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Rasmussen encephalitis: Response to early immunotherapy in a case of immune-mediated encephalitis.

Liba Z, Muthaffar O, Tang J, Minassian B, Halliday W, Branson H, Ann Yeh E - Neurol Neuroimmunol Neuroinflamm (2015)

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatric Neurology (Z.L.), 2nd Faculty of Medicine, Charles University in Prague, University Hospital Motol, Prague, Czech Republic; Division of Neurology (O.M., B.M., E.A.Y.), Division of Pathology (W.H.), and Division of Neuroradiology (H.B.), Hospital for Sick Children, Toronto, Ontario, Canada; and Department of Pediatrics (O.M., B.M., E.A.Y.), Department of Internal Medicine (J.T.), Department of Laboratory Medicine & Pathobiology (W.H.), and Department of Medical Imaging (H.B.), University of Toronto, Ontario, Canada.

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Rasmussen encephalitis (RE) is a severe immune-mediated neurologic disease characterized by unihemispheric inflammation, progressive neurologic deficits, and intractable focal epilepsy... Functional hemispherectomy is considered to be the only effective intervention for this devastating disease... Information regarding the effect of early and aggressive immunotherapy on cognitive function, MRI disease activity, and seizure control in this population is limited... A 6-year-old boy with an unremarkable past medical history experienced a sudden onset of focal seizures that gradually increased in frequency up to 20 times/day... He is cognitively intact and making progress in school and continues to have a normal neurologic examination... No serious side effects from any of the medications were observed... Search for a specific biologic marker for RE has been elusive, although specific antibodies and OB in the CSF have been described in some cases... In this case, OB were found in the CSF but not in the serum... This child had EEG evidence for generalized and focal seizures... The underlying inflammatory state may have triggered both seizure types... The relatively short follow-up of this patient suggests the need for caution in interpreting these results... However, recent data suggest a potential treatment window early in the context of the initial T-cell-mediated injury to the brain in RE... Larger studies with longer follow-up are necessary to confirm our findings.

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MRI, pathology, and EEG findings(A) Axial fluid-attenuated inversion recovery (FLAIR) MRI sequences of the brain showing right frontal and parietal cortical-subcortical hyperintensities at onset. (B) Axial FLAIR sequences 1 year later after therapy showing regression of the lesions. (C) Axial FLAIR sequences at the time of seizure recurrence after steroid withdrawal showing no new lesions. (D) Biopsy of the right parietal lesion with immunohistochemistry for CD8 showing a cortical cluster of inflammatory cells consisting primarily of cytotoxic T cells. (E) EEG showing right hemispheric ictal episode.
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Figure 1: MRI, pathology, and EEG findings(A) Axial fluid-attenuated inversion recovery (FLAIR) MRI sequences of the brain showing right frontal and parietal cortical-subcortical hyperintensities at onset. (B) Axial FLAIR sequences 1 year later after therapy showing regression of the lesions. (C) Axial FLAIR sequences at the time of seizure recurrence after steroid withdrawal showing no new lesions. (D) Biopsy of the right parietal lesion with immunohistochemistry for CD8 showing a cortical cluster of inflammatory cells consisting primarily of cytotoxic T cells. (E) EEG showing right hemispheric ictal episode.

Mentions: EEG showed (1) right hemispheric slowing and interictal discharges, (2) right hemispheric ictal events (figure 1E), and (3) generalized spike and slow wave discharges. MRI of the brain showed right frontal and parietal cortical-subcortical T2/fluid-attenuated inversion recovery (FLAIR) hyperintensities (figure 1A).


Rasmussen encephalitis: Response to early immunotherapy in a case of immune-mediated encephalitis.

Liba Z, Muthaffar O, Tang J, Minassian B, Halliday W, Branson H, Ann Yeh E - Neurol Neuroimmunol Neuroinflamm (2015)

MRI, pathology, and EEG findings(A) Axial fluid-attenuated inversion recovery (FLAIR) MRI sequences of the brain showing right frontal and parietal cortical-subcortical hyperintensities at onset. (B) Axial FLAIR sequences 1 year later after therapy showing regression of the lesions. (C) Axial FLAIR sequences at the time of seizure recurrence after steroid withdrawal showing no new lesions. (D) Biopsy of the right parietal lesion with immunohistochemistry for CD8 showing a cortical cluster of inflammatory cells consisting primarily of cytotoxic T cells. (E) EEG showing right hemispheric ictal episode.
© Copyright Policy - open-access
Related In: Results  -  Collection

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getmorefigures.php?uid=PMC4335812&req=5

Figure 1: MRI, pathology, and EEG findings(A) Axial fluid-attenuated inversion recovery (FLAIR) MRI sequences of the brain showing right frontal and parietal cortical-subcortical hyperintensities at onset. (B) Axial FLAIR sequences 1 year later after therapy showing regression of the lesions. (C) Axial FLAIR sequences at the time of seizure recurrence after steroid withdrawal showing no new lesions. (D) Biopsy of the right parietal lesion with immunohistochemistry for CD8 showing a cortical cluster of inflammatory cells consisting primarily of cytotoxic T cells. (E) EEG showing right hemispheric ictal episode.
Mentions: EEG showed (1) right hemispheric slowing and interictal discharges, (2) right hemispheric ictal events (figure 1E), and (3) generalized spike and slow wave discharges. MRI of the brain showed right frontal and parietal cortical-subcortical T2/fluid-attenuated inversion recovery (FLAIR) hyperintensities (figure 1A).

View Article: PubMed Central - PubMed

Affiliation: Department of Pediatric Neurology (Z.L.), 2nd Faculty of Medicine, Charles University in Prague, University Hospital Motol, Prague, Czech Republic; Division of Neurology (O.M., B.M., E.A.Y.), Division of Pathology (W.H.), and Division of Neuroradiology (H.B.), Hospital for Sick Children, Toronto, Ontario, Canada; and Department of Pediatrics (O.M., B.M., E.A.Y.), Department of Internal Medicine (J.T.), Department of Laboratory Medicine & Pathobiology (W.H.), and Department of Medical Imaging (H.B.), University of Toronto, Ontario, Canada.

AUTOMATICALLY GENERATED EXCERPT
Please rate it.

Rasmussen encephalitis (RE) is a severe immune-mediated neurologic disease characterized by unihemispheric inflammation, progressive neurologic deficits, and intractable focal epilepsy... Functional hemispherectomy is considered to be the only effective intervention for this devastating disease... Information regarding the effect of early and aggressive immunotherapy on cognitive function, MRI disease activity, and seizure control in this population is limited... A 6-year-old boy with an unremarkable past medical history experienced a sudden onset of focal seizures that gradually increased in frequency up to 20 times/day... He is cognitively intact and making progress in school and continues to have a normal neurologic examination... No serious side effects from any of the medications were observed... Search for a specific biologic marker for RE has been elusive, although specific antibodies and OB in the CSF have been described in some cases... In this case, OB were found in the CSF but not in the serum... This child had EEG evidence for generalized and focal seizures... The underlying inflammatory state may have triggered both seizure types... The relatively short follow-up of this patient suggests the need for caution in interpreting these results... However, recent data suggest a potential treatment window early in the context of the initial T-cell-mediated injury to the brain in RE... Larger studies with longer follow-up are necessary to confirm our findings.

No MeSH data available.


Related in: MedlinePlus