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Query: UMLS:C0014547 (focal epilepsy)
 1,627 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This report is a sequel to our 1958, 1960 and 1968 reports on a series of patients operated upon for focal epilepsy whose surgical specimens unexpectedly showed histological lesions typical of active encephalitis. None of these patients, now 27 in number, exhibited the clinical picture ordinarily associated with encephalitis. With one exception, all showed a severe focal seizure tendency beginning in infancy or childhood, often associated with episodes of epilepsia partialis continua. In addition, all except 2 showed slowly progressive neurological deterioration, usually hemiparesis and mental retardation, which advanced over periods of months or years before the progression became arrested. No infectious agent has yet been identified by standard viral studies carried out in the most recent 14 patients or by investigation for slow viruses in 6 patients operated upon between 1966 and 1971. The clinical course of this condition is outlined and the role, the timing and the results of treatment by craniotomy and cortical excision are discussed.
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PMID:Further observations on the syndrome of chronic encephalitis and epilepsy. 10 97

We report the results of a clinical trial of Magnetoencephalography (MEG) on spike foci in patients with epilepsy, which was performed from December 1990 to June 1991 at The University of Tokyo Hospital. Fifty patients with focal epilepsy; 26 primary epilepsy, 24 secondary epilepsy (7 brain tumor, 4 arteriovenous malformation, 4 encephalitis, 3 porencephaly, 2 arachnoid cyst, 1 brain abscess, 1 hemimegaloencephaly, 1 Lance-Adams syndrome, 1 hygroma), and ten normal subjects were enrolled in this study. MEG data were recorded using a 37-channel biomagnetometer system SMI-1001 (BTi Magnes, Biomagnetic Technologies, Inc., San Diego). A simultaneous 19-channel EEG recording with linked-ear reference was also obtained. The overall study was completed safely and none of the normal subjects showed abnormal paroxysmal MEG activity. Two patients showed interictal EEG spikes which would not have been noticed without first noting the presence of corresponding prominent MEG spikes. On the whole, the MEG signal seemed to have a wider frequency bandwidth than EEG. In most cases, the source localization predicted by MEG corresponded well with the EEG findings. The relative accuracy of MEG spike source localization was estimated to be within a cubic centimeter from the cases which showed tightly clustered localization of individual spikes. High-pass filtering reduced interference by superimposed slow wave activity, thereby improving the localization of spike sources. These results demonstrate that 37-channel biomagnetometer system could be a useful tool for analyzing epileptic spike sources.
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PMID:[Magnetoencephalographic studies on spike foci using a 37-channel biomagnetometer system]. 176 94

A new operative approach has been designed for the relief of medically intractable focal epilepsy. It is intended particularly to be used in those cases where the epileptogenic lesion lies in "unresectable" cortex; that is, those cerebral regions subserving speech, memory, and primary motor and sensory function. The procedure is based upon experimental evidence indicating 1) that epileptogenic discharge requires substantial side-to-side or horizontal interaction of cortical neurons, and 2) that the major functional properties of cortical tissue depend upon the vertical fiber connections of the columnar units. The technique requires severing of tangential intracortical fibers while preserving the vertical fiber connections of both incoming and outgoing nerve pathways and of the penetrating blood vessels which also have a vertical orientation. In this study, the effect of multiple subpial transection was assessed on both function and seizure control. The effect on function was reviewed in 32 cases; only 20 cases were evaluated with respect to seizure control, since a follow-up period of 5 years or more (5 to 22 years) is required before conclusions can be drawn. Multiple subpial transection was applied to the precentral gyrus in 16 cases, the postcentral gyrus in six, Broca's area in five, and Wernicke's area in five. With respect to function, the major finding was that none of the 32 patients has suffered a clinically significant behavioral deficit (although subtle deficits could be detected by careful neurological examination). Complete control of seizures has been obtained in 11 (55%) of the 20 cases evaluated. Nine patients developed recurrent seizures consequent to progressive disease unsuspected before operation (Rasmussen's encephalitis in five, tumor in three, and subacute sclerosing panencephalitis in one). In none of these cases, however, did the recurrent seizures arise in the transected zone. Thus, the results indicate that multiple subpial transection is about as effective as standard excisional therapy, and can be successfully employed when epileptogenic lesions encroach upon cortical territories, the removal of which would be functionally incapacitating.
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PMID:Multiple subpial transection: a new approach to the surgical treatment of focal epilepsy. 250 54

We treated 19 patients with Rasmussen's syndrome (chronic encephalitis and epilepsy)--a rare progressive disorder of unknown etiology causing focal epilepsy, hemiparesis, and intellectual deterioration--with intravenous immunoglobulins, high-dose steroids, or both, to control seizures and improve the end point of the disease. Ten of 17 patients receiving steroids, and eight of nine patients receiving immunoglobulins, had some reduction of seizure frequency in the short term. Improvement in hemiparesis was slight. The effect of these drugs in ameliorating the end point of the disease in the long term remains unknown, and further multicenter studies with standardized protocols are warranted.
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PMID:Medical treatment of Rasmussen's syndrome (chronic encephalitis and epilepsy): effect of high-dose steroids or immunoglobulins in 19 patients. 778 6

