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Child's Nervous System

, Volume 34, Issue 9, pp 1795–1798 | Cite as

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

  • Kosuke Tsuchida
  • Shinobu Fukumura
  • Akiyo Yamamoto
  • Yukinori Akiyama
  • Hiroshi Hirano
  • Hiroyuki Tsutsumi
Case Report

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.

Keywords

Hypertrophic pachymeningitis Brainstem lesion Brain death Pediatric 

Notes

Compliance with ethical standards

Conflict of interest

None.

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of PediatricsSapporo Medical University School of MedicineSapporoJapan
  2. 2.Department of NeurosurgerySapporo Medical University School of MedicineSapporoJapan
  3. 3.Department of Surgical PathologySapporo Medical University School of MedicineSapporoJapan

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