Moyamoya disease (MMD) was reported by first time in 1957 by Takeuchi and Shimizu as hypoplasia of the bilateral internal carotid arteries. The official Japanese name for the disease is Spontaneous occlusion of the circle of Willis, but in 1967 Suzuki and Takaku nicknamed it Moyamoya disease. In Japan, the estimated total number of the patients with this disease was 3900 in 1995 and the annual incidence is 0.35 per 100,000 populations. The incidence of the disease is high among Japanese and Koreans and far lower in Caucasians. On the other hand, in Western countries there is not reliable epidemiological data about it. The male and female ratio was 1:1.7 showing a slight female predominance. Solitary cases are much more frequent but familial cases were reported approximately in 10% with 13 cases of monovular twins. Moyamoya disease may cause cerebral ischemia (transient ischemic attacks and infarction) and haemorrhage (intracerebral, intraventricular and subarachnoid). Symptoms and signs in children are mainly TIA (hemiparesis, monoparesis and sensory disturbance), which occur repeatedly and occasionally. Headache, involuntary movements is and convulsive seizures may occur. Despite extensive studies over a long period, the aetiology of Moyamoya disease is still no clear. Because of the higher incidence of this disease in Orientals, very low incidence in Caucasians, and the presence of familial cases, multifactorial inheritance is considered to be causative. The treatments for Moyamoya disease are both medical and surgical. There are no medical treatments to prevent the progression of the illness. Direct bypass surgery (superficial temporal artery to middle cerebral artery, STA-MCA anastomosis) or indirect bypass surgery (placing the vascularized soft tissue flap on the surface of brain) can improve the decreased cerebral circulation and clinical signs. Indirect bypass surgery is preferred for young children. Within a few months after bypass surgery ischemic attacks disappear in most patients.
|Number of pages||7|
|Journal||Archivos de Neurociencias|
|Publication status||Published - 2009 Jan 1|
- Carotid arteries
ASJC Scopus subject areas
- Clinical Neurology