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)

A child with neurofibromatosis had hypertension and acute hemiplegia resulting from both renal and cerebral vascular occlusive disease. This case illustrates the importance of recognizing surgical antihypertensive therapy was attempted, and may have contributed to rapid neurologic deterioration. Children with neurofibromatosis and hypertension require a careful neurologic evaluation, including computed tomography, before therapy.
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PMID:Childhood hypertensive stroke with neurofibromatosis. 677 Feb 88

Moya-Moya disease is an obstruction of the internal carotids and of the afferent and efferent channels of Willis's polygon, which causes a collateral circulation, responsible for the typical angiographic image of a "puff of smoke" (Moya-Moya, in Japanese). Its etiology is unknown, and it might be congenital or acquired. It has been associated with congenital diseases such as Neurofibromatosis and disease of the falciform cells, among others. It usually appears as an alternating hemiplegia. We report here on the case of a 4-year-old boy with alternating hemiplegia (initially on the left, later on the right and then back on the left) and angiographic alterations which are characteristic of Moya-Moya. This patient had been examined in our hospital in the neonatal period as he had malformative stigmas, basically consistent with a flat angioma on the forehead, upper eyelids, and base of the nose, a previous wide fontanella which expanded to the whole of the methopic suture, aplasia, forehead level skin and abnormal implantation of the skin in that region. What draws attention in this case is the association of middle line craniofacial lesions (angioma, skin aplasia) and the later development of Moya-Moya.
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PMID:[Moya-moya disease in a child with multiple malformations]. 749

The authors report the case of a 39-year-old woman with type I neurofibromatosis who presented a right incomplete proportional hemiplegia which progressively worsened over a 6-month period. Left hemispheric atrophy with heterogeneous features, predominant in the temporoparietal region, was revealed by computerized tomography. Atrophy was associated with diffuse vascular lesions in the distal part of the left sylvian and anterior cerebral arteries, leading to major cortical hypoperfusion. Vascular examination showed no hypertension nor any sign of arterial involvement in another region. This case illustrates the nature of vasculopathy associated with neurofibromatosis. Its expression is polymorphous, with lesions inducing stenosis (the most common ones), aneurysmal lesions or veritable angiodysplasias (either hypo- or hyperplastic). The vascular expression of neurofibromatosis is often overlooked. However, in the presence of an inexplicable occlusive or aneurysmal vasculopathy, it is advisable to search for signs of neurofibromatosis since ill-defined forms exist.
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PMID:[Cerebral atrophy of vascular origin in the course of neurofibromatosis]. 785 77

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

Renovascular disease accounts for 8-10% of all cases of paediatric hypertension, whereas, in adults, its incidence is approximately 1%. The Turkish Paediatric Hypertension Group aimed to create the first registry database for childhood renovascular hypertension in Turkey. Twenty of the 28 paediatric nephrology centres in Turkey responded to the survey and reported 45 patients (27 girls, 18 boys) with renovascular hypertension between 1990 and 2005. The age at presentation ranged from 20 days to 17 years. The mean blood pressure at the diagnosis was 169/110 mmHg. Chief complaints of symptomatic patients were headache (38%), seizure (18%), epistaxis (4%), growth retardation (4%), cognitive dysfunction (4%), polyuria (2%), palpitation (2%), and hemiplegia (2%). Renovascular hypertension was found incidentally in 11 children. The diagnosis of renovascular hypertension was established with conventional angiography in 39 patients, MR angiography in three, CT angiography in two, and captopril diethylene triamine penta-acetic acid (DTPA) scintigraphy in one patient. Twenty-one children had bilateral renal artery stenosis and 24 had unilateral renal artery stenosis. Of these, 14 (31%) had fibromuscular dysplasia; 12 (27%) Takayasu's arteritis; six (13%) neurofibromatosis; two (5%) Williams syndrome; one (2%) Kawasaki disease; one (2%) mid-aortic syndrome; one (2%) extrinsic compression to the renal artery, and eight (18%) unspecified bilateral renal artery stenosis. Hypertension was controlled with antihypertensive drugs in 17 patients. Percutaneous transluminal angioplasty (PTRA) or surgery had to be performed in 28 patients: PTRA in 16 patients, PTRA + surgery in one patient and surgery in 11 patients (four nephrectomies). The importance of vasculitic disease, especially Takayasu's arteritis, should not be underestimated in children with renovascular hypertension.
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PMID:Reno-vascular hypertension in childhood: a nationwide survey. 1753 66