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November 2021; 8 (6) ArticleOpen Access

Infratentorial MRI Findings in Rasmussen Encephalitis Suggest Primary Cerebellar Involvement

View ORCID ProfileJohannes T. Reiter, View ORCID ProfileBastian David, Selma Enders, View ORCID ProfileConrad C. Prillwitz, View ORCID ProfileTobias Bauer, Deniz Atalay, View ORCID ProfileAnna Tietze, Angela M. Kaindl, View ORCID ProfileVera Keil, View ORCID ProfileAlexander Radbruch, View ORCID ProfileBernd Weber, View ORCID ProfileAlbert J. Becker, View ORCID ProfileChristian E. Elger, View ORCID ProfileRainer Surges, View ORCID ProfileTheodor Rüber
First published August 13, 2021, DOI: https://doi.org/10.1212/NXI.0000000000001058
Johannes T. Reiter
From the Department of Epileptology (J.T.R., B.D., S.E., C.C.P., T.B., C.E.E., R.S., T.R.), University Hospital Bonn; Charité–Universitätsmedizin Berlin (D.A., A.M.K.), Department of Pediatric Neurology; Charité–Universitätsmedizin Berlin (D.A., A.M.K.), Center for Chronically Sick Children; Charité–Universitätsmedizin Berlin (A.T.), Institute of Neuroradiology; Charité–Universitätsmedizin Berlin (A.M.K.), Institute for Cell Biology and Neurobiology; Department of Neuroradiology (V.K., A.R.), University Hospital Bonn; Department of Radiology and Nuclear Medicine (V.K.), Vrije Universiteit Amsterdam Medisch Centrum, The Netherlands; Institute of Experimental Epileptology and Cognition Research (B.W.), University Hospital Bonn; and Section for Translational Epilepsy Research (A.J.B.), Department of Neuropathology, University Hospital Bonn, Germany.
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Bastian David
From the Department of Epileptology (J.T.R., B.D., S.E., C.C.P., T.B., C.E.E., R.S., T.R.), University Hospital Bonn; Charité–Universitätsmedizin Berlin (D.A., A.M.K.), Department of Pediatric Neurology; Charité–Universitätsmedizin Berlin (D.A., A.M.K.), Center for Chronically Sick Children; Charité–Universitätsmedizin Berlin (A.T.), Institute of Neuroradiology; Charité–Universitätsmedizin Berlin (A.M.K.), Institute for Cell Biology and Neurobiology; Department of Neuroradiology (V.K., A.R.), University Hospital Bonn; Department of Radiology and Nuclear Medicine (V.K.), Vrije Universiteit Amsterdam Medisch Centrum, The Netherlands; Institute of Experimental Epileptology and Cognition Research (B.W.), University Hospital Bonn; and Section for Translational Epilepsy Research (A.J.B.), Department of Neuropathology, University Hospital Bonn, Germany.
MSc
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Selma Enders
From the Department of Epileptology (J.T.R., B.D., S.E., C.C.P., T.B., C.E.E., R.S., T.R.), University Hospital Bonn; Charité–Universitätsmedizin Berlin (D.A., A.M.K.), Department of Pediatric Neurology; Charité–Universitätsmedizin Berlin (D.A., A.M.K.), Center for Chronically Sick Children; Charité–Universitätsmedizin Berlin (A.T.), Institute of Neuroradiology; Charité–Universitätsmedizin Berlin (A.M.K.), Institute for Cell Biology and Neurobiology; Department of Neuroradiology (V.K., A.R.), University Hospital Bonn; Department of Radiology and Nuclear Medicine (V.K.), Vrije Universiteit Amsterdam Medisch Centrum, The Netherlands; Institute of Experimental Epileptology and Cognition Research (B.W.), University Hospital Bonn; and Section for Translational Epilepsy Research (A.J.B.), Department of Neuropathology, University Hospital Bonn, Germany.
MSc
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Conrad C. Prillwitz
