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神经结节病中的马尾综合征

Cauda Equina Syndrome in Neurosarcoidosis.

作者信息

Topiwala Karan, Rath Subhendu, Daniel Annie, Prasad Avinash

机构信息

Neurology, Hartford Hospital, Hartford, USA.

Neurology, University of Michigan School of Medicine, Ann Arbor, USA.

出版信息

Cureus. 2020 Aug 27;12(8):e10069. doi: 10.7759/cureus.10069.

Abstract

Neurosarcoidosis (NS) is a mimicker of many infectious, neoplastic, and inflammatory diseases. It most commonly involves the cranial nerves followed by meninges, ventricles, hypothalamic-pituitary axis, spinal cord, and brainstem/cerebellum. While NS myelopathy has been increasingly recognized, pathophysiological/prognostic and management principles in NS-mediated cauda equina (CE) and conus medullaris (CM) syndromes, which constitute a small and rare minority of this subset, remain elusive. We present the case of a 49 -year-old Hispanic man who developed a peripheral facial palsy and primary hypogonadism within a span of 12 months and eventually got diagnosed with NS after he presented with CE syndrome. We also performed an extensive literature review, with a discussion on the underlying pathophysiology and current management recommendations for NS-mediated CE/CM syndrome. CE/CM syndromes in a middle-aged man should prompt the consideration of NS as a possible differential diagnosis. While steroid responsive, the majority of NS-CE/CM patients are left with residual neurodeficits with quick relapses when steroids are tapered, making the case for early institution of immunosuppressive therapies.

摘要

神经结节病(NS)可模仿多种感染性、肿瘤性和炎性疾病。它最常累及颅神经,其次是脑膜、脑室、下丘脑 - 垂体轴、脊髓以及脑干/小脑。虽然NS脊髓病已越来越受到认可,但在NS介导的马尾(CE)和圆锥马尾(CM)综合征中,其病理生理/预后及管理原则仍不明确,而这两种综合征在该疾病亚组中占比小且罕见。我们报告了一例49岁的西班牙裔男性病例,该患者在12个月内出现周围性面瘫和原发性性腺功能减退,最终在出现CE综合征后被诊断为NS。我们还进行了广泛的文献综述,并讨论了NS介导的CE/CM综合征的潜在病理生理学和当前管理建议。中年男性出现CE/CM综合征时应考虑NS作为可能的鉴别诊断。虽然大多数NS - CE/CM患者对类固醇有反应,但当类固醇减量时,大多数患者会留下残余神经功能缺损且复发迅速,这使得早期应用免疫抑制疗法成为必要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bce4/7522054/d22b1ff9a970/cureus-0012-00000010069-i01.jpg

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