A Espeche1. 1. Department of Neurology, Salta Children's Hospital Niño Jesús de Praga, Salta, Sarmiento 625, CP, 4400 Salta, Argentina. albertoespeche@yahoo.com.ar
Abstract
INTRODUCTION: One idiopathic focal epileptic syndrome with onset during infancy is recognized, the benign infantile seizures (BIS). OBJECTIVE: To analyze the electroclinical features and evolution in patients with BIS and assess the difference between familial and non-familial infantile cases. PATIENTS AND METHODS: We performed a prospective follow-up study in 41 patients seen at our department between September 2002 and March 2004, with BIS from 2 to 12 months of age. Among the 41 cases 6 were excluded. RESULTS: Thirty five had BIS after the follow-up. The follow-up was 60-77 months (median 69 months). They were divided in Group 1: 14 patients had a family history of similar seizures in infancy and Group 2: 21 without familial history of infantile epilepsy. Both groups have similar electroclinical features and family history of seizures is the only clue to the differential diagnosis. Seizures were brief, and occurred in cluster in 30 patients (85%). Interictal EEGs were normal in 34 cases (97%) and neuroimaging in all children. No one had seizures after finishing the antiepileptic treatment. Neurological examination and developmental milestones remained normal after the follow-up. CONCLUSIONS: This study confirms the existence of BIS. Non-familial cases might represent novo mutations or sporadic cases of BIS. The recognition of BIS is possible at beginning of the epilepsy. To confirm the syndrome the follow-up is necessary. Copyright 2009 Elsevier B.V. All rights reserved.
INTRODUCTION: One idiopathic focal epileptic syndrome with onset during infancy is recognized, the benign infantile seizures (BIS). OBJECTIVE: To analyze the electroclinical features and evolution in patients with BIS and assess the difference between familial and non-familial infantile cases. PATIENTS AND METHODS: We performed a prospective follow-up study in 41 patients seen at our department between September 2002 and March 2004, with BIS from 2 to 12 months of age. Among the 41 cases 6 were excluded. RESULTS: Thirty five had BIS after the follow-up. The follow-up was 60-77 months (median 69 months). They were divided in Group 1: 14 patients had a family history of similar seizures in infancy and Group 2: 21 without familial history of infantile epilepsy. Both groups have similar electroclinical features and family history of seizures is the only clue to the differential diagnosis. Seizures were brief, and occurred in cluster in 30 patients (85%). Interictal EEGs were normal in 34 cases (97%) and neuroimaging in all children. No one had seizures after finishing the antiepileptic treatment. Neurological examination and developmental milestones remained normal after the follow-up. CONCLUSIONS: This study confirms the existence of BIS. Non-familial cases might represent novo mutations or sporadic cases of BIS. The recognition of BIS is possible at beginning of the epilepsy. To confirm the syndrome the follow-up is necessary. Copyright 2009 Elsevier B.V. All rights reserved.
Authors: Ingrid E Scheffer; Bronwyn E Grinton; Sarah E Heron; Sara Kivity; Zaid Afawi; Xenia Iona; Hadassa Goldberg-Stern; Maria Kinali; Ian Andrews; Renzo Guerrini; Carla Marini; Lynette G Sadleir; Samuel F Berkovic; Leanne M Dibbens Journal: Neurology Date: 2012-10-17 Impact factor: 9.910
Authors: Jan H Döring; Afshin Saffari; Thomas Bast; Knut Brockmann; Laura Ehrhardt; Walid Fazeli; Wibke G Janzarik; Annick Klabunde-Cherwon; Gerhard Kluger; Hiltrud Muhle; Manuela Pendziwiat; Rikke S Møller; Konrad Platzer; Joana Larupa Santos; Julian Schröter; Georg F Hoffmann; Stefan Kölker; Steffen Syrbe Journal: Neurol Genet Date: 2022-09-28