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)

Cerebrovascular accidents occurred in three children with homozygous sickle cell anemia. The first case was that of a young girl with three carotid aneurysms who developed a meningeal hemorrhage. Angiography in the second child, who had had recurrent hemiplegias, showed multiple stenoses of the cerebral arteries, with a moya-moya appearance. The third case was a boy with hemiplegia and signs of a lesion in the sylvian region on computed brain tomography. The outcome was favorable in cases 1 and 3, but there were marked sequelae in the second case. Published reports indicate a different frequency for these accidents in the USA (17 to 26 p. cent) and black Africa (less than 5 p. cent). The mechanism of these accidents is discussed. The presence of multiple aneurysms may be only coincidental, but they could result from the very widespread arterial lesions observed in sickle cell anemia. The moya-moya disease in this case is related to the fibromuscular dysplasia of the cerebral arteries occurring during homozygous sickle cell anemia. It is reported fairly frequently in the published literature and appears to run a more severe course than non-drepanocytic moya-moya cases, with many deaths and marked neurological sequelae. Care is required when conducting cerebral arteriography in order to detect these lesions in cases of homozygous sickle cell anemia, especially when computed brain tomography is not possible or does not supply confirmation. Exchange transfusion can prepare these patients for arteriography by reducing hemoglobins levels to 20 p. cent, and it is apparently also a useful adjuvant during therapy of these accidents.
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PMID:[Cerebrovascular accidents in children with homozygous sickle cell anemia: report on three cases (author's transl)]. 729 86

Four children with a complex brain malformation including absence of the septum pellucidum and porencephalies are reported. All patients presented with congenital hemiplegia or diplegia and mild to severe mental retardation. Ocular motor palsy and epilepsy were additional findings in two children. The diagnosis was made by pneumoencephalography (two patients), by CT scan (one patient) or by both techniques ((one patient). The neuroradiological images, especially those obtained by CT scan, are described. Previously reported cases with pathological confirmation are reviewed and compared with the present cases. The nosological situation of this syndrome is discussed in relation with holoprosencephaly and septo-optic dysplasia. We conclude that absence of the septum with porencephaly is a distinct and clinically recognizable syndrome.
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PMID:The syndrome of absence of the septum pellucidum with porencephalies and other developmental defects. 733 57

Stroke although rare in children, is an important cause of morbidity in the paediatric age group. Over a period of 8 years, 43 children (17 boys and 26 girls) in the age groups of 1-16 years (mean 8.02 yrs) presented with stroke which constituted 10% of all strokes in the young and 0.7% of all paediatric admissions. The chief clinical features were hemiplegia (86%), convulsions (27%), fever (23%), dysphasia (23%), headache (11%) and altered level of consciousness (11%). Routine laboratory tests were non-contributory. Cranial computerized tomography (CCT) on 21 patients was abnormal in 95% and was useful in revealing the extent of infarction. Infarction was confined to middle cerebral artery territory, often involving basal ganglionic structures and was associated with focal or diffuse atrophy. Angiograms were abnormal in 78% of the patients (18/23) and were complimentary to the CCT. Etiological factors identified were: Moya-moya disease 6, arteritis 5, fibromuscular dysplasia 2, scorpion sting 2, and venous sinus thrombosis and small vessel occlusion one each. Though 23% of the patients had fever at onset, no obvious evidence of systemic or CNS infection was noticed. Stroke in children continues to pose a diagnostic challenge.
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PMID:Cerebrovascular disease in children. 783 10

