Olivopontocerebellar degeneration. Clinicopathologic correlation of the associated retinopathy.
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.
D000328:Adult; D001927:Brain Diseases / Q000235:genetics; D002526:Cerebellar Diseases / Q000235:genetics* / Q000473:pathology; D002829:Choroid / Q000473:pathology; D003594:Cytoplasmic Granules; D004848:Epithelium / Q000473:pathology; D005260:Female; D005451:Fluorescein Angiography; D005654:Fundus Oculi; D006801:Humans; D007223:Infant; D008297:Male; D009847:Olivary Nucleus; D010860:Pigments, Biological; D011149:Pons; D012160:Retina / Q000473:pathology; D012164:Retinal Diseases / Q000209:etiology* / Q000235:genetics / Q000473:pathology; D013577:Syndrome
D010860:Pigments, Biological