Amyotrophic Lateral Sclerosis

Although amyotrophic lateral sclerosis is a relatively uncommon disorder, it has been the focus of considerable scientific scrutiny. First described by Charcot in 1869, the condition involves degeneration and loss of motor neurons in the cerebral cortex, brainstem, and spinal cord. Although different forms of the disease have been described, a definitive pathologic mechanism has yet to be delineated.

Various synonyms for amyotrophic lateral sclerosis (ALS) include Lou Gehrig's disease, Charcot disease, and motor neuron disease (Rowland, 1995, pp. 1251-1252). The disorder is caused by non-specific degenerative changes of the bulbar and spinal motor neurons and their corresponding cortico-spinal tracts. Moreover, other neuronal systems may become progressively involved. These can include the spinocerebellar pathways and nuclei, the frontal cortex, and the substantia nigra (Swash & Schwartz, 1992, pp. 4-13).

The disease's onset is insidious, and its outcome is typically fatal. Amyotrophic lateral sclerosis patients often present with asymmetrical and focal weakness. This complaint can involve only a few adjacent muscles: Leg weakness is slightly more common than hand weakness. About a third of patients present with bulbar symptoms. One characteristic feature of the disease is arm muscle fasciculation. Jokelainen (cited in Swash & Schwartz, 1992, pp. 4-13) found that patients with bulbar involvement survived for a mean period of 2.2 ye

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experience diffuse wasting of one leg. Juvenile ALS may occur in childhood or adolescence. Additionally, familial ALS can have either an adult or a juvenile onset. Comprising 5-10% of cases, familial ALS is transmitted according to an autosomal dominant pattern of inheritance (Swash & Schwartz, 1992, pp. 4-13). Men seem to be more vulnerable to ALS than women. Moreover, it seems to be more common among Caucasians. The disease's annual incidence is about 1.3/100,000 per year (Swash & Schwartz, 1992, pp. 4-13). The morbidity and mortality data, however, seem to suggest a recent increase in ALS deaths. Furthermore, this trend has also been observed for age-specific and age-adjusted rates (Kurtzke, 1991, pp. 245-267). Despite considerable research, a definitive mechanism for ALS pathogenesis has yet to become evident. The investigations are hampered by lack of an adequate animal model and few human subjects. Although there have been many epidemiological studies involving ALS, these have not delineated any noteworthy trends. In addition, no significant associations have been demonstrated between ALS and either life-style, social class, occupation, dietary habits, or another disease. While epidemiologic studies have show

Category: Medical - A