Evolution of MRI changes in Rasmussen's encephalitis.
ABSTRACT We studied the MRI findings in 16 patients with Rasmussen's encephalitis (RE), further analysed serial MRI changes in 11 of them and correlated it with clinical features.
The diagnosis of RE was based on the European consensus statement (Brain, 128, 2005, 454). Details related to demographical, clinical, MRI observations were analysed.
Forty MRIs of brain of 16 patients were reviewed. Eleven patients had undergone serial brain MRIs ranging from two to five occasions. All the patients had unihemispheric focal cortical atrophy, predominantly in the perisylvian region (n = 13). Other features were white matter signal changes (n = 14), and ipsilateral caudate (n = 6) and putamen (n = 4) atrophy. Signal alterations in putamen and caudate were noted in four each. In all the 11 patients with serial MRI, there was progression of cerebral atrophy and a trend towards increase in MRI staging. The MRI signal changes remained same in five patients, resolved in three patients, differential change in two patients and increased in one patient. Diffusion-weighted imaging showed facilitated diffusion (n = 5), and MR spectroscopy showed reduced N-acetyl-aspartate and elevated lactate (n = 2).
Pattern recognition of MRI findings and the changes in serial MRI might serve as a surrogate marker of disease viz. unihemispheric progressive focal cortical atrophy and signal changes predominantly in the perisylvian distribution and caudate followed by putamen involvement. This might assist in understanding and monitoring of the disease progression.
Article: Rasmussen's syndrome.[Show abstract] [Hide abstract]
ABSTRACT: Subacute focal encephalitis of Rasmussen involves cortical inflammatory infiltration which may on occasion be bilateral. The presence of anti-Glu R3 antibodies in some patients suggests that an autoimmune process may be involved. Although a small number of patients respond transiently to immunoglobulins and the condition of some is partially improved by steroids, only hemispherectomy seems to produce prolonged relief of seizures. Subpial cortical transection may be useful if hemiplegia has not developed.Current Opinion in Neurology 05/1996; 9(2):75-7. · 5.42 Impact Factor
- [Show abstract] [Hide abstract]
ABSTRACT: To correlate MRI and histopathologic findings in patients with Rasmussen's encephalitis (RE). MRI features of 10 patients with RE were studied on serial scans. In surgical specimens from these patients, densities of T lymphocytes, microglial cells and nodules, and reactive astrocytes were evaluated. Densities of T cells, microglial nodules, and astrocytes were inversely correlated to disease duration. MRI abnormalities had a focal onset and spread across one hemisphere. The following course of MRI abnormalities in a given brain region was observed: on very early scans, the cortex was swollen and showed a hyperintense T2/fluid-attenuated inversion recovery signal. Consecutively, progressive atrophy of the affected hemisphere occurred. Correlation of MRI features with quantitative histopathology revealed that there was a higher number of T cells and reactive astrocytes in the earlier MRI stages compared with the late (merely atrophic) stage. These data suggest a disease course in RE with the highest inflammatory intensity in the early stages and a subsequent decrease in inflammation. The MRI abnormalities and their characteristic sequence may help to identify patients with RE and to obtain informative biopsies.Neurology 02/2002; 58(2):250-7. · 8.30 Impact Factor
Article: Rasmussen's encephalitis.[Show abstract] [Hide abstract]
ABSTRACT: Rasmussen's encephalitis, a syndrome characteristically present-ing in children with the onset of partial motor seizures followed by progressive hemiparesis and cognitive impairment, and accompanied by unilateral cerebral atrophy, was described nearly 50 years ago, yet the cause and optimum treatment remain unclear. Although it was originally presumed to have a viral aetiology, the possible roles of antibody-mediated mechanisms and more recently cell-mediated immunity in its pathogenesis have come under increasing scrutiny in the last ten years. These developments are discussed, together with a review of the clinical features. The advances in treatment which have accompanied these changes are also assessed.Epileptic disorders: international epilepsy journal with videotape 10/2004; 6(3):133-44. · 1.17 Impact Factor