Supratentorial white matter blurring associated with voltage-gated potassium channel-complex limbic encephalitis
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Supratentorial white matter blurring associated with voltage-gated potassium channel-complex limbic encephalitis. / 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.
In: NEURORADIOLOGY, Vol. 57, No. 12, 12.2015, p. 1203-9.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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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
Y1 - 2015/12
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.
U2 - 10.1007/s00234-015-1581-x
DO - 10.1007/s00234-015-1581-x
M3 - SCORING: Journal article
C2 - 26293130
VL - 57
SP - 1203
EP - 1209
JO - NEURORADIOLOGY
JF - NEURORADIOLOGY
SN - 0028-3940
IS - 12
ER -
