Lesional perfusion abnormalities in Leigh disease demonstrated by arterial spin labeling correlate with disease activity

Matthew T. Whitehead, Bonmyong Lee, Andrea Gropman

Research output: Contribution to journalArticlepeer-review

Abstract

Background: Leigh disease is a metabolic disorder of the mitochondrial respiratory chain culminating in symmetrical necrotizing lesions in the deep gray nuclei or brainstem. Apart from classic gliotic/necrotic lesions, small-vessel proliferation is also characteristic on histopathology. We have observed lesional hyperperfusion on arterial spin-labeling (ASL) sequence in children with Leigh disease. Objective: In this cross-sectional analysis, we evaluated lesional ASL perfusion characteristics in children with Leigh syndrome. Materials and methods: We searched the imaging database from an academic children’s hospital for “arterial spin labeling, perfusion, necrosis, lactate, and Leigh” to build a cohort of children for retrospective analysis. We reviewed each child’s medical record to confirm a diagnosis of Leigh disease, excluding exams with artifact, technical limitations, and without ASL images. We evaluated the degree and extent of cerebral blood flow and relationship to brain lesions. Images were compared to normal exams from an aged-matche cohort. Results: The database search yielded 45 exams; 30 were excluded. We evaluated 15 exams from 8 children with Leigh disease and 15 age-matched normal exams. In general, Leigh brain perfusion ranged from hyperintense (n=10) to hypointense (n=5). Necrotic lesions appeared hypointense/hypoperfused. Active lesions with associated restricted diffusion demonstrated hyperperfusion. ASL perfusion patterns differed significantly from those on age-matched normal studies (P=

Original languageEnglish (US)
Pages (from-to)1-8
Number of pages8
JournalPediatric Radiology
DOIs
StateAccepted/In press - Apr 4 2016

Keywords

  • Arterial spin labeling
  • Children
  • Leigh disease
  • Magnetic resonance imaging
  • Metabolic disease
  • Perfusion

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Pediatrics, Perinatology, and Child Health

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