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Query: UMLS:C0018991 (hemiplegia)
 3,997 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Moyamoya is the name given to an angiographic picture consisting of bilateral intracranial carotid artery occlusion associated with telangiectatic vessels in the region of the basal ganglia. It appears that patients with this angiographic finding can be divided into two distinct clinical groups. The first consists of children with a "primary" form of moyamoya that causes alternating hemiplegia, frequently of nonprogressive nature. The second consists of children and adults with various underlying diseases who develop an "acquired," usually progressive form of moyamoya. The striking predominance of Japanese patients with primary moyamoya and its high familial occurrence suggests that the primary form represents a hereditary malformation of the cerebral vascular bed. We report three additional cases of moyamoya. One case represents the primary form and the two are examples of the acquired form in a child and an adult.
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PMID:Primary and acquired forms of moyamoya syndrome. A review and three case reports. 739 66

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.
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PMID:[Moyamoya disease associated with thrombotic thrombocytopenic purpura (TTP)]. 801 79

Three types of clinical features at the onset are well known to be characteristic of acute hemiplegia syndrome (AHS). Type 1 comprises status epilepticus of hemiconvulsions with fever. Representative diseases of this type are the infectious diseases of the central nervous system, acute encephalopathy and cerebral vascular diseases. Type 2 comprises status epilepticus of hemiconvulsions without fever. Cerebral vascular diseases and epilepsy are the major ones of this type. Type 3 comprises hemiplegia or hemiparesis of sudden onset without fever or convulsions. Most patients with this type had cerebral vascular diseases, about half of which were moyamoya disease in Japan. Recent progress in neuroimaging studies has allowed considerable elucidation of the etiology of AHS. Gadolinium-enhanced MRI showed minimal lesions such as capsular infarction more clearly than plain MRI. Acetazolamide test 99mTc-HMPAO SPECT imaging is one of the useful assisted diagnostic techniques for moyamoya disease, because it reveals the reserve capacity of the collaterals. [123I]IMP SPECT is useful for the diagnosis and follow-up of acute disseminated encephalomyelitis (ADEM), as the images of the lesions coincide well with the MRI ones. 99mTc-HMPAO SPECT in a case with alternating hemiplegia revealed normoperfusion in the ictal periods. Four cases of AHS are reported here.
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PMID:Acute hemiplegia syndrome in childhood. 805 23

Moyamoya disease is one of the cerebrovascular disorders and is characterized by bilateral stenosis or occlusion of the distal internal carotid arteries and their main branches. Repeated ischemic episodes in children and intracranial hemorrhage in adults with moyamoya disease is usually noted. In this report, we describe two children with moyamoya disease who presented with alternating hemiplegia. The final diagnosis was made by cerebral angiography and cranial computed tomography in these patients. Other neurological and radiological features of the patients were described.
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PMID:Moyamoya disease and alternating hemiplegia. A report of two cases. 816 Feb 80

We report on eight children who suffered from cerebrovascular ischemia or stroke at the age of 2 or up to 11 years. Antiphospholipid antibodies (APLA) were detected in two cases during the ischemic event and in six cases during follow-up examinations (after six weeks or within a span of six years). In two patients multiple stenoses of basal cerebral arteries were found; one of them suffered from moyamoya syndrome. The acute hemiplegia in one patient was linked to an asymptomatic mycoplasmal infection and APLA. In three cases, one of the parents was also APLA-positive. Seven patients were treated with acetylsalicylic acid, and in four cases immunoglobulin infusions were given. Transient ischemic attacks subsided after the child with the moyamoya syndrome received immunoglobulins. No effect of medication could be established in the other children. The concept of the antiphospholipid syndrome is still evolving. As none of the common risk factors pertaining to strokes in adults apply to children, pediatric research may offer a suitable platform for specific investigations on the causal, pathogenetic role of APLA. We propose that all children suffering from stroke or transient ischemic attacks should be tested for APLA.
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PMID:Antiphospholipid antibodies in cerebrovascular ischemia and stroke in childhood. 820 57

