Spikes, Spikes Go Away!

The child described in this chapter presented with seizures and developmental regression and had continuous spike-wave discharges activated by non–rapid-eye-movement (NREM) sleep consistent with epileptic encephalopathy with continuous spikes and waves during slow-wave sleep (E-CSWS), which is a rare age-dependent pediatric epilepsy syndrome. The CSWS electroencephalographic pattern in children with E-CSWS usually develops 1 to 2 years after seizure onset. While the CSWS EEG pattern in NREM sleep is diagnostic of E-CSWS, the cardinal clinical symptoms are a progressive decline in cognitive, behavioral, and/or psychiatric functioning. The pattern of CSWS is more often associated with neurodevelopmental problems, and the almost continuous discharges are thought to contribute or cause them. Encountering a CSWS EEG on an EEG or PSG requires clinical correlation to determine whether it is (or will become) symptomatic, and whether it should (or can) be treated. CSWS affects all cognitive domains, including language and communication, temporo-spatial orientation, attention, and social interaction. Antecedent and birth history are usually normal, although structural brain abnormalities are seen in 20% to 50%, most often prenatal or perinatal lesions, involving thalamus in some.