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Related Experiment Video

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Distinct Clinical and Radiographic Phenotypes in Pediatric Patients With Moyamoya.

Matsanga Leyila Kaseka1, Mahmoud Slim2, Prakash Muthusami3

  • 1Division of Neurology, Department of Pediatrics, Hospital for Sick Children, Toronto, Ontario, Canada.

Pediatric Neurology
|May 7, 2021
PubMed
Summary

Moyamoya disease (MMD) and moyamoya syndrome (MMS) present differently in children. MMD often involves frequent transient ischemic attacks (TIAs), while MMS subtypes have distinct features, influencing recurrence risk and outcomes.

Keywords:
Childhood moyamoyaNeurocognitive outcomeNeurofibromatosis type 1Outcome measurePediatric strokeSickle cell disease

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Area of Science:

  • Neurology
  • Pediatric Neurology
  • Vascular Neurology

Background:

  • Moyamoya disease (MMD) and moyamoya syndrome (MMS) are distinct cerebrovascular conditions.
  • Evidence suggests clinico-radiological differences between MMD and MMS.
  • Understanding these differences is crucial for managing pediatric cases.

Purpose of the Study:

  • To compare clinical and radiographic features of childhood MMD and MMS.
  • To identify predictors of ischemic event recurrence in pediatric moyamoya patients.
  • To differentiate phenotypes based on underlying etiologies.

Main Methods:

  • Retrospective review of a pediatric moyamoya cohort (2003-2019).
  • Abstraction of clinical and radiographic characteristics at diagnosis and follow-up.
  • Comparative analysis between MMD, MMS, MMS-NF1, and MMS-SCD.

Main Results:

  • MMD patients commonly presented with transient ischemic attacks (TIAs).
  • Symptomatic stroke presentation and bilateral disease were less common in MMS-NF1.
  • TIA recurrence was frequent in MMD; absence of ivy sign was noted in MMS-SCD.

Conclusions:

  • Moyamoya exhibits distinct phenotypes based on etiology.
  • MMD is associated with frequent TIAs; MMS-SCD with symptomatic stroke.
  • MMS-NF1 is characterized by unilateral disease and low infarct burden.