Amyotrophic Lateral Sclerosis: Lou Gehrig’s Disease

7 Pages 1645 Words

Amyotrophic Lateral Sclerosis: Lou Gehrig’s disease


Amyotrophic lateral sclerosis (ALS) – a devastating neuromuscular disease that strikes adults in the prime of life – has puzzled physicians since it was first described in medical literature more than 100 years ago. ALS was made famous by baseball legend Lou Gehrig – but anyone can get this disease. ALS is a fatal disorder that causes progressive muscle paralysis. ALS is a terminal neurological disorder that robs patients of their ability to dress themselves, eat, walk, and speak. Patients generally survive three to five years after being diagnosed, with death generally occurring due to respiratory failure. The cause of the deadly disease was and is still a mystery today.
Pathophysiology
The pathogenesis is unclear, but there are three general types of ALS: sporadic, familial and guamanian. (Neurology Channel, 2004) Sporadic is the most common in the United States with the cause believed to be excess levels of a neurotransmitter called glutamate. Glutamate in high levels acts as a neurotoxin that damages and kills nerve cells. Familial ALS (FALS) is linked to a genetic defect of chromosome 21. Mutated superoxide dismutase (SOD) causes motor neuron problems seen in ALS. (Brown, 2001) Guamanian ALS, first seen in Guam in the 1950’s and 1960’s, is suspected to be caused by the Cycad Cyas circinalis seed used to make flour, although not all researchers agree with this explanation. (Muscular Dystrophy Association, 2003) More recent research proposes a theory that bats, a delicacy of native Guamanians, who eat the nuts of the cycad tree, are cause of high rates of ALS in that country. If this holds true, then an environmental toxin in the diet may cause ALS and further investigation is needed. (Muscular Dystrophy Association Research, 2002)
Amyotrophic lateral sclerosis or Lou Gehrig’s disease is a progressive disease that attacks motor neurons, co...