Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018991 (hemiplegia)
 3,997 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We are reporting what we believe to be the first case of moya moya disease (hemiplegia associated with supraclinoid carotid stenosis and multiple cerebral telangiectasia) in a child with Down's syndrome. On cerebral angiography, multiple collateral vessels and rete mirabile (anastomosis of meningeal vessels with internal cerebral vessels) were noted, in addition to the supraclinoid carotid stenosis. Computerized tomography revealed nonobstructive hydrocephalus and findings consistent with multiple vascular insults or infarcts. It is not clear whether moya moya disease represents a true disease entity (congenital arterial dysplasia) or is a syndrome caused by nonspecific vascular reaction. Since abnormal vascular morphology has previously been described in children with trisomy 21, we suggest that the presence of these two disease entities may not be coincidental. It may represent a genetic predisposition in Down's syndrome toward vascular abnormalities, with variable expressivity which manifested itself in this case by abnormalities in the cerebral circulation.
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PMID:Acute hemiplegia and cortical blindness due to moya moya disease: report of a case in a child with Down's syndrome. 14 47

In a 32-year-old woman, cerebral angiography showed fibromuscular dysplasia of the left anterior and middle cerebral arteries. She had had epilepsy and subarachnoid hemorrhages with subsequent hemiplegia. Since she also had multiple enchondromas (Ollier disease), this may represent a case of Maffucci syndrome.
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PMID:Fibromuscular dysplasia of intracranial arteries in a patient with multiple enchondromas (Ollier disease). 56 16

Two children had abrupt onset of hemiplegia due to childhood fibromuscular dysplasia (FMD). Although FMD is a recognized cause of strokes in adults, the disorder has not, to our knowledge, been reported previously in children. Our report reviews the pathology and diagnosis of FMD and discusses the complexities of its treatment.
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PMID:Fibromuscular dysplasia as a cause of stroke in infancy and childhood. 86 42

A 16-year-old boy, who had sudden left-sided hemiplegia, died two weeks following onset of symptoms. A right carotid angiogram showed stenosis at the termination of the internal carotid artery. The middle cerebral artery had a beaded appearance and some of its branches were occluded. A basal "moyamoya" syndrome and transdural anastomoses were present. At autopsy, multiple intracranial dissecting aneurysms were found. Arteries of the body displayed fibromuscular dysplasia (FMD). The relevance of dysplastic changes of intracranial arteries and the relationship to moyamoya syndrome are discussed.
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PMID:Fibromuscular dysplasia and multiple dissecting aneurysms of intracranial arteries. A further cause of Moyamoya syndrome. 96 Jan 59

Moya-Moya disease has been associated to a number of disease entities including Down syndrome. Initial clinical manifestations in our patient were alternating lateralizing seizures with sudden onset hemiparesis. Ct scan demonstrated several infarcts in different stages of evolution, in the territory of left middle cerebral artery. Single digital subtraction angiography showed bilateral occlusion, predominantly on the left side of the supraclinoid portion of the interna carotid arteries with formation of collateral circulation in the diencephalic territory. The association of Moya-Moya disease and Down syndrome is not fortuitous and it is probably due to a congenital vascular dysplasia. It should be suspected in children with trisomy 21 (with or without congenital heart disease) who presents with alternating hemiplegia and convulsions or acute hemiplegia.
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PMID:[Acute hemiplegia in childhood and alternating hemiconvulsions secondary to Moya-Moya disease. Report of a case associated with Down's syndrome]. 228 71

