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[Moyamoya disease].

P G Peña-Tapia1, P Horn, P Schmiedek

  • 1Servico de Neurocirugía. Hospital Universitario Mannheim. Universidad de Heidelberg. Mannheim, Alemania. pablo.pena@nch.ma

Revista De Neurologia
|August 31, 2006
PubMed
Summary
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Moyamoya disease, a rare cerebrovascular condition, involves progressive blockage of the circle of Willis. Early diagnosis and neurosurgical revascularisation offer a cure, preventing severe neurological deficits.

Area of Science:

  • Neurology
  • Vascular Neurology
  • Neuroscience

Context:

  • Moyamoya disease is a rare, progressive cerebrovascular disorder.
  • It affects both children and adults globally, with unknown frequency.
  • Characterized by occlusion of the circle of Willis and development of collateral vessels.

Purpose:

  • To detail clinical manifestations of Moyamoya disease.
  • To synthesize current knowledge on its epidemiology, pathophysiology, histopathology, diagnosis, and treatment.

Summary:

  • The primary lesion involves thickening of the internal carotid artery's tunica interna, leading to reduced cerebral blood flow.
  • Clinical presentations include ischemic episodes and intracerebral hemorrhages.
  • Diagnosis relies on imaging like cerebral pan-angiography and xenon-CT.

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Impact:

  • Neurosurgical revascularization is the accepted treatment, offering potential for a permanent cure.
  • Timely diagnosis and intervention can prevent recurrent ischemic events and hemorrhages.
  • Successful treatment mitigates severe limitations on individual development.