We report a 62-year-old woman with a past history of painful central visual
loss who developed progressive quadriparesis and bulbar palsy, Neurologica
l examination revealed widespread upper and lower motor neuron signs in the
bulbar region and extremities. Electromyography demonstrated widespread ac
tive and chronic motor axon loss. Magnetic resonance neuroimaging studies r
evealed enhancing callosal and periventricular white matter lesions and cer
vical and thoracic cord hyperintensities. Cerebrospinal fluid analysis was
consistent with multiple sclerosis. The patient died of respiratory failure
two years after presentation, and autopsy revealed multifocal demyelinatio
n involving the corpus callosum, cerebellum and spinal cord as well as path
ologic findings typical of amyotrophic lateral sclerosis. A review of the l
iterature confirms the exceedingly unusual combination of amyotrophic later
al sclerosis with multiple sclerosis.