A 15-year-old boy suddenly developed a headache, followed by progressive weakness of the right extremities. A computed tomographic scan obtained on admission showed a low-density area in the left putamen extending to the internal capsule. No neurological deficits were observed other than slight dysarthria and right hemiparesis. Left carotid angiography showed the characteristic "string of beads" deformity of fibromuscular dysplasia (FMD) extracranially and complete occlusion of the internal carotid artery at the C2 portion. The right carotid artery and both vertebral arteries were normal. Anastomosis of the left superficial temporal and middle cerebral arteries was performed 1 month after the onset. The patient recovered quickly from the hemiplegia and was discharged with no neurological deficits. Angiograms obtained 3 months later showed not only patency of the bypass, but also recanalization of the intracranial internal carotid artery. Cervico-cephalic FMD mainly affects middle-aged women, and is uncommon in children; only nine childhood cases have been reported. This disorder may play an important role in the genesis of strokes among children.
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PMID:[Fibromuscular dysplasia with cerebral infarction in children. Case report]. 248 41

Neurological complications of arterial hypertension are analyzed in 31 children (mean age = 9 years). All patients presented a renal or renovascular disease (acute nephritis + hypoplastic dysplasia , transplantation = 58%) for which malignant hypertension was the first symptom in 16%. The mean +/- SD initial blood pressure was 189 +/- 33/113 +/- 25 mm Hg and was preceded by previous symptoms in 1 patient out of 6. Neurological abnormalities consisted in seizures (48%), acute intracranial hypertension (39%), cranial palsy (23%), coma (19%), hemiplegia/paresia (16%), retinal changes (6%) or aphasia (6%). The EEG was abnormal in 50% of the patients, sometimes showing permanent paroxysmal activity. Neuroradiologic investigations revealed hemorrhagic and/or ischemic lesions in 1/5 patients. On follow-up, hypertension disappeared in 41% of the children; a decrease in renal function was noted in 56% of the patients at the last examination; neurological sequellae were present in 40% (EEG anomalies +/- epilepsy, motor deficit, retinal changes, psychomotor delay, cranial palsy) and 1 patient died. The morbidity of malignant hypertension stresses the importance of early diagnosis and treatment (calcium channel blockers) when its prevention is not possible.
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PMID:[Neurologic manifestations of arterial hypertension in children]. 305 3

A case of acute infantile hemiplegia, probably in co-occurrence with intracranial fibromuscular dysplasia, is reported. A review of the literature is given and some remarks are made concerning the management of this disease.
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PMID:Fibromuscular dysplasia as a cause of acute infantile hemiplegia. 384 55

We have studied the natural history of spontaneous dislocation of the hip in cerebral palsy, with particular reference to the pattern of neurological involvement. In patients with bilateral hemiplegia and severe involvement of the upper limbs the incidence of dislocation was very high (59%), while in those with diplegia and little involvement of the upper limbs, only 6.5% were affected. There was no evidence of dysplasia or instability of the hip in any of the patients with unilateral hemiplegia. A strong correlation was found between the stability of the hip and the patients' ability to walk. These findings have a bearing on clinical surveillance and also on the indications for prophylactic surgery.
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PMID:Factors affecting the incidence of hip dislocation in cerebral palsy. 403 Aug 44

Fibromuscular dysplasia of the internal carotid artery is the most frequent extracranial localization of the disease. It can produce TIA or cerebral infarct through formation of fibrinous thrombi or complete occlusion of the artery. Seven cases are presented with disease localized in the distal segment of the carotid artery, usually considered inaccessible through standard exposure. A surgical approach is described to treat these lesions by performing a mandible osteotomy. This allows a resection of the internal carotid and its replacement with autologous saphenous vein graft as performed in six cases. The distal anastomosis was performed 1 or 2 cm. below the base of the skull. One case could not be corrected due to disease extending into the skull. All patients were operated on for TIA and one had a cerebral infarct. Six patients had an uneventful recovery and no further neurological symptoms. One patient had a postoperative hemiplegia. Pathologic specimens were described as fibromuscular dysplasia in all cases. Three of them had also a dissecting aneurysm, two of these also showed a ruptured intima. Intraluminal dilatation is regarded as a potentially risky procedure; resection and replacement through a mandible osteotomy is recommended for very distal internal carotid lesions.
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PMID:Fibromuscular dysplasia of the distal cervical internal carotid artery. 727 76


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