An autopsied case of alcoholic cerebellar degeneration with spastic paraplegia and neuropathy

A 45 year-old-female was admitted to Kanagawa Rehabilitation Center because of marked spastic paraplegia. There was no family history of neurological diseases. She had been drinking a great excess of alcohol since twenty years of age. Though she noticed the unsteadiness of gait several years prior to the admission, she had not been examined. On admission neurological examination revealed pyramidal weakness of both legs, and slight sensory impairment in distal part of the lower extremities. But she had neither difficulty in speech nor abnormal findings in the upper extremities. Her mentality was well preserved. Though computed tomography revealed cerebellar cortical atrophy, no signs of cerebellar impairment could not be found except spastic paraplegia. Blood chemistry failed to reveal liver dysfunction. There was no pernicious anemia. These symptoms were almost stationary until her death. At the age of 53, she died of gastric cancer. Postmortem examination disclosed marked degeneration of cerebellum and spinal cord. Cerebellar cortical degeneration, which was characterized by involvement of all layers of the cortex, showed unique distribution. The lingula, central lobule, culmen, superior portion of declive, anterior lobules and anteromedial half of simple lobule were severely degenerated, while other lobules were spared excluding moderate degeneration in inferomedian portion of the hemisphere. These cortical degeneration was prominent much more in vermis than in hemisphere. There was a mild myelin pallor in the lamellar white matter and slight fibrous gliosis. In the dentate nucleus, there were moderate neuronal loss with gliosis.(ABSTRACT TRUNCATED AT 250 WORDS)