Biomaterial Database

[Moyamoya disease associated with thrombotic thrombocytopenic purpura (TTP)]. 1994

H Hiyama, and R Kusano, and Y Muragaki, and N Miura

Department of Neurosurgery, Itabashi Central General Hospital.

A case of moyamoya disease associated with thrombotic thrombocytopenic purpura (TTP) was reported. A 26-year-old male patient was admitted on April 11, 1992, with sudden onset of right cerebral hemorrhage. Cerebral angiography revealed moyamoya disease and bilateral encephalo-duro-arterio-synangiosis (EDAS) was performed. In March, 1993, however, he suffered from left cerebral hemorrhage. Neurological examination on the second admission showed disturbance of consciousness, motor aphasia and right hemiplegia. Emergency operation for the hematoma removal was performed and neurological functions rapidly improved. However, on the day following the operation, he was in stupor and restlessness. Microangiopathic hemolytic anemia and severe thrombocytopenia were identified and he gradually sank into a comatose state. Systemic purpura, fever, renal dysfunction also appeared. CT scan 22 days after the onset demonstrated diffuse cerebral infarction in the region of the bilateral anterior and middle cerebral arteries, and cerebral angiography on the next day demonstrated the development of bilateral internal carotid stenosis. Though laboratory findings indicate gradual improvement, he has remained in very weak state. This is the first case of moyamoya disease associated with TTP. The etiology of both diseases was discussed.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D009072	Moyamoya Disease	A noninflammatory, progressive occlusion of the intracranial CAROTID ARTERIES and the formation of netlike collateral arteries arising from the CIRCLE OF WILLIS. Cerebral angiogram shows the puff-of-smoke (moyamoya) collaterals at the base of the brain. It is characterized by endothelial HYPERPLASIA and FIBROSIS with thickening of arterial walls. This disease primarily affects children but can also occur in adults.	Cerebrovascular Moyamoya Disease,Progressive Intracranial Occlusive Arteropathy (Moyamoya),Moya-Moya Disease,Moyamoya Disease, Classic,Moyamoya Disease, Primary,Moyamoya Disease, Secondary,Moyamoya Syndrome,Classic Moyamoya Disease,Disease, Classic Moyamoya,Disease, Moya-Moya,Disease, Primary Moyamoya,Moya Moya Disease,Moyamoya Diseases, Primary,Primary Moyamoya Disease,Primary Moyamoya Diseases,Secondary Moyamoya Disease
D011697	Purpura, Thrombotic Thrombocytopenic	An acquired, congenital, or familial disorder caused by PLATELET AGGREGATION with THROMBOSIS in terminal arterioles and capillaries. Clinical features include THROMBOCYTOPENIA; HEMOLYTIC ANEMIA; AZOTEMIA; FEVER; and thrombotic microangiopathy. The classical form also includes neurological symptoms and end-organ damage, such as RENAL FAILURE. Mutations in the ADAMTS13 PROTEIN gene have been identified in familial cases.	Moschkowitz Disease,Purpura, Thrombotic Thrombopenic,Thrombotic Thrombocytopenic Purpura, Congenital,Thrombotic Thrombocytopenic Purpura, Familial,Congenital Thrombotic Thrombocytopenic Purpura,Familial Thrombotic Thrombocytopenia Purpura,Familial Thrombotic Thrombocytopenic Purpura,Microangiopathic Hemolytic Anemia, Congenital,Moschcowitz Disease,Schulman-Upshaw Syndrome,Thrombotic Microangiopathy, Familial,Thrombotic Thrombocytopenic Purpura,Upshaw Factor, Deficiency of,Upshaw-Schulman Syndrome,Familial Thrombotic Microangiopathy,Microangiopathy, Familial Thrombotic,Schulman Upshaw Syndrome,Thrombocytopenic Purpura, Thrombotic,Thrombopenic Purpura, Thrombotic,Thrombotic Thrombopenic Purpura,Upshaw Schulman Syndrome
D002543	Cerebral Hemorrhage	Bleeding into one or both CEREBRAL HEMISPHERES including the BASAL GANGLIA and the CEREBRAL CORTEX. It is often associated with HYPERTENSION and CRANIOCEREBRAL TRAUMA.	Brain Hemorrhage, Cerebral,Cerebral Parenchymal Hemorrhage,Hemorrhage, Cerebral,Intracerebral Hemorrhage,Hemorrhage, Cerebrum,Brain Hemorrhages, Cerebral,Cerebral Brain Hemorrhage,Cerebral Brain Hemorrhages,Cerebral Hemorrhages,Cerebral Parenchymal Hemorrhages,Cerebrum Hemorrhage,Cerebrum Hemorrhages,Hemorrhage, Cerebral Brain,Hemorrhage, Cerebral Parenchymal,Hemorrhage, Intracerebral,Hemorrhages, Cerebral,Hemorrhages, Cerebral Brain,Hemorrhages, Cerebral Parenchymal,Hemorrhages, Cerebrum,Hemorrhages, Intracerebral,Intracerebral Hemorrhages,Parenchymal Hemorrhage, Cerebral,Parenchymal Hemorrhages, Cerebral
D002544	Cerebral Infarction	The formation of an area of NECROSIS in the CEREBRUM caused by an insufficiency of arterial or venous blood flow. Infarcts of the cerebrum are generally classified by hemisphere (i.e., left vs. right), lobe (e.g., frontal lobe infarction), arterial distribution (e.g., INFARCTION, ANTERIOR CEREBRAL ARTERY), and etiology (e.g., embolic infarction).	Anterior Choroidal Artery Infarction,Cerebral Infarct,Infarction, Cerebral,Posterior Choroidal Artery Infarction,Subcortical Infarction,Cerebral Infarction, Left Hemisphere,Cerebral Infarction, Right Hemisphere,Cerebral, Left Hemisphere, Infarction,Cerebral, Right Hemisphere, Infarction,Infarction, Cerebral, Left Hemisphere,Infarction, Cerebral, Right Hemisphere,Infarction, Left Hemisphere, Cerebral,Infarction, Right Hemisphere, Cerebral,Left Hemisphere, Cerebral Infarction,Left Hemisphere, Infarction, Cerebral,Right Hemisphere, Cerebral Infarction,Right Hemisphere, Infarction, Cerebral,Cerebral Infarctions,Cerebral Infarcts,Infarct, Cerebral,Infarction, Subcortical,Infarctions, Cerebral,Infarctions, Subcortical,Infarcts, Cerebral,Subcortical Infarctions
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328	Adult	A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available.	Adults