Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0917798 (cerebral ischemia)
17,036 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 34-year-old man with right-sided cluster headache presented with a stroke from right-sided moyamoya. Following surgery on the right, both moyamoya and cluster headache remitted, but eighteen months later a cluster attack and symptoms of cerebral ischemia from moyamoya recurred on the left. Again, following surgery on the left, both moyamoya symptoms and cluster attacks disappeared. Cluster headache secondary to moyamoya has not previously been described.
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PMID:Cluster headache associated with moyamoya. 1900 32

Compression of the brain by swollen temporal muscle used for indirect pial synangiosis is a rare complication after the revascularization surgery for moyamoya disease, and its mechanism and clinical presentation are undetermined. A 26-year-old woman, who had been suffering transient ischemic attack (TIA), underwent superficial temporal artery-middle cerebral artery anastomosis with encephalo-myo-synangiosis (EMS) on the affected hemisphere. The (123)I-IMP-SPECT 1 day after surgery demonstrated an improvement of cerebral blood flow (CBF) on the operated hemisphere. Two days later, however, she suffered fluctuating aphasia when computed tomography scan revealed marked swelling of the temporal muscle used for EMS. The (123)I-IMP-SPECT 4 days after surgery showed significant decrease in CBF by the compression of the brain. Then, we performed revision of EMS. The base of the temporal muscle was markedly compressed by the edge of the free bone flap, which resulted in swelling of the entire temporal muscle used for EMS. We drilled out the edge of the free bone flap for decompression. Her aphasia disappeared postoperatively, and CBF normalized 7 days after the initial surgery. Her TIA disappeared, and there was no deterioration during the follow-up period. The STA-MCA bypass has been patent since the initial surgery. Surgical revascularization including EMS has a substantial risk for cerebral ischemia owing to compression of the brain by temporal muscle swelling. Relative wide bone window for temporal muscle insertion is necessary to avoid this rare complication. Once the flow compromise is confirmed, we recommend early decompression by the revision of EMS.
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PMID:Cerebral ischemia owing to compression of the brain by swollen temporal muscle used for encephalo-myo-synangiosis in moyamoya disease. 1915 59

The surgical and endovascular treatment of cerebrovascular disorders (CVDs) in children, such as cerebral arteriovenous malformations, cavernous malformations, and moyamoya disease, have become commonplace and routine in many centers. As in the adult population, these procedures carry the risk of intraoperative cerebral ischemia. Therefore, similar strategies used to reduce the risk of cerebral ischemia in adults should be used in children. Unfortunately, there are no published studies on the intraoperative use of available techniques to identify, prevent, or potentially reverse cerebral ischemia. The goal of this article is to review the neurophysiologic techniques that may be useful and applicable in the surgical and endovascular treatment of pediatric CVDs, to describe the rationale and physiologic basis of their utility, to describe our experience in managing these cases, to present some of our results, and finally, to show the clinical utility of these techniques in the intraoperative management of CVDs.
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PMID:Neurophysiologic intraoperative monitoring of pediatric cerebrovascular surgery. 1927 96

The authors report on the case of a girl with cerebrovascular moyamoya disease born with severe respiratory failure caused by a congenital diaphragmatic hernia. Cardiopulmonary management included extracorporeal membrane oxygenation until the diaphragm defect was repaired. The right common carotid artery (CA) was interrupted and cannulated for extracorporeal membrane oxygenation. When she was 5 years of age, the patient experienced ischemic symptoms in her right extremities. Angiography revealed stenosis of the terminal portion of the internal CA (ICA) with the development of moyamoya vessels on the left side of the brain; the right ICA was supplied by extracranial anastomotic arteries. Indirect extracranial-intracranial bypass surgery was performed in the left hemisphere, and the hypoperfusion improved. The same change in the intracranial ICA with the development of moyamoya vessels occurred on her right side when she was 7 years old. Decreased cerebral blood flow occurred twice, and the moyamoya vessels developed to compensate for the cerebral ischemia. However, the occlusion of the extracranial common CA in infancy induced extracranial anastomosis rather than moyamoya vessel proliferation, and collateral circulation was formed at the lesion site. This finding indicates that neoangiogenesis requires both cerebral ischemia and growth factors derived from the lesion.
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PMID:Cerebrovascular moyamoya disease in a 5-year-old girl who underwent interruption of the right common carotid artery in the neonatal period. 1933 11

