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一例罕见的麻风病脑内可能的空泡变性合并节段性坏死性肉芽肿性神经炎及霍纳综合征病例。

A Rare Case of Possible Vacuolar Degeneration of Leprosy in Brain with Segmental Necrotizing Granulomatous Neuritis and Horner's Syndrome.

作者信息

Baveja Sukriti, Sandhu Sunmeet, Vashisht Deepak

机构信息

Department of Dermatology, Armed Forces Medical College, Pune, Maharashtra, India.

出版信息

Indian Dermatol Online J. 2019 Jul-Aug;10(4):444-446. doi: 10.4103/idoj.IDOJ_388_18.

DOI:10.4103/idoj.IDOJ_388_18
PMID:31334067
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC6615378/
Abstract

Leprosy has a predilection for peripheral nerves, but rarely does it involve the central nervous system (CNS). There is a single study of CNS involvement in leprosy showing vacuolar changes of motor neurons in medulla oblongata and spinal cord in autopsy findings. Besides this, there has been only one case report providing direct histopathological and molecular evidence of CNS involvement by leprosy in a living patient. Segmental necrotizing granulomatous neuritis (SNGN) is a rare condition affecting the peripheral nerves in leprosy usually seen as a complication of tuberculoid (TT) and borderline tuberculoid (BT) leprosy. We report the case of a 23-year-old male patient, a case of Hansen's disease (BT) who developed CNS involvement in the form of partial Horner's syndrome (right) and SNGN while on treatment. Magnetic resonance imaging of the brain revealed T2 hyperintense lesion on the dorsal aspect of left pontomedullary junction, suggestive of vacuolar degeneration of leprosy. Histopathology of greater auricular nerve (right) revealed SNGN.

摘要

麻风病好发于周围神经,但很少累及中枢神经系统(CNS)。仅有一项关于麻风病中枢神经系统受累的研究,其尸检结果显示延髓和脊髓运动神经元有空泡样改变。除此之外,仅有一例病例报告提供了麻风病累及活体患者中枢神经系统的直接组织病理学和分子证据。节段性坏死性肉芽肿性神经炎(SNGN)是一种罕见的麻风病累及周围神经的疾病,通常见于结核样型(TT)和界线结核样型(BT)麻风病的并发症。我们报告一例23岁男性患者,该患者为汉森病(BT型),在治疗期间出现以部分霍纳综合征(右侧)和SNGN形式的中枢神经系统受累。脑部磁共振成像显示左脑桥延髓交界处背侧T2高信号病变,提示麻风病的空泡样变性。右侧耳大神经组织病理学显示为SNGN。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f469/6615378/439f648f6632/IDOJ-10-444-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f469/6615378/cbedb12c6c69/IDOJ-10-444-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f469/6615378/5949f2af8c91/IDOJ-10-444-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f469/6615378/439f648f6632/IDOJ-10-444-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f469/6615378/cbedb12c6c69/IDOJ-10-444-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f469/6615378/5949f2af8c91/IDOJ-10-444-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f469/6615378/439f648f6632/IDOJ-10-444-g003.jpg

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