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Query: UMLS:C0018991 (hemiplegia)
 3,997 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Adams-Oliver syndrome (AOS) is a rare congenital disorder, characterized by aplasia cutis congenita (ACC) of the scalp and variable degrees of terminal transverse limb defects. In this article, a newborn infant diagnosed as AOS for a large scalp defect, acrania, and finger malformations is presented. The patient was hospitalized and the scalp defect was successfully repaired with several surgical operations. During the hospitalization septicemia, meningitis, and convulsions developed, but they were successfully treated with appropriate antibiotics, antifungal, and anticonvulsive agents, He was discharged five months after admission to the hospital. Now, he is 3 years old, and has microcephaly, moderate mental retardation, left spastic hemiplegia, and epilepsy.
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PMID:A case of Adams-Oliver syndrome associated with acrania, microcephaly, hemiplegia, epilepsy, and mental retardation. 1123 83

INTRODUCTION. This paper review the state of the art of alternating hemiplegia of childhood. This entity is a rare neurologic disorder of infancy characterize by transient hemiplegic spells shifting from one side to another, and occasionally affecting to both hemispheres at the same time. Usually start before 18 months, many cases exhibit neonatal symptoms related with the disorder. Early symptomatology include abnormal ocular movements, mainly nystagmus, and tonic or dystonic attacks generally beginning before 6 months of age. These symptoms are frequently misdiagnosis as epilepsy. Typical hemiplegic fits which disappeared when infant felt asleep appeared by 12 months of age. Diagnosis is on clinical basis after excluding any other causes of fluctuating and transitory hemiplegic attacks. Complementary investigations, such as electroencephalograms, TAC, MRI, angiographic MRI, CFS are strictly normal. Cerebral SPECT show controversial abnormalities in some studies. We perform SPECT study in a alert 12 months old girl during an interictal period resulting in a non significant asymmetry, with lesser perfusion in left frontal basal and anterior temporal gyrus. Nevertheless, we observed an striking hyperperfusion in middle occipital area. Treatment is symptomatic with flunarizine. Our patient experience a dramatic decrease in frequency, duration and severity of hemiplegic attacks.
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PMID:[Alternating hemiplegia in young babies]. 1198 11

There is a high risk of cerebral palsy (CP) following neonatal encephalopathy (NE) with fits, often attributed to intrapartum asphyxia. The evidence for the association is inconclusive and antepartum factors offer an alternative explanation. A cohort study was carried out to assess the evidence for and against hypoxic ischaemic injury as the cause of NE-associated CP in term infants. A total of 57 159 consecutive births were enrolled. There were 150 cases with NE, of whom 92 had at least one fit and 58 had no fits. The incidence of all NE was 2.62 per 1000 births and of NE with fits was 1.61 per 1000 births. Infants with NE were followed-up to identify those with cerebral palsy. There were 13 cases of four-limb cerebral palsy and three with hemiplegia among the survivors. In 12 of the 13 cases of four-limb CP, a combination of low Apgar scores, an early onset acute evolving encephalopathy, acidosis, renal dysfunction and the absence of antepartum factors suggested an acute intrapartum event as the immediate cause of the NE. An obstetric event likely to cause acute hypoxic injury was identified in four of the 12 cases. The clinical picture was similar in the four with and the eight without a specific obstetric event. The pathway leading to NE-associated CP remains unexplained, but intrapartum events appear to play a major role in most cases.
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PMID:The relationships between neonatal encephalopathy and cerebral palsy: a cohort study. 1252 75

