Pathoanatomic Changes in Amyotrophic Lateral Sclerosis on Guam

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Abstract
Introduction Since the original description of Charcot1 nearly a century ago, hundreds of papers have been written on amyotrophic lateral sclerosis (ALS). The pathologic lesions of ALS have been described in the classical case as progressive degeneration and loss of cells in the anterior horns of the spinal cord, in the motor nuclei of the brain stem, and, in many instances, in the Betz cells of the cortex. Demyelination of the lateral columns is generally present, but is not necessarily limited to the corticospinal and corticobulbar fibers2; posterior column demyelination, which has also been observed on occasion,3,4 has, however, not been associated with any obvious sensory impairment. The phenomenal incidence of ALS in the Mariana Islands of the Western Pacific, and particularly on Guam, has evoked special attention during the past 10 years because of the geographical isolation and the familial aggregation of the disease among the