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

A review of the recent Mayo Clinic experience with stroke in children having cerebral angiography revealed five patients with moyamoya disease. The disease commonly presents as recurrent strokes and only rarely as a seizure disorder. The angiographic pattern suggests that the telangiectasia characteristic of this condition represents normally present dilated vessels.
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PMID:Moyamoya disease in children. 62 48

30 micromalformations (MMF) of brain vessels that were not diagnosed either clinically or at the autopsy were found histologically in 25 autopsy cases and in 3 patients after the surgical operation. MMF were, in half of the cases, the cause of the stroke which was mistakingly considered a complication of the arterial hypertension, cerebral atherosclerosis or rupture of saccular aneurysm of the brain base arteries. Clinically, MMF were either latent or manifested as recurrent disturbances of the brain circulation of the ischemic or (more frequently) haemorrhagic type. When clinically manifested MMF were revealed at the age of 15-32 years; at the age of 65-74 years they were found incidentally at the autopsy. Their predominant localization was in the brain stem, contiguous ventricular wall and in the cerebellum with its tunic. The following histological types can be distinguished: telangiectasia, venous, cavernous, arteriovenous, nonclassified, varicosis. Clinico-anatomical characterization of each type is presented. The medico-legal importance of MMF and the principal possibility of their treatment (when duly diagnosed) is pointed out.
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PMID:[Micromalformations of the cerebral vessels and their role in the development of stroke]. 261 79

Over a 10-year period, 276 pulmonary arteriovenous malformations (PAVMs) were occluded with balloon embolotherapy in 76 patients, 67 (88%) of whom had hereditary hemorrhagic telangiectasia. Eleven patients (14%) were discovered by means of family screening with measurement of arterial blood gases and chest radiography. Epistaxis, dyspnea, hemoptysis, and hemothorax occurred in 79%, 71%, 13%, and 9% of patients, respectively. Clinical histories of strokes and transient ischemic attacks were present in 18% and 37% of patients, respectively. Computed tomographic scans of 59 patients showed stroke in 36%. Sixty-five percent of PAVMs were located in the lower lobes, which correlated with the finding of more pronounced hypoxemia in the upright position. After embolotherapy, symptomatic hypoxemia was corrected, and serial values have remained constant for 5 years. Complications were minimal, and no patient required surgery. Balloon embolotherapy is effective long-term therapy for PAVMs, and family screening should be pursued because of the possibility of a higher frequency of paradoxical embolization (stroke) than previously recognized.
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PMID:Pulmonary arteriovenous malformations: techniques and long-term outcome of embolotherapy. 318 89

Five cases of brain-stem hematoma are described. The cause of these hematomas was identified as "cryptic angioma" (1 cavernous angioma, 1 telangiectasia, 3 arteriovenous malformations). So, they are so-called "secondary hematoma", as opposed to brain-stem hematoma in relation with hypertension. Such secondary hematomas are reported in the literature: 37 operated on cases and 22 untreated cases were found. The clinical picture does not seem to be typical. The presentation appears to be either with the acute onset of a stroke, or with a subacute onset including relapsing symptoms. A progressive deterioration suggesting a pontine glioma or mimicking demyelination is not rare. The CT scanner appearance is often characteristic showing a high density area in the brain-stem which enhanced after injection of contrast medium with an aspect of "halo". Angiography is usually negative. The natural history of brain-stem hematoma due to rupture of a cryptic angioma is not well documented, but it seems that prognosis is very poor. So, the authors insist on surgical evacuation which is effective and safe allowing the diagnosis of brain-stem hematoma and in some cases the identification of the malformation.
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PMID:[Secondary hematoma of the brain stem. Apropos of 5 cases]. 376 36

Central nervous system arteriovenous malformations are uncommon in hereditary hemorrhagic telangiectasia. Identical twins are described with hereditary hemorrhagic telangiectasia and concordance for central nervous system arteriovenous malformations identified by angiography. One twin had a central nervous system hemorrhage in the seventh month of pregnancy and also had a pulmonary arteriovenous malformation. The other was asymptomatic. A previously reported association between HLA type A2 BW17 and hereditary hemorrhagic telangiectasia was not confirmed. Two recombinations were identified between the loci for HLA and hereditary hemorrhagic telangiectasia. The loci for HLA and hereditary hemorrhagic telangiectasia are not closely linked. Stroke in a young person should prompt an inspection for manifestations of hereditary hemorrhagic telangiectasia.
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PMID:Identical twins with hereditary hemorrhagic telangiectasia concordant for cerebrovascular arteriovenous malformations. 377

