence that, compared with general care programs, multidisciplinary programs lead to the use of a higher percentage of appropriate assistive devices or that patients in these programs have a higher quality of life with regard to social functioning and mental health. ^[21]

Speech therapists can teach patients with amyotrophic lateral sclerosis (ALS) with bulbar involvement to slow their speech and exaggerate articulation to improve comprehension for caregivers. For patients with respiratory insufficiency, the therapist can teach phrasing to promote energy conservation. Submaximal tongue strengthening exercises and diaphragmatic exercises also should be taught, to help improve articulation and voice projection.

As the patient's voice becomes more strained, the caregiver can be fitted with a hearing aid to promote communication with the patient. This also provides the patient with an "interpreter" who can assist in communicating with others. If the patient and caregiver already have their own form of communication (ie, gestures) that works well for them, encourage them to continue using their existing system as long as it is effective.

Dysarthria has a drastic impact on the quality of life of ALS patients. Communication systems improve or at least stabilize quality of life and mood in dysarthric ALS patients and should be provided early in the disease course. ^[22] Speech therapists should monitor patients and, as their speech declines, introduce augmentative communication devices, such as writing implements, communication boards, or computer-assistive technology. For patients with severe bulbar and limb involvement, eye gaze and blink generally are preserved. Therefore, the speech therapist can take advantage of these preserved functions by introducing blink-controlled voice synthesizers, communication boards, and computer-assistive devices that use eye-gaze techniques.

In patients with bulbar involvement, the speech therapist can perform an oropharyngeal assessment to evaluate for clinical signs of dysphagia. In many cases, a videofluoroscopic swallowing evaluation is necessary to identify specific problems during the various phases of swallowing. Speech therapists can provide interventions, such as alterations in the consistency, taste, or temperatures of foods, to improve oral intake and prevent aspiration. Straws may be used to encourage the recommended chin-tuck position for swallowing thin liquids. Enforce upright positioning at all meals, and, in patients with neck extensor weakness, recommend a soft cervical collar to improve positioning during meals.

Patients with ALS who predominantly have limb and respiratory weakness, accompanied by limited bulbar involvement, may prevent respiratory complications and placement of permanent tracheostomy through aggressive pulmonary care. Respiratory therapists can play a critical role in the management of these patients. ^{[23, 24]}

Recommend that patients perform routine measures of assisted peak cough flows to evaluate bulbar muscle function. Continued attainment of adequate peak cough flow is the most important goal for preventing serious pulmonary complications.

Routinely measure vital capacity in sitting and recumbent positions. Usually, recumbent vital capacity is the first to decline, resulting in nocturnal hypoventilation.

Oxyhemoglobin saturations (SaO₂) should be monitored, especially at night in patients complaining of nocturnal awakenings or sleep difficulties. Decreased overnight SaO₂ indicates nocturnal hypoventilation and the need for noninvasive IPPV at night. Despite low SaO₂, do not administer oxygen without invasive ventilation. In patients without invasive ventilation, oxygen administration decreases the respiratory drive and may thereby result in exacerbation of hypercapnia.

Respiratory therapists also are involved in educating patient and caregiver. They can instruct caregivers on proper performance of assisted cough techniques and teach submaximal respiratory strengthening exercises to the patient. Respiratory therapists also can provide instruction for proper use of expiratory muscle aids, such as the intermittent abdominal pressure ventilator and mechanical insufflator-exsufflator. In addition, they can teach patients and caregivers to use and manipulate inspiratory muscle aids, including noninvasive and invasive ventilation.

Finally, respiratory therapists can teach patients glossopharyngeal breathing. This involves gulping boluses of air into the lungs to add to the user's deep breath and can be useful in patients with nonbulbar ALS who do not have a tracheostomy. One glossopharyngeal breath contains 6-9 gulps and can provide the patient with weak inspiratory muscles and no vital capacity a safe way to breathe if, for any reason, a ventilator cannot be used or there is sudden ventilator failure.

Noninvasive intermittent positive pressure ventilation

Noninvasive IPPV is a form of mechanical ventilation that does not require placement of a tracheal tube. It can be delivered by mouth, oral-nasally, or nasally. The amount of bulbar involvement and the personal preference of the patient determine the type of interface used.

