Putaminal involvement in Rasmussen encephalitis

Rajesh, B; Kesavadas, Chandrasekharan; Radhakrishnan, Ashalatha

doi:10.1007/s00247-006-0176-4

Cited by 12 publications

(15 citation statements)

References 10 publications

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“…In our study group we found a preferential involvement of the frontal lobe or the insula in 75% of the patients. This is in agreement with previous studies reporting about MRI in RE (Bhatjiwale et al., 1998; Bien et al., 2002b; Chiapparini et al., 2003; Rajesh et al., 2006). However, in the remaining 25% the most severe atrophy was located in the parietal and occipital lobe, where it might be underestimated by HRplan (see below).…”

Section: Discussionsupporting

confidence: 94%

“…In contrast to several previous studies we did not find that the caudate nucleus was preferentially affected (Bhatjiwale et al., 1998; Chiapparini et al., 2003; Granata et al., 2003). This finding is supported by a study of Rajesh et al. (2006), who found predominant atrophy of both the putamen and the caudate nucleus in a group of 12 RE patients.…”

Section: Discussionsupporting

confidence: 66%

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Automated 3D MRI volumetry reveals regional atrophy differences in Rasmussen encephalitis

et al. 2012

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Summary Purpose: 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. Methods: 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 T1 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. Key Findings: 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. Significance: 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.

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Section: Discussionsupporting

confidence: 94%

Section: Discussionsupporting

confidence: 66%

Automated 3D MRI volumetry reveals regional atrophy differences in Rasmussen encephalitis

et al. 2012

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“…5 Seizures typically become frequent and medically refractory, and patients may suffer recurrent bouts of status epilepticus. 6 Multiple seizure semiologies emerge over time (e.g., in one series, 58% of patients developed multiple seizure types by 3 months from presentation). 4 Epilepsia partialis continua (EPC) is a rare seizure type with unusual clinical associations, including RE.…”

Section: Clinical Featuresmentioning

confidence: 99%

“…While several cortical patterns have been reported, frontal lobe and insula volume loss is commonly reported. 3,6,8,9,[11][12][13][14] In contrast, changes in posterior brain regions, especially the occipital lobe, are less frequently reported early in the clinical course and may be more common with younger age of onset. 12 Subcortical gray matter structures, including the ipsilateral amygdala, hippocampus, and striatum (especially the putamen and caudate nucleus), are frequently involved and may be among the earliest affected regions for some patients.…”

Section: Neuroimagingmentioning

confidence: 99%

“…12 Subcortical gray matter structures, including the ipsilateral amygdala, hippocampus, and striatum (especially the putamen and caudate nucleus), are frequently involved and may be among the earliest affected regions for some patients. 3,6,8,11,12 In particular, a flattening of the caudate head with a change in the contour of the frontal horn of the lateral ventricle on axial sections from that of "boomerang" to that of a "candle flame" is a conspicuous finding. 3 Hyperintense lesions on fluid-attenuated inversion recovery or T2-weighted sequences are another characteristic imaging finding.…”

Section: Neuroimagingmentioning

confidence: 99%

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Rasmussen Encephalitis: An Update

et al. 2020

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Rasmussen encephalitis (RE) is a rare, devastating, progressive pediatric epilepsy. First described 60 years ago, RE continues to present challenges in diagnosis and management. RE causes a unilateral focal epilepsy in children that typically becomes medically refractory, results in significant hemiparesis, and causes progressive cognitive decline. The etiology is a cell-mediated immune attack on one cerebral hemisphere, though the inciting antigen remains unknown. While the underlying histopathology is unilateral and RE is described as “unihemispheric,” studies have demonstrated (1) atrophy of the unaffected hemisphere, (2) electroencephalographic abnormalities (slowing and spikes) in the unaffected hemisphere, and (3) cognitive decline referable to the unaffected hemisphere. These secondary contralateral effects likely reflect the impact of uncontrolled epileptic activity (i.e., epileptic encephalopathy). Hemispheric disconnection (HD) renders 70 to 80% of patients seizure free. While it has the potential to limit the influence of seizures and abnormal electrical activity emanating from the pathological hemisphere, HD entails hemiparesis and hemianopia, as well as aphasia for patients with dominant HD. With the recent expansion of available immunomodulatory therapies, there has been interest in identifying an alternative to HD, though evidence for disease modification is limited to date. We review what is known and what remains unknown about RE.

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Neurology

Al-Tubaikh

2023

Internal Medicine

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Putaminal involvement in Rasmussen encephalitis

Abstract: Our study highlights frequent putaminal atrophy and signal changes in RE, which suggests a more extensive basal ganglion involvement than emphasized previously. Recognition of putaminal changes may be a useful additional tool in the radiological diagnosis of RE.

Cited by 12 publications

References 10 publications

Automated 3D MRI volumetry reveals regional atrophy differences in Rasmussen encephalitis

Automated 3D MRI volumetry reveals regional atrophy differences in Rasmussen encephalitis

Rasmussen Encephalitis: An Update

Neurology

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