A69yearoldmanwiths

A69-year-oldmanwithshufflinggait

A

B

C

Whatisyourdiagnosis?

TheanswerisOlivopontocerebellarAtrophy

Olivopontocerebellaratrophy,acerebellarsubtypeofmultiplesystemsatrophy(MSA-C),isasporadic,progressivemultisystemneurodegenerativedisorderpresentingwithparkinsonian,cerebellar,andautonomicsymptoms.MSA-Ciscausedbyintracellularαsynucleindeposition(synucleinopathy).TheclassicMRimagingfeaturesofMSA-Cincludepontocerebellaratrophy(A,arrows),degenerationofraphenucleiandtransversepontinefibers,whichgiverisetoaclassic“crossbunsign”appearanceonaxialT2WI(B,arrows),andatrophyofthemiddlecerebellarpeduncles(C,arrows).

中文版:

男性,69岁,拖行步态

诊断:橄榄体脑桥小脑萎缩

橄榄体脑桥小脑萎缩,属于多系统萎缩的小脑亚型,是一种散发、多系统进行性神经变性疾病,可表现为帕金森、小脑的症状以及自主症状。多系统萎缩是由细胞内α突触核蛋白沉积引起(共核蛋白病)。典型MRI表现包括:脑桥小脑萎缩(A图箭头),中继核、脑桥横向纤维变性,T2WI呈经典的“十字面包征”(B图箭头),同时可见小脑中脚萎缩(C图箭头)。

FromAJNRClassicCase

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