From the Department of Epileptology (J.T.R., B.D., S.E., C.C.P., T.B., C.E.E., R.S., T.R.), University Hospital Bonn; Charité–Universitätsmedizin Berlin (D.A., A.M.K.), Department of Pediatric Neurology; Charité–Universitätsmedizin Berlin (D.A., A.M.K.), Center for Chronically Sick Children; Charité–Universitätsmedizin Berlin (A.T.), Institute of Neuroradiology; Charité–Universitätsmedizin Berlin (A.M.K.), Institute for Cell Biology and Neurobiology; Department of Neuroradiology (V.K., A.R.), University Hospital Bonn; Department of Radiology and Nuclear Medicine (V.K.), Vrije Universiteit Amsterdam Medisch Centrum, The Netherlands; Institute of Experimental Epileptology and Cognition Research (B.W.), University Hospital Bonn; and Section for Translational Epilepsy Research (A.J.B.), Department of Neuropathology, University Hospital Bonn, Germany.
MD
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Tobias Bauer
From the Department of Epileptology (J.T.R., B.D., S.E., C.C.P., T.B., C.E.E., R.S., T.R.), University Hospital Bonn; Charité–Universitätsmedizin Berlin (D.A., A.M.K.), Department of Pediatric Neurology; Charité–Universitätsmedizin Berlin (D.A., A.M.K.), Center for Chronically Sick Children; Charité–Universitätsmedizin Berlin (A.T.), Institute of Neuroradiology; Charité–Universitätsmedizin Berlin (A.M.K.), Institute for Cell Biology and Neurobiology; Department of Neuroradiology (V.K., A.R.), University Hospital Bonn; Department of Radiology and Nuclear Medicine (V.K.), Vrije Universiteit Amsterdam Medisch Centrum, The Netherlands; Institute of Experimental Epileptology and Cognition Research (B.W.), University Hospital Bonn; and Section for Translational Epilepsy Research (A.J.B.), Department of Neuropathology, University Hospital Bonn, Germany.
BSc
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Deniz Atalay
From the Department of Epileptology (J.T.R., B.D., S.E., C.C.P., T.B., C.E.E., R.S., T.R.), University Hospital Bonn; Charité–Universitätsmedizin Berlin (D.A., A.M.K.), Department of Pediatric Neurology; Charité–Universitätsmedizin Berlin (D.A., A.M.K.), Center for Chronically Sick Children; Charité–Universitätsmedizin Berlin (A.T.), Institute of Neuroradiology; Charité–Universitätsmedizin Berlin (A.M.K.), Institute for Cell Biology and Neurobiology; Department of Neuroradiology (V.K., A.R.), University Hospital Bonn; Department of Radiology and Nuclear Medicine (V.K.), Vrije Universiteit Amsterdam Medisch Centrum, The Netherlands; Institute of Experimental Epileptology and Cognition Research (B.W.), University Hospital Bonn; and Section for Translational Epilepsy Research (A.J.B.), Department of Neuropathology, University Hospital Bonn, Germany.
MD
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Anna Tietze
From the Department of Epileptology (J.T.R., B.D., S.E., C.C.P., T.B., C.E.E., R.S., T.R.), University Hospital Bonn; Charité–Universitätsmedizin Berlin (D.A., A.M.K.), Department of Pediatric Neurology; Charité–Universitätsmedizin Berlin (D.A., A.M.K.), Center for Chronically Sick Children; Charité–Universitätsmedizin Berlin (A.T.), Institute of Neuroradiology; Charité–Universitätsmedizin Berlin (A.M.K.), Institute for Cell Biology and Neurobiology; Department of Neuroradiology (V.K., A.R.), University Hospital Bonn; Department of Radiology and Nuclear Medicine (V.K.), Vrije Universiteit Amsterdam Medisch Centrum, The Netherlands; Institute of Experimental Epileptology and Cognition Research (B.W.), University Hospital Bonn; and Section for Translational Epilepsy Research (A.J.B.), Department of Neuropathology, University Hospital Bonn, Germany.
MD, PhD
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Angela M. Kaindl