An 11-year-old girl with Down syndrome is reported with moyamoya syndrome; she presented with chorea and mental regression, but had no hemiplegia or convulsions. Magnetic resonance imaging and magnetic resonance angiography were valuable for diagnosis of moyamoya syndrome. It is suggested that moyamoya syndrome be considered as a possible cause of involuntary movements in Down syndrome patients.
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PMID:Moyamoya syndrome in a patient with Down syndrome presenting with chorea. 829 17

We have encountered 38 cases of juvenile ischemic strokes during the past 10 years, which included 16 cases of moyamoya disease and 22 cases of non-moyamoya strokes. The etiology could not be determined in most non-moyamoya strokes, except in 4 cases of cardiogenic embolism and 1 case of post-meningitic stroke. We further examined 13 cases of idiopathic strokes, which showed clinical symptoms of so-called acute infantile hemiplegia. Nine out of 13 patients developed strokes between 6 months and 2 years of age. Strong male preponderance was noted, since 11 out of 13 patients were boys. CT scan showed basal ganglionic infarction in the territory of the perforating branches of the middle cerebral artery in 12 patients. Recurrence of ischemic events occurred in none of the patients during the mean follow-up period of 48.4 months. Functional recovery was also good, since no neurological sequelae were found in 5 patients, and the other 8 disabled children were able to go to school without help and none of the children were mentally retarded. These results and a review of the literature suggest the importance of traumatic mechanisms in the etiology and pathogenesis of juvenile idiopathic ischemic strokes of the acute infantile hemiplegic type.
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PMID:[Ischemic stroke in childhood]. 832 52

Moyamoya is an obstructive cerebrovascular disease characterized by a cerebral angiographic picture of stenosis or occlusion of main cerebral arteries with an abnormal vascular network at the base of the brain. No definitive cause has been found for this disease and opinion is still divided between a congenital and an acquired etiology. Hemiplegia of sudden onset and epileptic seizures are the prevailing presentation in childhood, while subarachnoid bleeding occurs more frequently in adults. We report a new case of childhood moyamoya with clinical onset of the neurological symptoms within the 3rd year of life; during the child's illness the maternal grandmother presented with moyamoya disease too. Antiaggregating and calcium-antagonist drugs seem effective in preventing further vascular accidents, while a surgical approach is not possible. Computed tomography, single positron emission computed tomography, and magnetic resonance imaging are very useful in the diagnosis of this rare disease.
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PMID:Moyamoya disease in childhood: a familial case report. 840 3

Ocular ischemic syndrome is extremely rare in childhood. Patients with moyamoya disease may be particularly susceptible to the development of ocular ischemia due to the associated carotid occlusion. A 19-month-old boy presented with neurofibromatosis and signs of ocular ischemia. At 29 months of age, he developed dense right vitreous hemorrhage and eventually lost vision in that eye due to phthisis. At almost six years of age, he developed an acute hemiplegia and was then diagnosed with moyamoya disease. This rare instance of childhood ocular ischemia in conjunction with moyamoya disease and neurofibromatosis demonstrates the serious ocular and systemic sequelae of occlusive vascular disease.
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PMID:Ocular ischemic syndrome in a child with moyamoya disease and neurofibromatosis. 872 42

The significance of antiphospholipid antibodies was examined in patients with childhood strokes. Eight patients, aged 2-13 years, who presented with acute hemiplegia were studied. On the basis of magnetic resonance imaging, magnetic resonance angiography, single photon emission computed tomography, and cerebral angiographic findings, 3 children were diagnosed as having infarctions due to moyamoya disease, and the others as having idiopathic infarctions. Serologic studies revealed elevated anticardiolipin antibody (ACA) IgG in 3 of the 5 patients with idiopathic infarction; no such elevation was revealed in patients with moyamoya disease. Values for all other tests, including ACA IgM and lupus anticoagulant, were within normal limits or negative in all patients. ACA IgG, therefore, may be a more important causative agent of childhood strokes than was previously considered.
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PMID:Antiphospholipid antibody syndrome in childhood strokes. 877 Nov 68


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