Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease. Motor neurons are affected in certain patterns, such as cervical, thoracic, lumbar, and bulbar (facial) regions. Although initial presentations can vary, eventually upper and lower motor neurons are lost in the two types of ALS: familial and sporadic.
About 6000 people in the U.S. are diagnosed with ALS yearly. A French physician, Jean Charcot, identified ALS in 1869; initially, the disease was called “Charcot Disease.” However, in 1939, when the famous New York Yankees baseball player Lou Gehrig was diagnosed with ALS, it became known as “Lou Gehrig’s Disease”. (Gehrig died from ALS in 1941 at age 37.) It is estimated 300,000 Americans live with ALS in 2018. The median age of onset is 55, and disease indicence peaks between ages 70-75. More males are affected than females. Approximately 90% of ALS cases are determined to be sporadic, or accquired, while the remainder are considered familial, or hereditary.
Amyotrophic Lateral Sclerosis (ALS), also known as Lou Gehrig disease after the famous New York Yankees baseball player, is a devastating neurologic disorder that selectively affects motor function with an unknown origin, characterized by progressive muscle atrophy with hyperreflexia that results from denervation, and results in muscle weakness, disability, respiratory insufficiency, and eventually death.
ALS is a rare, debilitating, and incurable neurologic disease, has an annual incidence of 0.5-2 per 100,000 population, is primarily a disorder of middle to late adulthood, affecting people most frequently in their fifties, with men developing the disease nearly twice as often as women. The median survival duration for patient is approximately 3 years, yet approximately 20% of patients survive greater than 5 years, and 10% will survive for greater than 10 years.
As the main area of involvement is the motor neurons of the brain and spinal cord, ALS affects motor neurons in three locations as follows: the anterior horn cells (lower motor neurons – LMNs) of the spinal cord; the motor nuclei of the brain stem, particularly the hypoglossal nuclei; and the upper motor neurons (UMNs) of the cerebral cortex. The death of the LMNs leads to denervation, with subsequent shrinkage of musculature and muscle fiber atrophy. It is this fiber atrophy, called amyotrophy, which appears in the name of the disease. The loss of nerve fibers in lateral columns of the white matter of the spinal cord, along with fibrillary gliosis, imparts a firmness or sclerosis to this CNS tissue. The term lateral sclerosis designates these changes.
A remarkable feature of the disease is that the entire sensory system, the regulatory mechanisms of control and coordination of movement, and the intellect remain intact.
The three major subgroups: Alzheimer’s disease and other dementia, cerebrovascular disease, and amyotrophic lateral sclerosis (ALS) and related motor neuron disorders.
Alzheimer’s disease and other dementia
Inability to ambulate without assistance (FAST 7-C)
inability to speak or communicate meaningfully with speech limited to approximately a half-dozen or fewer intelligible or different words (FAST 7-B)
Loss of ADL functions including bathing and dressing (FAST 6)
Incontinence of bowel and bladder (FAST 6)
one or more of the following comorbid conditions in last 3-6 months
Pyelonephritis or upper urinary tract infection
Decubitus ulcers, usually multiple and stages II or IV
Fever, recurrent after antibiotics
An altered nutritional status as manifested by:
difficulty swallowing or refusal to eat such that sufficient fluid or caloric intake cannot be maintained and the patient refuses artificial nutritional support
If the patient is receiving artificial nutritional support (NG or G-tube or parenteral hyperalimentation), there must be evidence of an impaired nutritional status as defined in the General Guidelines (greater or equal 10% loss of body weight)
ALS is a rapidly progressing degenerative neuromuscular disease with an unknown origin, results in muscle weakness, disability, respiratory insufficiency, and eventually death. The median survival duration for patients is approximately 3 years, yet 10% will survive for > 10 years.