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MRI FINDINGS IN THE DIAGNOSIS OF RASMUSSEN’S ENCEPHALITIS. A ACHOUR, S JERBI OMEZZINE, S YOUNES 1 , S BOUABID, MH SFAR 1 , HA HAMZA. Department of Medical Imaging, Tahar Sfar University Hospital Center, Mahdia , Tunisia
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MRI FINDINGS IN THE DIAGNOSIS OF RASMUSSEN’S ENCEPHALITIS A ACHOUR, S JERBI OMEZZINE, S YOUNES1, S BOUABID, MH SFAR1, HA HAMZA. Department of Medical Imaging, TaharSfar University Hospital Center, Mahdia, Tunisia 1Department of InternalMedicine, TaharSfar University Hospital Center, Mahdia NR16
INTRODUCTION : • Rasmussen encephalitis (RE) is a chronicinflamatorydiseasewich affects mainlychildren , but alsoyoungadults. • RE ischaracterised by unilateralhemespheric progressive atrophy, consecutiveneurologicdeficits and severe focal epilepsy. • Pharmacoresistanceagainst anti epilepticdrugsisnotedearly in the course of the disease.
Diagnosisisbased on featuresfrom the electroencephalogram (EEG) , MRI and clinical and/or histologicalcharacteristics. Magnetic resonance (MR) findings, associated with clinical data and electroencephalogram (EEG), may indicate the diagnosis and could be an indicative of prognosis Currentapproaches of immuno-modulatory and surgical techniques are discussed. Onlysurgeryis able to providecompleteseizure control. Wepresent the case of RE and neuroradiologicalfindings.
Materials and methods • We report the longitudinal history of a 18-year-old women with RE who presented with seizures. Neurological symptoms included recurrent partial seizures with secondary generalized convulsions. • There was no concept of neonatal distress and psychomotor development was normal. • She presented partial seizures to clonic seizures type of predominantly straight-brachialcheiro with secondary generalization. Changes in several AEDs in combination was negative and there was an increase in seizure frequency that became multiple daily requiring hospitalization for 3 days in intensive care unit.
Results - The electroencephalogram (EEG) objectified slower background activity with a clear asymmetry of the plot. In fact, there was the left side of paroxysmal abnormalities in type of slow waves.- The brain MRI in T2 and flair showed atrophy of the right hemisphere with dilatation of the lateral ventricle ipsilateral cortical atrophy and a hyperintense white matter of the centrum ovale left.
brain MRI in T2 and flair showed atrophy of the right hemisphere with dilatation of the lateral ventricle ipsilateral cortical atrophy and a hyperintense white matter of the centrumovale left.
- The patient was treated with 3 antiepileptics in combination but no reduction in seizure frequency was observed. - Immunomodulatory therapy was then tried, it was based on corticosteroid associatedwithimmunoglobulins by intravenous - The evolution was marked by a disappearance of transitional seizure.
DISCUSSION Clinicalfeatrues : • Rasmussen’sencephalitis (RE) is an infrequent progressive and inflamatorydisease of the brainaffecting one hemesphere . • Rasmussen’sencephalitisistypicallyassociatedwithintractable focal epilepsy, cognitive decline and hemiparesis. • The ageatonsetis in childehood , between 6 and 8 years (range 1-13 years). • RE affects childrenwhowerepreviouselyhealthy. • Both sexes are equallyaffected.
-The diseasestartswith focal seizures. -RE ischaractirazed by polymorphusseizuresthereforeincludingsomatosensory , motor , visual, or psychomotorseizureswichbecamerapidlyresistant to antiepileptictreatment. -The pathologyisusually not lethal but leads to cognitive , motor and visualdefects . -The relation shipbetween the seizures’ frequency and the neurologicaldeteriorationiscomplex and not linear. -The course of the diseaseisdividedinto 3 stages. -Duringthis stage the seizuresfrequency and intensityprogressivelyincrease .
Histology : The aetiology and pathogenesis of RE stillremainunkown Threehypotheses have been forwarded: a direct viral insult , an autoimmuneprocesstrigerredthrough a viral agent , a primaryautoimmuneprocess . Diagnosis : Is based on featuresfrom the electroencephalogram(EEG) , MRI and clinical and / or histologicalcharacteristics . The EEG shows slowing and multiple epileptogenic anomalies, always in the samehemisphere.
Radiology: • -In the MRI , hyperintensesignals in the white matter of the affectedhemisphere and cortical swelling are seen , followed by cortical atrophy . • -RepetitiveMRIs in the begining of the disease are necessary to visualise the progression of and consolidate the diagnosis. • -The patient mayalsobenefitfrom a gadolinuim-MRI, MRI-angiography or angiography to excludedeffirentialdiagnosis of vascularitis , wichoccasionallypresents on only one hemisphere . • -Otherbrainimaging techniques including positron emissiontomography (PET) or single photon emissioncomputedtomography (SPECT) wichalsoshouws changes confined to only one hemisphere. • MR-spectroscopyusually shows decreased N-acetylaspartate a marker of neuronal integrity .
Treatment -RE ishighlypharmacoresistant in more than 80% of cases . -Antiepilepticdrugsreduced the risk of generalisedseizures . -Immunomodulatorytreatmentincludessteroids , immunoglobulins (IG) , plasmapherisis (PE) and immunosupressivetherapy . -Up to now, onlysurgeryallowsseizure control and remains the most efficient treatment .
Conclusion -Rasmussen’s encephalitis is a devastating syndrome of multifocal brain dysfunction and focal seizures. - Magnetic resonance (MR) findings, associated with clinical data and electroencephalogram (EEG), may indicate the diagnosis and could be an indicative of prognosis .