From the Department of Epileptology (J.T.R., B.D., S.E., C.C.P., T.B., C.E.E., R.S., T.R.), University Hospital Bonn; Charité–Universitätsmedizin Berlin (D.A., A.M.K.), Department of Pediatric Neurology; Charité–Universitätsmedizin Berlin (D.A., A.M.K.), Center for Chronically Sick Children; Charité–Universitätsmedizin Berlin (A.T.), Institute of Neuroradiology; Charité–Universitätsmedizin Berlin (A.M.K.), Institute for Cell Biology and Neurobiology; Department of Neuroradiology (V.K., A.R.), University Hospital Bonn; Department of Radiology and Nuclear Medicine (V.K.), Vrije Universiteit Amsterdam Medisch Centrum, The Netherlands; Institute of Experimental Epileptology and Cognition Research (B.W.), University Hospital Bonn; and Section for Translational Epilepsy Research (A.J.B.), Department of Neuropathology, University Hospital Bonn, Germany.
MD, PhD
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Vera Keil
From the Department of Epileptology (J.T.R., B.D., S.E., C.C.P., T.B., C.E.E., R.S., T.R.), University Hospital Bonn; Charité–Universitätsmedizin Berlin (D.A., A.M.K.), Department of Pediatric Neurology; Charité–Universitätsmedizin Berlin (D.A., A.M.K.), Center for Chronically Sick Children; Charité–Universitätsmedizin Berlin (A.T.), Institute of Neuroradiology; Charité–Universitätsmedizin Berlin (A.M.K.), Institute for Cell Biology and Neurobiology; Department of Neuroradiology (V.K., A.R.), University Hospital Bonn; Department of Radiology and Nuclear Medicine (V.K.), Vrije Universiteit Amsterdam Medisch Centrum, The Netherlands; Institute of Experimental Epileptology and Cognition Research (B.W.), University Hospital Bonn; and Section for Translational Epilepsy Research (A.J.B.), Department of Neuropathology, University Hospital Bonn, Germany.
MD
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Alexander Radbruch
From the Department of Epileptology (J.T.R., B.D., S.E., C.C.P., T.B., C.E.E., R.S., T.R.), University Hospital Bonn; Charité–Universitätsmedizin Berlin (D.A., A.M.K.), Department of Pediatric Neurology; Charité–Universitätsmedizin Berlin (D.A., A.M.K.), Center for Chronically Sick Children; Charité–Universitätsmedizin Berlin (A.T.), Institute of Neuroradiology; Charité–Universitätsmedizin Berlin (A.M.K.), Institute for Cell Biology and Neurobiology; Department of Neuroradiology (V.K., A.R.), University Hospital Bonn; Department of Radiology and Nuclear Medicine (V.K.), Vrije Universiteit Amsterdam Medisch Centrum, The Netherlands; Institute of Experimental Epileptology and Cognition Research (B.W.), University Hospital Bonn; and Section for Translational Epilepsy Research (A.J.B.), Department of Neuropathology, University Hospital Bonn, Germany.
MD, PhD, JD
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Bernd Weber
From the Department of Epileptology (J.T.R., B.D., S.E., C.C.P., T.B., C.E.E., R.S., T.R.), University Hospital Bonn; Charité–Universitätsmedizin Berlin (D.A., A.M.K.), Department of Pediatric Neurology; Charité–Universitätsmedizin Berlin (D.A., A.M.K.), Center for Chronically Sick Children; Charité–Universitätsmedizin Berlin (A.T.), Institute of Neuroradiology; Charité–Universitätsmedizin Berlin (A.M.K.), Institute for Cell Biology and Neurobiology; Department of Neuroradiology (V.K., A.R.), University Hospital Bonn; Department of Radiology and Nuclear Medicine (V.K.), Vrije Universiteit Amsterdam Medisch Centrum, The Netherlands; Institute of Experimental Epileptology and Cognition Research (B.W.), University Hospital Bonn; and Section for Translational Epilepsy Research (A.J.B.), Department of Neuropathology, University Hospital Bonn, Germany.
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Albert J. Becker
