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Multimodal Longitudinal Imaging of Focal Status Epilepticus

Published online by Cambridge University Press:  16 February 2016

Colin P. Doherty ,
Andrew J. Cole ,
P. Ellen Grant ,
Alan Fischman ,
Elizabeth Dooling ,
Daniel B. Hoch ,
Tessa Hedley White  and
G. Rees Cosgrove
Colin P. Doherty
Affiliation:
Epilepsy Service, Neurology Service, Massachusetts General Hospital and Harvard Medical School, BostonUSA
Andrew J. Cole*
Affiliation:
Epilepsy Service, Neurology Service, Massachusetts General Hospital and Harvard Medical School, BostonUSA
P. Ellen Grant
Affiliation:
Department of Neuroradiology, Massachusetts General Hospital and Harvard Medical School, BostonUSA
Alan Fischman
Affiliation:
Nuclear Medicine Unit, Department of Radiology, Massachusetts General Hospital and Harvard Medical School, BostonUSA
Elizabeth Dooling
Affiliation:
Pediatric Neurology, Department of Neurology, Massachusetts General Hospital and Harvard Medical School, BostonUSA
Daniel B. Hoch
Affiliation:
Epilepsy Service, Neurology Service, Massachusetts General Hospital and Harvard Medical School, BostonUSA
Tessa Hedley White
Affiliation:
Department of Neuropathology, Massachusetts General Hospital and Harvard Medical School, BostonUSA
G. Rees Cosgrove
Affiliation:
Epilepsy Surgery Unit, Neurosurgical Service, Massachusetts General Hospital and Harvard Medical School, BostonUSA
*
MGH Epilepsy Service, VBK-830, Massachusetts General Hospital, Fruit Street, Boston, Massachusetts 02114, USA
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Abstract

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Background:

Little is understood about the evolution of structural and functional brain changes during the course of uncontrolled focal status epilepticus in humans.

Methods:

We serially evaluated and treated a nine-year-old girl with refractory focal status epilepticus. Long-term EEG monitoring, MRI, MRA, SPECT, intraoperative visualization of affected cortex, and neuropathological examination of a biopsy specimen were conducted over a three year time span. Imaging changes were correlated with simultaneous treatment and EEG findings.

Results:

The EEG monitoring showed almost continuous spike discharges emanating initially from the right frontocentral area. These EEG abnormalities were intermittently suppressed by treatment with anesthetics. Over time, additional brain areas developed epileptiform EEG abnormalities. Serial MRI studies demonstrated an evolution of changes from normal, through increased regional T2 signal to generalized atrophy. An MRAdemonstrated dilatation of the middle cerebral artery stem on the right compared to the left with a broad distribution of flow-related enhancement. An 18FDG-PET scan showed a dramatically abnormal metabolic profile in the same right frontocentral areas, which modulated in response to treatment during the course of the illness. A right frontotemporal craniotomy revealed a markedly hyperemic cortical focus including vascular shunting. A sample of resected cortex showed severe gliosis and neuronal death.

Conclusions:

The co-registration of structural and functional imaging and its correlation with operative and pathological findings in this case illustrates the relentless progression of regional and generalized abnormalities in intractable focal status epilepticus that were only transiently modified by exhaustive therapeutic interventions. Increased flow through large vessels appeared to be shunted and did not translate into increased microvascular perfusion.

Type
Case Report
Information
Canadian Journal of Neurological Sciences , Volume 31 , Issue 2 , May 2004 , pp. 276 - 281
Copyright
Copyright © The Canadian Journal of Neurological 2004

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