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Spontaneous rapid resolution of acute basal ganglia changes in an untreated infant with propionic acidemia: a clue to

A Broomfield1, R Gunny, P Prabhakar

  • 1Department of Metabolic Disease, Great Ormond Street Hospital with UCL Institute of Child Health, London, UK. BroomA@gosh.nhs.uk

Neuropediatrics
|March 30, 2011
PubMed

Insights

Acute decompensation in propionic acidemia can cause basal ganglia lesions. These lesions in an infant resolved rapidly within 3 weeks, prompting a discussion on their pathogenesis.

Area of Science:

  • Neurology
  • Metabolic Disorders
  • Pediatrics

Background:

  • Propionic acidemia is a rare inherited metabolic disorder.
  • Acute decompensation can lead to neurological complications, including basal ganglia lesions.

Observation:

  • A case of an 8-month-old infant with propionic acidemia is presented.
  • The infant exhibited basal ganglia lesions upon initial presentation.

Findings:

  • Radiological imaging showed near-complete resolution of basal ganglia lesions within 3 weeks.
  • Resolution occurred without specific metabolic management interventions.

Implications:

  • The rapid resolution suggests a potentially reversible mechanism for basal ganglia lesions in propionic acidemia.
  • Further research into the pathogenesis of these lesions is warranted.
  • This case highlights the dynamic nature of brain lesions in acute metabolic crises.

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