Epileptic encephalopathy with continuous spike-and-wave during sleep (CSWS) and Landau-Kleffner syndrome are epilepsy syndromes characterized by frequent interictal spikes during sleep—termed electrical status epilepticus during sleep (ESES)—that are associated with cognitive and behavioral regression. This regression typically begins in early school-aged children and can cause severe, permanent learning delays that lead to lifelong dependency. Early treatment can be associated with a reduction in the spike frequency and significant improvement in cognition and behavior. Various medical treatments have been utilized to reduce spike frequency during sleep; however, optimal therapy has not been established.
The CSWS Epilepsy & Landau-Kleffner Syndrome – ESES Foundation was founded by parents with the purpose of expediting collaborative research projects, such as this one, that lead to a cure and better treatments for children impacted by this disease. The community is hopeful that better treatment will lead to improved cognition and quality of life for patients with this challenging condition. This proposal will enlist the ESES Foundation family stakeholders to work with physicians, neuropsychologists, social workers, and a statistician to develop collaborative research; the goal is to create comparative effectiveness research projects that improve care for patients living with ESES. A monthly teleconference will be undertaken to obtain caregiver input about how to best address their concerns and hopes during project creation. An in-person meeting will be arranged prior to the American Epilepsy Society Meeting in December 2017 in Washington, DC, to discuss our progress and formulate next steps toward creation of a funded comparative effectiveness trial.