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-19-74 10.15844/pedneurbriefs-19-10-2 Seizure Disorders Neurology Pediatrics Nervous System Diseases Child Development Brain Diseases Neurosurgery Child Infant Benign Infantile 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 10 2005 01 03 2016 19 10 74 74 Copyright: © 2005 The Author(s) 2005 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. Clinical features of benign infantile convulsions: familial and sporadic cases

The clinical features of 58 patients with benign infantile convulsions (BIC), seen at the Department of Pediatrics, University of Bologna, Italy, over a 20 year-period (1983-2003), are reviewed retrospectively, with reference to both familial and nonfamilial cases.

 Benign Infantile Convulsions Prevalent in Familial Heterogeneous Genetic Disorder

The clinical features of 58 patients with benign infantile convulsions (BIC), seen at the Department of Pediatrics, University of Bologna, Italy, over a 20 year-period (1983-2003), are reviewed retrospectively, with reference to both familial and nonfamilial cases. Seizure onset was between 4 and 24 months, with an average age of 10 and 11 months for girls and boys, respectively. The first seizure occurred before 1 year of age in 36 (62%) patients and during the second year in 22 (38%). The earlier age of onset was more prevalent in familial (BFIC) compared to nonfamilial (BNFIC) cases: of 17 BFIC patients, 13 (76%) were younger than 1 year at onset whereas of 41 BNFIC cases, 23 (56%) had seizures before age 1. In both familial and nonfamilial patients, seizures were in clusters, twice a day or more for 1 to 4 days, and sometimes recurred after 1 to 8 weeks. Seizure patterns were changeable among different children and also for individuals: psychomotor arrest, deviation of eyes, or hypertonia, with cyanosis. The EEG pattern during a seizure was variable but mainly focal spike and sharp wave discharges, central-parietal-temporal in location, and often followed by rapid generalization. Antiepileptic medications were prescribed in 80% of patients before 1990 and in only 30%, later. Seizures in clusters might prompt treatment, especially in BNFICs. Treatment is often withheld in familial cases, when the diagnosis and benign course are more obvious. A normal interictal EEG is not always indicative of a conclusive diagnosis. Only molecular studies will distinguish benign familial neonatal-infantile seizures (BFNIS) that overlap clinically with BNFIC. [1]

COMMENT. BFIC is a heterogeneous genetic disorder, with linkage to chromosomes 19q, 16pl2-ql2, and 2q24, and having no chromosome-related phenotypic differences [2]. BFIC and BNFIC have similar electroclinical findings and prognosis. In the above study, BFIC cases had an earlier onset than those with BNFIC, an observation that may be helpful in diagnosis.

Franzoni E Bracceschi R Colonnelli MC Errani A Ucchino V Malaspina E Clinical features of benign infantile convulsions: familial and sporadic cases Neurology 2005 Oct 11 65 7 1098 1100 10.1212/01.wnl.0000178898.31862.56 16217066 Malacarne M Gennaro E Madia F Pozzi S Vacca D Barone B Benign familial infantile convulsions: mapping of a novel locus on chromosome 2q24 and evidence for genetic heterogeneity Am J Hum Genet 2001 Jun 68 6 1521 1526 10.1086/320596 11326335