Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0025362 (mental retardation)
 15,878 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case of Turner's syndrome associated with Moyamoya disease in a 10-year-old female is presented. This patient had experienced two periods of syncope and transient right hemiparesis in 2 years. She was short in stature. She had cubitus vargus and mental retardation. The serum LH and FSH values were high. By chromosomal examination Turner's syndrome (karyotype: 45, X/46, X, i(Xq)) was diagnosed. An MRI (SE 2000/20) revealed abnormal vessels on the basal ganglia. Cerebral angiography showed occlusion of bilateral internal carotid arteries at the distal portion, bilateral posterior cerebral arteries at the proximal portion. Basal moyamoya well developed. EDAS (encephalo-duro-arterio-synangiosis) was performed bilaterally. We consider that this may be the first case report of Turner's syndrome associated with Moyamoya disease, and that there may be no relationship between Turner's syndrome and Moyamoya disease in this case.
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PMID:[A case of Turner's syndrome associated with moyamoya disease]. 140 55

A 4-year-old boy with right retinal hemorrhage, mental retardation, and multiple minor anomalies was referred to our hospital. Computed tomography scanning revealed a cystic brain tumor at the vermis. Angiography showed stenosis of both internal carotid arteries at the supraclinoid portion and the Moyamoya vessels. The right ophthalmic artery was dilated as wide as the internal carotid artery. Stenosis of the basilar artery was also observed. Collateral circulation via the posterior inferior cerebellar artery and Moyamoya vessels in the area of the posterior cerebral artery was observed.
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PMID:Atypical Moyamoya disease associated with brain tumor. 172 83

Ischemic cerebrovascular disease in children is relatively rare. To clarify the clinical features of ischemic stroke occurring in infants and children, we evaluated 54 cases of cerebral infarction, excluding cases of moyamoya disease, in patients less than 16 years old at 24 clinics in the Tohoku (northeast) district of Japan. We observed two incidence peaks, one in little children and the other in junior high school students. Infection and minor head trauma were more frequently seen prior to ischemic strokes than was heart disease. The middle cerebral artery region, including the basal ganglia, was most commonly affected (49 patients, 91%) on computed tomograms. Angiography was performed in 48 patients (89%) and showed various types of occlusive lesions, mostly affecting the middle cerebral artery. Hemiparesis was the most common form of disability following ischemic strokes (48 patients, 89%). Surgical treatment was carried out in seven patients (13%). The clinical course of these cases showed that the recovery of children after a stroke tends to be better than that of adults, but that permanent disabilities, such as hemiparesis or mental retardation, occur commonly. Further investigation of juvenile cerebrovascular disease is important to prevent ischemic strokes in children.
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PMID:Clinical survey of ischemic cerebrovascular disease in children in a district of Japan. 202 86

The authors have reported here fraternal twins of moyamoya disease. The one has the onset at the age of two years and six months. Then he had suffered from multiple cerebral infarction and resulting in severe neurological deficits. Now he has right hemiparesis, left homonymous hemianopsia, aphasia and mental retardation. The encephalomyo synangiosis was done to the boy bilaterally at the age of five years. The other one has the onset at the age of five years and five months. He had good physical and neurological development. The Superficial temporal artery-Middle cerebral artery anastomosis and Encephalomyo synangiosis were done bilaterally. Now his development has no problems. The twins and their younger sister all have the same HLA type. The hereditary and environmental factors may be completely related to the pathogenesis of this disease.
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PMID:[Moyamoya disease in fraternal twins]. 207 55

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

A case of adult Moyamoya disease, with formation of a transcranial external carotid-internal carotid (EC-IC) anastomosis through burr holes which had been made previously. A 43-year-old male suffered sudden headache and vomiting. Neurological examination revealed mild consciousness disturbance and dysarthria. The computed tomography (CT) scans showed intraventricular hemorrhage, which was drained through burr holes bifrontally. The diagnosis of Moyamoya disease was subsequently made by cerebral angiography. A month later he was discharged with mild gait disturbance and mental retardation. Seven years later he suddenly complained of gait disturbance, dysarthria and sensory disturbance involving the right upper extremity. A CT scan revealed a small hemorrhage in the left putamen. Carotid angiograms disclosed transcranial EC-IC anastomosis through the burr holes which had been made previously. It is suggested that revascularization can be expected after opening burr holes and incising the dura matter for Moyamoya disease in adults as well as, possibly, in children.
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PMID:[Adult moyamoya disease with a transcranial internal carotid-external carotid (EC-IC) anastomosis through burr holes]. 237 49

