How Can Neurologists Help Manage Symptoms in Patients With ALS?

Multidisciplinary care may promote survival and to improve quality of life in patients with ALS.

Neurology Reviews. 2017 August;25(8):30-31

August 2, 2017|Neurology Reviews

BOSTON—A multidisciplinary approach is the best way to provide care for patients with amyotrophic lateral sclerosis (ALS), said Michael D. Weiss, MD, Professor of Neurology at the University of Washington Medical Center in Seattle. Providing access to speech therapists, mental health specialists, dieticians, and pharmacists can promote survival in patients with ALS. In an overview presented at the 69th Annual Meeting of the American Academy of Neurology, Dr. Weiss discussed the utility of multidisciplinary care and addressed symptom management, palliative care, and hospice care.

Prognosis and Multidisciplinary Care

ALS is a rare degenerative disorder of motor neurons of the cerebral cortex, brainstem, and spinal cord that results in progressive wasting and paralysis of voluntary muscles. The median age of onset is 55, and the disease has a slight male predominance. Fifty percent of patients with ALS die within three years of symptoms onset; 90% of patients die within five years. Patients with bulbar-onset ALS are more likely to die sooner. Riluzole is the only FDA-approved disease-modifying therapy for patients with ALS. Studies have indicated that this drug extends median survival by two to three months.

In addition, data suggest that multidisciplinary care improves quality of life and survival in patients with ALS. Traynor et al found that survival increased by 7.5 months among all patients in multidisciplinary clinics; patients with bulbar onset lived 9.5 months longer.

Managing Muscle Cramps

Recent studies suggest that muscle cramps occur in 85% of patients with ALS. Cramps can vary in severity and can be debilitating, said Dr. Weiss. Some patients can have as many as 50 cramps per day. Few efficacious treatments for managing this symptom of ALS are available. A recent trial showed that patients who received either 300 mg or 900 mg of mexiletine experienced significant declines in cramping.

Spasticity

It is common for patients with ALS to develop spasticity. Several therapies that may reduce spasticity include baclofen, tizanidine, diazepam, and botulinum toxin injections. The baclofen pump might be more helpful than these therapies for patients who have upper motor neuron dominance.

Sialorrhea

Sialorrhea occurs when patients are unable to clear extra saliva due to weakness in the oropharyngeal muscles. Doses of 600 mg to 1,200 mg of guaifenesin twice per day may be beneficial in managing sialorrhea. Other drying agents such as atropine drops and glycopyrrolate may also be efficacious. These drying agents may cause urinary retention in older patients, Dr. Weiss cautioned.

Amitriptyline can also help manage sialorrhea. It also improves sleep and reduces depression. Hyoscyamine, the transdermal scopolamine patch, and botulinum toxin injections into the submandibular glands may also be beneficial for patients. A suction machine or a mechanical in-exsufflator can also help manage this symptom of ALS.

Emotional Incontinence and Depression

Pseudobulbar affect, also known as emotional incontinence, affects as much as 50% of patients with ALS and is more common in bulbar ALS. This condition causes patients to have uncontrollable episodes of laughing and crying that are inconsistent with the patients’ mood. A randomized controlled trial found that dextromethorphan–quinidine was beneficial in managing these emotional symptoms. Other treatments include tricyclic antidepressants and selective serotonin reuptake inhibitors.

Reactive clinical depression occurs in 9% to 11% of patients with ALS. Once the ALS diagnosis is confirmed, patients should be counseled about their prognosis; their spouses and family members should also be offered counseling. Antidepressants such as tricyclic antidepressants, selective serotonin reuptake inhibitors, and serotonin-norepinephrine reuptake inhibitors should be offered to all patients. These drugs may help to elevate mood, stimulate appetite, and improve sleep.

Respiratory Insufficiency and Falls

Respiratory insufficiency is one of the leading causes of death among patients with ALS. Patients with this condition may have morning headaches, vivid dreams and nightmares, frequent nocturnal arousals, fatigue, excessive daytime somnolence, and dyspnea on exertion. The American Academy of Neurology recommends that patients start noninvasive ventilation if their sniff nasal pressure is less than 40 cm, their maximal inspiratory pressure is less than –60 cm, or their forced vital capacity is less than 50%.

Bourke et al found that noninvasive ventilation was associated with improvement in quality of life and survival in patients with ALS. The median survival was increased by 205 days, and quality of life was maintained above 75% of baseline on the sleep apnea quality of life index score. In addition, patients who have a peak cough expiratory flow of less than 270 L/min should be offered a mechanical in-exsufflator suction device. If the patient does not tolerate noninvasive ventilation, then palliative medicine and hospice may be appropriate, said Dr. Weiss.

Evidence suggests that 2% of patients with ALS die from fall-related complications. Risk factors for falls in ALS include muscle weakness, deficits in gait or balance, and cognitive impairment. Assistive devices, wheelchairs, and physical therapy can help prevent falls. Some patients may need a brace to help stabilize their gait.