Amyotrophic lateral sclerosis (ALS), sometimes called Lou Gehrig’s disease, is a progressive,
invariably fatal neurological disease that attacks the nerve cells (motor neurons)
responsible for controlling voluntary muscles. ALS most commonly strikes people between
40 to 60 years of age, although it is possible for the disease to strike younger people,
The diagnosis of ALS is based on history, clinical findings consistent with the diagnosis,
and electrophysiological and neuroimaging studies to rule out other impairments that
may cause similar signs and symptoms. The diagnosis may also be supported by electromyography
(EMG) or nerve conduction studies, but these tests may be negative or only suggestive
of the diagnosis. There is no single test that establishes the existence of ALS. Other
neurological disorders that may have similar effects (such as neuropathy) should be
ruled out. The insidious onset of ALS can sometimes delay definitive diagnosis.
The sequence of emerging symptoms and the rate of disease progression and related
limitations vary from person to person. Eventually, however, all muscles under voluntary
control are affected and individuals lose the ability to move their arms and legs,
and to swallow, speak, and breathe. Most people with ALS die from respiratory failure,
usually within 3 to 5 years from the onset of symptoms.
Unlike Multiple Sclerosis, there are no exacerbations and remissions with this disease;
it is progressively debilitating until a person with ALS must depend on others for
all their personal needs.
There is no cure for ALS. The only disease-specific therapy is the drug riluzole (Rilutek),
which may slow the progression of ALS and prolong survival by several months. Other
treatments for ALS are designed to relieve symptoms and improve quality of life.
In July 2001, Congress eliminated the 24-month Medicare waiting period for individuals
found disabled due to ALS. This enables a claimant to get the medical help that is
required for this disease as soon as possible.