Mouthpiece IPPV most commonly is used in patients with nonbulbar ALS and is effective for 24-hour use. Lip-seal retention can be added to increase efficacy while the patient is sleeping. Nasal interfaces can be used in patients with bulbar involvement or for nocturnal ventilation. Some prefer nasal interfaces for daytime use as well, because they leave the mouth free for other activities. Custom-molded nasal interfaces or oral-nasal shells also can be made to increase efficacy and/or comfort. Increased risk of death is associated with intolerance to noninvasive IPPV, and increased survival has been documented in patients who can tolerate this form of ventilation.

Intermittent abdominal pressure ventilation

Another type of respiratory muscle aid is the intermittent abdominal pressure ventilator. This device assists expiratory muscles and provides daytime ventilatory support. The intermittent abdominal pressure ventilator uses an elastic air sac, which intermittently inflates to aid in expiration. The air sac is contained in a corset, which can be concealed under clothing. This device should not be used in patients with significant scoliosis or obesity. Some patients prefer its use to that of the mouthpiece intermittent positive pressure ventilator, as it leaves the mouth free for mouthstick and other activities.

Oxygen administration

Oxygen administration depresses the respiratory drive as in chronic obstructive pulmonary disease (COPD). The underlying reason for the patient's hypoxemia is not lack of perfusion but rather lack of adequate ventilation. Oxygen administration can lead to worsening hypercapnia; therefore, hypoxia in these patients initially should be treated by increasing ventilation, either noninvasively or invasively.

Reserve oxygen administration for patients whose hypoxemia is not reversed with ventilatory support. In such patients, usually an underlying process, such as pneumonia, has resulted in decreased pulmonary perfusion. This situation necessitates oxygen administration, but oxygen should be given only once the airway has been secured and the patient is invasively ventilated. Invasive ventilation in this instance should be temporary until the secondary process has been treated. Following adequate treatment, the patient should be able to return to his or her previous respiratory management.

Peak cough flows

As mentioned earlier, assisted peak cough flows should be performed regularly and used to monitor bulbar functioning. Once peak cough flows drop below 160 L/min, flows likely are inadequate to clear debris, thereby resulting in mucous plugging. Risk of mucous plugging is significantly increased during periods of intercurrent respiratory infection and can result in acute respiratory distress necessitating placement of an endotracheal tube. Patients with peak cough flows below this level may require permanent tracheostomy tube placement to allow suctioning and invasive ventilation when needed.

Suctioning alternative

An alternative (or adjunct) to suctioning can be provided with a mechanical insufflator-exsufflator. This respiratory aid assists in clearing debris by providing deep insufflation, immediately followed by a forced exsufflation to create artificially about 10 L/sec of expiratory flow (a normal cough expels air at approximately 16 L/sec). This can be administered directly via a tracheostomy tube with the cuff inflated or via an oral nasal mask.

The mechanical insufflator-exsufflator can be used acutely to relieve mucous plugs, but it also may be used routinely to provide assisted coughs and maintain clear airways. When used in combination with other respiratory muscle aids, it can avert tracheostomy tube placement, maintain quality of life, and prolong survival.

Weight loss is another common and serious issue in ALS and is usually related to the progression of dysphagia but cannot always be attributed to dysphagia. Weight loss had a negative impact on quality of life and was associated with shorter survival. ^[25] Weight loss also may develop in patients with and without bulbar involvement because of generalized fatigue, poor appetite, and associated depression. Malnutrition is a poor prognosticating factor and correlates with increased risk of death; therefore, once a patient has lost 5-10% of normal body weight, consider enteric feeding and discuss options with the patient and caregivers.

Symptomatic treatment of weight loss with high-calorie nutritional supplements and/or percutaneous endoscopic gastrostomy (PEG) should be offered more frequently. Consult a gastroenterologist early so that placement of a feeding tube is not delayed while weight loss progresses. PEG placement is preferred while the patient still is able to eat by mouth. In this situation, enteric feeding can be presented as a positive option, because it allows the patient to eat for enjoyment, rather than simply for nutritional necessity. This prevents the fatigue and frustration associated with mealtimes experienced by some of these patients.

Initially, nasogastric or orogastric feeding may be an option, but these methods can lead to traumatic irritation of the mucosa with prolonged use. For long-term feeding, options include standard gastrostomy, PEG, and jejunostomy. Preferred intervention is usually PEG tube placement, because other types of long-term feeding tubes require placement under general anesthesia, while a PEG tube can be placed under local anesthesia. ^[26] Increased risk of regurgitation is associated with gastrostomy/PEG tubes; therefore, some may prefer placement of a jejunostomy tube, which decreases the risk of aspiration.