Authors review 53 children, aged 0 to 14 years, affected with cerebrovascular ischemic strokes. Largest aetiological groups were: a) congenital heart disease, 16 patients; b) arteritis of unknown cause, 11; c) idiopathic arterial occlusion without arteritis images on angiography, 7; d) moyamoya disease, 6; and d) local or systemic infections, 5. The mode of onset was as completed stroke in 72% and stroke in evolution in 24%. After acute stage 17.6% of patients presented other definitive strokes, 11.7% suffered only transient ischemic strokes (TIA), and 4% reversible ischemic neurologic deficits (RIND). Mean follow-up was 4.36 years, 9.8% of patients died, 11.8% recovered completely and 52.9% improved after initial stroke. Poor global evolution was associated with heart disease (p less than 0.05) and with onset of strokes before age 2 (p less than 0.05). Most important sequelae, besides motor impairment, were epilepsy (49%) and mental retardation (50% got less than IQ 80). Late epilepsy was associated with seizures at onset (p less than 0.05). Clinical factors of adverse mental development were: a) seizures at onset, b) late epilepsy and c) stroke before age 2. 66% of cases had two or more arterial lesions in the same or in different arterial trees. Therefore, embolic and arteritic factors probably play an important role in infancy and childhood stroke.
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PMID:[Ischemic cerebrovascular accidents in childhood]. 340 Sep 36

We have reported the characteristic dynamic changes at the base of the brain in childhood Moyamoya disease in terms of follow-up angiography. So we have classified the angiographical findings of Moyamoya disease into six stages. However, there have been no report that proved these findings in long-term follow-up. On the other hand, these angiographical findings are mainly observed in children, we can seldom to find out any changes in adults. We performed long-term follow up angiography in adult cases whose onset was in childhood to clarify the natural course of this disease and to understand the difference between the cases of children and adults. Eleven cases (4 males and 7 females) of Moyamoya disease were investigated by angiography. Average onset was 5.1 years old. All of them were diagnosed by initial angiography in childhood (average age were 6.5 years old) and they grew up into adolescence (average age were 18.6 years old) at the time of follow-up study. Initial symptoms of them were all ischemic (TIA 7, RIND 2 or completed stroke 2). The symptoms at the time of follow-up were mental retardation in 5 patients, slight neurological deficits in 1, TIA in 2 and normal in 3. Among them, one patient had suffered from intraventricular hemorrhage. The results were as follows: the progression of angiographical stages was observed in 95% of sides. In 71% of sides, angiographical stage was in 5 or 6 which is thought to be an end stage of this disease.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Long-term follow-up angiography of moyamoya disease--cases followed from childhood to adolescence]. 395 60

Moyamoya syndrome is defined as the development of collateral anastomosis pathways at the base of the brain, associated with chronic progressive stenosis of the carotid fork. Both reconstructive vascular surgery and conservative strategies are used to treat this syndrome, but the latter cannot prevent the disease from progressing. We describe the procedure of encephalo-duro-arterio-myo-synangiosis (EDAMS), and report the results in 17 patients (28 sides) who underwent EDAMS. The clinical symptoms of moyamoya disease include transient ischemic attacks, reversible ischemic neurological deficits, stroke, seizures, Gerstmann's syndrome, involuntary movements, or mental retardation resulting from the lack of cerebral blood flow. The clinical results of EDAMS were poor in one patient, fair in two, good in five, excellent in eight, and fair on one side and excellent on the other side in one patient. Postoperative angiograms showed widespread collateral circulation on the ischemic brain surface in patients undergoing EDAMS.
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PMID:Surgical treatment of moyamoya disease: operative technique for encephalo-duro-arterio-myo-synangiosis, its follow-up, clinical results, and angiograms. 847 42

A new technique of indirect revascularization using the dural arterial supply to provide donor vessels is presented together with the results of an assay performed to determine the angiogenic activity of the dura. At surgery, a portion of the dura near the branches of the middle meningeal artery was split into outer and inner layers, and the split surface of the outer layer was attached to the cortical surface. This procedure, combined a with standard encephaloduroarteriosynangiosis, was applied to 25 hemispheres in 18 patients with pediatric moyamoya disease (mean age 6 years). All of the patients were symptom free by 1.5 years after surgery. The follow-up period ranged from 1 to 12 years (mean 6.5 years). Thirteen (81%) of 16 patients were able to lead normal lives and three were mildly handicapped due to mental retardation that existed preoperatively. Postoperative superselective angiograms demonstrated effective cortical revascularization through the dural arteries as well as from the scalp arteries. A histological study of the dura in cases of moyamoya disease showed an increased number of blood vessels in the outer layer. Angiogenic activity determined by chorioallantoic membrane assay was higher in the split surface of the dura than in the internal surface of the dura (the natural interface between the dura and cortex). The split duroencephalosynangiosis described in this report is a useful addition to indirect revascularization techniques, allowing extension of the area of revascularization in the ischemic hemispheres of patients with moyamoya disease.
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PMID:Use of a split dura for revascularization of ischemic hemispheres in moyamoya disease. 904 24


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