John Libbey Eurotext

Epileptic Disorders

The Educational Journal of the International League Against Epilepsy

The semiology of benign focal epilepsy with affective symptoms Volume 19, numéro 2, June 2017

Vidéo

  • The semiology of benign focal epilepsy with affective symptoms

Illustrations

  • Figure 1
  • Figure 2
Auteurs
1 Department of Neurology, Baylor College of Medicine, Houston, Texas
2 Swedish Neuroscience Institute, Pediatric Neuroscience Center, Swedish Medical Center, Seattle, Washington, USA
3 Neurological Institute of Curitiba (INC), Curitiba, Paraná, Brazil
* Correspondence: Fábio A Nascimento 7200 Cambridge Street, 9th floor, Houston, Texas, 77030, USA
  • Mots-clés : epilepsy, benign focal epilepsy with affective symptoms, benign psychomotor epilepsy, electroencephalography, video-electroencephalography
  • DOI : 10.1684/epd.2017.0910
  • Page(s) : 202-6
  • Année de parution : 2017

Benign focal epilepsy with affective symptoms (BFEAS) is a rare childhood epilepsy syndrome essentially characterized by “epileptic attacks with affective symptoms of a terrifying type”. Since the original description, approximately 50 cases have been reported. To our knowledge, however, none of the studies included video-EEG data. Herein, we detail the electroclinical features of a neurodevelopmentally normal 9-year-old boy with epilepsy since the age of 2 years. His seizure semiology essentially consisted of nocturnal focal seizures featuring abrupt fear and autonomic phenomena (such as excessive sweating, repeated swallowing, and coughing), associated with impaired consciousness. These seizures were often secondary generalized, and he had multiple episodes of convulsive status epilepticus. He has been seizure-free for the past year and a half on dual antiepileptic therapy with sulthiamine and valproate. His intellectual and social abilities are excellent (IQ of 116), although he does have difficulties particularly in language learning, and was recently diagnosed with phonological dyslexia with dysorthography. By presenting our patient's history and video-EEG, we intend to further detail the semiology of seizures with affective symptomatology. [Published with video sequence on www.epilepticdisorders.com]