Author(s): Carvalho, D ; Mendonça, C ; Carvalho, J ; Martins, A ; Leal, A
Date: 2023
Persistent ID: http://hdl.handle.net/10400.17/4353
Origin: Repositório do Centro Hospitalar de Lisboa Central, EPE
Subject(s): CSWS; CSWS; Epileptic Encephalopathy; Pediatric Epilepsy; Thalamus; HDE NEU PED
Objective: Continuous Spike-Wave during slow Sleep (CSWS) syndrome associates a clinically important neurocognitive regression with strong activation of non-REM sleep spikes. Its mechanisms remain unknown, but a contribution of rare perinatal thalamic injuries has been highlighted. We determine the incidence of such lesions in a cohort of CSWS patients. Methods: N = 65 patients with CSWS and a control group (N = 51) were studied. Spikes were quantified in long-term ambulatory EEGs, brain Magnetic Ressonance Imaging (MRI) structural lesions were assessed and thalamic volumetry was performed. A neurocognitive scale was used to assess dysfunction. Results: The most common etiologies in the control patients were not represented in the CSWS group. Structural lesions were detected in a minority of CSWS patients (25/53) but included a thalamic injury in the large majority (24/25). This ratio was 4/40 in controls. Lesions belonged to one of five types: 1. Circumscribed to the thalamus (N = 11); 2. Extending beyond the thalamus (N = 3); 3. Hypothalamic-Hamartomas (N = 4); 4. Periventricular-Leukomalacia (N = 4); 5. Hypoplasia-Polymicrogyria (N = 1). Most lesions were lateralized to one hemisphere, which in all cases corresponded to the lateralization of the CSWS. Significance: Thalamic lesions are present in most CSWS patients with abnormal MRIs, supporting an important role in its genesis.
