橄榄脑桥小脑萎缩与“十字征”

目的:探讨橄榄脑桥小脑萎缩(OPCA)的临床和影像学特点。方法:对1例橄榄脑桥小脑萎缩患者的临床资料进行回顾性分析并文献复习。结果:本病以小脑共济失调为首发表现,部分出现植物神经系统受损、锥体束或锥体外系症状。影像学除脑干变细、小脑体积变小外,可出现特征性的十字征表现。结论:因该病和某些小脑变性疾病表现相重叠,且无有意义的生化标记物,故其影像学十字征的发现对此病的诊断和鉴别诊断意义重大。

Oiivopontocerebellar atrophy with cross sign

Objective: To investigate the clinical manifestation and neuroimaging characteristic of olivopontocerebellar atrophy ( OPCA) .Methods: The clinical data of one OPCA patient was retrospectively analyzed associated with literature review. Results: The initial clinical manifestation of OPCA presents with cerebella ataxia. Autonomic nerve system damage and pyramidal tract and extrapyramidal system dysfunction are happened in part of patients. Brainstem and cerebella shrinking can be seen on neuroimaging, and cross sign is the characteristic appearance on neuroimaging. Conclusion: The clinical manifestations between OPCA and other cerebellar degeneration diseases overlap, and there is no biochemical marker of this disorder. Cross sign is important to the diagnosis and antidiastole of OPCA.