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Published on: February 23, 2016

Clinical features of adult moyamoya disease with special reference to the diagnosis

Jeong Eun Kim1, Kang Min Kim, Jeong Gyun Kim

  • 1Department of Neurosurgery, Seoul National University College of Medicine, Seoul, Republic of Korea.

Neurologia Medico-Chirurgica
|June 13, 2012
PubMed

Insights

Adult moyamoya disease (MMD) is a distinct entity, not a syndrome. Studies show progression from unilateral to bilateral MMD, necessitating different diagnostic and treatment strategies compared to pediatric cases.

Area of Science:

  • Neurology
  • Vascular Neurology
  • Neuroimaging

Background:

  • Moyamoya disease (MMD) is a rare cerebrovascular disorder.
  • Distinguishing adult MMD from other conditions with similar presentations is crucial for accurate diagnosis and management.

Purpose of the Study:

  • To analyze clinical features of adult MMD over 15 years.
  • To establish adult MMD as a distinct disease entity.
  • To evaluate diagnostic criteria and disease progression.

Main Methods:

  • Retrospective analysis of 348 adult MMD patients (1996-2010).
  • Comparison of laboratory data between adult MMD and atherosclerotic disease (ASD) patients.
  • Angiographic assessment of disease progression in unilateral MMD.

Main Results:

  • Adult MMD patients rarely present with childhood symptoms (0.8% over 40).
  • Laboratory tests did not differentiate adult MMD from ASD.
  • 11.5% of unilateral MMD cases progressed to bilateral MMD.

Conclusions:

  • Adult MMD is a distinct disease entity, not a syndrome.
  • Significant progression from unilateral to bilateral MMD occurs.
  • Diagnostic and therapeutic approaches for adult MMD should differ from pediatric cases.

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