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

Kosuke Tsuchida1, Shinobu Fukumura2, Akiyo Yamamoto2

  • 1Department of Pediatrics, Sapporo Medical University School of Medicine, South-1, West-16, Chuo-ku, Sapporo, 060-8543, Japan. k.tsuchida@sapmed.ac.jp.

Child'S Nervous System : Chns : Official Journal of the International Society for Pediatric Neurosurgery
|May 7, 2018
PubMed

Insights

Idiopathic hypertrophic pachymeningitis (HP) is a rare brain condition. This case report details the first fatal pediatric HP, highlighting its rapid progression and poor response to treatment.

Area of Science:

  • Neurology
  • Pediatric Neurology
  • Neuroinflammation

Background:

  • Hypertrophic pachymeningitis (HP) is a rare dural thickening disorder.
  • It typically affects adults and can cause neurological deficits.
  • Idiopathic HP in children is exceptionally rare.

Observation:

  • A 3-year-old female presented with idiopathic HP.
  • The condition affected her entire central nervous system, including the brainstem.
  • Lesions showed poor response to pulsed steroids and immunosuppressants.

Findings:

  • The brainstem lesion rapidly progressed, forming nodules.
  • This led to brain death in the patient.
  • This represents the first documented fatal pediatric case of HP.

Implications:

  • This case underscores the aggressive potential of pediatric HP.
  • It highlights the need for further research into effective treatments.
  • Understanding pediatric HP is crucial for early diagnosis and management.
Abstract

Keywords:
Brain deathBrainstem lesionHypertrophic pachymeningitisPediatric

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