Olivopontocerebellar Atrophies

Olivopontocerebellär atrofi

Svensk definition

En grupp av ärftliga och sporadiska sjukdomar med progressiv ataxi kombinerad med atrofi av lillhjärna, hjärnbrygga och inferior olivkärna. Andra kliniska kännetecken kan inkludera muskelstelhet, patologisk nystagmus, näthinneförtvining, muskelspasticitet, demens, urininkontinens och ögonförlamning. Den familjära formen har tidigare sjukdomsdebut (andra livsdekaden) och ryggmärgsatrofi kan uppträda. Den sporadiska formen tenderar att visa sig under femte eller sjätte livsdekaden och betraktas som en klinisk subtyp av multisystematrofi.

Engelsk definition

A group of inherited and sporadic disorders which share progressive ataxia in combination with atrophy of the CEREBELLUM; PONS; and inferior olivary nuclei. Additional clinical features may include MUSCLE RIGIDITY; NYSTAGMUS, PATHOLOGIC; RETINAL DEGENERATION; MUSCLE SPASTICITY; DEMENTIA; URINARY INCONTINENCE; and OPHTHALMOPLEGIA. The familial form has an earlier onset (second decade) and may feature spinal cord atrophy. The sporadic form tends to present in the fifth or sixth decade, and is considered a clinical subtype of MULTIPLE SYSTEM ATROPHY. (From Adams et al., Principles of Neurology, 6th ed, p1085)

Svenska synonymer

Dejerine-Thomas syndrom Presenil ataxi Olivopontocerebellär degeneration

Engelska synonymer

Atrophy, Olivopontocerebellar Olivopontocerebellar Atrophy Dejerine-Thomas Syndrome Dejerine Thomas Syndrome Syndrome, Dejerine-Thomas Olivo-Ponto-Cerebellar Degeneration Degeneration, Olivo-Ponto-Cerebellar Olivo Ponto Cerebellar Degeneration Olivo-Ponto-Cerebellar Degenerations Presenile Ataxia Ataxia, Presenile Presenile Ataxias Pontoolivocerebellar Atrophy Atrophy, Pontoolivocerebellar Pontoolivocerebellar Atrophies Olivopontocerebellar Degeneration Degeneration, Olivopontocerebellar Olivopontocerebellar Degenerations Olivo-Ponto-Cerebellar Atrophy Atrophy, Olivo-Ponto-Cerebellar Olivo Ponto Cerebellar Atrophy Nonfamilial Olivopontocerebellar Atrophy Atrophy, Nonfamilial Olivopontocerebellar Nonfamilial Olivopontocerebellar Atrophies Olivopontocerebellar Atrophies, Nonfamilial Olivopontocerebellar Atrophy, Nonfamilial Familial Olivopontocerebellar Atrophy Atrophy, Familial Olivopontocerebellar Familial Olivopontocerebellar Atrophies Olivopontocerebellar Atrophies, Familial Olivopontocerebellar Atrophy, Familial Inherited Olivopontocerebellar Atrophy Atrophy, Inherited Olivopontocerebellar Inherited Olivopontocerebellar Atrophies Olivopontocerebellar Atrophy, Inherited Olivopontocerebellar Atrophy, Idiopathic Atrophy, Idiopathic Olivopontocerebellar Idiopathic Olivopontocerebellar Atrophies Idiopathic Olivopontocerebellar Atrophy Olivopontocerebellar Hypoplasia Hypoplasia, Olivopontocerebellar Olivopontocerebellar Hypoplasias