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Query: UMLS:C0002895 (sickle cell disease)
 11,747 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

An opportunity to study cerebrovascular changes in sickle cell anemia (SCA) presented itself when a black child with this disorder died of bihemispheric strokes. Angiography demonstrated severe occlusive vascular disease involving primarily the circle of Willis and major bifurcations of both internal carotid arteries. Collateral circulation to the distal branches of the internal carotid arteries occurred through transdural anastomoses from the external carotid system and via the leptomeningeal route. Perfusion of the basal ganglia was accomplished by vessels arising from the proximal internal carotid arteries. These changes resembled those of Moyamoya disease. Autopsy showed old and recent cerebral infarcts. Two vascular processes were responsible for the arterial occlusions: (1) exuberant intimal hyperplasia, and (2) old and recent thrombi with partial recanalization. The former has been described only once before in SCA. Small vessels in the basal ganglia were exceptionally numerous and dilated. We conclude that intimal hyperplasia within large cerebral arteries may be responsible for infarction and small vessel prliferation in basal ganglia in patients with SCA.
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PMID:Cerebrovascular disease in sickle cell anemia: a clinical, pathological and radiological correlation. 62 45

Two cases of sickle cell disease with neurological complications are described. Cerebral angiograms were performed and a characteristic moyamoya-like angiographic pattern was demonstrated. This pattern results from a compensatory vascularization which may follow occlusion of the arteries at the base of the brain in several diseases. The risks of angiography and appropriate precautions are emphasized.
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PMID:[Sickle cell anemia: cerebral angiography changes]. 186 46

Clinical and necropsy findings in 11 patients with sickle cell anemia (SS) indicate that intracranial hemorrhage (IH) is a delayed sequela of the same vasculopathy that causes cerebral infarction during childhood. Evidence of prior cerebral infarction during childhood included hemiparesis, seizures, an episode of coma, or mental retardation. Computerized tomography (CT) scans showed cerebral infarcts with lucent areas and dilated ventricles or cerebral atrophy. CT or magnetic resonance imaging (MRI) scans after the intracranial hemorrhage demonstrated intraventricular or intracerebral hemorrhages. Angiography or autopsy in seven patients showed widespread vascular occlusion and narrowing of arterial vessels. Moyamoya with internal carotid artery occlusion was identified in two cases. At the time of the IH, three patients were being treated with prophylactic transfusion regimens. We hypothesize that the central nervous system vasculopathy progresses over time and that arterial narrowing in both large and small vessels secondary to endothelial hyperplasia is followed by neovascularization and hemorrhage. Recognition of this pattern of delayed intracranial hemorrhage following cerebral infarction should encourage more intensive evaluation aimed at developing rational interventional therapy prior to a terminal intracranial hemorrhage.
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PMID:Delayed intracranial hemorrhage following cerebral infarction in sickle cell anemia. 213 21

The criteria of the cerebrovascular Moyamoya disease is defined by the characteristic findings of its cerebral angiograms, as follows; 1) The internal carotid siphon is narrowed or obstructed bilaterally. 2) The "Moyamoya vessels" are observed at the base of the brain or the basal ganglionic regions. 3) Main trunks of the cerebral arteries such as the anterior, the middle, and/or the posterior cerebral arteries are often not or poorly visualized. 4) Its etiology is unknown. It has been known that the occlusion of the internal carotid fork with Moyamoya vessels is not infrequently seen in patients with tuberculous meningitis, sickle cell anemia, head trauma, and so on. In the definition of the disease, patients with known etiology and/or unilateral occlusion in the carotid fork must be excluded. However, the cases who cannot fulfil its criteria of the cerebrovascular Moyamoya disease, but have its characteristic Moyamoya vessels and collateral pathways have been reported. We investigated the findings of cerebral computed tomograms in 13 patients who did not fulfil the criteria of the cerebrovascular Moyamoya disease, but revealed the Moyamoya vessels. The subjects are 5 males and 8 females, ranging 15 to 70 years old. The past histories of 9 patients among them revealed hypertension, radiation therapy for pituitary adenoma, head trauma, aplastic anemia, and the Raynaud phenomenon. By angiographic evaluations, occlusions in the unilateral carotid forks were seen in 7 patients, and stenoses in those were in 5 patients. One patient showed only a severe stenosis in the horizontal portion of the middle cerebral artery.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Cases similar to cerebrovascular moyamoya disease--investigation by angiography and computed tomography]. 319 93

The cause of stroke in a young adult can usually be ascertained with proper workup. One of the most common causes is atherosclerotic cerebrovascular disease, and cigarette smoking is an important risk factor in young adults. Several types of nonatherosclerotic cerebral vasculopathy can also result in premature cerebral infarction; these include cervicocephalic arterial dissection, nonpenetrating traumatic arterial disease, moyamoya disease, fibromuscular dysplasia, vasculitis, and migraine. Cardiac embolism may play a more important role than was previously thought, and hematologic disorders (eg, sickle cell disease, polycythemia rubra vera, coagulation problems) are known to predispose patients to stroke. A careful history of risk factors and a thorough neurologic and cardiovascular examination followed by adequate testing, including angiography, are essential to diagnosis.
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PMID:Diagnosis of stroke in young adults. 356 68

We draw attention to the value of selective digital subtraction angiography (DSA) and ioxaglate meglumine (Hexabrix) in demonstrating extensive cerebral collateral circulation (moyamoya) in a patient with homozygous sickle cell disease.
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PMID:Moyamoya in sickle cell disease demonstrated by DSA and Hexabrix. 391 Jun 59

Sickle cell (drepanocytic) anemia is a hereditary blood disease occurring very rarely in Mexico. A 13-year-old Mexican boy with sickle cell anemia eventually died of a cerebrovascular accident of the brain stem, as shown by computerized tomography (CT). A characteristic moyamoya-like angiographic pattern was demonstrated on bilateral carotid and left vertebral arteriograms. Moyamoya disease has no known etiology, but the characteristic angiographic features of moyamoya have been observed in conjunction with some other disease of known origin (including sickle cell anemia). It is therefore my belief that these latter cases should be referred to as pseudomoyamoya and not true moyamoya.
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PMID:Pseudomoyamoya in sickle cell anemia. 716 62

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

Patients with sickle cell disease may develop intracranial vascular disease, with the occlusion or obstruction of the large or small arteries, which may lead to the secondary development of moyamoya disease. In this report, we describe the neurochemical changes in the brain before, during, and after an extracranial-intracranial bypass procedure on a patient with sickle cell disease and a moyamoya disease like pattern on angiography. We used the in vivo microdialysis technique to measure the on-line pH, lactate and amino acid concentrations in the extracellular fluid. There were relatively high resting glutamate levels and a lower-than-normal pH in the extracellular fluid prior to the bypass, associated with chronic ischemia. During the bypass there was a short-lived increase in the glutamate levels. After revascularization, there was a rapid decrease in the glutamate levels and an increase in the pH value. The patient's preoperative neurological deficit improved post-operatively, corresponding to the biochemical changes towards normal values. These changes after revascularization suggest that chronic biochemical abnormalities due to brain ischemia may improve after cerebral revascularization.
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PMID:EC-IC bypass improves chronic ischemia in a patient with moyamoya disease secondary to sickle cell disease: an in vivo microdialysis study. 909 Jun 39


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