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Young woman with a four-year history of epilepsy and progressive focal cortical atrophy - What is the diagnosis?

Pati S, Cobos-Sillero MI, Buchbinder B, Eskandar E, Cole AJ - Epilepsy Behav Case Rep (2014)

Bottom Line: The pathogenesis of disease progression in drug-refractory epilepsy is poorly understood.There was no consensus reached on the final diagnosis although the hypothesis of dual pathology (adult variant of Rasmussen's encephalitis and perinatal stroke) was considered.The possible role of inflammation in the progression of epilepsy caused by a "static" lesion (perinatal stroke) is discussed.

View Article: PubMed Central - PubMed

Affiliation: Epilepsy Service, Massachusetts General Hospital, Boston, MA, USA.

ABSTRACT
The pathogenesis of disease progression in drug-refractory epilepsy is poorly understood. We report the case of a young woman with a four-year history of epilepsy that progressed rapidly as evidenced by the development of progressive focal cortical atrophy. She underwent biopsy that showed perinatal ischemia and a prominent inflammatory response, including T-cell infiltration and microglial activation. There was no consensus reached on the final diagnosis although the hypothesis of dual pathology (adult variant of Rasmussen's encephalitis and perinatal stroke) was considered. The possible role of inflammation in the progression of epilepsy caused by a "static" lesion (perinatal stroke) is discussed.

No MeSH data available.


Related in: MedlinePlus

(a) Brain MRI axial FLAIR and (b) T2-weighted sequence showing encephalomalacia in the anterior right frontal lobe and another discrete noncontiguous focus of encephalomalacia involving the posterior right frontal operculum.
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f0005: (a) Brain MRI axial FLAIR and (b) T2-weighted sequence showing encephalomalacia in the anterior right frontal lobe and another discrete noncontiguous focus of encephalomalacia involving the posterior right frontal operculum.

Mentions: Magnetic resonance imaging of the brain performed at the time of initial presentation showed encephalomalacia affecting the anterior and the inferior portion of the right frontal lobe, with involvement of the anterior aspect of the right middle and perifrontal gyri as well as the right orbitofrontal gyrus. There was another discrete noncontiguous focus of encephalomalacia involving the posterior right frontal operculum. The hippocampi were symmetric and normal in size and signal intensity. Subsequent serial brain MRIs performed yearly showed progressive atrophy in the right anterior quadrant that was confirmed by quantitative volumetric analysis (Fig. 1, Fig. 2). Fluorine-18 fluorodeoxyglucose (18FDG) PET scan showed marked cortical hypometabolism in the right hemisphere, most pronounced proximal to the areas of encephalomalacia in the anterior right frontal lobe and in the right frontal operculum.


Young woman with a four-year history of epilepsy and progressive focal cortical atrophy - What is the diagnosis?

Pati S, Cobos-Sillero MI, Buchbinder B, Eskandar E, Cole AJ - Epilepsy Behav Case Rep (2014)

(a) Brain MRI axial FLAIR and (b) T2-weighted sequence showing encephalomalacia in the anterior right frontal lobe and another discrete noncontiguous focus of encephalomalacia involving the posterior right frontal operculum.
© Copyright Policy - CC BY-NC-ND
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4307882&req=5

f0005: (a) Brain MRI axial FLAIR and (b) T2-weighted sequence showing encephalomalacia in the anterior right frontal lobe and another discrete noncontiguous focus of encephalomalacia involving the posterior right frontal operculum.
Mentions: Magnetic resonance imaging of the brain performed at the time of initial presentation showed encephalomalacia affecting the anterior and the inferior portion of the right frontal lobe, with involvement of the anterior aspect of the right middle and perifrontal gyri as well as the right orbitofrontal gyrus. There was another discrete noncontiguous focus of encephalomalacia involving the posterior right frontal operculum. The hippocampi were symmetric and normal in size and signal intensity. Subsequent serial brain MRIs performed yearly showed progressive atrophy in the right anterior quadrant that was confirmed by quantitative volumetric analysis (Fig. 1, Fig. 2). Fluorine-18 fluorodeoxyglucose (18FDG) PET scan showed marked cortical hypometabolism in the right hemisphere, most pronounced proximal to the areas of encephalomalacia in the anterior right frontal lobe and in the right frontal operculum.

Bottom Line: The pathogenesis of disease progression in drug-refractory epilepsy is poorly understood.There was no consensus reached on the final diagnosis although the hypothesis of dual pathology (adult variant of Rasmussen's encephalitis and perinatal stroke) was considered.The possible role of inflammation in the progression of epilepsy caused by a "static" lesion (perinatal stroke) is discussed.

View Article: PubMed Central - PubMed

Affiliation: Epilepsy Service, Massachusetts General Hospital, Boston, MA, USA.

ABSTRACT
The pathogenesis of disease progression in drug-refractory epilepsy is poorly understood. We report the case of a young woman with a four-year history of epilepsy that progressed rapidly as evidenced by the development of progressive focal cortical atrophy. She underwent biopsy that showed perinatal ischemia and a prominent inflammatory response, including T-cell infiltration and microglial activation. There was no consensus reached on the final diagnosis although the hypothesis of dual pathology (adult variant of Rasmussen's encephalitis and perinatal stroke) was considered. The possible role of inflammation in the progression of epilepsy caused by a "static" lesion (perinatal stroke) is discussed.

No MeSH data available.


Related in: MedlinePlus