Amyotrophic Lateral Sclerosis With Polyglucosan Bodies

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Orthner et al. (1973) reported 2 sisters with onset of ALS at 38 and 39 years of age, and death after 14 and 26 months, respectively. Weakness began in the arms and later involved the legs. Bulbar signs and symptoms followed. Autopsy showed marked loss of motor neurons. Polyglucosan bodies were found in perikarya in the cortex and cerebellum. Barz et al. (1976) reported 2 sporadic cases.