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Query: UMLS:C0038454 (stroke)
 147,016 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Moyamoya disease is an uncommon clinical entity, characterized by bilateral occlusion of the internal carotid artery and the development of collateral arteries. An 18-year-old Saudi male with systemic lupus erythematosus (SLE) presented with mild right hemiparesis, followed by recurrent ischemic stroke. Cerebral angiography showed bilateral internal carotid artery stenosis associated with the development of collateral circulation (moyamoya vessels). There was no evidence of active SLE or other risk factors for cerebral occlusion, such as antiphospholipid antibody syndrome. Medical and surgical interventions did not influence the poor outcome of the recurrent ischemic insults.
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PMID:Systemic lupus erythematosus associated with moyamoya syndrome. 1103 39

Childhood stoke is increasingly recognized, but studies remain largely descriptive. Important differences from adult stroke include the following: (1) frequently delayed or missed diagnosis, (2) heterogenous and overlapping risk factors, and (3) developmental differences in the cerebrovascular, neurologic, and coagulation systems. These aspects limit the extrapolation of the results of adult stroke research and present challenges in caring for children with stroke. The incidence of childhood ischemic stroke exceeds 3.3 in 100,000 children per year, more than double the estimates from past decades. The increased incidence reflects, in part, increased survival in previously fatal conditions predisposing to stroke, including congenital heart disease, sickle cell anemia, and leukemia. Risk factors for stroke are recognized in more than 75% of children. Common risk factors include congenital heart disease and sickle cell disease. Progressive arteriopathies, including vasculitis and moyamoya syndrome, are rare in children with stroke; however, transient arteriopathies including post-varicella angiopathy are increasingly recognized. Prothrombotic abnormalities are frequently present but of unclear significance. Adverse outcomes after childhood stroke, including death in 10%, recurrence in 20%, and neurologic deficits in two thirds of survivors could be reduced with available stroke treatments. Aggressive prehospital emergency care and transfer could improve access to hyperacute stroke therapies including tPA. Currently, the diagnosis is delayed by more than 24 hours from onset in most children. As in adults, tPA will likely produce unacceptable rates of intracerebral hemmorrhage unless given within 3 hours of stroke symptom onset. The appropriate choices for in hospital treatment and secondary preventative strategies, including aspirin and anticoagulants, are controversial. Empiric recommendations are published; however, age-appropriate clinical trials are urgently needed. The large multinational networks of investigators necessary for designing and conducting these future trials are now being formed.
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PMID:Stroke in children: recognition, treatment, and future directions. 1120 20

Asymptomatic small hemorrhages were identified in hypertensive patients by T2*-weighted gradient echo magnetic resonance (MR) imaging to investigate the relationship between hypertensive intracerebral hemorrhage and asymptomatic minute hemorrhages. Forty-eight patients with hypertensive intracerebral hemorrhage or cerebral infarction with hypertension (these diseases were defined as stroke) were treated in National Defense Medical College from April 1998 to February 2000. All patients had no past history of stroke or head injury, underwent MR imaging within 6 months of the stroke attack, were aged from 40 to 80 years, and had no diagnosis of aneurysm, angioma, or moyamoya disease. Patients were divided into the infarction group and hemorrhage group. All foci over 2 mm in size appearing as hypointense on T2*-weighted MR imaging and unrelated to stroke areas were defined as minute hemorrhages. There were no significant differences between the two groups with respect to sex, age, and history of diabetes mellitus. The incidence of minute hemorrhages in the hemorrhage group (21/26) was greater than in the infarction group (9/22, p < 0.01). The incidence of minute hemorrhages in the basal ganglia (18/26) was greater in the hemorrhage group than in the infarction group (4/22, p < 0.001). Symptomatic intracerebral hemorrhage may be preceded by asymptomatic minute hemorrhage.
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PMID:Relationship between stroke and asymptomatic minute hemorrhages in hypertensive patients. 1121 34

Despite the number of potential causes, most children with ischemic cerebrovascular disease are classified into a few major categories: 1) cardioembolic stroke; 2) moyamoya syndrome; 3) complication(s) connected with systemic disease, with a known risk of a cerebrovascular event; 4) cervicocephalic arterial dissection; and 5) transient cerebral arteriopathy of undefined origin (probable arteritis). The etiological diagnosis is rapidly established by complementary investigations based on the initial clinical and imaging findings: cardiac exploration, magnetic resonance imaging and angiography or echo-doppler of the cervical arteries if cervical dissection is suspected; intra-arterial catheter cerebral angiography and analysis of the cerebrospinal fluid to investigate an intracranial arteriopathy. The outcome and treatment depend on the type of stroke, on its accurate identification, and on prediction regarding the risk of recurrence. Although there is a constitutional predisposition to cerebrovascular accidents, environmental triggers such as trauma, infectious disease and cardiac surgery also play a major role.
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PMID:[Specifics in pediatric arterial cerebral infarctions]. 1127 Feb 56

Spontaneous occlusion of the circle of Willis (moyamoya disease), a rare cerebrovascular disease, may lead to repeated hemodynamic stroke due to unremitting changes of the basal cranial vessels. Moyamoya can be diagnosed on its specific epidemiological, clinical, and radiological features. In patients with distinctive Moyamoya disease, improvement of cerebral blood supply by means of neurosurgical revascularization enables definitive treatment. This review summarizes current knowledge of the clinical and diagnostic features of this disease and illustrates its pathological and pathophysiological characteristics. Additionally, indications and techniques of current neurosurgical revascularization procedures are illustrated.
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PMID:[Spontaneous occlusion of the circle of Willis (moyamoya disease). Diagnosis and therapy]. 1143 99

