BOSTON—A multidisciplinary ap- proach 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
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 treat-
ments 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 signifi-
cant declines in cramping.
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 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
How Can Neurologists Help Manage
Symptoms in Patients With ALS?
Multidisciplinary care may promote survival and to improve quality of life in patients
Michael D. Weiss, MD