Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0022116 (ischemia)
 91,303 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We investigated the role of ischemic hypoxia in the appearance of re-build-up phenomenon on electroencephalography in childhood moyamoya disease using 99mTc-hexamethyl propyleneamine oxime and single photon emission computed tomography (99mTc-HMPAO SPECT). In the case reported on, critical reduction of regional cerebral blood flow (rCBF) was observed during re-build-up phenomenon in the bilateral parieto-occipital area where acetazolamide testing revealed severe impairment of the perfusion reserve. On electroencephalography, the re-build-up phenomenon originated in these areas. In addition, re-build-up phenomenon developed into the entire hemisphere 30 s later, even where the severe ischemia was not observed. These results suggest that rCBF reduction induced by hyperventilation plays a critical role in the appearance of the re-build-up phenomenon, but other factors such as ischemic hypoxia after hyperventilation are also important in the development of this phenomenon.
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PMID:Regional cerebral hemodynamics during re-build-up phenomenon in childhood moyamoya disease. An analysis using 99mTc-HMPAO SPECT. 869 60

Ocular ischemic syndrome is extremely rare in childhood. Patients with moyamoya disease may be particularly susceptible to the development of ocular ischemia due to the associated carotid occlusion. A 19-month-old boy presented with neurofibromatosis and signs of ocular ischemia. At 29 months of age, he developed dense right vitreous hemorrhage and eventually lost vision in that eye due to phthisis. At almost six years of age, he developed an acute hemiplegia and was then diagnosed with moyamoya disease. This rare instance of childhood ocular ischemia in conjunction with moyamoya disease and neurofibromatosis demonstrates the serious ocular and systemic sequelae of occlusive vascular disease.
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PMID:Ocular ischemic syndrome in a child with moyamoya disease and neurofibromatosis. 872 42

The first case of a child with an arteriovenous malformation (AVM) associated with moyamoya disease is reported. The patient presented ischemic symptoms and underwent indirect bypass surgery on both sides when she was 5 years old. Four years later she suffered from headache, and a small AVM of the left frontal lobe associated with the moyamoya vessels was detected. Single photon emission computed tomography (SPECT) was performed at age 11 and demonstrated low local cerebral blood flow (CBF) in the left frontal lobe and right temporal lobe, although the revascularization after the bypass surgery seemed to be effective, as judged on pancerebral angiography. We feel that brain ischemia due to the moyamoya disease may have played a causative role in the development of the AVM.
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PMID:Arteriovenous malformation associated with moyamoya disease. 929 67

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

Although parietal EDAS or STA-MCA anastomosis are effective in pediatric moyamoya disease, they do not adequately prevent ischemia in the frontal and occipital lobes. Some additional methods that can prevent ischemia in the frontal and occipital lobes are sometimes needed. We investigated whether EDAS using a frontal branch of the superficial temporal artery (frontal EDAS) or EDAS using the occipital artery (occipital EDAS) is preferable. Frontal or occipital EDAS was performed at 15 sites in seven patients with pediatric moyamoya disease. The outcome was estimated by angiography 3 months later, CT findings 3 months later, neurological findings during the follow up period and perioperative complications. The mean follow up period was 14 +/- 6 months after frontal or occipital EDAS. As results, good revascularization from frontal or occipital EDAS was shown in ten of fourteen surgical sites (71%) in angiography. None of the patients showed deterioration of symptoms after frontal or occipital EDAS during the follow up period. None of the patients developed surgical complications. In conclusion, multiple EDAS using the frontal branch of STA and the occipital artery is an effective and safe method for preventing ischemia in the frontal and occipital lobe in pediatric moyamoya disease.
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PMID:Multiple EDAS (encephalo-duro-arterio-synangiosis). Additional EDAS using the frontal branch of the superficial temporal artery (STA) and the occipital artery for pediatric moyamoya patients in whom EDAS using the parietal branch of STA was insufficient. 920 58

We describe two Caucasian women with the concurrence of Graves' disease and the moyamoya phenomenon (radiological evidence of collateral cerebral blood vessels like "puffs of smoke" due to cerebrovascular occlusive disease). One patient presented with acute cerebrovascular ischemia due to Moyamoya disease shortly after radioactive iodine therapy for Graves' disease and the second presented with Graves' disease 10 years after being diagnosed with moyamoya dysplastic cerebral vessels. The optimal treatment of hyperthyroidism in these patients is unknown; however, careful control of the hyperthyroidism by any modality seems reasonable. Our limited experience suggests that antithyroid drugs and radioactive iodine therapy are rational options. Thyroidectomy appears to be a safe therapeutic alternative, although long-term efficacy may be difficult to assure. Both of our patients had to be treated twice for hyperthyroidism. Whether Graves' disease and Moyamoya coexist because of an aggressive autoimmune mechanism is a concept that remains to be settled.
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PMID:Concurrence of Graves' disease and dysplastic cerebral blood vessels of the moyamoya variety. 929 53

