Olivopontocerebellar degeneration. Clinicopathologic correlation of the associated retinopathy

Arch Ophthalmol. 1975 Mar;93(3):169-72. doi: 10.1001/archopht.1975.01010020177001.

Abstract

In two siblings with olivopontocerebellar degeneration, the retinal pigment epithelium or the sensory retinal or both appeared to be damaged first. These layers were markedly involved before secondary changes in the choriocapillaris, the inner retinal layers, or the optic nerve occurred.

MeSH terms

  • Adult
  • Brain Diseases / genetics
  • Cerebellar Diseases / genetics*
  • Cerebellar Diseases / pathology
  • Choroid / pathology
  • Cytoplasmic Granules
  • Epithelium / pathology
  • Female
  • Fluorescein Angiography
  • Fundus Oculi
  • Humans
  • Infant
  • Male
  • Olivary Nucleus*
  • Pigments, Biological
  • Pons*
  • Retina / pathology
  • Retinal Diseases / etiology*
  • Retinal Diseases / genetics
  • Retinal Diseases / pathology
  • Syndrome

Substances

  • Pigments, Biological