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We examined five males with laboratory-confirmed ataxia-telangiectasia (AT),aged 9-28 years,several times by MRI(9 examinations:5 at 0.15 T,3 at 0.5T,1 at 1.5T).Intermediate,T1-T2-and T2*-weighted spin-echo and gradient-echo sequences were performed.All patients showed vermian atrophy,enlarged fourth ventricle and cisterna magna;four showed cerebellar hemisphere atrophy;two enlarged infracerebellar subarachnoid spaces and four patients had sinusitis.No focal areas of abnormal signal were seen in the brain,diffuse high signal was found in the central cerebral white matter of the oldest patient.When imaging is necessary,MRI should be preferred to CT in patients known or suspected to have AT and those with undefined pediatric ataxias of nontraumatic origin.If atrophy of only the cerebellum,especially the vermis,is noted,laboratory research should be performed to confirm the diagnosis of AT. |
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ataxia telangiectasia
cerebellar atrophy,primary
cerebellar degeneration
cerebellar vermis
cisterna magna
cisterna magna,enlarged
fourth ventricle,enlargement of
MRI
MRI,abnormal
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