Center for Epileptic Encephalopathies
At the Center for Epileptic Encephalopathies, part of NYU Langone’s Comprehensive Epilepsy Center, we help patients with seizures, loss of language, attention and behavioral problems, autistic-like behavior, and psychiatric and sleep problems related to epileptic conditions. An epileptic encephalopathy frequently affects children, but may occur in adults.
There are specific conditions included as epileptic encephalopathies, including:
- continuous spike waves in sleep
- Landau-Kleffner syndrome
- electrical status epilepticus of sleep
- autism with regression and electroencephalogram (EEG) abnormalities
- benign epilepsy variants, including benign Rolandic epilepsy, also referred to as atypical Rolandic epilepsy, benign Rolandic epilepsy variants, malignant Rolandic epilepsy, or borderland syndromes
- benign occipital epilepsies
An epileptic encephalopathy must be considered in any person with epilepsy who develops a loss of abilities, even when the actual seizures are under control.
Research and Diagnosis
Because patients may not respond to standard anti-seizure medications, research is an important part of the treatment for epileptic encephalopathies. We are studying the effects of high-dose benzodiazepines and pulse intravenous corticosteroids on the EEG, the neuropsychologic manifestations of electrical status epilepticus of sleep, and the genetics of the epileptic encephalopathies.
Seizures and epilepsy may be associated with cognitive and behavioral problems. In an epileptic encephalopathy, the epileptic activity itself contributes to cognitive and behavioral problems. An epileptic encephalopathy can cause progressive academic, behavioral, and even motor problems.
Management and Treatment
Classical seizure treatment mandates that we treat the patient, not the EEG. But in an epileptic encephalopathy, the problem extends beyond the clinical seizures, which may be easy to control, and must focus on reducing epileptiform activity—the doctor must also manage the epileptic EEG activity. This can be determined with prolonged EEG monitoring. Neuropsychological evaluation is critical to assess regression and improvement with therapy.
Treatment of an epileptic encephalopathy starts with a neurologic evaluation to identify the underlying cause. Most epileptic encephalopathies occur in people with known epilepsies. However, infectious, metabolic, or autoimmune disorders may be responsible.
The seizure treatment starts with a standard anti-seizure medication to suppress and control seizures. Treatment is assessed by control of seizures and improvement in cognitive function that usually follows EEG improvement. Behavioral problems may not always improve along with the EEG and may require specific psychopharmacologic treatment.
Successful treatment requires the teamwork of various specialists, including an epileptologist, psychopharmacologist, neuropsychiatrist, neuropsychologist, nurse practitioner, and dietician.