Treatment of Moyamoya Disease




Essentially, moyamoya disease is progressive disease with unknown etiology. Thus, there is no cure and the treatment for moyamoya disease is directed to palliative and supportive care.


Treatment in the acute stage


Treatment for the patients with moyamoya disease in the acute stage is same for the stroke patients, either brain hemorrhage or ischemia, with other etiologies. Adult patients with brain hemorrhage might be treated with drainage of the bloody cerebrospinal fluid from the ventricular system or with surgical removal of the hematoma by craniotomy.


Treatment in the chronic stage


Ischemic cases


There is no consensus on the medical treatment with aspirin, other anti-platelet drugs, anticoagulants, or vasodilator to prevent the future ischemic attacks. Thus, some institutes in Japan, aspirin or ticlopidine (anti-platelet drug) is prescribed to the moyamoya patients in the selected situation, but in the other institutions, like us, do not give them to the moyamoya patients.

Aspirin and its adverse effects

In order to eliminate ischemic symptoms, bypass surgery is accepted as the treatment of choice. Vascular anastomosis is classified as direct or indirect one. In direct anastomosis, the superficial temporal artery in the scalp is anastomosed with the middle cerebral artery on the brain surface under a microscope. In indirect anastomosis, the dura mater or fascia of the temporal muscle is placed over the brain surface, expecting the development of the spontaneous anastomosis. In direct anastomosis, indirect one is usually combined. In the rare situation, multiple burr hole surgery is performed, in which multiple small holes are made on the skull bone, expecting the development of spontaneous anastomosis. Another rare surgery is omental transplantation.


Further details on surgical anastomosis

Perioperative management for pediatric moyamoya patients

Many Indirect Anastomoses for Moyamoya

Surgical treatment

Hemorrhagic cases

Contrary to the treatment for ischemic cases, bypass surgery does not necessarily reduce the future hemorrhagic complication. At present, the efficacy of bypass surgery for hemorrhagic cases are under investigation in Japan.

Japan Adult Moyamoya (JAM) trial for the hemorrhagic adult moyamoya patients


Incidental or asymptomatic cases


Familial incidence of moyamoya disease is about 10%. Screening examination using MRI is frequently performed. Incidentally moyamoya disease is diagnosed in the MR examination performed for the other purpose. It does not follow that these incidental or asymptomatic disease will become symptomatic in the future. Thus, there is no consensus in the preventive treatment for these patients.


Rehabilitation


According to the neurological impairment, rehabilitation with physical therapy, occupational therapy, and speech therapy should be considered. The extent of rehabilitation depends upon the degrees of the patient's impairment.


Pregnancy and delivery of the female patients with moyamoya disease