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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Rasmussen encephalitis is a disease consisting of chronic encephalitis with progressive neurologic deficits and focal intractable seizure activity. The etiology is unknown, but pathologic specimens revealed changes consistent with viral encephalitis. Even though neuro-imaging techniques, such as positron emission tomography and magnetic resonance imaging, offer the prospect of specific, presurgical diagnostic criteria, the initial diagnosis usually is made on a clinical basis. Treatment modalities, including a wide variety of antiepileptic drug therapies and surgical interventions, may result in significant physical and mental impairments. We summarize the clinical presentation, diagnostic considerations, and different treatment protocols in a patient with this rare and debilitating disorder.
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PMID:Rasmussen encephalitis: epilepsia partialis continua secondary to chronic encephalitis. 212 55

Six of 81 (7.4%) patients with medically intractable epilepsy treated by selective cortical excision at The Hospital for Sick Children, Toronto, Canada, since 1974 were found to have inflammatory changes in the excised specimens of cerebral cortex. The clinical and histopathological findings in these cases resemble the chronic encephalitic syndrome described by Rasmussen. We confirm the unsatisfactory seizure control and the guarded neurological and intellectual prognosis that has been associated with Rasmussen syndrome.
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PMID:Chronic focal encephalitis (Rasmussen syndrome): six cases. 313 Nov 34

Authors report about a boy with Rasmussen syndrome. This is a chronic localised encephalitis with focal epileptic seizures causing progressive neurologic deficits and mental impairment. The etiology and the clinical features of the syndrome are discussed, and the diagnostic role of the new imaging modalities is emphasized. This is the first report about this entity in Hungary.
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PMID:[Rasmussen syndrome]. 825 73

The surgical treatment of chronic pharmacoresistant epilepsies is increasing rapidly. Although several studies have reported on histopathological findings in temporal lobe epilepsy, little is known about the surgical pathology of other seizure disorders. Here we report the histopathological findings in 63 consecutive surgical specimens of patients who were operated for chronic pharmacoresistant epileptic seizures other than temporal lobe epilepsy (37 corticectomies, 19 functional hemispherectomies, 5 lobectomies, 1 multilobectomy, and 1 frontal lobe deafferentiation combined with a temporal lobectomy). There were structural lesions in 85.7% of the specimens. In 16 cases (25.4%) the predominant lesions were malformative (focal glioneuronal hamartias and hamartomas, vascular malformations, abundant ectopic neurons in the white matter, microgyria, and arachnoid cyst). Lesions indicating pre- or perinatal necrosis such as porencephaly, ulegyria, and congenital hemiatrophy were present in 7 cases (11.1%). Twelve specimens (19.0%) contained low-grade neoplasms (7 gangliogliomas, 3 astrocytomas, 1 oligodendroglioma and 1 oligoastrocytoma). There were 3 cases of Rasmussen encephalitis, 1 specimen with atrophy and gliosis due to previous herpetic encephalitis and 1 case with an old abscess wall. Posttraumatic or postoperative changes were the predominant finding in 7 specimens (11.1%). In 7 patients there were only nonspecific changes such as cortical atrophy and gliosis or old hemorrhage. No structural alterations were identified in 9 specimens (14.3%). The findings suggest that the structural lesions observed in the great majority of the specimens were closely related to the pathogenesis of intractable seizures.
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PMID:Surgical pathology of chronic epileptic seizure disorders: experience with 63 specimens from extratemporal corticectomies, lobectomies and functional hemispherectomies. 831 Jul 97

Knowledge about the role of viruses in encephalitis and other diseases of the central nervous system has been greatly expanded since the advent of polymerase chain reaction testing. It is known that the spectrum of disease caused by herpes simplex virus, for example, is much wider than was previously recognized. Evidence is mounting that infectious agents may have a causative role in diseases such as recurrent febrile seizures and Rasmussen encephalitis. This review examines the recent advances in treating viral encephalitis and puts these developments into perspective.
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PMID:Viral encephalitis in children. 868 May 7

Rasmussen syndrome (RS) is a severe and progressive focal epilepsy of unknown etiology that leads to deterioration of motor and cognitive function. We report a 14-year-old girl who developed epilepsia partialis continua involving the left hand, mild hemiparesis, and secondarily generalized seizures. RS was confirmed by brain biopsy. The patient has been treated with intravenous gamma globulin every 4 months for 46 months. The clinical course throughout this time has been distinctly atypical for RS, with no progression in motor or cognitive deficits and rare secondarily generalized seizures. Although the mechanism for action for gamma globulin in RS is not known, an immunomodulatory role has been postulated. Evidence of an immunologically mediated process in RS and clinical experience with a growing number of patients who benefit from immunomodulatory therapy suggest that a systematic study of the efficacy of gamma globulin in comparison with other forms of medical therapy is warranted.
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PMID:Rasmussen syndrome and long-term response to gamma globulin. 870 28