Seckel syndrome is an autosomal recessive disorder characterized by intrauterine and postnatal growth delay, microcephaly with mental retardation, and facial dysmorphisms including micrognathia, a recessed forehead, and a large beaked nose. Occurring in 1 in 10,000 children without sex preference, it is the most common primordial microcephalic osteodysplastic dwarfism and has been associated with a variety of congenital brain malformations and intracranial aneurysms. Moyamoya syndrome is an idiopathic, chronic, progressive cerebrovascular disorder marked by stenosis of the intracranial internal carotid arteries and concurrent development of hypertrophied collateral vessels. These tortuous arterial collaterals appear radiographically as "puffs of smoke," giving the syndrome its name. In this report, the authors describe the case of a 16-year-old girl with coincident Seckel and moyamoya syndromes. To their knowledge, this is the first reported case of such an association being treated with surgical revascularization. The patient presented with persistent headaches and a 2-year history of progressive hand, arm, and face numbness. Imaging studies revealed multiple completed cerebral infarcts, global ischemic changes, and vascular anatomy consistent with moyamoya syndrome. Bilateral pial synangioses successfully revascularized each hemisphere with resolution of the patient's symptoms. The patient died 1 year later of complications related to treatment of a rapidly progressing intracranial aneurysm. This report documents the first case associating moyamoya and Seckel syndromes. In addition, the report reveals the rapid development of an intracranial aneurysm in a patient with this syndrome. When coupled with previous reports of other types of cerebrovascular disease in patients with Seckel syndrome or other primordial dwarfisms, the authors' findings are important because they suggest that physicians treating patients with dwarfism should consider the diagnosis of moyamoya syndrome when symptoms suggestive of cerebral ischemia are present. Prompt diagnosis and treatment of moyamoya syndrome, including the use of proven surgical revascularization procedures such as pial synangiosis, may significantly improve the long-term outcomes of these patients.
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PMID:Seckel syndrome and moyamoya. 1933 12

Primary central nervous system vasculitis (PCNSV) and moyamoya disease (MMD) represent rare and poorly-understood causes of stroke. Both may present with similar clinical and auxiliary findings, but differentiation is extremely important because they require different treatment regimens. Our cohort included 21 white patients with PCNSV and 21 white patients with MMD. Clinical and diagnostic features were obtained by retrospective chart review; follow-up information and outcome were obtained prospectively. Data were compared between patients with PCNSV and MMD using Chi square test or Fisher's exact test for categorical data and Mann-Whitney U test for continuous data. The mean age at symptom onset was 42.48 years in PCNSV and 31.0 years in MMD (p = 0.008). All patients with MMD presented with ischemic events while cerebral ischemia was observed in only 14 of 21 patients (66.7%) with PCNSV (p = 0.004). There was no significant difference regarding the frequency of headaches, which represented an important symptom in both conditions. Conventional cerebral angiography verified correct diagnosis in 13 of 17 patients (76.5%) with PCNSV while angiogram verified correct diagnosis in all patients with MMD (p = 0.032). MRI and cerebrospinal fluid studies were appropriate to differentiate between the inflammatory and the non-inflammatory disease. Three PCNSV patients and two MMD patients died within documented follow-up. Despite important pathophysiological and angiographic differences, PCNSV and MMD may present with similar clinical and auxiliary findings. An intensive workup including MRI, conventional cerebral angiography and CSF studies is required to avoid misdiagnosis.
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PMID:Primary central nervous system vasculitis and moyamoya disease: similarities and differences. 2003 65

