Suppr超能文献

希尔德弥漫性髓鞘破坏硬化症

Schilder's myelinoclastic diffuse sclerosis.

作者信息

Poser C M, Goutières F, Carpentier M A, Aicardi J

出版信息

Pediatrics. 1986 Jan;77(1):107-12.

PMID:3940347
Abstract

The term "Schilder's disease" has been used to describe conditions as disparate as adrenoleukodystrophy, myelinoclastic diffuse sclerosis, and postinfectious and postvaccinal encephalomyelitis. The eponymic designation should be reserved for instances of myelinoclastic diffuse sclerosis that correspond to the case described by Schilder in 1912. The diagnosis cannot be made unless adrenoleukodystrophy has been ruled out by analysis of the long-chain fatty acids of plasma cholesterol esters. Schilder's myelinoclastic diffuse sclerosis, a variant of multiple sclerosis, is a very rare disease that occurs in children and adults of both sexes and appears to respond to vigorous treatment with corticosteroids and/or corticotropin. A case of this disease is reported and the recent literature of cases that have been called Schilder's disease is reviewed.

摘要

术语“席尔德病”曾被用于描述多种不同的病症,如肾上腺脑白质营养不良、髓鞘破坏型弥漫性硬化症以及感染后和疫苗接种后脑脊髓炎。该名称应仅用于那些与席尔德在1912年所描述病例相符的髓鞘破坏型弥漫性硬化症实例。除非通过分析血浆胆固醇酯的长链脂肪酸已排除肾上腺脑白质营养不良,否则无法做出诊断。席尔德的髓鞘破坏型弥漫性硬化症是多发性硬化症的一种变体,是一种非常罕见的疾病,可发生于男女儿童及成人,似乎对皮质类固醇和/或促肾上腺皮质激素的积极治疗有反应。本文报告了一例该疾病,并对近期被称为席尔德病的病例文献进行了综述。

文献AI研究员

20分钟写一篇综述,助力文献阅读效率提升50倍。

立即体验

用中文搜PubMed

大模型驱动的PubMed中文搜索引擎

马上搜索

文档翻译

学术文献翻译模型,支持多种主流文档格式。

立即体验