An open study of tacrolimus therapy in Rasmussen encephalitis

Bien, Christian G.; Gleißner, Ulrike; Sassen, Robert; Widman, Guido; Urbach, Horst; Elger, Christian E.

doi:10.1212/01.wnl.0000128044.94294.87

Cited by 87 publications

(69 citation statements)

References 10 publications

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“…1a) [17][18][19]. Although the rate of progression of atrophic changes is variable, volumetric studies indicate the hemispheric volume loss peaks in the first 8 months of disease [18,20,21]. Interestingly, topographic analysis of brain atrophy showed that the frontal lobe and the insula were preferentially involved by the atrophic process.…”

Section: Introductionmentioning

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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Section: Introductionmentioning

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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“…6,7 Based on the prevalence of cytomegalovirus genome in the brains of these patients 8 , we began using ganciclovir, an inhibitor of viral replication as treatment. We have treated a total of eight patients but the three in this report are the only ones treated within three months of onset of the illness.…”

Section: Discussionmentioning

confidence: 99%

Early Treatment of a Progressive Rasmussen's Like Syndrome with Ganciclovir

McLachlan

Diosy

Levin

2011

Can. J. Neurol. Sci.

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Background:Escalating focal sensorimotor seizures, progressive neurologic deficit and cognitive decline with associated typical magnetic resonance imaging (MRI) findings or pathological confirmation constitute the syndrome of Rasmussen's encephalitis.Methods:Three patients with this clinical scenario had long-term follow-up after being given the antiviral drug ganciclovir 10 mg/kg/day IV for ten days within one to three months of disease onset.Results:Seizures occurred at least hourly and were localized to the Rolandic region. The MRIs were normal in one patient and in two showed changes consistent with ongoing seizures. Two patients, one whose short-term outcome was reported previously, had immediate and sustained cessation of seizures and resolution of their neurologic deficit. One patient with seizures from both hemispheres did not respond and went on to hemispherectomy that confirmed chronic encephalitis. Seizures continued in five other patients treated 6 to 72 months after disease onset.Conclusion:The sustained seizure control after ganciclovir in two of three patients suggests this drug may be effective when given early in the course of this subacute intractable focal epilepsy syndrome.

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“…Association has not proved better. Steroids, plasmapheresis, intravenous immunoglobulins, ␣ -interferon and tacrolimus have been used to stop the inflammatory process [4,10,11] . Immunoglobulins seem more eligible as a first-line treatment.…”

Section: Discussionmentioning

confidence: 99%

“…It is widely accepted as a sort of focal autoimmune encephalitis, resulting in a particular epileptic encephalopathy. This hypothesis is based on several facts: (1) clinical resemblance to Russian spring-summer encephalitis of viral origin, which causes epilepsia partialis continua; (2) presence of lymphocyte inflammation and glial nodules in brain biopsy; (3) inconstant finding in serum and cerebrospinal fluid (CSF) of antibodies against subunit 3 of ionotropic glutamate receptor; (4) in some cases, good response to steroids, immunoglobulins and plasmapheresis [2][3][4][5][6][7][8][9][10][11] .We present a patient whose RE apparently began at age 51. We have focused on clinical findings (posterior hemispheric involvement with absence of epilepsia partialis continua), neuroimaging evolution, brain biopsy and good response to intravenous inmunoglobulins.…”

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confidence: 99%