title: Computed tomography in hereditary ataxias.
abstract: Cranial CT in 39 patients (23 belonged to 8 families) with four different groups of hereditary ataxia (HA) showed mainly three combinations of atrophic findings: (1) cerebellar ataxia (CA, n = 17) had marked atrophy of the cerebellum and/or the brain stem combined with moderate cerebral atrophy; (2) an intermediate group consisting of hereditary spastic paraplegia (HSP, n = 10) and Friedreich's ataxia (FA, n = 7), both with moderate infra- and supratentorial atrophy; (3) atrophy was hardly demonstrated in the group of Charcot-Marie-Tooth disease (CMT, n = 5). HA cases with atrophy could be distinguished from multiple sclerosis (MS) by CT.
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001259:Ataxia / Q000000981:diagnostic imaging* / Q000235:genetics; D001921:Brain / Q000000981:diagnostic imaging; D002524:Cerebellar Ataxia / Q000000981:diagnostic imaging; D002607:Charcot-Marie-Tooth Disease / Q000000981:diagnostic imaging; D002648:Child; D002675:Child, Preschool; D005260:Female; D005621:Friedreich Ataxia / Q000000981:diagnostic imaging; D006801:Humans; D007223:Infant; D008297:Male; D008875:Middle Aged; D009128:Muscle Spasticity / Q000235:genetics; D010264:Paraplegia / Q000235:genetics; D014057:Tomography, X-Ray Computed