moyamoya disease

(redirected from Moyamoya syndrome)

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 ?
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.
Individuals with DS can get neurological complications from strokes, autoimmune disorders, moyamoya syndrome, endocrine dysfunction, epilepsy, or cervical cord compression, but these were excluded in our patient by appropriate testing.
Evaluation leads to the eventual diagnosis of stroke secondary to Moyamoya syndrome. This is an unusual presentation of stroke and highlights the need to expand the differential diagnosis to include rare diseases in children with predisposing conditions.
Although rare, other reported features are moyamoya syndrome, osteosarcoma, and polyarteritis nodosa [26].
Long-term outcome in children with moyamoya syndrome after cranial revascularization by pial synangiosis.
Anesthetic management of children with moyamoya syndrome. Anesth Analg 1993; 77 (5): 1066-70.
Postinfectious vasculopathy with evolution to moyamoya syndrome. J Neurol Neurosurg Psychiatry.
Keywords: Leigh disease, Moyamoya syndrome, Childhood, MRI.
Moyamoya syndrome and Graves' disease in a young woman
Moyamoya syndrome is a cerebral arteriopathy characterised by bilateral progressive stenosis of the terminal portions of the ICAs.
Smoke in the Brain, Moyamoya Syndrome in Sickle Cell Anemia: Case Report
Also moyamoya syndrome, a disease in which certain arteries in the brain are constricted, may accompany morning glory disc anomaly.