Cerebral dominance in an unusual case of Landau-Kleffner syndrome

Landau-Kleffner syndrome (LKS) is described by the International Classification of Epileptic Syndromes since 1985 as a constellation of clinical and electrographic signs, including acquired aphasia, regression of language milestones and seizures, along with sleep-activated paroxysms on electroencephalogram which can progress to electrographic status epilepticus of sleep. In this case, a 7-year-old boy presented with an atypical history of new-onset aphasia and regression of language milestones with rare seizures. However, there was an electrographic mismatch in the form of right-sided epileptiform activity and continuous spike and wave of sleep pattern. Detailed speech analysis and perusal of the history revealed a possibly ambidextrous child with right hemispheric language dominance, and he was diagnosed with LKS and treated. This report illustrates the many pitfalls in the diagnosis and treatment of this rare epileptic syndrome.

Clinical Speech fMRI in Children and Adolescents

Purpose In patients with drug-resistant focal epilepsy, surgical resection is often the only treatment option to achieve long-term seizure control. Prior to brain surgery involving potential language areas, identification of hemispheric language dominance is crucial. Our group developed and validated a functional magnetic resonance imaging (fMRI) battery of four pediatric language tasks. The present study aimed at optimizing fMRI data acquisition and analysis using these tasks. Methods We retrospectively analyzed speech fMRI examinations of 114 neuropediatric patients (age range 5.8–17.8 years) who were examined prior to possible epilepsy surgery. In order to evaluate hemispheric language dominance, 1–4 language tasks (vowel identification task VIT, word-chain task WCT, beep-story task BST, synonym task SYT) were measured. Results Language dominance was classified using fMRI activation in the 13 validly lateralizing ROIs (VLR) in frontal, temporal and parietal lobes and cerebellum of the recent validation study from our group: 47/114 patients were classified as left-dominant, 34/114 as bilateral and 6/114 as right-dominant. In an attempt to enlarge the set of VLR, we then compared for each task agreement of these ROI activations with the classified language dominance. We found four additional task-specific ROIs showing concordant activation and activation in ≥ 10 sessions, which we termed validly lateralizing (VLRnew). The new VLRs were: for VIT the temporal language area and for SYT the middle frontal gyrus, the intraparietal sulcus and cerebellum. Finally, in order to find the optimal sequence of measuring the different tasks, we analyzed the success rates of single tasks and all possible task combinations. The sequence 1) VIT 2) WCT 3) BST 4) SYT was identified as the optimal sequence, yielding the highest chance to obtain reliable results even when the fMRI examination has to be stopped, e.g., due to lack of cooperation. Conclusion Our suggested task order together with the enlarged set of VLRnew may contribute to optimize pediatric speech fMRI in a clinical setting.