1043-3155 Pediatr Neurol Briefs pedneurbriefs Pediatr Neurol Briefs Pediatric Neurology Briefs Pediatr Neurol Briefs 2166-6482 1043-3155 2166-3155 Pediatric Neurology Briefs Publishers Chicago, IL, USA PNB-8-34 10.15844/pedneurbriefs-8-5-3 Seizure Disorders Neurology Pediatrics Nervous System Diseases Child Development Brain Diseases Neurosurgery Child Infant Benign Familial Convulsions http://orcid.org/0000-0002-0173-7931 Millichap J. Gordon MD 1 2 * Division of Neurology, Children's Memorial Hospital, Chicago, IL Departments of Pediatrics and Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL Correspondence: Dr. J. Gordon Millichap, E-mail: jgmillichap@northwestern.edu 05 1994 01 06 2016 8 5 34 35 Copyright: © 1994 The Author(s) 1994 This work is licensed under the Creative Commons Attribution 4.0 International License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Benign infantile familial convulsions are not an allelic form of the benign familial neonatal convulsions gene

Results of linkage analysis between benign infantile familial convulsions (BIFC) and two linked DNA markers, D20S19 and D20S20, in 52 members from eight BIFC pedigrees are reported from centers in Montpellier and Paris, France, and Rome and Treviso, Italy.

 Benign Infantile Familial Convulsions Benign Familial Neonatal Convulsions DNA Markers

Results of linkage analysis between benign infantile familial convulsions (BIFC) and two linked DNA markers, D20S19 and D20S20, in 52 members from eight BIFC pedigrees are reported from centers in Montpellier and Paris, France, and Rome and Treviso, Italy. The gene responsible for benign familial neonatal convulsions (BFNC) has been mapped to chromosome 20q in the close vicinity of these two DNA markers. Several recombinants were observed betweeen the BIFC locus and D20S19-D20S20 markers, whereas none appeared between the BFNC locus and the markers in 11 BFNC families. The gene responsible for BFNC is not implicated in BIFC. [1]

COMMENT. The authors distinguish BFNC and BIFC by clinical and genetic markers, as follows: 1) Onset of BFNC is before 3 months and BIFC, after 3 months of age; 2) Seizures, generalized in BFNC and partial in BIFC; and 3) genetic heterogeneity.

Seizure patterns cannot be used to differentiate these benign familial convulsions without documentation by ictal EEG recordings. Ictal EEGs demonstrated a seizure of right frontal onset with secondary generalization and one of right frontal onset which remained focal in a neonate with BFNC presenting with seizures at 50 hours of age, and reported from the Prince of Wales Children’s Hospital, Randwick, Australia [2]. BFNC is heterogeneous in clinical and EEG features and cannot be distinguished from BIFC on the basis of clinical seizure patterns.

 Malafosse A Beck C Bellet H Di Capua M Dulac O Echenne B Benign infantile familial convulsions are not an allelic form of the benign familial neonatal convulsions gene Ann Neurol 1994 Apr 35 4 479 82 8154876 10.1002/ana.410350417 Bye AM Neonate with benign familial neonatal convulsions: recorded generalized and focal seizures Pediatr Neurol 1994 Mar 10 2 164 5 8024668 10.1016/0887-8994(94)90052-3