MRI brain volumetry in Rasmussen encephalitis: The fate of affected and "unaffected" hemispheres

Department of Neuropathology, University of Bonn, Bonn, Germany.
Neurology (Impact Factor: 8.29). 04/2005; 64(5):885-7. DOI: 10.1212/01.WNL.0000152895.23010.52
Source: PubMed


Rasmussen encephalitis (RE) is regarded as a unihemispheric disease. Serial three-dimensional MRIs of 18 patients under immunotherapy were analyzed volumetrically and planimetrically. Median volume loss was significantly higher in the affected than in the unaffected hemispheres (29.9 cm3/y vs 6.8 cm3/y). Correlation of the planimetrically and volumetrically assessed hemispheric ratios (HRs) was significant. The results support the concept of RE as a basically unilateral disease. Planimetric HR assessment is valid and time efficient.

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    • "We did not find HRvol values >1.0 as an indicator of hemispheric swelling, which has been reported to occur especially in the early stage of the disease (Chiapparini et al., 2003). The HRvol change per year in our study was smaller than reported by Larionov et al. (2005) (median )2.7%/year vs. )4.0%/year). A study of Bien et al. (2004) reported about HRplan changes in two patient groups with RE: one was treated with tacrolimus and the other received no immunosuppressive therapy. "
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    ABSTRACT: Rasmussen encephalitis is a chronic immune-mediated disease leading to unilateral atrophy on magnetic resonance imaging (MRI) and progressive neurologic deficits. Until now, quantitative parameters describing the course of the disease on MRI require manual intervention and are, therefore, time-consuming and observer-dependent. Furthermore, regional atrophy differences cannot be evaluated with the previously published methods. In this study we present a fully automated volumetric approach applied to serial MRI scans of 12 patients with Rasmussen encephalitis. We retrospectively analyzed 12 patients with Rasmussen encephalitis with a disease onset between 2001 and 2008. All patients underwent a total of 66 serial MRI scans including a three-dimensional T(1) data set. The volumetric analysis was based on standard procedures of the freely available software FMRIB Software Library (FSL) and required about 45 min per scan. Furthermore, planimetric analyses were performed on 51 scans as described previously. The relative and absolute volume loss of the affected hemisphere was significantly higher compared to the unaffected hemisphere. Referring to regional atrophy differences our results show that the frontal lobe and the insula were preferentially involved in the atrophic process. The degree of hemispheric, parietal, and occipital atrophy was negatively correlated with the age at disease onset, indicating a more aggressive and outspread disease in young children compared to adolescents. Volumetric hemispheric ratio and planimetric hemispheric ratio correlated significantly, but planimetric hemispheric ratio underestimated the real degree of hemiatrophy, especially in patients with predominant affections outside the frontoinsular region. The volumetric analysis presented here offers a precise assessment of the disease progression in Rasmussen encephalitis in an observer-independent and time-efficient manner and gives an interesting insight into the course of the disease on MRI. The degree of atrophy evaluated with this method correlates with clinical parameters and is comparable to atrophy rates in patients receiving immunotherapy in preceding planimetric MRI studies.
    Full-text · Article · Feb 2012 · Epilepsia
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    • " 2002d ) . However , it may , in some cases , go on for several years ( Bhatjiwale et al . , 1998 ; Chiapparini et al . , 2003 ) . In 11 immunotreated RE patients , volumetric assessment of serial MRIs during early disease stages revealed a median tissue loss of 29 . 9 cm 3 per year in the affected and of 6 . 8 cm 3 in the unaffected hemispheres ( Larionov et al . , 2005 ) . Totally normal findings on very early scans have been reported , but are rare ( Geller et al . , 1998 ; Kaiboriboon et al . , 2000 ; Lee et al . , 2001 ) . Gadolinium enhancement is very rare in RE ( Nakasu et al . , 1997 ; Yacubian et al . , 1997 ; Bien et al . , 2002c ; Chiapparini et al . , 2003 ) ."
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    ABSTRACT: Rasmussen encephalitis (RE) is a rare but severe immune-mediated brain disorder leading to unilateral hemispheric atrophy, associated progressive neurological dysfunction and intractable seizures. Recent data on the pathogenesis of the disease, its clinical and paraclinical presentation, and therapeutic approaches are summarized. Based on these data, we propose formal diagnostic criteria and a therapeutic pathway for the management of RE patients. © The Author (2005). Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved.
    Full-text · Article · Apr 2005 · Brain
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    ABSTRACT: Die Rasmussen-Enzephalitis (RE) ist eine ebenso schwerwiegende wie pathophysiologisch faszinierende Erkrankung. Die chronische Entzündung befällt eine der beiden Großhirnhemisphären und zerstört diese in einem Monate bis Jahre dauernden Krankheitsprozess. Die Patienten — überwiegend Kinder — leiden an häufigen pharmakoresistenten Anfällen, oft in Form einer Epilepsia partialis continua. Parallel zur Atrophie der betroffenen Hemisphäre verschlechtern sich zunehmend die von dieser Hirnhälfte getragenen neurologischen Funktionen. Es resultiert im Endstadium ein meist hochgradiges sensomotorisches Hemisyndrom, eine Hemianopsie, eine kognitive Einschränkung und — bei Befall der sprachdominanten Hemisphäre — eine Aphasie. In den letzten 5 Jahren haben neue Forschungsergebnisse zu einem vertieften Verständnis der Pathogenese geführt. Formelle diagnostische Kriterien wurden etabliert, und es wurden neue Therapieoptionen aufgezeigt, durch die der Erkrankungsfortschritt gestoppt oder zumindest verlangsamt werden kann. Dieser Beitrag resümiert diese aktuellen Resultate vor dem Hintergrund der älteren Erkenntnisse und gibt Empfehlungen zum diagnostischen und therapeutischen Vorgehen.
    No preview · Article · Dec 2005 · Der Nervenarzt
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