An autopsied case of idiopathic late cortical cerebellar atrophy--comparison with other cortical cerebellar atrophy

A 68-year-old man without familial history developed ataxic gait and sensory disturbance in the lower extremities. At the age of 74, neurological examination revealed marked cerebellar ataxia of all limbs, dysarthria, sensory disturbance of glove and stocking type in the extremities, and slight neurogenic muscular atrophy. There were no mental deterioration and dysautonomia. He died of pneumonia at the age of 74. Neuropathological findings. The cerebellum was decreased in size. Microscopically, there were severe disappearance of Purkinje cells in the cerebellar vermis and hemispheres. The molecular layer, granular cell layer, and cerebellar white matter were preserved. Neurons of the inferior olivary nuclei were also spared. In the spinal cord, there was myelin pallor in the posterior column predominant in Goll's fascicule and moderate atrophy of neurons in the anterior horn. Degeneration of the posterior roots was greater than that of the anterior roots. No abnormal findings were found in the extrapyramidal system, cranial nerves, and cerebrum. We compared this case clinicopathologically with other diseases with cortical cerebellar atrophy; alcoholic cerebellar degeneration, phenytoin intoxication, neuroleptic malignant syndrome, and subacute paraneoplastic cerebellar degeneration. In conclusion, idiopathic late cerebellar cortical atrophy (LCCA) was different clinicopathologically from the other diseases. Especially, LCCA showed the characteristic topography of Purkinje cells loss sparing the molecular and granule cell layers.