Med.Sci 532

Structure-Function

This is a rare, autosomally-inherited disease that leads to the progressive degeneration of the cerebellar cortex and the inferior olivary nucleus within the medulla. Onset is generally in the fourth decade. The disease results in ataxic gait, dysarthria, and tremor of the limbs.

Autopsy reveals pronounced atrophy of the cerebellum.  There is almost complete loss of Pukinje cells and an associated astrocytosis. Shrinkage is not as prominent in the olives.   It is thought that the disease begins in the cerebellum and that in the inferior olivary nucleus the neuronal loss is secondary since the Purkinje cell is the synaptic target of the inferior olivary nucleus neurons. There is some similarity pathologically between this disease and chronic alcoholic degeneration in that both affect the cerebellum.

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References

Holmes,Gordon

1876-1966