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Bilateral Rasmussen's encephalitis associated with type II focal cortical dysplasia: Dormant 'second' epileptogenic zone in contralateral disease.

Frigeri T, Hemb M, Paglioli E, Hoefel JR, Silva V, Vinters H, Palmini A - Epilepsy Behav Case Rep (2013)

Bottom Line: We demonstrated histopathological evidence of RE and type II FCD in the left hemisphere, which led to EPC on the right hemibody at presentation.This is highly compatible with RE (+/- FCD) in the right hemisphere as well.Although the association of FCD and RE - as well as the occasional occurrence of bilateral RE - has already been reported [3-5], this is the first such case in which bilateral RE and FCD co-occur.

View Article: PubMed Central - PubMed

Affiliation: Porto Alegre Epilepsy Surgery Program, Hospital São Lucas da Pontifícia Universidade Catolica do Rio Grande do Sul (PUCRS), Porto Alegre, Brazil.

ABSTRACT
Rasmussen's encephalitis (RE) is an inflammatory, probably autoimmune disorder manifested by refractory seizures and progressive deterioration of one cerebral hemisphere [1]. Here, we describe the unfortunate history of a girl with a progressive disorder which, upon clinical, neuroimaging, and histopathological evaluation, proved to be bilateral RE associated with type II focal cortical dysplasia. Whether the second pathology is relevant for the extent of the disease is discussed. We demonstrated histopathological evidence of RE and type II FCD in the left hemisphere, which led to EPC on the right hemibody at presentation. In addition, there was unequivocal progressive cortical and subcortical atrophy of the right hemisphere, which accounted for the EPC on the left hemibody. This is highly compatible with RE (+/- FCD) in the right hemisphere as well. Although the association of FCD and RE - as well as the occasional occurrence of bilateral RE - has already been reported [3-5], this is the first such case in which bilateral RE and FCD co-occur.

No MeSH data available.


Related in: MedlinePlus

Control MRI showing progressive atrophy of the contralateral hemisphere (nonoperated).
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f0020: Control MRI showing progressive atrophy of the contralateral hemisphere (nonoperated).

Mentions: The girl became seizure-free for 1 year with remarkable improvement in global functioning, including walking and better cognition. One year later, in the context of high fever, this girl started with EPC in the other (left) hemibody. She quickly deteriorated, and a new MRI showed severe diffuse atrophy of the left hemisphere (where operation had been performed) with increased cortical and subcortical signal extending way beyond the margins of the previous resection. There was also cortical and subcortical atrophy of the right hemisphere, which was unequivocal upon comparison with the MRI performed a few years earlier (Fig. 4).


Bilateral Rasmussen's encephalitis associated with type II focal cortical dysplasia: Dormant 'second' epileptogenic zone in contralateral disease.

Frigeri T, Hemb M, Paglioli E, Hoefel JR, Silva V, Vinters H, Palmini A - Epilepsy Behav Case Rep (2013)

Control MRI showing progressive atrophy of the contralateral hemisphere (nonoperated).
© Copyright Policy - CC BY-NC-SA
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4150617&req=5

f0020: Control MRI showing progressive atrophy of the contralateral hemisphere (nonoperated).
Mentions: The girl became seizure-free for 1 year with remarkable improvement in global functioning, including walking and better cognition. One year later, in the context of high fever, this girl started with EPC in the other (left) hemibody. She quickly deteriorated, and a new MRI showed severe diffuse atrophy of the left hemisphere (where operation had been performed) with increased cortical and subcortical signal extending way beyond the margins of the previous resection. There was also cortical and subcortical atrophy of the right hemisphere, which was unequivocal upon comparison with the MRI performed a few years earlier (Fig. 4).

Bottom Line: We demonstrated histopathological evidence of RE and type II FCD in the left hemisphere, which led to EPC on the right hemibody at presentation.This is highly compatible with RE (+/- FCD) in the right hemisphere as well.Although the association of FCD and RE - as well as the occasional occurrence of bilateral RE - has already been reported [3-5], this is the first such case in which bilateral RE and FCD co-occur.

View Article: PubMed Central - PubMed

Affiliation: Porto Alegre Epilepsy Surgery Program, Hospital São Lucas da Pontifícia Universidade Catolica do Rio Grande do Sul (PUCRS), Porto Alegre, Brazil.

ABSTRACT
Rasmussen's encephalitis (RE) is an inflammatory, probably autoimmune disorder manifested by refractory seizures and progressive deterioration of one cerebral hemisphere [1]. Here, we describe the unfortunate history of a girl with a progressive disorder which, upon clinical, neuroimaging, and histopathological evaluation, proved to be bilateral RE associated with type II focal cortical dysplasia. Whether the second pathology is relevant for the extent of the disease is discussed. We demonstrated histopathological evidence of RE and type II FCD in the left hemisphere, which led to EPC on the right hemibody at presentation. In addition, there was unequivocal progressive cortical and subcortical atrophy of the right hemisphere, which accounted for the EPC on the left hemibody. This is highly compatible with RE (+/- FCD) in the right hemisphere as well. Although the association of FCD and RE - as well as the occasional occurrence of bilateral RE - has already been reported [3-5], this is the first such case in which bilateral RE and FCD co-occur.

No MeSH data available.


Related in: MedlinePlus