Successful ECT in a Patient With Wiedemann-Steiner Syndrome and Insights From Twin-Based EEG Analysis

We describe a young man with Wiedemann-Steiner syndrome (WSS) suffering from severe depression with psychotic and catatonic features, who showed a marked and lasting response to electroconvulsive therapy (ECT). Resting-state electroencephalogram (EEG) was recorded before and after the ECT course in the patient, and-at comparable time points-in his monozygotic twin brother. This offered unique neurophysiological insights into candidate mechanisms underlying ECT's therapeutic effects. After 16 ECT sessions, the patient completely remitted. Pretreatment EEG of the patient showed a lower amplitude, less fluctuating and more symmetrical posterior dominant rhythm, which partly normalized after the successful ECT course. Quantitative EEG analyses using a computational corticothalamic model suggested that thalamic intrinsic responsiveness in the recovered patient decreased, normalizing toward his twin brother's values after the ECT course. These findings may point to normalization of thalamic inhibitory function as a candidate mechanism of the therapeutic effect of ECT in treating catatonic psychotic depression.