Guidelines for the diagnosis and treatment of spontaneous occlusion of the circle of Willis (`Moyamoya' disease)1

https://doi.org/10.1016/S0303-8467(97)00082-6Get rights and content

Section snippets

Diagnostic criteria

(A) Cerebral angiography is indispensable for the diagnosis, and should present at least the following findings:

  • 1.

    Stenosis or occlusion at the terminal portion of the internal carotid artery and/or at the proximal portion of the anterior and/or the middle cerebral arteries.

  • 2.

    Abnormal vascular networks in the vicinity of the occlusive or stenotic lesions in the arterial phase.

  • 3.

    These findings should present bilaterally.

(B) When magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA)

Concept

Spontaneous occlusion of the circle of Willis is a cerebrovascular disorder, which is frequently seen in Japanese, with an unknown etiology. Because the Japanese term `Moyamoya' means puffy, hazy, obscure or vague and the abnormal vascular network at the base of the brain gives a `Moyamoya' appearance on the angiogram, the term `Moyamoya disease' has also been used. The onset age distribution pattern has two peaks with a higher peak at 5 years of age and a lower peak from 30 to 49. The former

Treatment and limitations at primary medical institutions

Because the cause of this disease is unknown, there is still no known curative treatment. Most treatments are symptomatic in the acute phase as well as for sequelae and protection against a recurrent attack. Especially the occurrence of cerebral stoke in children strongly suggests the presence of this disease.

In the acute phase of ischemia and hemorrhagic attack, symptomatic treatment is necessary to improve the cerebrovascular function by reducing the intracranial pressure, by controlling

Guidelines for making a diagnosis using MRI and MRA

(A) When magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) clearly demonstrate all the findings described below, conventional cerebral angiography is not mandatory:

  • 1.

    Stenosis or occlusion at the terminal portion of the internal carotid artery and at the proximal portion of the anterior and middle cerebral arteries.

  • 2.

    An abnormal vascular network in the basal ganglia.

  • 3.

    (1) and (2) are seen bilaterally.

(B) Imaging methods and judgment

  • 1.

    More than a 1.0 tesla magnetic field strength is

First page preview

First page preview
Click to open first page preview

References (0)

Cited by (0)

1

Modified and established in 1996 by the Research Committee on Spontaneous Occlusion of the Circle of Willis (Moyamoya Disease) of the Ministry of Health and Welfare, Japan.

View full text