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

Neuroradiology 2015 Dec

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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. Two experienced neuroradiologists independently evaluated signal abnormalities on FLAIR MRI in 79 patients with LE while unaware on the antibody type. 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). SWMB is a newly described MRI sign rather specific for VGKC-LE.

Citation

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. Supratentorial white matter blurring associated with voltage-gated potassium channel-complex limbic encephalitis. Neuroradiology. 2015 Dec;57(12):1203-9