moyamoya disease

(redirected from Moyamoya)

moy·a·moy·a dis·ease

[MIM*252350] Because moyamoya is not a proper name, it is spelled with a lowercase m.
A cerebrovascular disorder occurring predominantly in the Japanese, in which the vessels of the base of the brain become occluded and revascularized with a fine network of vessels; it occurs commonly in young children and is manifested by convulsions, hemiplegia, mental retardation, and subarachnoid hemorrhage; the diagnosis is made by the angiographic picture.
[Jap. addlebrained]

moyamoya disease

An idiopathic disease pattern characterised by bilateral stenosis of the internal carotid arteries, accompanied by a network of abnormal collateral vessels that bypass the stenosis. It is named for the “smoky” (in Japanese, moya-moya) angiographic appearance of prominent collateral vessels of the basal ganglia that accompany narrowed and distorted cerebral arteries with thin collateral vessels, which arise from the circle of Willis, with progressive occlusion.
 
Clinical findings
Moyamoya disease is more common in females who are presenting as TIAs. Initially described in the Japanese and characterised by a familial tendency, it is also well described in Caucasians. It may follow a febrile illness and present with an abrupt onset of hemiparesis, transient aphasia and convulsions,

DiffDx, “puff of smoke” pattern
Occlusive intracranial atherosclerosis, radiation arteritis, intravascular tumour proliferation, tuberculous meningitis.
 
Management
Revascularisation using the parietal branch of the superficial temporal artery.
References in periodicals archive ?
The couple first thought Riley was suffering from epilepsy, however they later found out he was suffering from three separate conditions - rare disease moyamoya, a brain tumour and gelastic seizures, which cause giggling fits.
The mechanisms of brain injury include traumatic brain injury (n = 23), spontaneous cerebral hemorrhage (n = 23), Moyamoya disease (n = 2), vascular malformation (n = 1), and tumor (n = 1).
Doctors are working to determine if he suffers from Moyamoya disease, a rare, progressive cerebrovascular disorder that would require specialized intervention, she said.
To the Editor: Clinical symptom spectrum of paroxysmal nocturnal hemoglobinuria (PNH) is consisted of chronic intravascular hemolysis, hemoglobinuria, relative bone marrow failure, and the rarity of thrombosis.[1] The neurological complications generated by PNH were almost exclusively a result of cerebral venous thrombosis.[1],[2],[3] However, moyamoya syndrome (MMS) secondary to PNH (PNH-MMS) is rarely described in the literature.[2],[3],[4],[5] We report herein a case of PNH-MMS to raise the awareness of this disease in PNH patients presenting with acute neurological deficits.
"They discovered he had a rare disease called Moyamoya. He's doing great now.
Moyamoya disease associated with Graves disease: special considerations regarding clinical significance and management.
Amman, Apr 17 (Petra) A neurosurgery team at the Al Hussein Medical Centre have successfully conducted the first operation to treat a seven-year-old child suffering from Moyamoya disease.
A battery of tests found she had moyamoya disease, a rare condition first identified in Japan.
Vessels of such small diameter may be used for the treatment of moyamoya disease in children.
This phenomenon not only involves the coronary arteries but also reported cerebrovascular occlusive disease (Moyamoya phenomenon) in uncontrolled Graves' disease that may share the same mechanism for acute vasospasm of the cerebral arteries [2].
Moyamoya is a rare progressive cerebrovascular disease that presents as a hemorrhagic or ischemic event in adults.