Supratentorial white matter blurring associated with voltage-gated potassium channel-complex limbic encephalitis

H. Urbach; S. Rauer; I. Mader; S. Paus; J. Wagner; M. P. Malter; H. Prüss; J. Lewerenz; J. Kassubek; H. Hegen; M. Auer; F. Deisenhammer; F. Ufer; C. G. Bien; A. Baumgartner

doi:10.1007/s00234-015-1581-x

Supratentorial white matter blurring associated with voltage-gated potassium channel-complex limbic encephalitis

H. Urbach^*, S. Rauer, I. Mader, S. Paus, J. Wagner, M. P. Malter, H. Prüss, J. Lewerenz, J. Kassubek, H. Hegen, M. Auer, F. Deisenhammer, F. Ufer, C. G. Bien, A. Baumgartner

^*Corresponding author for this work

Institute of Health Sciences

14 Citations (Scopus)

Abstract

Introduction: Limbic encephalitis (LE) associated with voltage-gated potassium channel-complex antibodies (VGKC-LE) is frequently non-paraneoplastic and associated with marked improvement following corticosteroid therapy. Mesial temporal lobe abnormalities are present in around 80 % of patients. If associated or preceded by faciobrachial dystonic seizures, basal ganglia signal changes may occur. In some patients, blurring of the supratentorial white matter on T2-weighted images (SWMB) may be seen. The purpose of this study was to evaluate the incidence of SWMB and whether it is specific for VGKC-LE. Methods: Two experienced neuroradiologists independently evaluated signal abnormalities on FLAIR MRI in 79 patients with LE while unaware on the antibody type. Results: SWMB was independently assessed as present in 10 of 36 (28 %) compared to 2 (5 %) of 43 non-VGKC patients (p = 0.009). It was not related to the presence of LGI1 or CASPR2 proteins of VGKC antibodies. MRI showed increased temporomesial FLAIR signal in 22 (61 %) VGKC compared to 14 (33 %) non-VGKC patients (p = 0.013), and extratemporomesial structures were affected in one VGKC (3 %) compared to 11 (26 %) non-VGKC patients (p = 0.005). Conclusion: SWMB is a newly described MRI sign rather specific for VGKC-LE.

Original language	English
Journal	Neuroradiology
Volume	57
Issue number	12
Pages (from-to)	1203-1209
Number of pages	7
ISSN	0028-3940
DOIs	https://doi.org/10.1007/s00234-015-1581-x
Publication status	Published - 01.12.2015

Funding

SR receives consulting and lecture fees, grant, and research support from Bayer Vital GmbH, Biogen Idec, Merck Serono, Novartis, Sanofi-Aventis, Baxter, RG, and Teva. SR is a founding executive board member of ravo Diagnostika GmbH. MPM received payments for lectures, manuscript preparation, and travel expenses from EISAI and UCB. JL received honoraria for lectures sponsored by Euroimmun. HH has participated in meetings sponsored by or received honoraria for lectures from Bayer Schering, Biogen Idec, Merck Serono, and Novartis. CGB gave scientific advice to Eisai (Frankfurt, Germany) and UCB (Monheim, Germany); undertook industry-funded travel with support of Eisai (Frankfurt, Germany), UCB (Monheim, Germany), Desitin (Hamburg, Germany), and Grifols (Frankfurt, Germany); obtained honoraria for speaking engagements from Eisai (Frankfurt, Germany), UCB (Monheim, Germany), Desitin (Hamburg, Germany), diamed (Köln, Germany), and Fresenius Medical Care (Bad Homburg, Germany); and received research support from Astellas Pharma (München, Germany), Octapharma (Langenfeld, Germany), diamed (Köln, Germany), and Fresenius Medical Care (Bad Homburg, Germany). CGB’s employer (Krankenhaus Mara, Bielefeld, Germany) runs a laboratory for the detection of auto-antibodies including those described in this study; external senders are charged for antibody diagnostics. AB has received consulting and lecture fees, grant, and research support from Bayer Vital GmbH, Biogen Idec, Merck Serono, Novartis, Sanofi-Aventis, and Teva.

Research Areas and Centers

Health Sciences

DFG Research Classification Scheme

2.23-05 Experimental Models for the Understanding of Nervous System Diseases
2.23-07 Clinical Neurology, Neurosurgery and Neuroradiology
2.23-08 Human Cognitive and Systems Neuroscience

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This output contributes to the following UN Sustainable Development Goals (SDGs)

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Urbach, H., Rauer, S., Mader, I., Paus, S., Wagner, J., Malter, M. P., Prüss, H., Lewerenz, J., Kassubek, J., Hegen, H., Auer, M., Deisenhammer, F., Ufer, F., Bien, C. G., & Baumgartner, A. (2015). Supratentorial white matter blurring associated with voltage-gated potassium channel-complex limbic encephalitis. Neuroradiology, 57(12), 1203-1209. https://doi.org/10.1007/s00234-015-1581-x

