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We used magnetic resonance imaging(MRI)to study brain and spinal cord morphology in hereditary and idiopathic ataxia.Our interest was in whether the classical neuropathologic categories-cerebellar cortical atrophy(CCA), olivopontocerebellar atrophy(OPCA),and spinal atrophy(SA)-could be identified in vivo and which vermis,cerebellar hemispheres fourth ventricle,middle cerebellar peduncles,basis pontis,medulla oblongata,and cervical spine cord on T1-weighted images of 61 patients and 24 healthy controls.Five patients with Friedreich's ataxia(n=7)and all with late- onset Freidreich's ataxia(n=3)had SA without major involvement of the brainstem or cerebellum.Morphologic findings in patients with early-onset cerebellar ataxia with retained tendon reflexes(n=11)were heterogeneous; six patients had MRI findings compatible with CCA,and two patients had a combination of SA and CCA.The three remaining patients had an atypical pattern of atrophy.Similarly,the morphologic changes in patients with autosomal-dominant cerebellar ataxia with additional noncerebellar symptoms(ADCA-I;n=13)were nonuniform;atrophic changes typical for CCA, OPCA,or SA were each present in one case,four patients had a combination of OPCA and SA,and the remaining patients could not be assigned to one of the morphologic categories.In autosomal-dominant cerebellar ataxia with a pure cerebellar syndrome(ADCA-III;n=6),all patients except one had CCA. Patients with idiopathic cerebellar ataxia(IDCA)fell into two clinically and morphologically distinct groups:the majority of patients with additional noncerebellar symptoms(IDCA-P;n=14)had OPCA,whereas almost all patients with a pure cerebellar syndrome(IDCA-C;n=7)had CCA. |
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ataxia ataxia,cerebellar ataxia,hereditary brainstem,atrophy cerebellar ataxia,hereditary cerebellar ataxia,primary cerebellar atrophy,primary degenerative diseases of CNS familial Friedreich's ataxia MRI MRI,abnormal myelomalacia spinocerebellar ataxia type 1
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