Cerebral infarction is a frequent, severe complication of sickle cell anaemia. During childhood, most strokes are due to infarction with the majority resulting from occlusion of the large cerebral arteries. Risk factors include transient ischaemic attacks, acute chest syndrome, severe anaemia and elevated blood pressure. Less certain is the association with leucocytosis, or protection provided by alpha-thalassaemia or fetal haemoglobin. Children who have one stroke are at significant risk for having subsequent events that can be substantially reduced by maintaining haemoglobin S below 30%. It has not yet been possible to identify individuals for whom transfusion can be safely stopped. Haemosiderosis is a consequence of intensive and long term transfusion therapy, which requires chelation with deferoxamine. Iron accumulation can be minimised using erythrocytapheresis but this is technically difficult in children, expensive and results in increased donor exposure. In addition to lesions associated with strokes, an additional 17% of patients can be shown to have clinically silent cerebral infarcts. Although these are termed 'silent', those affected have mild neuropsychological deficits. Their relationship to stroke or risk for recurrence is unknown. Transfusion therapy has been shown to provide primary stroke prevention for children who have elevated cerebral artery velocity. Finally, intracranial haemorrhages, more commonly found in adults, also affect children. Subarachnoid haemorrhage is frequently found to result from cerebral artery aneurysms. A condition that mimics the moyamoya syndrome radiographically, as well as for its risk of haemorrhage, can be found in children with partly occluded cerebral arteries either as a result of stroke or silent infarct.
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PMID:Stroke in children with sickle cell anaemia: aetiology and treatment. 1143 87

A 3-year-old male patient with hereditary spherocytosis who developed moyamoya syndrome, presenting hemiplegia, and slurred speech is reported. Transient ischemic attacks occurred repeatedly with hemolytic crises. Magnetic resonance imaging and angiography revealed bilateral occlusion of the internal carotid and middle cerebral arteries with the formation of moyamoya vessels and multiple infarctions in the basal ganglia. Although splenectomy can increase the risk of stroke, no stroke occurred after splenectomy. On aspirin and dipyridamole therapy the patient has been free of neurologic deficits and progression of the vasculopathy for 5 years. This rare observation suggests that anemic hypoxia more greatly contributes to the progression of moyamoya syndrome than postsplenectomy thrombocytosis or reduced deformability of spherocytes.
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PMID:Moyamoya syndrome with spherocytosis: effect of splenectomy on strokes. 1148 2

We conducted a retrospective study to determine whether the presence of moyamoya collaterals influenced the risk of recurrence of cerebrovascular events (CVEs: stroke or transient ischemic attack) in patients with sickle cell disease placed on chronic transfusions after a stroke. Forty-three patients with homozygous sickle cell anemia (HbSS) and 1 with HbSO(Arab) (16 females, 28 males) who had suffered strokes while under the age of 18 were studied. All patients had been on transfusions aimed at maintaining the sickle hemoglobin (HbS) level below 30%. They were followed for a mean of 6.6 years (2.2 to 20.4 years). The presence of collaterals was diagnosed based on either magnetic resonance angiography or conventional angiography. Eighteen (41%) of the 44 patients suffered recurrent CVEs. Nineteen (43%) (6 females, 13 males) patients had moyamoya collaterals. Eleven (58%) of these 19 experienced 21 total recurrent CVEs, including 4 strokes in 4 patients (21%). In comparison, 7 (28%) of 25 patients without moyamoya collaterals experienced 9 recurrent CVEs (P <.05) with only 1 recurrent stroke (4%). Moyamoya patients were also more likely to have 2 recurrent CVEs (42% vs 8%, P <.05) as well as poorer neuropsychological testing results. A proportional hazards regression analysis indicated that patients with moyamoya were more than twice as likely to incur a subsequent CVE (hazard ratio, 2.40; 95% confidence interval, 0.85, 6.75). We conclude that up to 41% of patients with sickle cell disease experience recurrent CVEs after an initial stroke despite chronic transfusions and that the risk of recurrence is significantly higher for those who have moyamoya collaterals.
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PMID:Moyamoya syndrome in childhood sickle cell disease: a predictive factor for recurrent cerebrovascular events. 1196 76

Five cases of paediatric stroke are reported. Two patients presented with idiopathic stroke, another following vertebral artery dissection, one secondary to Moyamoya disease, and one patient with the syndrome of mitochondrial encephalopathy, lactic acidosis, and stroke-like episodes. The presentation, investigation, and management of paediatric stroke are discussed.
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PMID:Paediatric stroke: case series. 1205 70

We present the case of a 29-year-old woman with Down syndrome who developed bilateral frontal ischemic stroke. Cerebral angiography demonstrated an occlusion of the both supraclinoid internal carotid arteries associated with dilated collateral vessels, consistent with moyamoya disease. We review the clinical and radiological features of moyamoya disease associated with Down syndrome and discuss a few major physiopathologic hypotheses to explain this association.
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PMID:Moyamoya disease and Down syndrome: case report and review of the literature. 1216 1


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