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孤立性延髓麻痹:神经结节病的一种罕见表现。

Isolated Bulbar Palsy: A Rare Presentation of Neurosarcoidosis.

作者信息

Chaudhry Hammad S, Ata Fateen, Abdelghani Mohamed, Kazman Rashid, Rahil Ali

机构信息

Internal Medicine, Hamad Medical Corporation, Doha, QAT.

Cardiology, Heart Hospital, Hamad Medical Corporation, Doha, QAT.

出版信息

Cureus. 2020 Feb 16;12(2):e7010. doi: 10.7759/cureus.7010.

DOI:10.7759/cureus.7010
PMID:32206474
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC7077743/
Abstract

Sarcoidosis, which is a non-caseating granulomatous chronic inflammatory disease, can affect virtually any organ system, including the central nervous system (CNS). Very rarely, patients may present solely with neurosarcoidosis. It commonly presents with unilateral or bilateral seventh nerve palsy. Rarely it can present as dangerous progressive bulbar palsy and is a diagnostic and clinical challenge. We present a case of sarcoidosis with a unique presentation of isolated bulbar palsy. A 38-year-old male presented to the emergency with a sudden onset of dysphonia followed by dysphagia for both solids and liquids for one week and 5 kg weight loss in two months. The rest of the CNS exam was unremarkable. On labs, he had hypercalcemia and suppressed parathyroid hormone (PTH) intact. Detailed radiological investigations, lab tests, and lymph node biopsy helped confirm the diagnosis of neurosarcoidosis. The patient did not respond to first-line steroid therapy and hence received intravenous immunoglobulin (IVIG) subsequently with adequate response and complete neurologic recovery, confirmed by a follow-up visit.

摘要

结节病是一种非干酪样肉芽肿性慢性炎症性疾病,几乎可累及任何器官系统,包括中枢神经系统(CNS)。极罕见情况下,患者可能仅表现为神经结节病。其通常表现为单侧或双侧面神经麻痹。罕见情况下可表现为危险的进行性延髓麻痹,是一种诊断和临床挑战。我们报告一例具有孤立性延髓麻痹独特表现的结节病病例。一名38岁男性因突发声音嘶哑就诊于急诊科,随后出现固体和液体吞咽困难1周,2个月内体重减轻5kg。中枢神经系统其他检查无异常。实验室检查显示,他有高钙血症且完整甲状旁腺激素(PTH)水平受抑制。详细的影像学检查、实验室检查及淋巴结活检有助于确诊神经结节病。患者对一线类固醇治疗无反应,因此随后接受静脉注射免疫球蛋白(IVIG)治疗,反应良好且神经功能完全恢复,随访证实了这一点。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8af5/7077743/35b1bf755524/cureus-0012-00000007010-i08.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8af5/7077743/5495b3a82f58/cureus-0012-00000007010-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8af5/7077743/7f42a40a1495/cureus-0012-00000007010-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8af5/7077743/853b6ec398cc/cureus-0012-00000007010-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8af5/7077743/315c3c021c1a/cureus-0012-00000007010-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8af5/7077743/d712fbb84cdf/cureus-0012-00000007010-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8af5/7077743/0796d4ef89ac/cureus-0012-00000007010-i06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8af5/7077743/a6c0b9662b79/cureus-0012-00000007010-i07.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8af5/7077743/35b1bf755524/cureus-0012-00000007010-i08.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8af5/7077743/5495b3a82f58/cureus-0012-00000007010-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8af5/7077743/7f42a40a1495/cureus-0012-00000007010-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8af5/7077743/853b6ec398cc/cureus-0012-00000007010-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8af5/7077743/315c3c021c1a/cureus-0012-00000007010-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8af5/7077743/d712fbb84cdf/cureus-0012-00000007010-i05.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8af5/7077743/0796d4ef89ac/cureus-0012-00000007010-i06.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8af5/7077743/a6c0b9662b79/cureus-0012-00000007010-i07.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8af5/7077743/35b1bf755524/cureus-0012-00000007010-i08.jpg

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本文引用的文献

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