Perinatal thalamic injury: MRI predictors of electrical status epilepticus in sleep and long-term neurodevelopment

van den Munckhof, Bart, Zwart, Anne F, Weeke, Lauren C, Claessens, Nathalie H P, Plate, Joost D J, Leemans, Alexander, Kuijf, Hugo J, van Teeseling, Heleen C, Leijten, Frans S S, Benders, Manon J N, Braun, Kees P J, de Vries, Linda S, Jansen, Floor E


Neuroimage: Clinical [E] 26


Objective: Perinatal thalamic injury is associated with epilepsy with electrical status epilepticus in sleep (ESES). The aim of this study was to prospectively quantify the risk of ESES and to assess neuroimaging predictors of neurodevelopment. Methods: We included patients with perinatal thalamic injury. MRI scans were obtained in the neonatal period, around three months of age and during childhood. Thalamic and total brain volumes were obtained from the three months MRI. Diffusion characteristics were assessed. Sleep EEGs distinguished patients into ESES (spike-wave index (SWI) >85%), ESES-spectrum (SWI 50–85%) or no ESES (SWI < 50%). Serial Intelligence Quotient (IQ)/Developmental Quotient (DQ) scores were obtained during follow-up. Imaging and EEG findings were correlated to neurodevelopmental outcome. Results: Thirty patients were included. Mean thalamic volume at three months was 8.11 (±1.67) ml and mean total brain volume 526.45 (±88.99) ml. In the prospective cohort (n = 23) 19 patients (83%) developed ESES (-spectrum) abnormalities after a mean follow-up of 96 months. In the univariate analysis, larger thalamic volume, larger total brain volume and lower SWI correlated with higher mean IQ/DQ after 2 years (Pearson's r = 0.74, p = 0.001; Pearson's r = 0.64, p = 0.005; and Spearman's rho -0.44, p = 0.03). In a multivariable mixed model analysis, thalamic volume was a significant predictor of IQ/DQ (coefficient 9.60 [p < 0.001], i.e., corrected for total brain volume and SWI and accounting for repeated measures within patients, a 1 ml higher thalamic volume was associated with a 9.6 points higher IQ). Diffusion characteristics during childhood correlated with IQ/DQ after 2 years. Significance: Perinatal thalamic injury is followed by electrical status epilepticus in sleep in the majority of patients. Thalamic volume and diffusion characteristics correlate to neurodevelopmental outcome.