Over a 15-month period ending in July 1992, 9 patients (7 male, 2 female; mean age 67 years) with recurrent stenosis of the internal carotid artery underwent transluminal angioplasty (TLA). The mean interval between endarterectomy and treatment of recurrent stenosis was 45 months (range: 9 to 84 months). All patients were symptomatic except one with extensive bilateral carotid dysplasia. Three patients had recurrence proximal to the endarterectomized segment (Group I); in one of these patients the narrowing was extensive. The other 6 patients (Group II) demonstrated stenoses distally. In one of these latter patients, the narrowing developed in a vein bypass. Balloon dilation was performed by the surgical route in 3 patients and percutaneously in the other 6. Perioperative transcranial Doppler (TCD) monitoring was employed in all procedures. Postoperative treatment consisted of oral Ticlopicine. In Group I (proximal recurrence), immediate complications included one case of reversible spasm and two dissections that led to acute thrombosis treated by emergency bypass and to sylvian artery embolism complicated by transient hemiplegia. In Group II (distal disease), dissection was not encountered, and only one case of transient neurologic manifestations due to cerebral edema following reperfusion was observed. Mean follow-up has been 18 months (range: 9 to 24 months). All patients are presently asymptomatic. In Group II, 2 patients presented with secondary recurrence at 6 months and were treated again by angioplasty. In one of these cases, a Palmaz stent was placed to prevent restenosis by elastic recoil.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Transluminal angioplasty for recurrent stenosis after carotid endarterectomy. Prognostic factors and indications. 790 85

A 44-year-old patient, without remarkable medical history, was admitted with a head trauma with initial loss of consciousness and a thoracic trauma. The initial treatment included the insertion of a chest drain for evacuation of a pneumothorax and intrapleural analgesia with bupivacaine. The day after admission, the patient experienced a generalized epileptic crisis, without prodomes. Later, a left proportional hemiplegia with aphasia was recognized. The CT scan obtained immediately after the crisis, as well as the carotid Doppler ultrasonography and echocardiography were normal. The bilateral carotid angiography showed an image of fibromuscular dysplasia of the extracranial segment of the right internal carotid artery. The migration of a carotid thrombus initiated by the trauma was hypothetized. A treatment with a platelet aggregation inhibiting drug was started and associated 20 days later with low molecular weight heparin. The patient recovered a normal motility within 10 days; only the aphasia remained. Trauma of the carotid artery is not a frequent cause of cerebrovascular accident. The occurrence of the latter is favoured by a pre-existing lesion of this artery. This case demonstrates that in a trauma patient not all central nervous system manifestations are initiated by a head trauma.
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PMID:[Post-traumatic hemiplegia in a patient with fibromuscular dysplasia of the carotid artery]. 831 57

Functional hemispherectomy, indicated for the control of pharmacologically refractory seizures, has been used at the Montreal Neurological Hospital since 1974. We have used this technique in 18 children suffering from intractable seizures secondary to conditions such as infantile hemiplegia, chronic encephalitis, head trauma, cerebrovascular accident, brain dysplasia and Sturge-Weber angiomatosis. None has developed superficial cerebral hemosiderosis often seen following the classical anatomical hemispherectomy. Eighty-two per cent (82%) of patients have been seizure-free since hospital discharge while another 11.5% have had at least 80% reduction in their seizure frequency. Most patients have shown an improvement in their intellectual capacity and sociability.
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PMID:Functional hemispherectomy in children. 847 13

