TN 2 (09-03)

Citations:

Appendix 1 to Subpart P of Part 404, Section 11.00 20 CFR 416.934

A. BACKGROUND

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, including children.

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.

B. POLICY

1. Listing 11.10

Listing 11.10 (DI 34001.030) provides that anyone with ALS established by clinical and laboratory findings as described in 11.00O will be found disabled. For purposes of meeting the listing, documentation of the diagnosis must be by generally accepted methods consistent with the prevailing state of medical knowledge and clinical practice.

2. Evidence

The evidence should include documentation of a clinically appropriate medical history, neurological findings consistent with the diagnosis of ALS, and the results of any electrophysiological and neuroimaging testing. A negative test result will not preclude a diagnosis of ALS if other clinical findings are present that are consistent with ALS and other potential disorders have been ruled out.

3. Title XVI Applicants

For Title XVI applicants, an allegation of ALS will warrant a finding of presumptive disability as described in DI 23535.000.