What is Moyamoya Disease?

What is Moyamoya Disease?

• Moyamoya Disease: Brain blood vessel disorder.
• Moyamoya Disease: A chronic cerebral vasculopathy primarily occurring in childhood and characterized by slowly progressive carotid artery narrowing and occlusion at the base of the brain. An extensive network of anastomoses forms between branches of the carotid arteries, resulting in a characteristic angiographic appearance. The condition may be idiopathic (classic moyamoya disease) or occur in association with ANEMIA, SICKLE CELL; DOWN SYNDROME; CRANIOCEREBRAL TRAUMA; RADIOTHERAPY induced arterial injury; NEUROFIBROMATOSIS; and other diseases. Clinical manifestations include hemiparesis, headache, seizures, and delayed mental development. In older individuals, this disease may present as SUBARACHNOID HEMORRHAGE. (From Adams et al., Principles of Neurology, 6th ed, p831; Clin Neurol Neurosurg 1997 Oct;99 Suppl 2:S36-8)
  Source - Diseases Database

Moyamoya Disease is listed as a "rare disease" by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH). This means that Moyamoya Disease, or a subtype of Moyamoya Disease, affects less than 200,000 people in the US population.
Source - National Institutes of Health (NIH)

Ophanet, a consortium of European partners, currently defines a condition rare when it affects 1 person per 2,000. They list Moyamoya Disease as a "rare disease".
Source - Orphanet

Moyamoya Disease: Introduction

Types of Moyamoya Disease:

Types of Moyamoya Disease:

• Familial moyamoya disease - a genetic form of the condition.
• more types...»

Broader types of Moyamoya Disease:

• Blood vessel conditions
• Brain conditions
• more types...»

How many people get Moyamoya Disease?

Prevalance of Moyamoya Disease: rare

Who gets Moyamoya Disease?

Patient Profile for Moyamoya Disease: Typically children to young adults, but older cases can occur.

Profile for Moyamoya Disease: It primarily affects children, adolescents, and young adults, although it has also been seen in people beyond these ages. Females are more frequently affected than males. (Source: excerpt from NINDS Moyamoya Disease Information Page: NINDS)

Gender Profile for Moyamoya Disease: Females more common.

How serious is Moyamoya Disease?

Prognosis of Moyamoya Disease: Poor. Progressive mental deterioration. Stroke and death.
Complications of Moyamoya Disease: see complications of Moyamoya Disease
Prognosis of Moyamoya Disease: Progressive deterioration of cognitive function is seen in the majority of individuals with moyamoya disease. Death usually results from intracerebral hemorrhage. (Source: excerpt from NINDS Moyamoya Disease Information Page: NINDS)

What causes Moyamoya Disease?

Causes of Moyamoya Disease: see causes of Moyamoya Disease
Causes of Moyamoya Disease: The cause of the disease is unknown. Researchers suspect a genetic link because of the 9 percent incidence of the disease found in certain Japanese families. A gene for familial moyamoya disease has been located on chromosome 17q25 and further study of the gene may reveal the cause of the disorder. (Source: excerpt from NINDS Moyamoya Disease Information Page: NINDS)

What are the symptoms of Moyamoya Disease?

Symptoms of Moyamoya Disease: see symptoms of Moyamoya Disease

Complications of Moyamoya Disease: see complications of Moyamoya Disease

Moyamoya Disease: Testing

Diagnostic testing: see tests for Moyamoya Disease.

Misdiagnosis: see misdiagnosis and Moyamoya Disease.

How is it treated?

Treatments for Moyamoya Disease: see treatments for Moyamoya Disease
Research for Moyamoya Disease: see research for Moyamoya Disease

Organs Affected by Moyamoya Disease:

Organs and body systems related to Moyamoya Disease include:

• Brain
• Blood vessels

Name and Aliases of Moyamoya Disease

Main name of condition: Moyamoya Disease

Source - Diseases Database

Spontaneous occlusion of the circle of Willis, Moyamoya disease 1, MYMY, MYMY1
Source - Office of Rare Diseases (ORD) of the National Institutes of Health (NIH)

Research the causes of these diseases that are similar to, or related to, Moyamoya Disease:

• Thrombosis
• Cerebral ischemia
• Transient ischemic attack
• Sensorimotor paralysis
• Atherosclerosis
• Circle of Willis

 » Next page: Prevalence and Incidence of Moyamoya Disease

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