Long‐term outcome after cognitive and behavioral regression in nonlesional epilepsy with continuous spike‐waves during slow‐wave sleep

Abstract

Summary Purpose: To present the long‐term follow‐up of 10 adolescents and young adults with documented cognitive and behavioral regression as children due to nonlesional focal, mainly frontal, epilepsy with continuous spike‐waves during slow wave sleep (CSWS). Methods: Past medical and electroencephalography (EEG) data were reviewed and neuropsychological tests exploring main cognitive functions were administered. Key Findings: After a mean duration of follow‐up of 15.6 years (range, 8–23 years), none of the 10 patients had recovered fully, but four regained borderline to normal intelligence and were almost independent. Patients with prolonged global intellectual regression had the worst outcome, whereas those with more specific and short‐lived deficits recovered best. The marked behavioral disorders resolved in all but one patient. Executive functions were neither severely nor homogenously affected. Three patients with a frontal syndrome during the active phase (AP) disclosed only mild residual executive and social cognition deficits. The main cognitive gains occurred shortly after the AP, but qualitative improvements continued to occur. Long‐term outcome correlated best with duration of CSWS. Significance: Our findings emphasize that cognitive recovery after cessation of CSWS depends on the severity and duration of the initial regression. None of our patients had major executive and social cognition deficits with preserved intelligence, as reported in adults with early destructive lesions of the frontal lobes. Early recognition of epilepsy with CSWS and rapid introduction of effective therapy are crucial for a best possible outcome.

The tower of Babel: Survey on concepts and terminology in electrical status epilepticus in sleep and continuous spikes and waves during sleep in North America

“…However, one third of our respondents diagnose CSWS based on EEG assessment alone and two thirds of respondents considered that regression was typical but not required for the diagnosis of CSWS. In addition, previous literature has shown that normal or near‐normal outcome is rare in CSWS, and at least half of the patients remain severely impaired (Tassinari et al., 2000; Nickels & Wirrell, 2008; Loddenkemper et al., 2011; Sánchez Fernández et al., 2012c; Seegmuller et al., 2012). Although most respondents consider CSWS as a devastating epileptic encephalopathy with severe sequelae even if treated correctly, around one fourth have indicated that it did not leave sequelae if epilepsy was treated early and around one tenth suggested that cognitive problems resolved with age.…”

“…Although most respondents consider CSWS as a devastating epileptic encephalopathy with severe sequelae even if treated correctly, around one fourth have indicated that it did not leave sequelae if epilepsy was treated early and around one tenth suggested that cognitive problems resolved with age. Based on available data, regression may be considered an integral part of the definition of CSWS, and cognitive prognosis is generally poor (Tassinari et al., 2000; Loddenkemper et al., 2011; Sánchez Fernández et al., 2012c,e; Seegmuller et al., 2012).…”

Continuous Spikes and Waves during Sleep: Electroclinical Presentation and Suggestions for Management

“…Some patients present with moderate improvements after seizure freedom. However, most patients remain severely impaired [2, 3, 6, 47]. The impact of interictal spikes on neurocognitive features is a matter of debate, and it is not clear whether an increased amount of epileptiform activity is associated with a worse cognitive outcome [4, 48, 49].…”

Primary Sleep Disorders in People with Epilepsy

“…96,97 However, NFLE can present with RLS-like symptoms. 98 Nonconvulsive status epilepticus can present as recurrent hypersomnia. 99 Partial seizures can mimic sleep terrors.…”