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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

Preoperative MRI showing the alterations described above. Note that there is no hint of pathology on the contralateral hemisphere.
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f0005: Preoperative MRI showing the alterations described above. Note that there is no hint of pathology on the contralateral hemisphere.

Mentions: This girl had an uneventful history until starting with partial motor seizures at age 4.5 years. The attacks involved the entire right hemibody and progressed to medically refractory epilepsia partialis continua (EPC). At age 6, her right arm and hand were nonfunctional, she could not walk, swallowing was difficult, and cognition was markedly affected by unstoppable seizures and recurrent ICU admissions. MRI showed a high-intensity lesion in the left precentral gyrus, suggestive of FCD, and also a more diffuse atrophy of the left hemisphere, including cortical (insula) and subcortical (caudate and hippocampus) structures (Fig. 1).


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)

Preoperative MRI showing the alterations described above. Note that there is no hint of pathology on the contralateral hemisphere.
© Copyright Policy - CC BY-NC-SA
Related In: Results  -  Collection

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

f0005: Preoperative MRI showing the alterations described above. Note that there is no hint of pathology on the contralateral hemisphere.
Mentions: This girl had an uneventful history until starting with partial motor seizures at age 4.5 years. The attacks involved the entire right hemibody and progressed to medically refractory epilepsia partialis continua (EPC). At age 6, her right arm and hand were nonfunctional, she could not walk, swallowing was difficult, and cognition was markedly affected by unstoppable seizures and recurrent ICU admissions. MRI showed a high-intensity lesion in the left precentral gyrus, suggestive of FCD, and also a more diffuse atrophy of the left hemisphere, including cortical (insula) and subcortical (caudate and hippocampus) structures (Fig. 1).

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