We report an unusual case of encephalotrigeminal angiomatosis in which the facial and oral angioma was bilateral, and several teeth were congenitally absent. The developmental nature of the anomaly is reviewed. Encephalotrigeminal angiomatosis is commonly referred to as the Sturge-Weber syndrome, after Sturge and Weber who first described this affliction in 1879. The main clinical features of this syndrome are 1. venous angiomatosis of the leptomeninges of the cerebral-cortex, usually unilaterally 2. ipsilateral facial angiomatosis that often follows in outline the distribution of the trigeminal nerve (Fig 1) 3. ipsilateral gyriform calcification of the cerebral cortex 4. epileptic convulsions (contralateral focus) or other seizures 5. ocular defects (choroidal angioma, glaucoma hemianopia) 6. mental retardation 7. contralateral hemiplegia 8. obesity 9. oral mucosal and gingival involvement. Other less typical features are 1. association with hypomelanosis of 1 to 10 2. leptomeningeal angioma contralateral to the facial nevus 3. leptomeningeal angioma without facial vascular naevus 4. association with gastro intestinal hemorrhage 5. paranasal sinus enlargement. This syndrome that affects males and females equally, is a rare congenital disorder, apparently hamartomatous in nature, from persistence of a primitive embryonal vascular plexus. During the sixth week of intra-uterine life this plexus develops around the cephalic portion of the neural tube and under the ectoderm in the region destined to become facial skin. In the Sturge-Weber syndrome, the vascular plexus fails to regress, as is normal during the ninth week, resulting in angiomatosis of the related tissues. Variation in the degree of persistence or regression of the vascular plexus accounts for unilaterality or bilaterality of involvement, and also for an incomplete syndrome in which the leptomeninges, but not the facial tissues are affected. Leptomeningeal angiomatosis is the primary abnormality of encephalotrigeminal angiomatosis, all other features of the syndrome probably being secondary to this. Calcification of the cortex is a poorly understood phenomenon which may result from stasis of blood in the angioma, associated with altered local metabolism. Epilepsy and other neurological seizures, and mental retardation are probably, in their turn, secondary to the cortical calcification. The most striking clinical feature of the Sturge-Weber syndrome is the facial vascular naevus which generally follows the distribution of innervation of one or more divisions of the trigeminal nerve, whence the term encephalotrigeminal angiomatosis. However, the naevus may be more extensive, down the neck and even onto the chest. The oral tissues underlying the affected facial tissues are invariably also angiomatous and may be considerably enlarged as a result. Alterations in eruption of teeth have also been noted. Histologically, affected soft tissues are very vascular, resembling a pyogenic granuloma or a capillary, or cavernous hemangioma. Yukna, Cassingham and Carr noted that affected bone was partially replaced by a delicate fibrous tissue containing thin-walled vascular spaces. Neither inflammatory cells, nor fatty or haemopoietic marrow was noted.
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PMID:Encephalotrigeminal angiomatosis. 1496 51

Herpes Simplex Encephalitis (HSE) is an uncommon but severe disease with high mortality and morbidity. The major clinical manifestations are deteriorating consciousness with confusion, drowsiness or coma, altered behaviour, convulsions and a variety of neurological signs (hemiplegia, aphasia, ataxia, etc.). An uncommon complication of HSE is Kluver Bucy syndrome (KBS), characterized by hyperorality, bulimia and changes in emotional behaviour. Neuroimaging studies frequently show an involvement of the temporal lobes and limbic areas. Another uncommon complication of HSE is central diabetes insipidus as a result of herpes simplex infection of the hypothalamus. We report two pediatric cases of HSE complicated with Kluver Bucy syndrome and central diabetes insipidus.
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PMID:[Kluver Bucy syndrome and central diabetes insipidus: two uncommon complications of herpes simplex encephalitis]. 1527 70

Alternating hemiplegia of childhood (AHC) is a rare chronic neurological disorder characterized by alternating transient attacks of hemiplegia. Evidence from recent studies suggests the disorder is characterized by tonic fits, dystonic posturing, and ocular motor abnormalities. Although widely observed, deficits in cognitive functioning are among one of the least described features of AHC. Most studies describe global deficits, however, no study has provided a comprehensive neuropsychological assessment of a child with AHC. This study presents neuropsychological findings from a comprehensive assessment of a single child with AHC. Results showed significant deficits in intellectual, academic, memory, attention, and executive functioning. Additional deficits in language, psychomotor abilities, and psychosocial functioning were noted. Preliminary longitudinal data are also offered to examine the progressive nature of this disorder. Cognitive strengths and weaknesses are discussed at length as well as recommendations for the classroom and future research.
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PMID:Alternating hemiplegia of childhood: a study of neuropsychological functioning. 1578 23

