Olivopontocerebellar atrophy with retinal degeneration. An electroretinographic and histopathologic investigation.

@article{To1993OlivopontocerebellarAW,
  title={Olivopontocerebellar atrophy with retinal degeneration. An electroretinographic and histopathologic investigation.},
  author={King W. To and Michael Adamian and Frederick Albert Jakobiec and Eliot L. Berson},
  journal={Ophthalmology},
  year={1993},
  volume={100 1},
  pages={15-23}
}
BACKGROUND Olivopontocerebellar atrophy is an uncommon disorder with variable clinical manifestations that affects the cerebellum, the spinocerebellar tracts, and other structures of the brainstem. A deficiency of glutamate dehydrogenase, which results in an excess of glutamate, has been suggested to play a role in the pathogenesis of olivopontocerebellar atrophy. In experimental animals, toxic levels of glutamate are known to cause a selective loss of the b-wave on electroretinographic (ERG… CONTINUE READING
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