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Adult onset moyamoya disease: institutional experience

Swati Dayanand Chinchure1, Hima Shriniwas Pendharkar, Arun Kumar Gupta

  • 1Department of Imaging Sciences and Interventional Radiology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, India.

Neurology India
|October 25, 2011
PubMed

Insights

Adult-onset moyamoya disease, a rare condition affecting brain arteries, can cause serious bleeding or stroke. This study highlights its importance in diagnosing adult intracranial hemorrhage.

Area of Science:

  • Neurology
  • Vascular Neurology
  • Neuroimaging

Background:

  • Moyamoya disease is a rare, progressive steno-occlusive condition affecting the major arteries at the base of the brain.
  • It is characterized by the formation of a delicate collateral vascular network, diagnosed via angiography.
  • Adult-onset moyamoya disease presents unique diagnostic challenges.

Observation:

  • This study analyzed 11 patients diagnosed with adult-onset moyamoya disease.
  • The primary clinical presentations were intracranial hemorrhage (6 patients) and focal ischemia (5 patients).
  • Angiographic findings included bilateral internal carotid artery involvement (8 patients) and unilateral involvement (3 patients), with posterior cerebral artery involvement in 3 patients.

Findings:

  • Adult-onset moyamoya disease can manifest as severe neurological events like intracerebral and intraventricular hemorrhage.
  • The disease predominantly affects the internal carotid arteries, but posterior circulation can also be involved.
  • Aneurysms are an uncommon but possible associated finding.

Implications:

  • Adult-onset moyamoya disease should be considered in the differential diagnosis of spontaneous intracranial hemorrhage in adults.
  • Early recognition and diagnosis are crucial for appropriate management and to prevent further neurological damage.
  • Further research into the specific mechanisms and optimal treatment strategies for adult-onset moyamoya disease is warranted.

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