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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

Histopathological analysis again confirming RE.
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f0025: Histopathological analysis again confirming RE.

Mentions: In extremis, she underwent functional hemispherectomy of the left hemisphere, complementing the initial resection, in the hope that this might interfere with disease progression. Nonetheless, left-sided EPC and cognitive deterioration persisted. She is currently tetraparetic, bed-ridden, and does not communicate. Pathology again confirmed RE (Fig. 5).


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)

Histopathological analysis again confirming RE.
© Copyright Policy - CC BY-NC-SA
Related In: Results  -  Collection

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

f0025: Histopathological analysis again confirming RE.
Mentions: In extremis, she underwent functional hemispherectomy of the left hemisphere, complementing the initial resection, in the hope that this might interfere with disease progression. Nonetheless, left-sided EPC and cognitive deterioration persisted. She is currently tetraparetic, bed-ridden, and does not communicate. Pathology again confirmed RE (Fig. 5).

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