[Olivo-pontocerebellar atrophy. Report of a case (author's transl)]

A case of hereditary olivo-pontocerebellar atrophy is reported. The patient presented with intentional tremor and slurred speech, and physical examination disclosed a bi-hemispherical cerebellar syndrome, involvement of the IX and X cranial nerves, and retinitis pigmentosa. Computerized axial tomography demonstrated a supra and infratentorial atrophy, predominating in the brain stem and cerebellum. The literature on olivo-pontocerebellar atrophy is reviewed, verifying the ample clinical spectrum of this disorder amongst the spinocerebellar degeneration syndromes, and emphasizing the diagnostic value of computerized axial tomography.