From the Department of Epileptology (J.T.R., B.D., S.E., C.C.P., T.B., C.E.E., R.S., T.R.), University Hospital Bonn; Charité–Universitätsmedizin Berlin (D.A., A.M.K.), Department of Pediatric Neurology; Charité–Universitätsmedizin Berlin (D.A., A.M.K.), Center for Chronically Sick Children; Charité–Universitätsmedizin Berlin (A.T.), Institute of Neuroradiology; Charité–Universitätsmedizin Berlin (A.M.K.), Institute for Cell Biology and Neurobiology; Department of Neuroradiology (V.K., A.R.), University Hospital Bonn; Department of Radiology and Nuclear Medicine (V.K.), Vrije Universiteit Amsterdam Medisch Centrum, The Netherlands; Institute of Experimental Epileptology and Cognition Research (B.W.), University Hospital Bonn; and Section for Translational Epilepsy Research (A.J.B.), Department of Neuropathology, University Hospital Bonn, Germany.
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Christian E. Elger
From the Department of Epileptology (J.T.R., B.D., S.E., C.C.P., T.B., C.E.E., R.S., T.R.), University Hospital Bonn; Charité–Universitätsmedizin Berlin (D.A., A.M.K.), Department of Pediatric Neurology; Charité–Universitätsmedizin Berlin (D.A., A.M.K.), Center for Chronically Sick Children; Charité–Universitätsmedizin Berlin (A.T.), Institute of Neuroradiology; Charité–Universitätsmedizin Berlin (A.M.K.), Institute for Cell Biology and Neurobiology; Department of Neuroradiology (V.K., A.R.), University Hospital Bonn; Department of Radiology and Nuclear Medicine (V.K.), Vrije Universiteit Amsterdam Medisch Centrum, The Netherlands; Institute of Experimental Epileptology and Cognition Research (B.W.), University Hospital Bonn; and Section for Translational Epilepsy Research (A.J.B.), Department of Neuropathology, University Hospital Bonn, Germany.
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Rainer Surges
From the Department of Epileptology (J.T.R., B.D., S.E., C.C.P., T.B., C.E.E., R.S., T.R.), University Hospital Bonn; Charité–Universitätsmedizin Berlin (D.A., A.M.K.), Department of Pediatric Neurology; Charité–Universitätsmedizin Berlin (D.A., A.M.K.), Center for Chronically Sick Children; Charité–Universitätsmedizin Berlin (A.T.), Institute of Neuroradiology; Charité–Universitätsmedizin Berlin (A.M.K.), Institute for Cell Biology and Neurobiology; Department of Neuroradiology (V.K., A.R.), University Hospital Bonn; Department of Radiology and Nuclear Medicine (V.K.), Vrije Universiteit Amsterdam Medisch Centrum, The Netherlands; Institute of Experimental Epileptology and Cognition Research (B.W.), University Hospital Bonn; and Section for Translational Epilepsy Research (A.J.B.), Department of Neuropathology, University Hospital Bonn, Germany.
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Theodor Rüber
From the Department of Epileptology (J.T.R., B.D., S.E., C.C.P., T.B., C.E.E., R.S., T.R.), University Hospital Bonn; Charité–Universitätsmedizin Berlin (D.A., A.M.K.), Department of Pediatric Neurology; Charité–Universitätsmedizin Berlin (D.A., A.M.K.), Center for Chronically Sick Children; Charité–Universitätsmedizin Berlin (A.T.), Institute of Neuroradiology; Charité–Universitätsmedizin Berlin (A.M.K.), Institute for Cell Biology and Neurobiology; Department of Neuroradiology (V.K., A.R.), University Hospital Bonn; Department of Radiology and Nuclear Medicine (V.K.), Vrije Universiteit Amsterdam Medisch Centrum, The Netherlands; Institute of Experimental Epileptology and Cognition Research (B.W.), University Hospital Bonn; and Section for Translational Epilepsy Research (A.J.B.), Department of Neuropathology, University Hospital Bonn, Germany.
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Full PDF
Citation
Infratentorial MRI Findings in Rasmussen Encephalitis Suggest Primary Cerebellar Involvement
Johannes T. Reiter, Bastian David, Selma Enders, Conrad C. Prillwitz, Tobias Bauer, Deniz Atalay, Anna Tietze, Angela M. Kaindl, Vera Keil, Alexander Radbruch, Bernd Weber, Albert J. Becker, Christian E. Elger, Rainer Surges, Theodor Rüber
Neurol Neuroimmunol Neuroinflamm Nov 2021, 8 (6) e1058; DOI: 10.1212/NXI.0000000000001058