Among the variable manifesting conditions of neuronal migration disorders, mental retardation, motor disturbance and epilepsy are the main features of developmental disabilities. We analyzed the relationship between clinical symptoms and magnetic resonance (MR) images, including surface anatomy scan (SAS). Thirty nine patients (23 males, 16 females; mean age 6.1 years) with neuronal migration disorders were studied. The diagnoses were cerebral palsy in 23 cases, mental retardation in 4. West syndrome in 4, Fukuyama type congenital muscular dystrophy (FCMD) in 6. Walker-Warburg syndrome in 1 and Dubowitz syndrome in 1. Cortical dysplasias were classified into the following 7 groups, mainly based on the SAS findings: complete agyria (AG 1), mixture of agyria and pachygyria (AG 2), bilateral complete pachygyria (BP 1), diffuse pachygyria with marked widening of the bilateral superior frontal gyrus (BP 2), unilateral pachygyria with hemispheric atrophy or hemimegalencephaly UP), focal cortical dysplasia (FP) and other findings such as solitary schizencephaly (Others). Most cases of AG 1 and AG 2 showed spastic quadriplegia (6/7) and symptomatic generalized epilepsy (5/7), whereas cases of BP1 showed spasticity only in 1/8 and epilepsy in 7/8. Hemiplegia was observed in 6/7 of UP, 2/8 of FP and 2/4 of Others. Partial epilepsy was observed in 2/7 of UP and 1/8 of FP. Intellectual level was variable in BP 1, UP, FP and Others, but all cases showed severe mental retardation in AG 1, AG 2 and BP 2. BP 2 was observed in all cases of typical FCMD (5/5). The birth weight was less than 2,500 g in 6/7 of UP. The structural findings well correlated with clinical symptoms and epileptic seizure types. The surface anatomy scan was a very useful technique for detecting cortical dysplasias.
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PMID:[The relationship between MR images and clinical findings in neuronal migration disorders]. 924 87

A 51-yr-old woman was transferred to the emergency department with nonspecific interscapular pain and a progressive right-sided hemiparesis. Physical examination and laboratory examination revealed moderate right-sided hemiparesis, with no other focal neurologic deficits. A computed axial tomography scan of the brain was negative. Emergency arteriography revealed bilateral highgrade fibromuscular dysplasia involving the origins of the left and right common carotid arteries. The patient was admitted with the presumptive diagnosis of embolic cerebrovascular accident. Subsequently, the patient developed a left-sided sensory deficit, and magnetic resonance imaging of the cervical spine revealed a C5-T2 epidural hemorrhage. The patient underwent emergent surgical evacuation of the clot and recovered without incident. Spinal epidural hematomas are rare and typically present as cord compressions with or without pain rather than as unilateral hemiplegia.
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PMID:Spinal epidural hematoma progressing to Brown-Sequard syndrome: report of a case. 925 79

We studied the clinical details of 14 children with intractable epilepsies, all of whom underwent epilepsy surgery before age 18 years. All 14 suffered catastrophic seizures, which were resistant to the full range of available medical treatments. The ages at operation ranged from 4 years 7 months to 17 years 2 months, with a mean of 9 years 11 months. In nine patients, the age at onset of epilepsy was less than 2 years. The seizure disorders were classified as temporal lobe epilepsy in two patients, extratemporal lobe epilepsy in 10, and symptomatic generalized epilepsy in two. Eight patients had a hemicorporeal deficit (hemiparesis or hemiplegia) preoperatively. All 14 patients showed localized magnetic resonance imaging (MRI), single photon emission computer tomography (SPECT) and/or positron emission tomography (PET) abnormalities, providing crucial information regarding the epileptic focus. As to the surgical outcomes, four patients became seizure-free and the other 10 showed significant improvement during a mean follow-up period of 2 years 5 months. As to etiology, cortical dysplasia was identified in seven patients. Epilepsy surgery should be considered for intractable childhood epilepsy based on individual clinical characteristics, including seizure status, cognitive development, and evidence indicating location of the seizure focus, rather than age.
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PMID:Study on surgical treatment of intractable childhood epilepsy. 962 91

A 12-year-old, right-handed boy experienced a pathological fit of laughter before a sudden right hemiplegia. Magnetic resonance imaging showed a left basal ganglia infarction, induced by a left internal carotid dissection. Arteriography revealed an underlying fibromuscular dysplasia. This case study demonstrates that cerebral artery dissection can occur in children and that a basal ganglia infarction may be preceded by pathological laughter called 'fou rire prodromique'. The clinical and anatomical relationship of this paroxysmal event are discussed.
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PMID:'Fou rire prodromique' as the heralding symptom of lenticular infarction, caused by dissection of the internal carotid artery in a 12-year-old boy. 1046 70


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