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)

Hemispherectomy has been performed in the treatment of epilepsy in association with hemiplegia for over 50 years. However, the optimal timing of surgery with respect to age at presentation and the influence of underlying pathology on outcome is only slowly emerging. This study reports on the clinical course and outcomes of 33 children who underwent hemispherectomy at Great Ormond Street Hospital, London, between 1991 and 1997. Age at surgery was 0.33-17 years (median 4.25) with 1-8 years follow-up (median 3.4). The underlying pathology was developmental in 16 (10 hemimegalencephaly, two polymicrogyria, two focal cortical dysplasia, one diffuse cortical dysplasia and one microdysgenesis), acquired in 11 (six middle cerebral artery infarct, three post encephalitis/trauma, and one each of hemiconvulsion-hemiplegia epilepsy and perinatal ischaemic insult) and progressive in six children (four Rasmussen encephalitis, two Sturge-Weber syndrome). At follow-up, 52% were seizure free, 9% experienced rare seizures, 30% showed >75% reduction in seizures and 9% showed <75% seizure reduction or no improvement. Seizure freedom was highest in those with acquired pathology (82%), followed by those with progressive pathology (50%) and those with developmental pathology (31%). However, seizure freedom, rare seizures or >75% reduction in seizures occurred in 100% of those with progressive pathology, 91% of those with acquired and 88% of those with developmental pathology, indicating a worthwhile seizure outcome in all groups. Hemiplegia remained unchanged following surgery in 22 out of 33 children, improved in five and was worse in six. No significant cognitive deterioration or loss of language occurred, and four children showed significant cognitive improvement. Behavioural improvement was reported in 92% of those who had behaviour problems pre-operatively.
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PMID:Clinical outcomes of hemispherectomy for epilepsy in childhood and adolescence. 1256 77

We describe a case of Rasmussen syndrome in a 7-year-old boy, presenting with epilepsia partialis continua, hemiplegia, and progressive mental deterioration. The initial MRI examination was normal, followed by progressive left hemispheric cortical atrophy and abnormal high signal intensity over the left occipital, parietal, and cingulate gyral areas over an 18-month period. On the basis of the clinical diagnosis and biopsy findings of Rasmussen syndrome, functional hemispherectomy was carried out at 7.5 years of age with alleviation of clinical seizures for the following 44 months. The follow-up MRI demonstrated atrophic changes involving the remaining left hemisphere with increased signal and cortical volume loss, as well as the absence of abnormal signal in the right hemisphere at 10 years of age. Our MRI findings are consistent with the progression of Rasmussen syndrome in the ipsilateral hemisphere even after functional-hemispherectomy without clinical seizures.
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PMID:What is the fate of disconnected brain tissue in a child with Rasmussen syndrome? A case report. 1268 10

Rasmussen syndrome or Rasmussen encephalitis is a rare, progressive gray matter disease of childhood. A case of 4-year-old boy is reported here, who presented with recurrent episodes of status epilepticus of simple partial fits along with progressive left sided hemiplegia. EEG showed focal discharges from right hemisphere. Serial MRI brain showed progressive atrophy of right cerebral hemisphere with dilatation of ipsilateral ventricle.
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PMID:Rasmussen encephalitis. 1845 4

Parry-Romberg syndrome (PRS) is a sporadic disease of unknown etiology with typical onset in childhood or in young adults. It is characterized by a slow and progressive atrophy affecting one side of the face, the skin, the subcutaneous tissue, the muscles, the cartilages, and the underlying bony structures. The neurological symptoms usually include focal epilepsy, migraine, and unilateral brain lesions on the same side as the atrophy. A common neuroimaging finding of the syndrome is white matter high signal intensity on brain magnetic resonance (MR) imaging. Rasmussen encephalitis (RE) is a rare and chronic inflammatory disease of the brain that begins in the first decade of life and more rarely in adolescents and adults. It usually involves one hemisphere with focal cortical inflammation. Neurologic symptoms are intractable seizures and progressive hemiplegia. Both PRS and RE are often associated with other inflammatory or autoimmune disorders and only 1 case of both syndromes has been reported in literature. We report the clinical and neuroradiological findings in a 6-year-old boy, presenting with focal hemifacial and arm motor seizures and progressive facial hemiatrophy. Serial MR imaging studies revealed progressive brain hemispheric signal alterations and atrophy. This would thus suggest acoexistence of PRS and RE.
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PMID:Parry-Romberg syndrome and Rasmussen encephalitis: possible association. Clinical and neuroimaging features. 1955 4

Brain magnetic resonance imaging (MRI) studies in patients with Dravet syndrome and SCN1A mutations have shown abnormal findings in a small minority of patients. The origin of the structural abnormalities--such focal brain atrophy, cortical dysplasia, and hippocampal sclerosis--observed in some children remains unclear. There seems to be no correlation between the presence of MRI abnormalities and duration of epilepsy, age at seizure onset, or the frequency of episodes of status epilepticus having occurred early in life. Recent descriptions of Rasmussen syndrome and of the hemiconvulsion-hemiplegia syndrome in isolated patients with SCN1A mutations are of uncertain meaning but might indicate that co-occurring immunomediated or seizure-induced structural changes can, in turn, become a substrate for the severe epileptic encephalopathy. The few available neuropathologic studies of Dravet syndrome have provided inconsistent findings, including evidence of subtle brain malformation. However, the underlying dysfunction of the SCN1A gene might confer to the brain a unique profile of vulnerability whose consequences are not easily disclosed by neuropathology and require specific experimental settings to be fully appreciated. There would seem to be value in studies in animal models of these aspects, as well as prospective studies in humans, with a particular view to establishing if earlier diagnosis and efforts at seizure control may influence the development of any clinical, imaging, or pathologic deterioration.
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PMID:Neuroimaging and neuropathology of Dravet syndrome. 2146 76