The usefulness of dynamic perfusion computerized tomography for early diagnosis of stroke is well established. However, limited data exists to assess the value of PCT in the evaluation of patients with chronic cerebral ischemia. The purpose of the study was to assess the utility of perfusion computerized tomography with acetazolamide challenge (PCTA) in the evaluation of cerebral perfusion and cerebrovascular reserve (CVR) in adult Moyamoya patients. From 2005-2008, 41 patients with Moyamoya were scrutinized by PCTA. Of these, 22 patients (12 females, ten males, mean age 41 years) with ischemic symptoms from Moyamoya disease were found to have no response or decreased cerebrovascular flow post-acetazolamide indicating compromised CVR. Eight had bilateral symptoms. Based on matching symptoms and decreased CVR on PCTA, those 22 patients underwent 30 superficial temporal artery-to-middle cerebral artery (STA-MCA) by-pass (eight with bilateral abnormal PCTA had bilateral STA-MCA by-pass). After a mean follow-up of 14 months (range, 3-36 months), no new strokes or symptoms were reported. Graft patency, as documented by postoperative computerized tomography angiography at a mean 12 weeks after surgery was 100%. Eight patients had postoperative PCTA documenting improved CVR after STA-MCA by-pass. At latest follow-up, none of the 19 patients with PCTA findings suggestive of normal CVR had any ischemic symptoms. CVR testing by means of PCTA may have a key role in patient selection for revascularization surgery and postoperative follow-up. These promising results warrant further testing of the technique.
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PMID:Patient selection for revascularization procedures in adult Moyamoya disease based on dynamic perfusion computerized tomography with acetazolamide challenge (PCTA). 2014 Jun 34

Aim of the study was to demonstrate the possibility of effective surgical revascularization of the brain in children with moya-moya disease. 3 year old girl with moya-moya disease underwent encephalo-duro-arteriosynangiosis. At 14 months follow-up signs and symptoms of cerebral ischemia totally resolved. Angiography confirmed brain supply from external carotid artery via the newly formed anastomotic network. Authors emphasize the necessity to seek moyamoya disease in children with symptoms of brain ischemia to allow timely and effective surgical treatment.
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PMID:[Successful treatment of moya-moya disease using encephalo-duro-arteriosynangiosis]. 2042 66

Moyamoya syndrome is an increasingly recognized arteriopathy associated with cerebral ischemia and has been associated with approximately 6% of childhood strokes. It is characterized by chronic progressive stenosis at the apices of the intracranial internal carotid arteries, including the proximal anterior cerebral arteries and middle cerebral arteries and ultimately results in decreased cerebral blood flow with an increased resultant risk of stroke. This article discusses the epidemiology, presentation, and diagnosis of this condition in children.
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PMID:Moyamoya: epidemiology, presentation, and diagnosis. 2056 2

Moyamoya disease is a chronically progressive and occlusive cerebrovascular disease. Steno-occlusion of the terminal portion of the internal carotid artery occurs bilaterally, and fine vascular network gradually develops at the base of the brain and they act as collaterals to the distal portion of the anterior and middle cerebral arteries. Steno-occlusion of the posterior cerebral artery develops in accord with progression of the disease. Intracranial omental transplantation is one of the indirect bypass surgeries for the treatment of cerebral ischemia, especially in the territories of the anterior/posterior cerebral artery in moyamoya disease. In the present report, one of sixty-seven moyamoya disease patients who had been treated with intracranial omental transplantation, showed chronological change of the mass of the transplanted omentum in accord with decrease/increase in the patient's body weight. In addition, the patient experienced headache which appeared and disappeared in accord with increase and decrease in the patient's body weight, respectively. The transplanted omentum was thought to act in the same way as fat tissue in other portions of the body. Measurement of body weight is thought to be as important as radiological examination using computed tomography/magnetic resonance imaging in the follow-up period after intracranial omental transplantation in moyamoya disease.
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PMID:[Chronological mass change of intracranial transplanted omentum in accord with decrease/increase in a patient's body weight--case report]. 2116 Jan 3


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