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    Figure 1 Results of Voxel-Based Morphometry (VBM) Group Comparisons

    (A) Mapping of results from 3-dimensional voxel-wise statistics onto a flattened cerebellar surface reveals widespread bilateral clusters of lower cerebellar gray matter volume in patients with RE compared with CON. Jet colormap indicates raw t values. Hot colormap indicates FWE-corrected p values. (B) Projections on the flatmap show bilaterally lower cerebellar gray matter volume in both more acute (ACUTE) and more chronic (CHRONIC) patients with RE compared with CON. (C) White matter differences between patients with RE and CON. Hot colors indicate FWE-corrected p values. z indicates z coordinate in Montreal Neurological Institute and Hospital space. CON = controls; FWE = family-wise error; RE = Rasmussen encephalitis.

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    Figure 2 Morphometric and FLAIR Intensity AIs

    (A) Morphometric AIs of cerebellar hemispheres show a greater variance in patients with RE than in CON (F = 11.093, ***p < 0.001; dotted horizontal lines indicate double SD of morphometric AIs of control). Thirty patients showed positive AIs, whereas 27 showed negative asymmetry values. MR images show 2 representative cases of the predominantly ipsilesional and contralesional atrophy patterns. (B) Correlation of FLAIR intensity AIs and morphometric AIs significantly differed in patients with predominantly ipsilesional and contralesional cerebellar atrophy (z = 2.70, **p < 0.01). A strong correlation between AIs was found in patients with predominantly ipsilesional cerebellar atrophy (r = 0.86, p < 0.001). MR images show 2 representative cases of predominantly ipsilesional and contralesional atrophy patterns. AIs = asymmetry indices; CON = controls; FLAIR = fluid-attenuated inversion recovery.

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    Figure 3 Comparison of FA and Tractography of CPC Tracts

    (A) Mean FA of CPC fiber tracts connecting the ipsilesional cerebrum with the contralesional cerebellum showed significantly lower values than opposite tracts (*p < 0.05). Dashed lines indicate individual tract pairs where voxel-wise FA values between tracts differed significantly (2-sample unpaired t test, *p < 0.05). (B) Exemplary CPC fiber tracts of 1 patient weighted according to the corresponding local FA values. CPC = cortico-ponto-cerebellar; FA = fractional anisotropy.

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    Figure 4 Group Comparison of ICA Patterns

    Eight of 30 components showed a significant difference in their loading coefficients between patients with RE and CON (FDR-corr p < 0.05) and indicated decreased GM volume in patients. ICA results are distinguishable in bilateral, strictly contralesional, and strictly ipsilesional patterns. A threshold of z > 3 was applied to all spatial component maps. Colormap indicates the normalized loading coefficients. CON = controls; FDR = false discovery rate; ICA = independent component analysis; RE = Rasmussen encephalitis.

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