Dorst et al studied the effect of high-caloric food supplements in ALS patients. ^[27] In their study of 26 ALS patients, high-caloric food supplements with high fat and high carbohydrate content are both suitable to stabilize the body weight. The effect of a high-fat diet might be more pronounced. They propose that since body weight is an independent prognostic factor in ALS, it is possible that a high-caloric food supplement improves survival in ALS patients.

Spasticity occurs in ALS patients from loss of inhibition from upper motor neurons. The development of spasticity in people with ALS adds significantly to their functional decline and to the reduction in quality of life for the individual. Nonpharmacological treatments should be tried first before adding medications; these include regular stretching and other types of exercises. A Cochrane review found only one randomized controlled trial in the world literature that looked at the treatment of spasticity in motor neuron disease, which implies there is a huge gap in our knowledge of this area. ^[28]

Baclofen acts as a gamma-amino butyric acid (GABA) agonist and is the drug of choice for the treatment of spasticity in patients with ALS. Alpha2-adrenergic agonists, such as tizanidine and clonidine, also may be effective, but hypotension is a potential adverse effect that may limit their use.

Dantrolene interferes with excitation-contraction coupling and usually is used as an adjunct agent. Dantrolene does have potential for hepatotoxicity; therefore, monitor liver function tests closely in patients treated with this medication. Benzodiazepines facilitate GABA in the central nervous system (CNS) and are effective in the treatment of spasticity.

Use these medications with extreme caution in patients with ALS because of their adverse effect of respiratory depression. Patients may already be compromised from a respiratory standpoint, and this class of medications may worsen residual respiratory function. Carefully monitor the use of antispasticity agents, because they can cause sedation, dizziness, increased weakness, or other adverse effects that may be potentially harmful to the patient.

In severe cases, an intrathecal baclofen pump can be considered. In a case series by McClelland et al, 8 patients with ALS with severe spasticity and pain were treated with intrathecal baclofen pump. Following intrathecal baclofen pump placement, the average reduction of pain was 54%. Six patients (75%) experienced pain score reduction, 3 of whom had complete pain relief. ^[29]

Pain is quite common in patients with ALS; its prevalence is reported to be 67% in one population-based controlled study ^[30] and 72 % in another cross-sectional study. ^[31] Its occurrence is directly proportional to disease progression. ^[32] Pain can occur for many reasons, but in these patients, it is usually musculoskeletal in origin. Pain may be caused by muscle spasms, contractures, or altered joint biomechanics. Involvement of either peripheral or central sensory mechanisms in ALS causing pain is still under debate. ^[33]

First-line treatment for pain is with nonsteroidal anti-inflammatory drugs (NSAIDs). If the patient is intolerant to these medications, use acetaminophen. Muscle cramps occur frequently. Traditional treatment was with quinine, but this drug is no longer recommended because of its adverse effect profile. NSAIDs and acetaminophen may provide some benefit. Antispasticity medications discussed above are recommended for treatment of pain due to muscle spasm and spasticity and usually are effective.

In some instances, pain may be neuropathic in origin, and anticonvulsant medications such as gabapentin and pregabalin may be beneficial. Tricyclic antidepressants such as nortriptyline and serotonin norepinephrine reuptake inhibitors (SNRIs) such as duloxetine also are indicated for this type of pain.

For severe pain unresponsive to the above treatments, opioid pain medications are indicated. Use them with caution owing to their multiple adverse effects; they too can cause respiratory depression, especially at higher doses.

Fasciculations can be a very early marker of ALS and portend motor unit instability or reinnervation, consistent with a very early phase of increased axonal excitability. ^[34] Fasciculations can be troublesome or even embarrassing for some patients. Avoiding caffeine and nicotine can reduce them, but in those patients with severe symptoms, benzodiazepines can be effective. Use these medications with caution because of their adverse effect profile.