Chronic progressive focal encephalitis or Rasmussen encephalitis (RE) is chronic brain disease of viral etiology. It is manifested by either partial motor or myoclonic seizures combined with hemiparesis. RE was described in detail in a girl of 8.5 years. The disease debut was observed at the age of 5.5 and manifested in the form of simple partial motor fits with serial episodes of secondary generalization. Post-attack hemiparesis was observed from the age of 7.5 and became permanent after 8. From this time myoclonic fits became constant, changed for the worse, memory and attention deteriorated. NMR tomography revealed widening and deepening of the Sylvian fissure as well as a focal decrease of left temporal area density. The patient was resistant to anticonvulsant, hormonal and immunosuppressive drugs. The criteria of RE diagnosis, its difference from Kozhevnikov's epilepsy are provided. Estimation of drug and surgical treatment effectivity is given. The necessity of early surgical treatment (functional hemisphere ectomy) is emphasized.
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PMID:[Rasmussen's chronic progressive focal encephalitis]. 875 34

This prospective case study examined social communication (i.e., formal thought disorder, cohesion), language, positron emission tomography glucose utilization, and neuropathology in four children with Rasmussen encephalitis who achieved seizure control following right hemispherectomy. Prior to hemispherectomy, all four children had illogical thinking, loose associations, cohesive deficits, and impaired performance on formal language tests. Their postoperative improvement in social communication and language appeared to be related to age of onset, duration of illness, and postsurgical reversibility of hypometabolism in the nonresected prefrontal cortex. These changes were not associated with increase in IQ scores. The variability in the type and extent of pathologic change across subjects reflected the severity and duration of the illness. The study's findings imply that early surgical intervention might have mitigated certain aspects of the social communication and linguistic deficits found in these children.
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PMID:Pediatric Rasmussen encephalitis: social communication, language, PET and pathology before and after hemispherectomy. 889 14

Acute herpes simplex encephalitis (HSE) carries significant morbidity and mortality even after early treatment with antiviral agents (7). As well as causing acute neurological disease, Herpes viruses are associated with relapsing--remitting (Varicella--Zoster, Epstein-Barr) and chronic (Rasmussen encephalitis) disease processes (1). A two-year-old girl developed acute HSE which was followed by a 10-year neurologic illness characterised by asymmetric spastic tetraparesis, pseudobulbar palsy, the opercular syndrome of Foix-Chavany-Marie (4) and seizures. The neurological signs remained static until the child died suddenly 12 years after disease onset. Neuropathologic examination demonstrated active chronic encephalitis. Herpes simplex virus (HSV) DNA was recovered from formalin-fixed paraffin-embedded brain tissue. This case provides additional evidence for the development of chronic neurological disease attributable to persistence of herpes simplex virus type 1.
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PMID:Chronic active destructive herpes simplex encephalitis with recovery of viral DNA 12 years after disease onset. 970 20

Six patients admitted to the Department of Pediatric Neurology at Hacettepe University Children's Hospital between 1992 and 1997 with a clinical diagnosis of Rasmussen encephalitis received surgical treatment for their intractable epilepsy. MRI, SPECT and WADA tests were performed in patients with an epileptic focus demonstrated on routine or long-term video EEG monitoring. Viral studies using the PCR methodology were performed in cases with histopathological evidence of Rasmussen encephalitis. The ages of these patients ranged between 7 and 16 years, and the mean age at onset of seizures was 7.1+/-2.2 years. In four patients seizures presented as epilepsia partialis continua and were refractory to anticonvulsive drug therapy. In three cases intravenous immunoglobulin therapy yielded temporary and partial improvement in seizure control. The mean presurgical follow-up duration was 2.04+1.74 years, and early surgical intervention for epilepsy was performed in one case. The surgical approach selected for the treatment of epilepsy was resective surgery with electrocorticography. The mean postoperative follow-up duration was 32.3+17.2 months. Seizures were fully controlled in one patient, in whom surgery was performed 3 months after the seizures first started. Early surgical intervention may provide histopathological evidence for diagnosis as well as effective seizure control.
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PMID:Rasmussen encephalitis in childhood. 1044 11


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