Further Observations on the Syndrome of Chronic Encephalitis and Epilepsy

Rasmussen, Theodore P.

doi:10.1159/000102395

Cited by 46 publications

(48 citation statements)

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“…Chronic encephalitis of Rasmussen with occipital lobe seizures was reported as the initial seizure type in one patient (22). Occipital lobe seizures have also been reported early in the course of Lafora and Kufs disease (23).…”

Section: Etiology Of Symptomatic Occipital Lobe Epilepsymentioning

confidence: 92%

Symptomatic Occipital Lobe Epilepsy

Kuzniecky

1998

Epilepsia

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Summary: Symptomatic occipital lobe epilepsy is increasingly recognized among patients with partial-onset seizures. Although traditional clinical and electroencephalographic criteria had defined occipital lobe epilepsy in the past, new neuroimaging techniques and the recognition of specific syndromes associated with occipital lobe epilepsy have improved the diagnosis and management of these patients. These syndromes include, among others, lesional occipital lobe epilepsy (congenital vs. acquired), MELAS, and epilepsy with bilateral occipital calcifications. The diagnosis of symptomatic occipital lobe epilepsy is improving as functional and structural neuroimaging techniques enable the detection of subtle abnormalities in such patients. This has had a direct impact on the correct classification of patients with benign occipital lobe epilepsy, basilar migraine, and symptomatic occipital lobe epilepsy. The common clinical symptoms, EEG patterns, and neuroimaging findings of these patients are discussed. Key Words: Occipital lobe epilepsy-congenital vs. acquired syndromes-EEG patterns-Neuroimaging.Advances in the diagnosis of epilepsy have led to refinements in the classification of the epilepsies. This has been primarily possible because of recent major advances in neuroimaging techniques, which are now able to detect pathology in many patients with temporal lobe epilepsy, particularly in those with mesial temporal lobe sclerosis. This capability, in addition to the wealth of new noninvasive imaging techniques, has directed efforts toward the better definition of extratemporal lobe epilepsies. This article describes the clinical features associated with symptomatic occipital lobe epilepsy and reviews the etiologic factors, imaging and electrographic characteristics, and outcome. EPIDEMIOLOGIC ASPECTSThe relative incidence of symptomatic occipital lobe epilepsy is unknown (1). No prospective studies are available, and most retrospective series have selected patients on the basis of EEG abnormalities or of EEG and clinical features suggestive of occipital lobe epilepsy, and have included those with benign syndromes. Furthermore, previous studies have included children and adults and have been influenced by the referral patterns of the reporting centers. An EEG-based survey reported 8% of the population as having occipital seizures. An Italian study (2) reported that occipital epilepsy consti- tuted 11% of symptomatic partial epilepsies in children.Other centers report frequencies ranging from 4 to 13%.In contrast, data from epilepsy surgery centers suggest that occipital lobe resections constitute less than 5% of operated patients (3). The available data, however, are an underestimation of the relative frequency of occipital seizures. This is a reflection of the fact that a sizable number of patients with occipital lobe epilepsy are not correctly diagnosed or are not candidates for epilepsy surgery. CLINICAL SYMPTOMS OF OCCIPITAL LOBE EPILEPSYThe symptomatology of occipital lobe epilepsy was described as early as 188...

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Section: Etiology Of Symptomatic Occipital Lobe Epilepsymentioning

confidence: 92%

Symptomatic Occipital Lobe Epilepsy

Kuzniecky

1998

Epilepsia

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“…A localized and slowly progressive viral infection may explain the unihemispheric encephalitic changes in RE. However, extensive studies to investigate the presence of viruses associated with well-known encephalitis (e.g., herpes simplex virus, cytomegalovirus, Epstein-Barr virus, enterovirus) have failed to demonstrate causal involvement in RE [53][54][55][56][57][58]. It is very likely that new nucleic acid deep sequencing technologies, microbiome, and metagenomic studies of the brain will revisit issues related to viral infections in RE.…”

Section: Infection and Viral Hypothesis In Rementioning

confidence: 99%

Mechanisms of Epileptogenesis in Pediatric Epileptic Syndromes: Rasmussen Encephalitis, Infantile Spasms, and Febrile Infection-related Epilepsy Syndrome (FIRES)

2014

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The mechanisms of epileptogenesis in pediatric epileptic syndromes are diverse, and may involve disturbances of neurodevelopmental trajectories, synaptic homeostasis, and cortical connectivity, which may occur during brain development, early infancy, or childhood. Although genetic or structural/metabolic factors are frequently associated with agespecific epileptic syndromes, such as infantile spasms and West syndrome, other syndromes may be determined by the effect of immunopathogenic mechanisms or energy-dependent processes in response to environmental challenges, such as infections or fever in normally-developed children during early or late childhood. Immune-mediated mechanisms have been suggested in selected pediatric epileptic syndromes in which acute and rapidly progressive encephalopathies preceded by fever and/or infections, such as febrile infection-related epilepsy syndrome, or in chronic progressive encephalopathies, such as Rasmussen encephalitis. A definite involvement of adaptive and innate immune mechanisms driven by cytotoxic CD8 +

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“…RE is a rare, progressive and catastrophic disease of unknown etiology that begins in the first decade of life, affecting previously normal individuals [4]. It is associated with damage to a single cerebral cortical hemisphere, and characterized by intractable unilateral focal seizures, progressive neurological deficits, and variable intellectual impairment [4,5]. Hemispheric atrophy, loss of cortical neurons, and chronic inflammatory changes are characteristic of RE histopathology [6,7].…”

Section: Introductionmentioning

confidence: 99%