Neuropediatrics 2006; 37 - THP112
DOI: 10.1055/s-2006-945935

ELECTRO-CLINICAL STUDY OF FOUR FIRST NATIONS FAMILIES WITH BENIGN FAMILIAL INFANTILE SEIZURES

M Demos 1, M Connolly 1, L Arbour 1, K Farrell 1
  • 1Children's & Women's Health Centre of BC, University of BC, Vancouver, BC, Canada

Objectives: The syndrome of benign familial infantile seizures (BFIS) is characterized by normal development, no remote neurological insult, a positive family history and onset in infancy of seizures typically localized to the occipito-parietal region.

Methods: We report the electro-clinical features of a form of BFIS affecting twelve patients from four First Nations families located within the northwest region of British Columbia.

Results: The mean age of seizure onset was 4 months (3 days –8 months) and the mean age of the last seizure was 7 months (4–10 months). Seizures were brief (less than 2 minutes) and had a tendency to cluster and occur during sleep. The response to antiepileptic medication was good and psychomotor development remained normal. The inheritance pattern appeared to be autosomal dominant with incomplete penetrance. Eight interictal and 2 ictal video-EEGs were performed on eight patients. The interictal EEG demonstrated frontal epileptiform activity in three, frontal slowing in two and was normal in three. Both ictal video-EEGs demonstrated bilateral tonic posturing followed by fine jerking movements associated with diffuse attenuation and postictal frontal slowing. Similar seizure semiology was described in the other affected family members.

Conclusion: The clinical features and age of onset in these four families is similar to those described for BFIS. However, the ictal and interictal clinical and EEG features are suggestive of frontal lobe seizures.