Cerebellar ataxia, dementia, pyramidal signs, cortical cataract of the posterior pole and a raised IgG index in a patient with a sporadic form of olivopontocerebellar atrophy.
Middle-aged patients who initially present with a progressive cerebellar ataxia, in the absence of a known familial pattern are often referred to under the descriptive diagnosis of 'idiopathic' late onset cerebellar ataxia. If these patients in time develop additional pyramidal or extrapyramidal features then they should be labeled as olivopontocerebellar atrophy (sOPCA). This case report describes a patient with OPCA with cerebellar ataxia as the presenting and most prominent feature in combination with dementia, pyramidal signs, cortical cataract of the posterior pole and a raised IgG index in cerebrospinal fluid. To the best of our knowledge this combination of signs and symptoms have not been described before.
Schelhaas
H J
HJ
Department of Neurology, Medical Spectrum Twente, Enschede, The Netherlands.
Hageman
G
G
Post
J G
JG
D000328:Adult; D001284:Atrophy; D001706:Biopsy; D001921:Brain / Q000473:pathology; D002386:Cataract / Q000175:diagnosis / Q000235:genetics* / Q000276:immunology; D002531:Cerebellum / Q000473:pathology; D002540:Cerebral Cortex / Q000473:pathology; D003704:Dementia / Q000175:diagnosis / Q000235:genetics* / Q000276:immunology; D005260:Female; D006801:Humans; D006942:Hypergammaglobulinemia / Q000175:diagnosis / Q000235:genetics* / Q000276:immunology; D007074:Immunoglobulin G / Q000134:cerebrospinal fluid*; D008279:Magnetic Resonance Imaging; D018482:Muscle, Skeletal / Q000473:pathology; D009460:Neurologic Examination; D009483:Neuropsychological Tests; D009849:Olivopontocerebellar Atrophies / Q000175:diagnosis / Q000235:genetics* / Q000276:immunology; D010375:Pedigree; D011712:Pyramidal Tracts / Q000473:pathology / Q000503:physiopathology; D013132:Spinocerebellar Degenerations / Q000175:diagnosis / Q000235:genetics* / Q000276:immunology
D007074:Immunoglobulin G