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
13 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 languageEnglish
JournalNeuroradiology
Volume57
Issue number12
Pages (from-to)1203-1209
Number of pages7
ISSN0028-3940
DOIs
Publication statusPublished - 01.12.2015

Research Areas and Centers

  • Health Sciences

DFG Research Classification Scheme

  • 206-05 Experimental Models for Investigating Diseases of the Nervous System
  • 206-07 Clinical Neurology Neurosurgery and Neuroradiology
  • 206-08 Cognitive and Systemic Human Neuroscience

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