Acute Magnetic Resonance Imaging Findings in Young Children With Moyamoya Disease

Shingo Numoto1, Syuntaro Takasu2, Tomomi Nakamura3

  • 1Department of Pediatrics, Aichi Medical University, Nagakute, Aichi, Japan; Department of Child Neurology, Seirei Hamamatsu General Hospital, Hamamatsu, Japan.

Pediatric Neurology
|May 15, 2024
PubMed

Insights

Diffusion-weighted imaging (DWI) in young children with moyamoya disease (MMD) shows characteristic cortical lesions, often gyral or atypical territorial, differing from typical strokes. These findings are crucial for diagnosing MMD in this age group.

Area of Science:

  • Pediatric Neurology
  • Neuroradiology
  • Neuroimaging

Background:

  • Moyamoya disease (MMD) is a rare cerebrovascular disorder.
  • Understanding acute diffusion-weighted imaging (DWI) findings in young children with MMD is essential for early diagnosis and management.
  • Previous studies have not fully characterized DWI findings in the acute phase of MMD in pre-school aged children.

Purpose of the Study:

  • To describe the diffusion-weighted imaging (DWI) findings in young children (under six years) with moyamoya disease (MMD) during the acute symptomatic period.
  • To correlate DWI findings with the severity of arterial stenosis in this pediatric population.

Main Methods:

  • Retrospective analysis of clinical data from 12 children under six years old with MMD.
  • DWI scans obtained within one week of MMD symptom onset were reviewed.
  • DWI abnormalities were classified into patterns: gyral, atypical territorial, honeycomb, classical territorial, multiple-dot, border zone, and deep lacunar.
  • Arterial stenosis severity was graded using established angiographic stages.

Main Results:

  • DWI abnormalities were predominantly located in the cerebral cortex in 11 out of 12 children.
  • Gyral lesions were observed in seven children, and atypical territorial lesions in five, distinct from typical arterial stroke patterns.
  • All 12 children had internal carotid artery stenosis, mostly mild (11 children).
  • The severity of arterial stenosis did not consistently correlate with the location or pattern of ischemic lesions.

Conclusions:

  • Acute DWI findings in young children with MMD are typically confined to the cerebral cortex.
  • Lesions are often gyral or atypical territorial, differentiating them from classic arterial territory infarcts.
  • These characteristic cortical lesions on DWI are a key feature of MMD in very young children.
Abstract