Why Are Children With Epileptic Encephalopathies Encephalopathic?

The epileptic encephalopathies are devastating conditions characterized by frequent seizures, severely abnormal electroencephalograms (EEGs), and cognitive slowing or regression. The cognitive impairment in the epileptic encephalopathies may be more concerning to the patient and parents than the epilepsy itself. There is increasing recognition that the cognitive comorbidity can be both chronic, primarily due to the underlying etiology of the epilepsy, and dynamic or evolving because of recurrent seizures, interictal spikes, and antiepileptic drugs. Much of scholars' understanding of the neurophysiological underpinnings of cognitive dysfunction in the epileptic encephalopathies comes from rodent studies. Frequent seizures and interictal EEG discharges in rats lead to considerable spatial and social-cognitive deficits. Paralleling these cognitive deficits are dyscoordination of dynamic neural activity within and between the neural networks that subserve normal cognitive processes.