A 34-year-old man demonstrated rapidly progressive motor neuron diseas
e and, late in his 9-month clinical course, exhibited ophthalmoplegia
and dysautonomic symptoms. Neuropathology showed spinal and bulbar mot
or neuron disease with severe involvement of extraocular motor nuclei,
degeneration of spinal sympathetic and bulbar parasympathetic nuclei,
striatonigral degeneration, and early olivopontocerebellar atrophy. T
his case underscores the diversity of multiple system atrophy and demo
nstrates an unusually rapid course in a young patient.