Olivopontocerebellar Atrophy (Heredocerebellar Ataxia of Marie) with Encephalographic Findings

Author and Disclosure Information


Hassin and Harris1 have stated that heredocerebellar ataxia and olivopontocerebellar atrophy is one disease process. This observation was based upon their review of Keiller’s2 work, wherein cerebellar atrophy was observed clinically in 10 members of the same family. The neuropathologic changes were alike in the cases studied and were typical of olivopontocerebellar atrophy. The outstanding changes noted were: diminished size of the cerebellar folia, atrophy of the olivary bodies and of the pons.

Thus, while olivopontocerebellar atrophy is a pathologic entity, clinically it resembles heredocerebellar ataxia of Marie so closely that it seems to have no definite differentiating features. The presence of a hereditary or familial history of this condition was regarded as thè differentiating point between Marie’s heredocerebellar ataxia and olivopontocerebellar atrophy. However, these authors feel that olivopontocerebellar atrophy may be both hereditary and familial, having demonstrated that, in the patients with the typical clinical picture of heredocerebellar ataxia, there were also typical pathologic findings of olivopontocerebellar atrophy.

With encephalography it is often possible to determine in vivo gross pathologic changes in the brain. At least one of the pathologic changes present in cerebellar atrophy is often gross enough to be detected by encephalographic study. Accordingly, encephalographic study was made of a patient who had the typical clinical picture of heredocerebellar ataxia of Marie (olivopontocerebellar atrophy), and this pathologic change was noted. A lateral view of the encephalogram is presented (figure), while the case report follows.

Case Report

A white man, aged 35, rather stocky and obese, complained of. . .



Next Article: