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持久性动眼神经麻痹作为慢性炎性脱髓鞘性多发性神经病的首发症状

Long-Lasting Cranial Nerve III Palsy as a Presenting Feature of Chronic Inflammatory Demyelinating Polyneuropathy.

作者信息

Spataro Rossella, La Bella Vincenzo

机构信息

ALS Clinical Research Center, Bio.Ne.C, University of Palermo, 90129 Palermo, Italy.

出版信息

Case Rep Med. 2015;2015:769429. doi: 10.1155/2015/769429. Epub 2015 Apr 16.

Abstract

We describe a patient with chronic inflammatory demyelinating polyneuropathy (CIDP) in which an adduction deficit and ptosis in the left eye presented several years before the polyneuropathy. A 52-year-old man presented with a 14-year history of unremitting diplopia, adduction deficit, and ptosis in the left eye. At the age of 45 a mild bilateral foot drop and impaired sensation in the four limbs appeared, with these symptoms showing a progressive course. The diagnostic workup included EMG/ENG which demonstrated reduced conduction velocity with bilateral and symmetrical sensory and motor involvement. Cerebrospinal fluid studies revealed a cytoalbuminologic dissociation. A prolonged treatment with corticosteroids allowed a significant improvement of the limb weakness. Diplopia and ptosis remained unchanged. This unusual form of CIDP presented as a long-lasting isolated cranial nerve palsy. A diagnostic workup for CIDP should therefore be performed in those patients in which an isolated and unremitting cranial nerve palsy cannot be explained by common causes.

摘要

我们描述了一名慢性炎性脱髓鞘性多发性神经病(CIDP)患者,其左眼内收功能障碍和上睑下垂在多发性神经病出现前数年就已出现。一名52岁男性,有14年左眼持续性复视、内收功能障碍和上睑下垂的病史。45岁时出现轻度双侧足下垂和四肢感觉障碍,这些症状呈进行性发展。诊断性检查包括肌电图/神经电图,显示传导速度减慢,双侧对称性感觉和运动受累。脑脊液检查显示细胞蛋白分离。长期使用皮质类固醇治疗使肢体无力有显著改善。复视和上睑下垂无变化。这种不寻常的CIDP形式表现为长期孤立的颅神经麻痹。因此,对于那些孤立且持续性颅神经麻痹无法用常见原因解释的患者,应进行CIDP的诊断性检查。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/3040/4415652/3f7fa5230cd7/CRIM2015-769429.001.jpg

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