Rasmussen’s encephalitis

Counce, Dianne; Limdi, Nita A.; Kuzniecky, Ruben

doi:10.1007/s11940-001-0018-5

Cited by 6 publications

(3 citation statements)

References 38 publications

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“…RE is chiefly a disease of children but occasionally occurs in adulthood (10%) and can be fatal depending on the severity of the disease. Treatment options for RE are limited to controlling the seizures with anti-epileptic drugs together with radical neurosurgical interventions (corticectomy or hemispherectomy) associated with major postoperative neurologic deficits including hemiparesis and hemianopsia [5]. Therapeutic immunomodulation and/or immunoablation in RE as well as antiviral therapies have been reported with inconclusive results [6,7].…”

Section: Introductionmentioning

confidence: 99%

Inflammasome induction in Rasmussen’s encephalitis: cortical and associated white matter pathogenesis

Ramaswamy

Walsh

Sinclair

et al. 2013

J Neuroinflammation

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BackgroundRasmussen’s encephalitis (RE) is an inflammatory encephalopathy of unknown cause defined by seizures with progressive neurological disabilities. Herein, the pathogenesis of RE was investigated focusing on inflammasome activation in the brain.MethodsPatients with RE at the University of Alberta, Edmonton, AB, Canada, were identified and analyzed by neuroimaging, neuropsychological, molecular, and pathological tools. Primary human microglia, astrocytes, and neurons were examined using RT-PCR, enzyme-linked immunosorbent assay (ELISA), and western blotting.ResultsFour patients with RE were identified at the University of Alberta. Magnetic resonance imaging (MRI) disclosed increased signal intensities in cerebral white matter adjacent to cortical lesions of RE patients, accompanied by a decline in neurocognitive processing speed (P <0.05). CD3ϵ, HLA-DRA, and TNFα together with several inflammasome-associated genes (IL-1β, IL-18, NLRP1, NLRP3, and CASP1) showed increased transcript levels in RE brains compared to non-RE controls (n = 6; P <0.05). Cultured human microglia displayed expression of inflammasome-associated genes and responded to inflammasome activators by releasing IL-1β, which was inhibited by the caspase inhibitor, zVAD-fmk. Major histocompatibility complex (MHC) class II, IL-1β, caspase-1, and alanine/serine/cysteine (ASC) immunoreactivity were increased in RE brain tissues, especially in white matter myeloid cells, in conjunction with mononuclear cell infiltration and gliosis. Neuroinflammation in RE brains was present in both white matter and adjacent cortex with associated induction of inflammasome components, which was correlated with neuroimaging and neuropsychological deficits.ConclusionInflammasome activation likely contributes to the disease process underlying RE and offers a mechanistic target for future therapeutic interventions.

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Section: Introductionmentioning

confidence: 99%

Inflammasome induction in Rasmussen’s encephalitis: cortical and associated white matter pathogenesis

Ramaswamy

Walsh

Sinclair

et al. 2013

J Neuroinflammation

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“…Progression is slowed with immunomodulatory treatment. 21,22 The EEG in RE evolves over time, initially comprising lateralized epileptiform discharges from the affected hemisphere, and progressing to multifocal contralateral epileptiform discharges later in the disease course -termed ''false lateralisation''. 23,24 Other syndromes sharing this pattern include Sturge-Weber, focal cortical dysplasia, hemiconvulsionhemiplegia-epilepsy-syndrome and PCDH19 related epilepsies.…”

Section: Discussionmentioning

confidence: 99%

Immunoglobulin-responsive refractory epilepsy – 3 cases with a similar EEG pattern

Dionisio

Brown

Lander

et al. 2013

Seizure

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“…efficacy. 8,9 Hemispherectomy has shown to provide the best long-term results. 1,10,11 We report a patient with Rasmussen's encephalitis who underwent an exhaustive evaluation but required additional functional neuroimaging, in the form of single photon emission computed tomography (SPECT), for further lateralization of seizure onset.…”

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confidence: 99%

Utility of Ictal SPECT in the Presurgical Evaluation of Rasmussen's Encephalitis

et al. 2006

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: Background: Rasmussen's encephalitis is a progressive disease characterized by severe progressive neurological and cognitive deficits. It is a difficult condition to treat with conventional medical treatment and requires, in most of the cases, surgical intervention. Methods: We present the case of a patient with Rasmussen's encephalitis who presented with epilepsia partialis continua, who did not respond to antiepileptic treatment and was evaluated for possible surgical intervention. Results: The electroencephalographic studies revealed bihemispheric involvement during seizures, although magnetic resonance imaging (MRI) showed a marked right hemispheric atrophy. Hyperperfusion over the right hemisphere was seen with the performance of ictal single photon emission computed tomography (SPECT), which finally guided a right functional hemispherectomy and subsequent seizure control. Conclusion: Ictal SPECT could be a useful tool in the armamentarium of tools used for the localization of the epileptogenic focus in patients with Rasmussen's encephalitis and other localization-related epilepsies.

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Rasmussen’s encephalitis

Cited by 6 publications

References 38 publications

Inflammasome induction in Rasmussen’s encephalitis: cortical and associated white matter pathogenesis

Inflammasome induction in Rasmussen’s encephalitis: cortical and associated white matter pathogenesis

Immunoglobulin-responsive refractory epilepsy – 3 cases with a similar EEG pattern

Utility of Ictal SPECT in the Presurgical Evaluation of Rasmussen's Encephalitis

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