Abstracts

Idiopathic Focal Epilepsy with Frontal Spikes: A Forgotten Syndrome?

Abstract number : 1.148
Submission category :
Year : 2001
Submission ID : 311
Source : www.aesnet.org
Presentation date : 12/1/2001 12:00:00 AM
Published date : Dec 1, 2001, 06:00 AM

Authors :
A. De Saint-Martin, MD, Pediatric, INSERM U398, Strasbourg, France; E. Raffo, MD, Pediatric, Nancy, France; R. Carcangiu, MD, Epilepsy Unit, Neurology, France; C. Seegmuller, Neuropsychology, Neurology, France; F. Picard, MD, Neurology, Geneva, Switzerlan

RATIONALE: Beaumanoir et al. described in 1983 a [dsquote]benign frontal epilepsy[dsquote] in eleven children, characterized by the following features : i) diurnal and nocturnal polymorphous focal seizures ii) unilateral or bilateral frontal spike and waves (SW) and generalized SW iii) absence of cerebral lesion or neurological impairment iiii) spontaneous recovery at adolescence. We report here six new cases.
METHODS: Our patients are issued from a cohort of 80 patients followed prospectively. Five girls and one boy underwent repeated clinical, neuropsychological assessments and wake and sleep video EEG studies. MRI were performed in all of them.
RESULTS: Age of onset varied between 2 and 7 years, after a normal previous development. The seizures were diurnal or nocturnal and their semiology was polymorphous: loss of contact with vegetative symptoms, prolonged confusion with automatisms, repeated short motor seizures, nocturnal generalized seizures. MRI was normal in all patients. Interictal EEG showed unilateral or bilateral frontal high amplitude SW, sometimes associated with generalized SW, and rare rolandic diphasic SW. Those interictal abnormalities were activated by sleep. During the active phase of their epilepsy, four children experimented learning impairments or behavioural disorders. Seizures and EEG abnormalities were age related and disappeared before adolescence.
CONCLUSIONS: Those features are concordant with the previous description of Beaumanoir et al. Some of those children were initially misdiagnosed as: i)Idiopathic generalized epileposy as their EEG showed frequent generalized SW,however sleep EEG always demonstrated frontal focal abnormalities ii)Cryptogenic frontal lobe epilepsy iii) Atypical benign partial epilepsy.