The Pathology of Rasmussen Syndrome: Stages of Cortical Involvement and Neuropathological Studies in 45 Hemispherectomies

Pardo, Carlos A.; Vining, Eileen P.G.; Guo, Liping; Skolasky, Richard L.; Carson, Benjamin S.; Freeman, John M.

doi:10.1111/j.0013-9580.2004.33103.x

Cited by 195 publications

(197 citation statements)

References 26 publications

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“…The progression of the symptoms to significant neurological impairment usually occurs within months to few years. 17 Although RE causes are unkown, an autoimmune process has been suggested 2,9,12,16 and surgery is the treatment of choice to try to avoid mental retardation, dementia and death. 1 Electroencephalographic usually show a pattern of recurrent seizures, with activity over the central motor area.…”

Section: Introductionmentioning

confidence: 99%

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Rasmussen encephalitis: long-term outcome after surgery

et al. 2009

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Background and Purpose: Rasmussen Encephalitis (RE) is characterized by intractable epilepsy, progressive hemiparesis and unilateral hemispheric atrophy. The progression of the symptoms usually occurs within months to few years. Antiepileptic drugs are usually not effective to control disease progression and epilepsy surgery in the form of hemispheric disconnection has been considered the treatment of choice. This work describes the clinical and electrographic analyses, as well as the post-operative evolution of patients with RE. Patients and Methods: This work includes all the patients with RE evaluated from January 1995 to January 2008 by the Ribeirão Preto Epilepsy Surgery Program (CIREP) considering demographic data, interictal and ictal electroencephalographic (EEG) findings; anatomo-pathological findings and clinical outcome. Results: Twenty-five patients were evaluated, thirteen were female. Mean age of epilepsy onset was 4.4±2.0 years. There were no differences between patients with slow and fast evolution with respect to age of epilepsy onset (p=0.79), age at surgery (p=0.24), duration of epilepsy (0.06), and follow-up (p=0.40). There were no correlations between the presence of bilateral EEG abnormalities or the absence of spikes and post-operative seizure outcome (p=0.06). Twenty-three patients underwent surgery. The mean follow-up was 75.3 months. Eleven patients had total seizure control. Twelve individuals persisted with seizures consisting of mild facial jerks (6 patients), occasional hemigeneralized tonic-clonic seizures (3 patients), and frequent tonic-clonic seizures (3 patients). Mental and language impairment was observed in 15 and 12 patients, after surgery, respectively. Conclusions: This retrospective study reported the clinical and electrographic analysis, as well as the evolution of 23 patients with RE. Fourteen patients achieved satisfactory seizure control, three patients had partial response to surgery, and five patients had maintenance of the pre-operative condition. All patients with left side involvement presented with some language and cognitive disturbance.

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Section: Introductionmentioning

confidence: 99%

“…7,17 In this study, we have reviewed the clinical, electrographic and post-operative evolution of patients with RE, considering long-term prognosis.…”

Section: Introductionmentioning

confidence: 99%

Rasmussen encephalitis: long-term outcome after surgery

et al. 2009

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“…Indeed, consequences of perforin-and/or Fas-mediated killing are likely more relevant in advanced stages of inflammatory CNS disease. Neuronal loss is a dominant feature in chronic but not acute stages of RE (Bien et al, 2002;Pardo et al, 2004) and was observed only late after challenge (Fig. 1).…”

Section: Discussionmentioning

confidence: 93%

Neuroprotective intervention by interferon-γ blockade prevents CD8+ T cell-mediated dendrite and synapse loss

Kreutzfeldt¹,

Bergthaler²,

Fernandez³

et al. 2013

J Cell Biol

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Neurons are postmitotic and thus irreplaceable cells of the central nervous system (CNS). Accordingly, CNS inflammation with resulting neuronal damage can have devastating consequences. We investigated molecular mediators and structural consequences of CD8(+) T lymphocyte (CTL) attack on neurons in vivo. In a viral encephalitis model in mice, disease depended on CTL-derived interferon-(IFN-) and neuronal IFN-signaling. Downstream STAT1 phosphorylation and nuclear translocation in neurons were associated with dendrite and synapse loss (deafferentation). Analogous molecular and structural alterations were also found in human Rasmussen encephalitis, a CTL-mediated human autoimmune disorder of the CNS. Importantly, therapeutic intervention by IFN-blocking antibody prevented neuronal deafferentation and clinical disease without reducing CTL responses or CNS infiltration. These findings identify neuronal IFN-signaling as a novel target for neuroprotective interventions in CTLmediated CNS disease.

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“…Pathologically, the salient features include chronic meningeal and parenchymal inflammation, consisting primarily of benign appearing T lymphocytes, both diffuse and nodular microglial cell proliferation, gliosis, and neuronal cell death. 36 The underlying pathogenesis of the disorder is still of some debate. Evidence supporting GluR3 autoantibody-induced injury is conflicting and there are cases in which such autoantibodies do not appear to exist; however, there is evidence to support a T-cell-mediated cytotoxicity targeting neurons in the disorder.…”

Section: Discussionmentioning

confidence: 99%

Rasmussen's Encephalitis - Chronic Focal Encephalitis (CFE) - A Rare Entity

Kumar¹

2017

jmscr

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Rasmussen's encephalitis, also known as chronic focal encephalitis (CFE), is a rare inflammatory neurological disease, characterized by frequent and severe seizures, loss of motorskills and speech, hemiparesis (paralysis on one side of the body), encephalitis (inflammation of the brain), and CASE REPORTA 12 year old male child presented with history of focal seizures in the form of jerky movements of left upper limb and left lower limb associated with history of up-rolling of eyeball, frothing from mouth and deviation of angel of mouth to right side. There was no associated history of tongue bite, loss of consciousness, altered sensorium, post-ictal confusion, involuntary urination or defecation during episode. These complains were slowly progressive with increasing weakness on left half of body. There was no significant past history and family history related to patients complains.

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The Pathology of Rasmussen Syndrome: Stages of Cortical Involvement and Neuropathological Studies in 45 Hemispherectomies

Cited by 195 publications

References 26 publications

Rasmussen encephalitis: long-term outcome after surgery

Rasmussen encephalitis: long-term outcome after surgery

Neuroprotective intervention by interferon-γ blockade prevents CD8+ T cell-mediated dendrite and synapse loss

Rasmussen's Encephalitis - Chronic Focal Encephalitis (CFE) - A Rare Entity

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