Hemispherectomy is the most dramatic type of brain resection performed for epilepsy. Introduced by McKenzie in 1938,(1) it is indicated for intractable seizures that predominantly first occur in infancy and early childhood, arise diffusely from one hemisphere, and are associated with unihemispheric insults. These include hemimegalencephaly, other multilobar cortical dysplasias, perinatal strokes, Sturge-Weber syndrome, and Rasmussen encephalitis. Improvements in surgical techniques and patient selection criteria have resulted in lower complication rates, including lower mortality. Because hemispherectomy is often performed in children who already have moderate to severe hemiplegia, the added motor deficits from the surgery are generally low. Furthermore, given the plasticity in the developing brain, surgery when done early may allow reorganization and preservation of both motor and cognitive function.
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PMID:Big epilepsy surgery for little people: what's the full story on hemispherectomy? 2322 41

We report a longitudinal case study of a left-handed girl who underwent left hemispherotomy at 7 years for Rasmussen encephalitis (RE). Presurgical evaluation showed mild hemiparesis, no visual defect, and light neuropsychological impairment with short-term memory weakness. Language fMRI showed a right hemispheric dominance. Postoperatively, the patient exhibited right hemiplegia and hemianopsia but preserved intellectual capacities. She became seizure-free, and antiepileptic medication was discontinued. Long-term follow-up showed very high verbal intelligence at 11 years of age (VCI of 155) and improvement in working memory as well as language and reading abilities. Furthermore, a significant visuoverbal discrepancy became increasingly pronounced. Thus, early surgical treatment of epilepsy avoided the global cognitive deterioration usually associated with RE. Finally, such a high level of verbal functioning combined with low spatial reasoning with a single hemisphere provides additional information on the neurocognitive profile of children with RE after hemispherotomy.
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PMID:Exceptional verbal intelligence after hemispherotomy in a child with Rasmussen encephalitis. 2447 81

Rasmussen encephalitis (RE) is a severe epileptic and inflammatory encephalopathy of unknown etiology, responsible for focal neurological signs and cognitive decline. The current leading hypothesis suggests a sequence of immune reactions induced by an indeterminate factor. This sequence is thought to be responsible for the production of autoantibody-mediated central nervous system degeneration. However, these autoantibodies are not specific to the disease and not all patients present with them. We report the case of a 4-year-old girl suffering from RE displaying some atypical features such as fast evolution and seizures of left parietal onset refractory to several antiepileptics, intravenous immunoglobulins, and corticosteroids. Serum autoantibodies directed against voltage-gated potassium channels (VGKC) were evidenced at 739 pM, a finding never previously reported in children. This screening was performed because of an increased signal in the temporolimbic areas on brain magnetic resonance imaging, which was similar to what is observed during limbic encephalitis. The patient experienced epilepsia partialis continua with progressive right hemiplegia and aphasia. She underwent left hemispherotomy at the age of 5.5 years after which she became seizure free with great cognitive improvement. First described in adults, VGKC autoantibodies have been recently described in children with various neurological manifestations. The implication of VGKC autoantibodies in RE is a new observation and opens up new physiopathological and therapeutic avenues of investigation.
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PMID:Voltage-gated potassium channels autoantibodies in a child with rasmussen encephalitis. 2506 71

Rasmussen encephalitis (RE) is a rare and severe brain disorder that is associated with unilateral hemispheric atrophy and manifests as severe refractory epilepsy, hemiplegia, defects of motor and speech functions, and cognitive impairment. Treatment of RE, especially in adult patients, is extremely challenging. Herein, we report the case of an adult patient with RE who was treated with a functional hemispherectomy and achieved a favorable prognosis. A 29-year-old woman presented with a 24-year history of epileptic seizures. Neurological examination showed hemiplegia, homonymous hemianopsia, and right muscular atrophy. Neuropsychological examination demonstrated cognitive disorders. Serial magnetic resonance imaging (MRI) and computed tomography (CT) scans showed progressive encephalatrophy in the left hemisphere and ventriculomegaly in the left lateral ventricle. The Wada test showed that the right hemisphere was dominant for language. A functional hemispherectomy was performed. Postoperatively, no antiepileptic drugs were administered, and the patient remained seizure-free without aggravation of hemiplegia. Over a 2-year follow-up, cognitive functions improved. In an adult patient with RE, a favorable prognosis was achieved after functional hemispherectomy. The safety and efficacy of functional hemispherectomy in patients with RE is highlighted.
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PMID:Functional Hemispherectomy for Adult Rasmussen Encephalitis: A Case Report and Literature Review. 2909 Dec 50