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Published in: Child's Nervous System 9/2018

01-09-2018 | Case Report

Rapidly progressive fatal idiopathic hypertrophic pachymeningitis with brainstem involvement in a child

Authors: Kosuke Tsuchida, Shinobu Fukumura, Akiyo Yamamoto, Yukinori Akiyama, Hiroshi Hirano, Hiroyuki Tsutsumi

Published in: Child's Nervous System | Issue 9/2018

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Abstract

Background

Hypertrophic pachymeningitis (HP) is a rare disorder characterized by diffuse thickening of the dura mater with resultant neurologic deficits. HP develops secondary to various conditions or idiopathically usually in adults but rarely in children.

Case report

We describe a 3-year-old female child with idiopathic HP. Her HP involved the entire central nervous system with progression into the brainstem. The lesion responded poorly to pulsed steroids or any immunosuppressants. The brainstem lesion grew rapidly and formed various nodules that ultimately resulted in brain death. This is the first fatal case of HP in a child.
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Metadata
Title
Rapidly progressive fatal idiopathic hypertrophic pachymeningitis with brainstem involvement in a child
Authors
Kosuke Tsuchida
Shinobu Fukumura
Akiyo Yamamoto
Yukinori Akiyama
Hiroshi Hirano
Hiroyuki Tsutsumi
Publication date
01-09-2018
Publisher
Springer Berlin Heidelberg
Published in
Child's Nervous System / Issue 9/2018
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI
https://doi.org/10.1007/s00381-018-3819-5

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