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title = "Supratentorial white matter blurring associated with voltage-gated potassium channel-complex limbic encephalitis",

abstract = "Introduction: Limbic encephalitis (LE) associated with voltage-gated potassium channel-complex antibodies (VGKC-LE) is frequently non-paraneoplastic and associated with marked improvement following corticosteroid therapy. Mesial temporal lobe abnormalities are present in around 80 \% of patients. If associated or preceded by faciobrachial dystonic seizures, basal ganglia signal changes may occur. In some patients, blurring of the supratentorial white matter on T2-weighted images (SWMB) may be seen. The purpose of this study was to evaluate the incidence of SWMB and whether it is specific for VGKC-LE. Methods: Two experienced neuroradiologists independently evaluated signal abnormalities on FLAIR MRI in 79 patients with LE while unaware on the antibody type. Results: SWMB was independently assessed as present in 10 of 36 (28 \%) compared to 2 (5 \%) of 43 non-VGKC patients (p = 0.009). It was not related to the presence of LGI1 or CASPR2 proteins of VGKC antibodies. MRI showed increased temporomesial FLAIR signal in 22 (61 \%) VGKC compared to 14 (33 \%) non-VGKC patients (p = 0.013), and extratemporomesial structures were affected in one VGKC (3 \%) compared to 11 (26 \%) non-VGKC patients (p = 0.005). Conclusion: SWMB is a newly described MRI sign rather specific for VGKC-LE.",

author = "H. Urbach and S. Rauer and I. Mader and S. Paus and J. Wagner and Malter, \{M. P.\} and H. Pr{\"u}ss and J. Lewerenz and J. Kassubek and H. Hegen and M. Auer and F. Deisenhammer and F. Ufer and Bien, \{C. G.\} and A. Baumgartner",

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doi = "10.1007/s00234-015-1581-x",

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Urbach, H, Rauer, S, Mader, I, Paus, S, Wagner, J, Malter, MP, Prüss, H, Lewerenz, J, Kassubek, J, Hegen, H, Auer, M, Deisenhammer, F, Ufer, F, Bien, CG & Baumgartner, A 2015, 'Supratentorial white matter blurring associated with voltage-gated potassium channel-complex limbic encephalitis', Neuroradiology, vol. 57, no. 12, pp. 1203-1209. https://doi.org/10.1007/s00234-015-1581-x

TY - JOUR

T1 - Supratentorial white matter blurring associated with voltage-gated potassium channel-complex limbic encephalitis

AU - Urbach, H.

AU - Rauer, S.

AU - Mader, I.

AU - Paus, S.

AU - Wagner, J.

AU - Malter, M. P.

AU - Prüss, H.

AU - Lewerenz, J.

AU - Kassubek, J.

AU - Hegen, H.

AU - Auer, M.

AU - Deisenhammer, F.

AU - Ufer, F.

AU - Bien, C. G.

AU - Baumgartner, A.

PY - 2015/12/1

Y1 - 2015/12/1

N2 - Introduction: Limbic encephalitis (LE) associated with voltage-gated potassium channel-complex antibodies (VGKC-LE) is frequently non-paraneoplastic and associated with marked improvement following corticosteroid therapy. Mesial temporal lobe abnormalities are present in around 80 % of patients. If associated or preceded by faciobrachial dystonic seizures, basal ganglia signal changes may occur. In some patients, blurring of the supratentorial white matter on T2-weighted images (SWMB) may be seen. The purpose of this study was to evaluate the incidence of SWMB and whether it is specific for VGKC-LE. Methods: Two experienced neuroradiologists independently evaluated signal abnormalities on FLAIR MRI in 79 patients with LE while unaware on the antibody type. Results: SWMB was independently assessed as present in 10 of 36 (28 %) compared to 2 (5 %) of 43 non-VGKC patients (p = 0.009). It was not related to the presence of LGI1 or CASPR2 proteins of VGKC antibodies. MRI showed increased temporomesial FLAIR signal in 22 (61 %) VGKC compared to 14 (33 %) non-VGKC patients (p = 0.013), and extratemporomesial structures were affected in one VGKC (3 %) compared to 11 (26 %) non-VGKC patients (p = 0.005). Conclusion: SWMB is a newly described MRI sign rather specific for VGKC-LE.

AB - Introduction: Limbic encephalitis (LE) associated with voltage-gated potassium channel-complex antibodies (VGKC-LE) is frequently non-paraneoplastic and associated with marked improvement following corticosteroid therapy. Mesial temporal lobe abnormalities are present in around 80 % of patients. If associated or preceded by faciobrachial dystonic seizures, basal ganglia signal changes may occur. In some patients, blurring of the supratentorial white matter on T2-weighted images (SWMB) may be seen. The purpose of this study was to evaluate the incidence of SWMB and whether it is specific for VGKC-LE. Methods: Two experienced neuroradiologists independently evaluated signal abnormalities on FLAIR MRI in 79 patients with LE while unaware on the antibody type. Results: SWMB was independently assessed as present in 10 of 36 (28 %) compared to 2 (5 %) of 43 non-VGKC patients (p = 0.009). It was not related to the presence of LGI1 or CASPR2 proteins of VGKC antibodies. MRI showed increased temporomesial FLAIR signal in 22 (61 %) VGKC compared to 14 (33 %) non-VGKC patients (p = 0.013), and extratemporomesial structures were affected in one VGKC (3 %) compared to 11 (26 %) non-VGKC patients (p = 0.005). Conclusion: SWMB is a newly described MRI sign rather specific for VGKC-LE.

UR - https://www.scopus.com/pages/publications/84947484670

U2 - 10.1007/s00234-015-1581-x

DO - 10.1007/s00234-015-1581-x

M3 - Journal articles

C2 - 26293130

AN - SCOPUS:84947484670

SN - 0028-3940

VL - 57

SP - 1203

EP - 1209

JO - Neuroradiology

JF - Neuroradiology

IS - 12

ER -

Supratentorial white matter blurring associated with voltage-gated potassium channel-complex limbic encephalitis

Abstract

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UN SDGs

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