Moyamoya Disease

Moyamoya disease is a cerebrovascular disease which, as it progresses, is characterized by a particular angiographic appearance of the cerebral collateral network. Moyamoya disease is also defined as spontaneous occlusion of the circle of Willis with extensive basal collateral vessels.

Early clinical symptoms of the disease, which may first occur in childhood or adulthood, consist of transient ischemic attacks and/or stroke and, less frequently, migraine-like headaches, seizures, or hemorrhage. All patients with true moyamoya disease show clinical or radiological evidence of hemorrhage or ischemia and infarction.

Surgical treatment (encephalo-duro-arterio-synangiosis: EDAS) is effective for prevention of recurrent ischemic attacks. The effectiveness of indirect revascularization for hemorrhagic moyamoya disease is less clear.

Airway clearance complications of moyamoya disease occur secondary to neurological sequelae of the disease and mainly as a result of aspiration of gastric contents and/or oral secretions. The introduction of bacteria from aspirated saliva and gastric contents into normally sterile airways can result in serious, potentially fatal episodes of pneumonia. At particular risk for aspiration are patients who experience:

  • Dysphagia, or inadequate control of the swallowing muscles
  • Gastroesophageal reflux
  • Seizures resulting in dyscoordinated swallowing and breathing

Patients who are immobilized by stroke or other neurological manifestations of moyamoya disease cannot use exercise to maintain aerobic capacity, bellows function, and lung volume. They are at risk for developing restrictive lung disease and consequent impaired airway clearance.

For those patients with pulmonary manifestations of moyamoya disease, daily Airway Clearance Therapy can be a vital component in disease management.

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Sources

Manceau E, Giroud M, Dumas R. Moyamoya Disease in Children: A Review of the Clinical and Radiologic Features and Current Treatment. Child’s Nerv Syst 1997; 13: 595-600. Choi JU, Kim DS, Kim EY, Lee KC. Natural History of Moyamoya disease: Comparison of Activity of Daily Living in Surgery and Non-Surgery Groups. Clin Neurol Neurosurg 1997 Sept; 99 Suppl 2:S11-8. Peerless SJ. Risk Factors of Moyamoya Disease in Canada and the USA. Clin Neurol Neurosurg 1997 Oct: 99 Suppl 2: S45-8.

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