Amyotrophic Lateral Sclerosis: Clinicopathological Studies of a Family

摘要: Twenty-one members of a family through four generations have experienced syndrome similar to amyotrophic lateral sclerosis, with additional features sensory loss and prolonged course. Five patients were examined, two whom had glove stocking distribution impairment. Autopsy on one showed demyelination gliosis in the spinocerebellar tracts Goll's posterior columns addition changes cells anterior horns. The average course lasted more than 11 years. Two other cases presented as progressive muscular atrophy without signs spasticity. All consistently began leg weakness, followed by arm bulbar both upper motor neuron degeneration. mode transmission was autosomal dominant complete penetrance.