Three children with pronounced livedo reticularis present since birth (cutis marmorata-telangiectasia congenita) have been followed to the ages of eight, 17 and 21 years. During childhood they developed frequent recurrent transient stroke-like hemipareses, affecting either side of the body, associated with ipsilateral pain, headache, visual symptoms, dysphasia, fits and confusion. Intellectual failure and, in one, progressive spasticity have followed. Attacks were more frequent in winter. Other problems have included abnormal peripheral vascular responses to temperature change, gastro-intestinal bleeding, glaucoma, local tissue hypertrophy and, in the two older patients, renal involvement with hypertension. Their condition represents a form of congenital vasculopathy. Anticonvulsants, anti-migraine agents, anti-platelet drugs and flunarizine have been ineffective. Nifedipine prevented further attacks in one patient and reduced attacks in another, but has not helped the third child. Adequate clothing and warmth may also be important.
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PMID:Congenital livedo reticularis and recurrent stroke-like episodes. 840 21

A study was performed to assess the natural history, prognostic factors, and lipid and apolipoprotein abnormalities of idiopathic ischemic childhood stroke. A case series of 42 children, retrospectively identified with idiopathic ischemic strokes, were reassessed an average of 7.4 years (range, 1 to 19 years) after presentation. Patients were interviewed and examined, and fasting serum was obtained for lipid and apolipoprotein analysis. Poor outcome was defined as moderate to severe hemiparesis, special educational needs, epilepsy, recurrent stroke, or stroke-related death. Eighteen (43%) of the patients had a poor outcome. Among them were moderate to severe hemiparesis in 14 (78%), recurrent strokes in seven (39%), and one death. Poor outcome was evident early in their clinical course. Independent of outcome, lipid abnormalities including an elevated triglyceride and low-density lipoprotein cholesterol, and a depressed high-density lipoprotein cholesterol were seen in one third of all patients. A depressed ratio of apolipoprotein A-1 to apolipoprotein B (using adult normative values) was seen in half of the entire cohort. Clinical features of children with unexplained ischemic strokes at presentation and their subsequent course are described. Significant risk factors for a poor outcome include (1) persistence of hemiparesis 1 month after the stroke, (2) cortical as opposed to subcortical location, and (3) bilateral occlusive disease with telangiectasia on cerebral angiography. Previously described risk factors for an unfavorable prognosis, including occurrence during infancy and presentation with seizures, were not substantiated. Lipid abnormalities occur at an increased frequency in children after unexplained ischemic strokes. Prospective assessment of lipoprotein profiles are needed to further assess clinical significance. Assessing apolipoproteins may provide further insight than lipid values alone.
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PMID:Natural history, prognosis, and lipid abnormalities of idiopathic ischemic childhood stroke. 880 16

Intracerebral hemorrhage (ICH) is a common and serious type of stroke. Recent studies have shown that inherited factors that affect the development of the vessel wall can increase the risk of ICH. We studied endoglin as a candidate gene in patients with sporadic ICH, since mutations in this gene can cause telangiectasia formation. One hundred three patients with sporadic ICH and 202 controls were studied. The polymerase chain reaction and single-strand conformational polymorphism analysis were used to screen for mutations in exon 7 of the endoglin gene. No coding mutations in exon 7 were identified in the ICH patients or controls. A 6-base intronic insertion was found 26 bases beyond the 3' end of exon 7. The homozygous form of the insertion was present in 9 of 103 (8.7%) ICH patients compared with 4 of 202 (2.0%) controls, p = 0.012 (odds ratio 4.8 [95% confidence interval, 1.28, 21.60]). Analysis of the endoglin transcript around the insertion did not reveal any changes in the RNA sequence. There were no obvious clinical features that distinguished the ICH patients with the homozygous insertion from the other patients. The pathophysiologic mechanism underlying this association remains to be determined.
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PMID:Endoglin gene polymorphism as a risk factor for sporadic intracerebral hemorrhage. 915 32

An unusual and rare case of a central pulmonary arteriovenous fistula (PAVF) with concomitant cerebral arteriovenous malformation (CAVM) in the absence of hereditary hemorrhagic telangiectasia is reported. Presentation was in the form of a cerebrovascular accident (CVA). Clinical presentations and management strategies of PAVF with associated CAVM are broadly discussed along with a pertinent literature review.
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PMID:Pulmonary arteriovenous fistula with cerebral arteriovenous malformation without hereditary hemorrhagic telangiectasia. Unusual case report and literature review. 946 Dec 79

Chronic illness played a major role in the life and literary success of Robert Louis Stevenson. However, the exact nature of his chronic illness remains unclear. It is possible that Stevenson had hereditary hemorrhagic telangiectasia (Osler-Rendu-Weber Syndrome). This would explain his chronic respiratory complaints, recurrent episodes of pulmonary hemorrhage, and his death, at age 44 years, of probable cerebral hemorrhage. It would also explain his mother's hitherto unreported but apparent stroke, at age 38 years. Further support for this hypothesis might come from new details about the health of Stevenson and his relatives or from molecular analysis of tissue specimens remaining from him.
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PMID:Did Robert Louis Stevenson have hereditary hemorrhagic telangiectasia? 1075 Oct 91


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