Sialorrhea (drooling), the unintentional loss of saliva from the mouth, occurs in up to 25% of patients with motor neuron disease and affects almost all patients with bulbar involvement. It can significantly affect quality of life. It is caused by dysphagia with inability to swallow secretions because of tongue spasticity; weakness of face, mouth, and pharyngeal muscles; and loss of oropharyngeal coordination and function and is not due to increased production of saliva. There are 2 main types of saliva produced by the 3 major salivary glands: serous saliva, which is thin and watery and is mediated by cholinergic receptors, and mucoidal saliva, which is thicker and more viscous and is mediated by beta-adrenergic receptors. ^[35]

The treatment of sialorrhea is largely a matter of trial and error, using a variety of drug treatments, natural remedies, upright positioning, intermittent oral suction, and more invasive approaches, such as injection of botulinum toxin into salivary glands, radiotherapy to salivary glands, or ligation of salivary gland ducts. Acetylcholine is the active neurotransmitter, binding at muscarinic receptors in the salivary glands. ^[36] Thus, direct cholinergic muscarinic receptor antagonists such as atropine, scopolamine, glycopyrronium bromide, and tricyclic antidepressants can be used to treat sialorrhea in motor neuron disease. ^[37] Beta-blockers have been shown to produce relief from thick secretions in 12 of 16 people with bulbar motor neuron disease. ^[35]

Use of botulinum toxin to reduce salivary flow in motor neuron disease was first hypothesized by Bushara in 1997. ^[38] Botulinum toxin injections reduce sialorrhea by blocking the release of acetylcholine at the cholinergic neurosecretory junction of salivary glands. There are several studies reported in the literature, utilizing different preparations of botulinum toxin (botulinum toxin type A and botulinum toxin type B) and treatment regimens, administered by direct injection or a transductal approach, with varying outcome measures. ^{[39, 40, 41, 42, 43, 44, 45]} A Cochrane review on treatment of sialorrhea in ALS patients concluded that there is some evidence for use of botulinum toxin injections to salivary glands for the treatment of sialorrhea in motor neuron disease. ^[46]

Radiotherapy to the salivary glands, either once or in divided fractions, has been used effectively to manage sialorrhea in ALS patients. ^{[47, 48, 49, 50]}

Psychological symptoms are common and are indirectly related to the disease and its progression. Patients with amyotrophic lateral sclerosis (ALS) generally have preserved cognitive functioning with a progressive loss of control over their disease process and daily functioning. This can be very distressing to the patient and his/her caregivers, and symptoms of fear, anxiety, and depression may manifest. If left untreated, these symptoms may result in a decline in the patient's functioning out of proportion to disease severity. ^{[1, 51]}  A study by Körner et al found that in patients with ALS, depression influenced quality of life, independent of physical impairment. The study involved 159 patients with ALS, who were evaluated with the Beck Depression Inventory-II, the 36-Item Short Form Health Survey, and the ALS Functional Rating Scale-Revised (ALSFRS-R). ^[52]

Early recognition of psychological disorders is important. This allows appropriate interventions to be made to provide support to the patient and caregivers. Interventions can be provided through individual and family psychotherapy, as well as through psychoactive medications. Selective serotonin reuptake inhibitors (SSRIs) most commonly are used for the treatment of depression. In some patients with anxiety, SSRIs also may be beneficial, but in patients with severe anxiety that limits function, anxiolytic medications, including benzodiazepines, should be considered. Thought should be given to the potential respiratory depressive effects of any medication used.

Assistance of a psychologist is recommended early on to help identify and treat clinical symptoms. ^[53] Psychotherapy and counseling can be used alone or in combination with medications, but treatment with medications alone is not recommended. Symptoms such as anxiety and fear can be treated through role-playing and relaxation techniques. Other potential problems, such as caregiver burnout and patient loss of control, also may be identified and treated through the assistance of a psychologist. In addition, psychologists can aid the patient in developing advance directives, addressing financial concerns, and assisting in planning for total care.

Incidence of suicide is surprisingly rare in patients with ALS. This may be attributed to the fact that in the early stages of the disease, the patient either denies or does not fully comprehend the impact of the disease. Once the patient realizes fully the devastation caused by his or her illness, the individual may be too weak to perform the act of suicide.

One study looked at attitudes of patients with ALS and their primary caregivers toward physician-assisted suicide. ^[54] Among the patients studied, mean duration of illness was 4 years from onset of symptoms. Researchers found that 56% percent of ALS patients studied were willing to consider assisted suicide. Of these patients, 79% felt that, if physician-assisted suicide were legal, they would request a lethal prescription from a physician. Most said they probably would keep the prescription available for potential use in the future, although only 1 patient expressed readiness to request the prescription with the intent of taking it to cause death in the next month. Of the caregivers studied, 73% had attitudes toward assisted suicide similar to those of the patients, and 76% were able to predict accurately the patient's willingness to consider taking a lethal dose of medication.

