Morgan Catherine, Fuller Geraint, Wakerley Benjamin R
Department of Neurology, Gloucester Royal Hospital, Gloucester, GL1 3NN, UK.
Nuffield Department of Clinical Neurosciences, University of Oxford, Oxford, UK.
Muscle Nerve. 2016 May;53(5):818-22. doi: 10.1002/mus.25028. Epub 2016 Mar 15.
Bifacial weakness with paresthesias is a rare subtype of Guillain-Barré syndrome (GBS), characterized by facial diplegia in the absence of any other cranial neuropathies, limb weakness, or ataxia. Frequently, patients also complain of distal limb paresthesias before or at the time they develop facial weakness.
We describe a man who developed post-infective isolated symmetric facial diplegia associated with distal paresthesias. Nerve conduction studies were conducted at 4 time-points over 6 months.
A monophasic disease course and presence of cerebrospinal fluid albuminocytological dissociation supported a diagnosis of bifacial weakness with paresthesias. Serial nerve conduction studies demonstrated an evolving demyelinating neuropathy with evidence of distal and proximal demyelination without conduction block, which partially resolved over time. Despite complete resolution of facial weakness within weeks, distal paresthesias persisted beyond 6 months.
This study suggests that neuropathy in patients with bifacial weakness and paresthesias is demyelinating and diffuse.
伴有感觉异常的双侧面肌无力是吉兰-巴雷综合征(GBS)的一种罕见亚型,其特征为双侧面瘫,且无任何其他颅神经病变、肢体无力或共济失调。患者常于出现面部无力之前或之时诉说肢体远端感觉异常。
我们描述了一名出现感染后孤立性对称性双侧面瘫并伴有远端感觉异常的男性患者。在6个月内的4个时间点进行了神经传导研究。
单相病程及脑脊液蛋白细胞分离现象支持伴有感觉异常的双侧面肌无力的诊断。系列神经传导研究显示为一种进行性脱髓鞘性神经病,有远端和近端脱髓鞘的证据,但无传导阻滞,且部分随着时间推移而缓解。尽管面部无力在数周内完全缓解,但远端感觉异常持续超过6个月。
本研究提示,伴有感觉异常的双侧面肌无力患者的神经病变为脱髓鞘性且呈弥漫性。