Moyamoya Disease

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?????? ?????? ?-MOYA MOYA
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Moyamoya Disease

• Moyamoya is a chronic cerebrovascular disease.
• It was first described in Japan in 1957.
• Severe bilateral stenosis or occlusion of the
  arteries around the circle of Willis with
  collateral circulation.
• The term describes the smoky angiographic
  appearance of the vascular network (cloud of
  smoke referred to an extensive basal cerebral
  rete mirabile).

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Etiology

• The etiology is unknown .
• High incidence among Japanese and Asian
  population.
• Genetic factors- familial , linkage to chromosome
  3p,6q,17q, 8q, several alleles of HLA , in
  families with Down S. , NFM and sickle cell
  anemia.
• High levels of fibroblast growth factor which may
  stimulate arterial growth.
• Transforming growth factor beta-1 which mediates
  neovascularization.
• Autoimmune disease with association to URI
  tonsillitis (no AB).

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Moyamoya Phenomenon
Few reports Changes characteristic of Moyamoya
arteries developed in adults within one year.
The angiographic findings of Moyamoya have been
demonstrated in patients with severe vasospasm
after subarachnoid hemorrhage , vasculitis in TB
meningitis and atherosclerotic D. This has led
some authors to propose the term Moyamoya
phenomenon as an entity separate from true
Moyamoya D. or Moyamoya like vasculopathy.
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Clinical features
Ischemic events more frequent in
children. Hemorrhagic stroke Epilepsy. In
children 77-ischemic events
59-TIA 5-ICH In adults
69-ICH 27-TIA ischemic
stroke Epilepsy 25- children , 5 -adults.

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Prognosis
The natural history tends to be progressive with
extensive intracranial large artery occlusion and
collateral circulation. The natural history may
be more benign in US compared to Asian
population. Moyamoya D. is one of the D.D. of
stroke in children and young adults.
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Treatment
Surgery is recommended in most patients .
Surgical treatment is intended to restore the
circulation for the ischemic brain area
. Perivascular and superior cervical
sympathectomy have been performed. A direct
vascular anastomosis , superficial temporal-MCA
bypass is used by many centers. Other procedures
are STA-ACA, arteriosynangiosis or
durasynangiosis (conecting a vascular muscle flap
or omentum containing the STA to the pial
surface of the frontal lobe for
neovascularization) . Surgery appears to be more
effective in children than in adults.