Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A boy with sickle cell anemia underwent bone marrow transplantation (BMT). He was normal on neurological examination, but had radiologic evidence of an old left frontal lobe infarct, multiple cerebral vascular stenoses and moyamoya collaterals. After BMT he developed seizures with extension of the infarct and subarachnoid hemorrhage. One year later angiography revealed worsening stenosis of the M1 segments of both middle cerebral arteries. At that time an increase in von Willebrand's factor with decreased large molecular weight multimers (LvWF) was observed. We speculate that LvWF dependent, shear-induced platelet aggregation, together with endothelial damage may have contributed to the development of neurologic complications in this patient.
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PMID:Neurologic complications following bone marrow transplantation for sickle cell disease. 870 95

We report a black girl with sickle cell anemia. On prophylactic exchange transfusion protocol, she experienced cerebrovascular accidents at 3 and 3.5 years of age, both associated with transient right hemiparesis. At 7.5 years of age, she presented with a partial motor seizure and a left hemiparesis. A cerebral angiogram demonstrated stenosis at the origins of both middle and anterior cerebral arteries bilaterally with extensive basal collateralization. She underwent uncomplicated bilateral encephaloduroarteriosynangiosis (EDAS) procedures using both superficial temporal arteries. At age 9 years, the patient presented with a severe headache and tunnel vision secondary to a stenosis of both posterior cerebral arteries. She underwent bilateral EDAS procedures using both occipital arteries. No complication was encountered. Postoperative cerebral angiogram demonstrated impressive neovascularity at the sites of all four EDAS procedures. Different treatment options of moyamoya disease are discussed.
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PMID:Encephaloduroarterio-synangiosis in a child with sickle cell anemia and moyamoya disease. 873 7

Moyamoya syndrome is a chronic cerebrovascular disorder that is characterized by a progressive narrowing of the intracranial internal carotid arteries and their main branches and a compensatory dilation of smaller arteries (ie, moyamoya vessels) at the base of the brain. Common signs and symptoms in children include recurrent episodes of cerebral ischemia, transient hemiparesis, seizures, and cerebrovascular accidents (CVAs). The diagnosis is made primarily by cerebral angiography, and surgical treatment consists of pial synangiosis, whereby surgeons suture the superficial temporal artery to the pla mater under microscopic visualization. The goal of surgical intervention is to promote the development of collateral circulation to the brain and decrease the risk of CVAs, transient ischemic attacks, and seizures. Follow-up angiography demonstrates that pial synangiosis results in excellent postoperative collaterization of ischemic areas of the brain.
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PMID:Pial synangiosis for treatment of Moyamoya syndrome in children. 889 61

A case of moyamoya disease is presented. The patient is a 15-year-old white Spanish girl who had the onset of the neurological symptoms at 8 months of age with seizures, transient ischemic attacks and residual left hemiparesis. Cerebral arteriography performed at the age of 1 year revealed all the features of moyamoya disease in the territory of both carotids and in the vertebrobasilar arteries. The presence of an embryonic tentorial artery, the Bernasconi-Cassinari artery, originating from the right internal carotid artery, was disclosed at 1 year of age but did not appear in an arteriogram performed at 6 years of age and it was revisualized by magnetic resonance arteriography (MRA) performed at the age of 15 years. This finding seems to indicate a very early intrauterine onset of the disease in this case and demonstrates the superiority of MRA over conventional arteriography to discover anomalies of intracranial vessels. Administration of nicardipine, a calcium channel blocker, added to conventional antiepileptic drugs that the patient had previously taken, improved the epileptic and the neurological disease.
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PMID:Moyamoya disease with a marked collateral supply through the artery of Bernasconi-Cassinari. 890 48

A 2-year-old child who acutely developed hemiplegia and seizure was found to have moyamoya disease and heterozygous protein S deficiency. This case report should alert physicians to the possible coexistence of moyamoya disease and protein S deficiency, even in the case of typical moyamoya disease. The intimate relationship between the two require further study.
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PMID:Moyamoya disease and protein S deficiency: a case report. 936 1

Moyamoya disease usually presents itself in children as recurrent episodes of transient cerebral ischemia, such as acute motor and sensory deficits, speech disturbance, headache and seizures. Its initial symptoms rarely include mental disturbance. We experienced a nine-year-old girl with Moyamoya disease who showed choreic involuntary movements of the left upper and lower limbs. In spite of mental disturbance which began around the age of four, she had never visited hospital. CT and MRI detected multiple ischemic lesions in the frontal, parietal and occipital areas. SPECT demonstrated low perfusion of these regions as well as of the right corpora striata, which appeared to be normal in MRI. She underwent right and left superficial temporal artery-middle cerebral artery (STA-MCA) anastomoses with an interval of three months. The choreic movement completely disappeared but her intelligence showed no improvement. The cerebral blood flow increased, but there was no change in the infarction area. Moyamoya disease should be considered in the differential diagnosis of mental disturbance in young children.
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PMID:[Moyamoya disease presenting initially with mental disturbance]. 939 2

