Hereditary Amyotrophic Lateral Sclerosis

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Abstract
One autopsy case of hereditary amyotrophic lateral sclerosis (ALS) with family background and history was reported. The spinal cord exhibited degeneration in the median portion of the faciculus gracilis and slight degeneration of the pyramidal tracts. Degeneration of the anterior horn cells was marked, and many hyaline inclusions were observed in the cytoplasm of the remaining nerve cells. Most of the inclusion bodies consisted of a halo and core, and histochemically the cores were composed of protein and lipids including sphingomyelin. Electron microscopic examination findings revealed that the haloes were composed of radial filaments, as well as the cores of filaments and granular bodies. The inclusion bodies resembled the Lewy's body in many respects. Heredity was of the autosomal dominance type.