alexa Moyamoya disease | Sweden | PDF | PPT| Case Reports | Symptoms | Treatment

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Moyamoya Disease

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  • Moyamoya disease

    Pathophysiology:
    Moyamoya disease is a rare, progressive cerebrovascular disorder caused by blocked arteries at the base of the brain in an area called the basal ganglia. The name “moyamoya” means “puff of smoke” in Japanese and describes the look of the tangle of tiny vessels formed to compensate for the blockage. It primarily affects children, but it can also occur in adults. In children, the first symptom of Moyamoya disease is often stroke, or recurrent transient ischemic attacks.

  • Moyamoya disease

    Statistics:
    On identification of 51 children (23 boys and 28 girls; median age 13) the average annual incidence of disese was 1.8 per 100000 children. It was found in 51% of the children, HS in 41% and cerebral sinus venous stroke in 8%. 1/3rd of the children had underlying diseases, and one-third had vascular malformations.Two children died in the acute stage (4%), and 40/49 (82%) had some neurological dysfunction at discharge.

  • Moyamoya disease

    Treatment:
    There are several types of revascularization surgery that can restore blood flow to the brain by opening narrowed blood vessels or by bypassing blocked arteries. Children usually respond better to revascularization surgery than adults, but the majority of individuals have no further strokes or related problems after surgery.

  • Moyamoya disease

    Major Research:
    Recent investigations have established that both moyamoya disease and arteriovenous fistulas (AVFs) of the lining of the brain, the dura, are associated with dural angiogenesis. These factors may represent a mechanism for ischemia contributing to the formation of dural AVFs. At least one case of simultaneous unilateral moyamoya disease and ipsilateral dural arteriovenous fistula has been reported at the Barrow Neurological Institute.

  • Moyamoya disease

    Major Research:
    Recent investigations have established that both moyamoya disease and arteriovenous fistulas (AVFs) of the lining of the brain, the dura, are associated with dural angiogenesis. These factors may represent a mechanism for ischemia contributing to the formation of dural AVFs. At least one case of simultaneous unilateral moyamoya disease and ipsilateral dural arteriovenous fistula has been reported at the Barrow Neurological Institute.

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