To both clarify the current clinico-epidemiological features of Moyamoya disease in Korea as well as compare these cases with Japanese patients, 451 cases were collected from 26 Korean major neurosurgical institutes and 296 definite cases were analyzed statistically. Although the Korean age distribution patterns of Moyamoya disease showed two peaks, similar to Japanese patients, the Korean pattern was shifted to the right thus indicating Korean adult population to be 20% higher than that of Japanese patients. The female/male ratio was 1:3, which was slightly less than that for Japanese. The family occurrence rate in Koreans was 1.8%. The incidence of cerebral infarction and bleeding in Koreans was higher while transient ischemic attack (TIA) and seizure were less than that of Japanese. The incidence of infarction in children and of hemorrhage in children and adults were also statistically higher in Koreans. The incidence of hemorrhage was higher in females than in males. Both the age at onset and sex affected the disease type. Single encephalo-duro-arterio-synangiosis (EDAS) was performed on 87.6% of all surgical cases. Although the incidence of bleeding was higher in Korea, the outcomes of the patients were similar to that of the Japanese patients. Although Korean Moyamoya disease showed a relatively higher incidence of hemorrhage and adult onset, the overall clinical background was similar to that of Japanese patients.
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PMID:Epidemiological survey of moyamoya disease in Korea. 940 96

A total of 34 Italian patients (15 males and 19 females) suffering from Moyamoya disease (MMD) and selected by a questionnaire survey in 12 neuropediatric and neuroradiologic departments were studied in a multicentric study. The onset of the disease appeared either in childhood (27 patients, aged 0-16 years, mean 5.4 years) or in adulthood (seven patients, aged 25-55 years, mean 35 years). The early clinical symptoms consisted of transient ischemic attacks and/or stroke (20 cases), recurrent migraine-like headaches (seven cases), seizures (six cases) and hemorrhage (one case). A total of four familial cases were found. The final diagnosis was based in all cases on the conventional angiographic findings and more recently also on the magnetic resonance angiography (ten patients). The mean lag time between the first clinical manifestation and the angiographic diagnosis was about 2 years. A medical treatment (vasodilators, antiplatelet agents, calcium channel blockers) was followed by 21 patients, while five cases underwent a surgical revascularization. The follow-up ranges from 1 to 15 years (mean 6 years): A motor (16 cases) and/or mental impairment (14 cases) was detected especially in the childhood onset MMD; only one patient died. In nine cases the long-term outcome persisted without neurological deficit.
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PMID:Clinical and neuroradiological findings of moyamoya disease in Italy. 940 6

To determine the clinical characteristics and the effectiveness of encephalo-duro-arterio-synangiosis (EDAS) in adulthood-onset Moyamoya disease (MMD), the authors retrospectively reviewed 26 patients suffering from MMD who were admitted to Seoul National University Hospital between 1987 and 1995. When they showed major symptoms, all were more than 16 years-old. The most common presenting symptom was intracranial hemorrhage (ICrH), found in 12 patients or 46% of the total; the second was infarction and transient ischemic attack, each found in seven or 27% of them. Only one patient was found to have seizures, which were associated with a cerebral infarction. The Suzuki angiographic stage 3 and less than stage 3 accounted for 73% of all 52 hemispheres. A total of 15 patients underwent single photon emission computed tomography (SPECT) preoperatively. When the derangement of cerebral perfusion was estimated with four SPECT grades (SG), 70% of their hemispheres revealed normal (SG1) or localized decreased-perfusion (SG2). The other 30% had extensive decreased-perfusion or localized perfusion defects (SG3). There was no case who had extensive perfusion defects (SG4). A total of 17 patients underwent EDAS operations (EDAS group) and nine did not undergo any operation (no-op group). The EDAS group had significantly better clinical outcomes than the no-op group after a 12-month median follow-up period (P < 0.05). The angiographic and SPECT follow-up studies comprised six and seven cases, respectively. There was also satisfactory angiographic revascularization in all follow-up cases and improvement in cerebral perfusion at SPECT follow-up in six of seven cases. It is concluded that the involvement of posterior circulation of MMD is not frequent and cerebral perfusion is preserved in adulthood-onset MMD patients. These findings may explain the reason why hemorrhages are frequent and the late onset of symptoms in adulthood-onset MMD. Surgical treatment with EDAS seems to be effective in adulthood-onset MMD in terms of clinical improvement.
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PMID:Moyamoya disease in adults: characteristics of clinical presentation and outcome after encephalo-duro-arterio-synangiosis. 940 27

Two cases of moyamoya disease observed in two children are reported. The two cases recall the frequency of ischemic strokes, transient ischemic attacks, and seizures revealing the disease. Diagnosis is made by cerebral arteriography showing carotid stenosis and the dense deep arterial collateral as seen in these two cases. Magnetic resonance imaging makes it possible to identify this vascular disease as it reveals vascular varicosities in the basal ganglia, as in case 1. Strokes can sometimes be prevented by constructing an anastomosis between the superficial temporal artery and the middle cerebral artery, as demonstrated in case 1. Thus, the diagnosis of moyamoya disease has been improved by magnetic resonance imaging and there is now a surgical treatment to prevent ischemic stroke.
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PMID:Moyamoya disease in children. A review of the clinical and radiological features and current treatment. 945 75


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