Moyamoya disease

Common Name(s)

Moyamoya disease

Moyamoya disease is a rare, progressive, blood vessel disease caused by blocked arteries at the base of the brain in an area called the basal ganglia. The name "moyamoya" means "puff of smoke" in Japanese and describes the look of the tangled vessels that form to compensate for the blockage. This condition usually affects children, but can affect adults. Affected people are at increased risk for blood clots, strokes, and transient ischemic attacks (TIAs) which are frequently accompanied by seizures and muscular weakness, or paralysis on one side of the body. Affected people may also have disturbed consciousness, speech deficits (usually aphasia), sensory and cognitive impairments, involuntary movements, and vision problems. Researchers believe that Moyamoya disease is an inherited condition because it tends to run in families.

Moyamoya syndrome is a related term that refers to cases of moyamoya disease that occur in association with other conditions or risk factors, such as neurofibromatosis, tuberculosis meningitis, sickle cell disease, leptospirosis, brain tumors, Sturge-Weber syndrome, and tuberous sclerosis.

 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Moyamoya disease" for support, advocacy or research.

There are currently no organizations listed in Disease InfoSearch that support this condition. Create a listing.

 

 

General Support Organizations

Not finding the support you need? Show General Support Organizations

 
 
Top

How do you compare to others with this condition?

Privately answer questions about your health. Let resources, you select, come to you.

Anonymously share and see how your answers compare with others with this condition while privately providing key pieces of information to medical researchers, disease advocacy groups, and others ONLY YOU select to help speed up cures and better alternatives.

 
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Moyamoya disease" for support, advocacy or research.

There are currently no organizations listed in Disease InfoSearch that support this condition. Create a listing.

 

 

General Support Organizations

Not finding the support you need? Show General Support Organizations

 
 
 
 
Top

Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Moyamoya disease" returned 325 free, full-text research articles on human participants. First 3 results:

Ivy Sign on Fluid-Attenuated Inversion Recovery Images in Moyamoya Disease: Correlation with Clinical Severity and Old Brain Lesions.
 

Author(s): Kwon-Duk Seo, Sang Hyun Suh, Yong Bae Kim, Ji Hwa Kim, Sung Jun Ahn, Dong-Seok Kim, Kyung-Yul Lee

Journal: Yonsei Med. J.. 2015 Sep;56(5):1322-7.

 

Leptomeningeal collateral, in moyamoya disease (MMD), appears as an ivy sign on fluid-attenuated inversion-recovery (FLAIR) images. There has been little investigation into the relationship between presentation of ivy signs and old brain lesions. We aimed to evaluate clinical significance ...

Last Updated: 10 Aug 2015

Go To URL
Cortical Microvascularization and Leptomeningeal Collaterals in Moyamoya Disease.
 

Author(s): Shunji Mugikura, Miki Fujimura, Shoki Takahashi

Journal: Eur. Neurol.. 2015 ;73(5-6):351-2.

 

Last Updated: 19 Jun 2015

Go To URL
Systematic Validation of RNF213 Coding Variants in Japanese Patients With Moyamoya Disease.
 

Author(s): Yosuke Moteki, Hideaki Onda, Hidetoshi Kasuya, Taku Yoneyama, Yoshikazu Okada, Kengo Hirota, Maki Mukawa, Tadashi Nariai, Shohei Mitani, Hiroyuki Akagawa

Journal:

 

A founder variant of RNF213, p.R4810K (c.14429G>A, rs112735431), was recently identified as a major genetic risk factor for moyamoya disease (MMD) in Japan. Although the association of p.R4810K was reported to be highly significant and reproducible, the disease susceptibility of other ...

Last Updated: 12 May 2015

Go To URL

Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Moyamoya disease" returned 25 free, full-text review articles on human participants. First 3 results:

Current status of revascularization surgery for Moyamoya disease: special consideration for its 'internal carotid-external carotid (IC-EC) conversion' as the physiological reorganization system.
 

Author(s): Miki Fujimura, Teiji Tominaga

Journal: Tohoku J. Exp. Med.. 2015 ;236(1):45-53.

 

Moyamoya disease is a chronic cerebrovascular disease with unknown etiology, which is characterized by bilateral steno-occlusive changes at the terminal portion of the internal carotid artery and an abnormal vascular network formation at the base of the brain. Moyamoya disease is ...

Last Updated: 14 May 2015

Go To URL
Treatment strategies for aneurysms associated with moyamoya disease.
 

Author(s): Lei Zhang, Kan Xu, Yandong Zhang, Xin Wang, Jinlu Yu

Journal:

 

The treatment of aneurysms associated with moyamoya disease (MMD) is difficult for neurosurgeons, and little is known of strategy options. This report constitutes a comprehensive review of the literature. We summarize the known treatments and their clinical outcomes according to the ...

Last Updated: 14 Feb 2015

Go To URL
Management of distal choroidal artery aneurysms in patients with moyamoya disease: report of three cases and review of the literature.
 

Author(s): Kangmin He, Wei Zhu, Liang Chen, Ying Mao

Journal:

 

Prevention of rebleeding plays an important role in the treatment of hemorrhagic moyamoya disease, because rebleeding results in high mortality and morbidity. We discuss possible treatment for patients with moyamoya disease accompanied with distal choroidal artery aneurysms and review ...

Last Updated: 26 Aug 2013

Go To URL
 
 
Top

Symptoms, Diagnosis, and Treatment

There are currently no related results available in GeneReviews.

 
 
Top

Clinical Trial Information This information is provided by ClinicalTrials.gov

Moyamoya Disease Biomarkers in Patients With Intracranial Atherosclerotic Stroke
 

Status: Recruiting

Condition Summary: Moyamoya Disease; Intracranial Atherosclerotic Stroke; Intracranial Steno-occlusive Disease

 

Last Updated: 26 Feb 2014

Go to URL
Neurocognitive and Radiological Assessments in Adult Moyamoya Undergoing Surgery
 

Status: Not yet recruiting

Condition Summary: Moyamoya Disease

 

Last Updated: 10 Jul 2015

Go to URL
Sevoflurane and Hyperperfusion Syndrome
 

Status: Not yet recruiting

Condition Summary: Hyperperfusion Syndrome; Moyamoya Disease

 

Last Updated: 26 Jul 2015

Go to URL