A 3-year-old girl developed right hemiplegia with acute onset, followed by generalized convulsion, choreic movement, and severe motor deterioration. She became bed-ridden a few months after the onset. Intravenous cyclophosphamide pulse therapy resulted in a remarkable improvement of her clinical symptoms. At age of six, she has recovered markedly with the residual symptom of minimum dysarthria. Autoimmune mechanism similar to the antiphospholipid syndrome was considered to be the pathogenesis of this case.
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PMID:[A child with choreic movement, generalized convulsion and severe neurological deterioration responded to cyclophosphamide]. 1602 99

We reported 2 patients with top of the basilar syndrome manifested by onset seizure. Patient 1 was a 76-year-old man. When he was sleeping, suddenly he gave a loud cry and went into convulsions. So he was brought to our hospital by ambulance. On admission, he had unconsciousness and left hemiplegia. Patient 2 was a 70-year-old man. When he was bathing, he lost his consciousness and brought to our hospital by ambulance. On admission, he had convulsion and tetraplegia. Both patients had convulsions and palsy when they had brain infarctions. Brain diffusion weighted MR image showed high intensity area in bilateral internal thalamus, brainstem and cerebellum and no high intensity area in cerebral cortex. We know well that Todd's palsy is palsy after convulsion, but vertebro-basilar occlusion also shows convulsion and palsy. Therefore attention should be paid in the case of the patients who had convulsion and palsy.
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PMID:[Two cases of top of the basilar syndrome with onset seizure]. 1624 96

Hemiconvulsions-hemiplegia-epilepsy syndrome (HH/HHE) is a rare epileptic syndrome consisting of a prolonged unilateral convulsion producing a persisting hemiplegia, sometimes followed by epilepsy. We report on a 13-month-old male who presented with febrile left-sided HH syndrome with right hemispheric unilateral cytotoxic oedema followed by hemispheric atrophy on magnetic resonance imaging (MRI). Six months later the child progressively developed refractory focal epilepsy, including right hemiclonic seizures, and nearly continuous left frontal rhythmic spikes, suggesting the presence of a focal cortical dysplasia (FCD). A repeat MRI at 2 years of age showed left frontal FCD. This unusual case of dual pathology--right HH syndrome and left FCD--suggests that some other factor than the malformation determined the prolonged status and brain atrophy. The kinetics of regional cortical maturation could explain this unusual condition.
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PMID:Atypical case of hemiconvulsions-hemiplegia-epilepsy syndrome revealing contralateral focal cortical dysplasia. 1628 74

The incidence of epilepsy in 110 patients with cerebral palsy (the majority with spastic tetraplegia) was 46,4%. Almost half of the patients with spastic tetraplegia (45%) and hemiplegia (52%) had epilepsy. The incidence was lower in patients with spastic diplegia (32%). Half of epilepsy in spastic hemiplegia were partial seizures and the other half consisted of generalized seizures, while generalized tonic-clonic episodes predominated in all other forms of C.P. A high incidence of West syndrome was observed in patients with spastic tetraplegia. Etiological factors of C.P. were perinatal in 75%. 16,65% had neonatal antecedents of convulsions; most of them had spastic tetraplegia (75%) and a significant mental retardation. A low intelligence quotient (I.Q.) was seen in most of the children with epilepsy, and patients with tetraplegia had significantly lower intelligence quotient than other groups.
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PMID:[Epilepsy in children with cerebral palsy]. 1678 10


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