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一名63岁女性以全身性舞蹈徐动症为表现的亚急性硬化性全脑炎

Subacute Sclerosing Panencephalitis in a 63-Year-Old Woman Presenting as Generalized Choreoathetosis.

作者信息

Mondal Ritwick, Deb Shramana, Mahata Manoj, Saha Somesh, Lahiri Durjoy, Benito-León Julián

机构信息

Department of Clinical Pharmacology and Therapeutic Medicine, IPGMER and SSKM Hospital, Kolkata, India.

Department of Neuroscience, S.N.Pradhan Centre for Neuroscience, Kolkata, India.

出版信息

Neurohospitalist. 2023 Oct;13(4):381-393. doi: 10.1177/19418744231177105. Epub 2023 May 19.

Abstract

The persistence of measles virus infection in childhood and early adolescence can rarely lead to a fatal progressive neurodegenerative disorder known as subacute sclerosing panencephalitis (SSPE), characterized by behavioral disturbances and intellectual disability followed by myoclonic jerks and occasional negative myoclonus. Movement disorders are rarely presenting manifestations in SSPE. We herein report a 63-year-old woman with generalized choreoathetosis as the presenting manifestation of stage-I SSPE. Our case was atypical for the patient's age and clinical presentation with generalized choreoathetosis and bilateral putaminal and caudate nucleus signal hyperintensity. Though highly uncommon, neurologists should keep SSPE as a differential diagnosis among patients with movement disorders. Measles-endemic countries should be more vigilant to the atypical and rare presentations of SSPE, such as generalized choreoathetosis.

摘要

儿童期和青春期早期麻疹病毒感染的持续存在很少会导致一种致命的进行性神经退行性疾病,即亚急性硬化性全脑炎(SSPE),其特征为行为障碍和智力残疾,随后出现肌阵挛性抽搐和偶尔的负性肌阵挛。运动障碍在SSPE中很少作为首发表现。我们在此报告一名63岁女性,以全身性舞蹈徐动症作为I期SSPE的首发表现。我们的病例在患者年龄以及全身性舞蹈徐动症和双侧壳核及尾状核信号高强化的临床表现方面均不典型。尽管极为罕见,但神经科医生在患有运动障碍的患者中应将SSPE作为鉴别诊断之一。麻疹流行国家应更加警惕SSPE的非典型和罕见表现,如全身性舞蹈徐动症。

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