An unusual association of dentato-rubral degeneration with spinal ataxia, ophthalmoplegia and multiple cranial nerve palsies.
Clinical and neuropathological data of a 50-year-old woman with an unusual multisystem degeneration are presented. Clinically the illness was characterized by progressive ataxia with ophthalmoplegia and multiple cranial nerve palsies. Neuropathological investigation showed a severe and selective degeneration of the dentato-rubral system, of the posterior columns and of several cranial nerve nuclei. The problems of differential diagnosis and classification are discussed.
D001259:Ataxia / Q000150:complications* / Q000473:pathology; D001927:Brain Diseases / Q000150:complications / Q000473:pathology; D002526:Cerebellar Diseases / Q000150:complications* / Q000473:pathology; D003389:Cranial Nerve Diseases / Q000150:complications*; D005260:Female; D006801:Humans; D008875:Middle Aged; D009410:Nerve Degeneration; D009886:Ophthalmoplegia / Q000150:complications*; D010243:Paralysis / Q000150:complications*; D012012:Red Nucleus / Q000473:pathology; D013122:Spinal Diseases / Q000150:complications* / Q000473:pathology