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Lower motor neuron disease with spinocerebellar degeneration
Author(s) -
Page Robert W.,
Moskowitz Roland W.,
Nash Robert E.,
Roessmann Uros
Publication year - 1977
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.410020613
Subject(s) - degeneration (medical) , motor neuron , atrophy , medicine , weakness , lower motor neuron , polymyositis , pathology , muscle biopsy , autopsy , amyotrophic lateral sclerosis , disease , neuroscience , biopsy , psychology , anatomy
A patient with polymyositis responded initially to steroid therapy. A muscle biopsy disclosed features of primary myopathy and group atrophy. The patient became refractory to therapy and died with relentlessly progressive weakness. The autopsy disclosed lower motor neuron involvement and degeneration of the spinocerebellar tracts. There was loss of Purkinje cells, which may have occurred secondary to an anoxic episode prior to death. The case is unique because of the limited involvement of the lower motor and spinocerebellar systems.