Involuntary Thumb Flexion on Neurological Examination: An Unusual Form of Upper Limb Dystonia in the Faroe Islands

Involuntary Thumb Flexion on Neurological Examination: An Unusual Form of Upper Limb Dystonia in the Faroe Islands
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DOI:
10.7916/tohm.v0.686
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发表时间:
2019-08-20
影响因子:
2.2
通讯作者:
Louis, Elan D.
Louis, Elan D.
中科院分区:
其他
文献类型:
--
作者:
Kim, Christine Y.;Petersen, Maria Skaalum;Louis, Elan D.

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背景:肌张力障碍的患病率在世界范围内各不相同。先前的一份报告表明,法罗群岛人群中局灶性肌张力障碍的患病率很高,这可能反映了创始人效应。作为正在进行的法罗群岛神经流行病学研究的一部分,在标准化的神经学检查中,我们注意到一部分受试者在重复手部运动时拇指屈曲的异常现象,并试图定义其现象学。方法:我们征求了肌张力障碍专家小组对运动现象学的评论。这些专家回顾了录像的神经学检查。结果:在专家中,肌张力障碍是主要的诊断。其他原因也被考虑,但被认为不太可能。讨论:诊断肌张力障碍需要仔细的临床评估和考虑相关特征。我们报告了一种新的肌张力障碍形式,据我们所知,在这个与世隔绝的人群中没有描述过。有必要对法罗群岛肌张力障碍的流行情况进行进一步研究,以确定其在该人群中的负担及其具体的临床特征。
Background: The prevalence of dystonia varies worldwide. A prior report suggested a high prevalence of focal dystonia in the Faroese population, possibly reflecting a founder effect. During standardized neurological examination as part of an ongoing neuroepidemiologic study in the Faroe Islands, we noted an unusual phenomenon of thumb flexion during repetitive hand movements in a subset of subjects and sought to define its phenomenology.Methods: We requested commentary from a panel of dystonia experts regarding the phenomenology of the movements. These experts reviewed the videotaped neurological examination.Results: Among the experts, dystonia was the leading diagnosis. Alternate causes were considered, but deemed less likely.Discussion: Diagnosis of dystonia requires careful clinical assessment and consideration of associated features. We report a novel form of dystonia, not previously described to our knowledge, in this isolated population. Further studies of dystonia prevalence in the Faroe Islands are merited to characterize its burden in this population and its specific clinical characteristics.