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伴有肥厚性橄榄体变性(HOD)的进行性共济失调和腭震颤(PAPT):一例报告。

Progressive ataxia and palatal tremor (PAPT) with hypertrophic olivary degeneration (HOD): A case report.

作者信息

Marrakchi Salma, Laasri Khadija, Moufakkir Rim, Guelzim Yousra, Hadj Hsain Ihssan, El Yousfi Zakia, Fenni Jamal El, En-Nouali Hassan

机构信息

Radiology Department, Mohammed V Military Teaching Hospital, Faculty of Medicine and Pharmacy, Mohammed V University, Rabat, Morocco.

Neurology Department, Ibn Sina University Hospital Center, Faculty of Medicine and Pharmacy, Mohammed V University, Rabat, Morocco.

出版信息

Radiol Case Rep. 2024 Mar 20;19(6):2264-2267. doi: 10.1016/j.radcr.2024.01.054. eCollection 2024 Jun.

Abstract

Palatal tremor has been subdivided into essential (EPT) and symptomatic palatal tremor (SPT). Progressive ataxia and palatal tremor syndrome (PAPT) is a subgroup of symptomatic palatal tremor (SPT). It can be divided into familial and sporadic forms. Sporadic PAPT is characterized by progressive cerebellar degeneration. The cause of sporadic PAPT remains uncertain. MRI examination found an enlarged appearance of the olivary nuclei with increased signal intensity on T2 and FLAIR images. Here we report a case of a mid-adult-onset man which presents a worsening cerebellar progressive ataxia with palatal tremor, in whom imaging reveals abnormalities of the olivary nuclei with tardive cerebellar atrophy which has been diagnosed as a sporadic PAPT.

摘要

腭震颤已被细分为原发性(EPT)和症状性腭震颤(SPT)。进行性共济失调和腭震颤综合征(PAPT)是症状性腭震颤(SPT)的一个亚组。它可分为家族性和散发性形式。散发性PAPT的特征是进行性小脑变性。散发性PAPT的病因仍不确定。MRI检查发现橄榄核外观增大,在T2和FLAIR图像上信号强度增加。在此,我们报告一例中年发病男性病例,该患者出现伴有腭震颤的进行性小脑性共济失调加重,其影像学显示橄榄核异常伴迟发性小脑萎缩,已被诊断为散发性PAPT。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1dcb/10965476/c41f373bc86e/gr1.jpg

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