Rare Spontaneous Attenuation of Childhood Inflammatory Cerebral Adrenoleukodystrophy

Hyoung Won Choi, Gerald Vincent Raymond, Weston Miller

Research output: Contribution to journalArticle

Abstract

X-linked adrenoleukodystrophy (ALD) is a neurodegenerative peroxisomal disorder with variable clinical phenotypes. Childhood cerebral ALD (CCALD) is at the most severe end of the disease spectrum. In CCALD, the clinical manifestations include increasing deficits in behavior, vision, hearing, coordination, and motor function, as well as seizures. Without treatment, CCALD often results in apparent vegetative state within 1 to 2 years of appearance of initial signs and symptoms. We present the case of a boy with classic inflammatory CCALD who exhibited spontaneous attenuation in disease progression. While extremely rare, spontaneous arrest of disease progression may occur in boys with inflammatory CCALD.

Original languageEnglish (US)
Pages (from-to)106-109
Number of pages4
JournalJournal of Pediatric Neurology
Volume18
Issue number2
DOIs
StatePublished - Apr 1 2020

Keywords

  • cerebral adrenoleukodystrophy
  • inflammatory
  • spontaneous arrest

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

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