Chronic encephalitis and epilepsy (Rasmussen's encephalitis) is a rare progressive disorder of uncertain etiology that usually occurs in children, producing focal epilepsy, hemiparesis, and intellectual deterioration. We identified 13 patients in whom seizures developed in adolescence or adulthood with a pathologic picture of chronic encephalitis. The clinical characteristics were more variable than those occurring in children, with the patients falling into three groups: five patients developed seizures in adulthood, but otherwise showed many resemblances to the childhood form; five developed seizures in adolescence, with similar presentation but rather more benign course than in the younger patients; and three presented with clinical features initially suggestive of a tumor. Occipital onset to the seizures appeared to be more common than in the childhood form, and bilateral disease also occurred.
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PMID:Chronic encephalitis and epilepsy in adults and adolescents: a variant of Rasmussen's syndrome? 904 Jul 32

The clinical, virological and immunocytochemical features of three children who recovered from acute herpes simplex encephalitis (HSE) before the age of 2 years, and who developed secondary severe focal epilepsy after a symptom-free period, leading to neurosurgery 3-10 years later are described. In one child, relapse of HSE occurred immediately after surgery. In all three patients, brain sample biopsies showed abundant CD3-positive T lymphocytes with a majority of CD8 cells, and abundant activated macrophage-microglial cells, a pattern similar to that found in acute HSE. Herpes simplex virus DNA was retrieved from the tissue biopsy in one case. The long-term persistent cerebral inflammatory process observed after HSE differed from that observed in another chronic viral disease, subacute sclerosing panencephalitis. This inflammatory reaction may be a result either of low-grade viral expression or self-induced immune activation. The role of inflammation in triggering epilepsy remains hypothetical. Solving these issues should have major therapeutic implications. Herpes simplex virus DNA latency in brain may be the source of replicative HSE relapse.
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PMID:Immunocytochemical characterization of long-term persistent immune activation in human brain after herpes simplex encephalitis. 1088 86

Cases are described of chronic zoster encephalitis presenting with the leading progressive focal epilepsy syndrome. The clinical presentation of the trouble can include both general cerebral signs and those of the focal affection of the central nervous system only. The progressive course and duration of remission suggest to us a persisting infection manifesting against the background of immune deficiency.
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PMID:[The clinical characteristics of progressive focal epilepsy with a herpetic etiology]. 1103 68

Eight patients with Rasmussen's encephalitis, 40 patients with noninflammatory focal epilepsy, 104 patients with various neurologic diseases, and 16 healthy donors were tested for the prevalence of antibodies against the GluR3 receptor in serum and CSF. Reactivities against different peptides derived from various portions of this glutamate receptor subtype were detectable in a significantly higher number of patients with focal epilepsy than in those with other neurologic diseases, but they were not specific for the diagnosis of Rasmussen's encephalitis.
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PMID:GluR3 antibodies: prevalence in focal epilepsy but no specificity for Rasmussen's encephalitis. 1167 4

Rasmussen's syndrome is a chronic encephalitis characterized by intractable focal epilepsy and progressive neurologic deterioration with lateralized brain destruction. In the early stages of the disease, the diagnosis can be difficult to make, and brain biopsy is often performed. We evaluated the patterns of cerebral glucose metabolism using 2-deoxy-2-[18F]-fluoro-D-glucose positron emission tomography (PET) in 15 children (age range 2.9-15.4 years, mean age 8.7 +/- 4.3 years) with Rasmussen's syndrome. In 6 patients evaluated early (< or = 1 year of onset of seizures), the PET scan showed areas of abnormal metabolism restricted mostly to the frontal and temporal regions, whereas the posterior cortex was preserved. Pathologic changes seen in the resected cortex were more pronounced in cortical areas of abnormal metabolism than in regions showing normal metabolism. In 9 patients evaluated later (>1 year after onset of seizures), the PET scan showed more diffuse hemispheric metabolic abnormalities including the occipital cortex, but the abnormalities remained highly lateralized. These patterns of glucose metabolic abnormalities in the early and late stages of the disease may facilitate the diagnosis of Rasmussen's syndrome and assist guidance of biopsy in early cases, when structural neuroimaging is still normal.
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PMID:Patterns of cerebral glucose metabolism in early and late stages of Rasmussen's syndrome. 1173 64

Rasmussen's encephalitis (RE) is a cryptogenic progressive inflammatory disorder of the brain that causes severe neurological problems, including intractable focal epilepsy. In select patients, aggressive treatment using cerebral hemispherectomy may ameliorate the devastating cognitive decline that accompanies this disease, even if the epileptic focus appears broadly distributed. We present a case of histopathologically-confirmed RE evaluated using a multimodal process that explored the physical and functional aspects of the associated epilepsy. This process included magnetic resonance imaging, single photo emission computed tomography, electroencephalography, and magnetoencephalography (MEG). The findings indicate that functional brain imaging data may greatly assist the surgical treatment decision-making process in RE, especially when structural imaging fails to reveal definitive localizing information. In addition, MEG may provide insights about the cortical reorganization of somatosensory cortex following hemispherectomy.
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PMID:Multimodality functional imaging evaluation in a patient with Rasmussen's encephalitis. 1201 67


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