以坐骨神经单肢神经病为主表现的慢性炎症性脱髓鞘性多发性神经根神经病
Chronic inflammatory demyelinating polyradiculoneuropathy presenting as predominantly sciatic monomelic neuropathy.
作者信息
El-Wahsh Shadi, Cappelen-Smith Cecilia, Spies Judith
机构信息
Department of Neurology, Royal Prince Alfred Hospital, Camperdown, New South Wales, Australia.
South Western Sydney Clinical School, University of New South Wales, Sydney, New South Wales, Australia.
出版信息
BMJ Neurol Open. 2020 Apr 8;2(1):e000045. doi: 10.1136/bmjno-2020-000045. eCollection 2020.
BACKGROUND
Chronic inflammatory demyelinating polyneuropathy (CIDP) is a common yet underdiagnosed cause of potentially treatable chronic sensorimotor neuropathy. The classical form of the disease is characterised by symmetrical weakness in both distal and proximal muscle groups accompanied by sensory dysfunction and diminished tendon reflexes lasting more than 2 months.
METHOD
The diagnosis of CIDP is supplemented by electrodiagnostic studies and biopsy findings confirming demyelination, in accordance with well-established diagnostic criteria. Atypical presentations of CIDP often pose a diagnostic challenge.
RESULTS
In this paper, we present a case of isolated lower limb involvement due to CIDP to raise awareness of this focal lower limb variant. Of particular, significance is the use of lumbosacral plexus MRI to assist in the diagnosis.
CONCLUSION
Focal CIDP is an atypical presentation that should be considered in patients presenting with chronic monomelic neuropathy and should be investigated with electrodiagnostic studies, lumbar puncture, nerve biopsy and MRI of the nerve roots and plexuses.
背景
慢性炎症性脱髓鞘性多发性神经病（CIDP）是潜在可治疗的慢性感觉运动性神经病常见但诊断不足的病因。该疾病的经典形式表现为远端和近端肌群对称性无力，伴有感觉功能障碍和腱反射减弱，持续超过2个月。
方法
根据公认的诊断标准，CIDP的诊断需辅以电诊断研究和活检结果以证实脱髓鞘。CIDP的非典型表现常带来诊断挑战。
结果
在本文中，我们报告一例因CIDP导致孤立性下肢受累的病例，以提高对这种局灶性下肢变异型的认识。特别重要的是使用腰骶丛MRI辅助诊断。
结论
局灶性CIDP是一种非典型表现，对于表现为慢性单肢神经病的患者应予以考虑，并应通过电诊断研究、腰椎穿刺、神经活检以及神经根和神经丛的MRI进行检查。
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