The present study was undertaken to reach a clearer understanding of the natural history of Moyamoya disease. Follow-up studies were performed in 88 patients with Moyamoya disease. They were divided into the ischemia group and the hemorrhage group. The activity of daily living (ADL) of each groups were followed up and compared between those who were surgically treated and conservatively managed. Ischemic manifestations were more common in the younger children and tended to be recurrent, whereas hemorrhagic manifestations were more common in the adults. Follow-up duration of the 36 patients, who were surgically treated, ranged from 6 to 86.4 months (mean: 28.8 months). During the follow-up period, ADL was improved in 17 of 31 ischemic Moyamoya patients (55%); the condition was unchanged in nine (29%); and aggravated in five (16%). Follow-up duration of the 52 patients who were managed without surgery ranged from 12 to 216 months (mean: 67.2 months). In 35 patients of the ischemia group, ADL was aggravated in 49% and improved in only 26% during the follow-up period. However, ADL was aggravated in 12% of 17 hemorrhagic patients, but improved in 53%. Our result suggest that indirect revascularization procedures are effective for prevention of recurrent ischemic attacks which is common in pediatric patients. However, the effectiveness of indirect revascularization for hemorrhagic Moyamoya disease is not clear and requires extended follow-up study.
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PMID:Natural history of moyamoya disease: comparison of activity of daily living in surgery and non surgery groups. 940 97

Over the past 28 years, 39 patients with Moyamoya disease or syndrome defined as spontaneous occlusion of the circle of Willis with extensive basal collateral vessels have been treated by the author in Canada and the USA. All patients presented with clinical or radiologic evidence of hemorrhage (23) or ischemia and infarction (16). A total of 12 patients had associated cerebral aneurysms and seven of these patients with aneurysms presented with subarachnoid hemorrhage. The patients ages ranged from 5 to 47 years. Of these 58% were female. The patients racial origin included North American Indian, Innuit, East Indian/Pakistani, Japanese, Chinese, Filipino, Korean, Malayasian, Hispanic, African American and Caucasian. Familial clustering was seen in North American Indian, Innuit and Caucasian patients. Associated disorders (tuberculosis, pharyngitis, thalassemia, fibromuscular hyperplasia, polycystic kidney, sickle cell trait and hypertension) were common in these patients, as was the use of tobacco, alcohol and in the adult females, oral contraceptives. It may be concluded from this series that the etiology of Moyamoya disease or syndrome is probably multifactorial, but that some racial and familial groups are more susceptible. Furthermore, in that the clinical and angiographic features are identical, the separation between Moyamoya disease and syndrome may not be helpful in understanding the etiology and pathophysiology of this disorder.
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PMID:Risk factors of moyamoya disease in Canada and the USA. 940 4

Moyamoya syndrome is a vaso-occlusive disease involving the intracranial vessels of the circle of Willis which is accompanied by an intense compensatory recruitment of new vessels. Angiogenic substances such as basic fibroblast growth factor (bFGF) present in the cerebrospinal fluid (CSF) have been proposed as possible mediators of the neovascular response. We analyzed CSF samples collected intraoperatively from predominantly pediatric patients with moyamoya and other conditions such as Chiari malformation (Ch), tethered cord (TC), arteriovenous malformation (AVM), brain tumor (BT) and hydrocephalus (HCP). We found that CSF bFGF was significantly elevated in patients with moyamoya (141 pg/ml, n = 37), Ch (56.7 pg/ml, n = 22), TC (55.1 pg/ml, n = 23), AVM (354 pg/ml, n = 5), and BT (208 pg/ml, n = 5) compared to patients with HCP (5.5 pg/ml, n = 7) and controls (1.6 pg/ml, n = 25; p < 0.05). There was no dependence of CSF bFGF on patient age or gender. Although CSF bFGF in the moyamoya group showed no correlation with the Suzuki radiographic stage at either pre- or post-operative (1-year follow-up) angiography, it showed a trend with the Matsushima angiographic score with increasing collateral vascularization from the synangiosis developing at higher levels of CSF bFGF. Our findings suggest that CSF bFGF may be playing a wide-ranging role in a number of central nervous system conditions associated with ischemia and hypervascularity. Although not a specific marker for moyamoya, elevated CSF bFGF may serve as a weak predictor of the extent of angiogenesis to be expected in indirect revascularization procedures.
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PMID:Elevation of cerebrospinal fluid levels of basic fibroblast growth factor in moyamoya and central nervous system disorders. 957 71

Schimke immunoosseous dysplasia (SID) is an autosomal recessive spondyloepiphyseal dysplasia that was first described by Schimke et al. [1971: Lancet 2:1088-1089]. It is associated with premature arteriosclerosis and cerebral ischemia; however, the cerebral vascular abnormalities causing ischemia have not been described [Spranger et al., 1991: J Pediatr 119:64-72; Ehrich et al., 1995: Clin Nephrol 43:89-95]. Based on magnetic resonance angiography (MRA) and magnetic resonance venography (MRV), we now report on 2 girls with SID who have cerebral ischemia associated with moyamoya phenomenon. In addition, one patient also has an absent or occluded left transverse sinus and diffuse aortic narrowing. This is the first characterization of the cerebral vascular abnormality found in SID and raises the possibility that cerebral moyamoya may represent another major manifestation of the underlying genetic defect in SID.
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PMID:Schimke immunoosseous dysplasia complicated by moyamoya phenomenon. 967


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