Surprisingly, incidence of depression was not associated with the patient's willingness or unwillingness to consider assisted suicide. Patients who were willing to consider assisted suicide tended to be male, had more years of education, had higher scores of hopelessness, and rated their quality of life as lower than did those who opposed assisted suicide. Patients and caregivers who would consider assisted suicide were less likely to be religious on all measures.

A study conducted in Switzerland, where assisted suicide is legal, showed that a liberal legal setting does not necessarily promote the wish for assisted suicide. However, the desire to discuss assisted suicide is prevalent in ALS patients. ^[55]

A survey of Canadian ALS health-care providers conducted to assess attitudes concerning physician-assisted death (PAD) concluded that the majority of ALS health-care providers support the Supreme Court of Canada's 2015 decision invalidating Criminal Code provision prohibiting PAD, with most believing that patients with moderate to severe ALS with physical or emotional suffering should have the option of PAD. However, the great majority of clinicians were unwilling to directly provide PAD and believed that PAD training modules and guidelines are needed. ^[56]

These studies have strong implications for physicians, multidisciplinary team members, and caregivers who work closely with patients with ALS. In the first study cited above, most caregivers were able to predict whether patients would consider assisted suicide if it were offered to them. Discussing these matters with the patient and his/her primary caregiver is important. By recognizing signs of hopelessness and diminished quality of life in these patients, physicians can institute cognitive therapies and other interventions in an effort to help the patient find meaning in the future, reduce fears, and avoid focusing on the worst outcomes.

Patients with amyotrophic lateral sclerosis who have respiratory failure due to bulbar and respiratory muscle involvement may need a permanent tracheostomy. Severe bulbar involvement may also preclude oral feeding due to significant aspiration and, therefore, a PEG feeding tube may be necessary for adequate nutritional support.

Patients with amyotrophic lateral sclerosis (ALS) who have severe, generalized spasticity resulting in pain or impaired function may initially be given oral medications discussed previously; however, if these medications are ineffective or poorly tolerated at higher doses, consider referral for placement of an intrathecal baclofen pump.

Patients with severe spasticity limited to specific muscle groups may be treated with isolated nerve or motor point blocks, using phenol or botulinum toxin. The type of block generally used is based on the size of the muscle groups involved, risk of paresthesias with phenol injections, cost, and physician preference.

An inpatient stay in the acute care setting may be necessary at onset of symptoms if the diagnosis of amyotrophic lateral sclerosis (ALS) is uncertain and a full diagnostic workup is indicated to rule out diseases that may mimic ALS.

Once a diagnosis has been made, inpatient stays in an acute care hospital may be necessary in times of acute decompensation related to intercurrent illnesses, such as pneumonia.

Additionally, an acute stay may be indicated as respiratory function declines and appropriate ventilatory options are explored and implemented.

Rehabilitation Hospitalization

The patient's rehabilitation program may be initiated in the inpatient setting, at the time of diagnosis, or at a follow-up outpatient visit with a physiatrist.

An inpatient rehabilitation stay is indicated when the patient has had a significant decline in function and appropriate resources or equipment have not been set up in the patient's home yet to provide for current needs.

An inpatient stay also may be necessary after noninvasive or invasive ventilation has been initiated to monitor the patient and allow time to educate the patient and caregivers on the appropriate use of equipment while the ventilator and other respiratory aids are being set up in the home.

Outpatient Care

Coordinate care in the outpatient setting with a multidisciplinary team to provide the patient with amyotrophic lateral sclerosis (ALS) with timely interventions appropriate for his or her current and upcoming needs.

Visits with the physiatrist should include a thorough history and repeat clinical examination to monitor the patient's disease progression.

At each visit, discuss code status, as studies have shown that patient preferences regarding code status may change within a 6-month period because of changes in physical functioning and increased medical needs.

Patient Education

Provide patient education at the time of diagnosis and throughout the course of disease. There are many resources available to patients with amyotrophic lateral sclerosis (ALS) and their families. Provide the names and numbers of local support groups as soon as the diagnosis has been confirmed. Some excellent online resources for physicians and patients include the ALS Association, World Federation of Neurology - Amyotrophic Lateral Sclerosis, and American Academy of Neurology.

For patient education information, see the Dementia Center and the Brain and Nervous System Center, as well as Amyotrophic Lateral Sclerosis (Lou Gehrig’s Disease), and Dementia in Amyotrophic Lateral Sclerosis (Lou Gehrig’s Disease).