Betül Tekin Güveli1, Özlem Çokar2, Nimet Dörtcan3, Gülçin Benbir4, Veysi Demirbilek4, Ayşın Dervent4. 1. Bakirkoy Research and Training Hospital for Psychiatry, Neurology, Neurosurgery, Department of Neurology, Istanbul, Turkey. Electronic address: betultekin2013@gmail.com. 2. Haseki Research and Training Hospital, Department of Neurology, Istanbul, Turkey. 3. Fatih Sultan Mehmet Research and Training Hospital, Department of Neurology, Istanbul, Turkey. 4. Istanbul University, Cerrahpasa Faculty of Medicine, Department of Neurology, Pediatric Neurology, Istanbul, Turkey.
Abstract
PURPOSE: Nearly half of all patients with seizure onset in the first year of life suffer from West syndrome (WS). The prognosis of epilepsy and psychosocial outcomes in children with WS are variable. This study was performed to examine the factors influencing the outcome of this patient population. METHODS: A total of 109 patients with WS followed up regularly for at least 3 years were included in the study. Relevant clinical, laboratory, and imaging data were collected. RESULTS: The male/female ratio was 65/44 (59.6%/40.4%). The mean age at onset of infantile spasm (IS) was 6 ± 6 (1-36) months. With regard to neuro-developmental and social conditions during the final evaluation, 29.4% of the patients were socially dependent on caregivers, 61.8% needed assistance, and 8.8% were normal. Among the patients, 5.9% were free of epilepsy and antiepileptic drugs (AED) for at least 2 years, 49.0% had no seizures with AEDs, and 45.1% had uncontrollable seizures. Parameters with significant negative effects on the long-term outcomes included symptomatic etiology, presence of developmental retardation before the onset of IS, persistence of active epilepsy, and male gender. CONCLUSION: In this study, 37 (33.9%) patients had severe consequences as a result of WS. The majority of the rest could cope with daily life with varying degrees of assistance. Eight percent of the patients had a normal development. These results draw attention to the two-thirds of patients with WS who have the chance of an acceptable quality of life (QoL) with early diagnosis and therapeutic measures.
PURPOSE: Nearly half of all patients with seizure onset in the first year of life suffer from West syndrome (WS). The prognosis of epilepsy and psychosocial outcomes in children with WS are variable. This study was performed to examine the factors influencing the outcome of this patient population. METHODS: A total of 109 patients with WS followed up regularly for at least 3 years were included in the study. Relevant clinical, laboratory, and imaging data were collected. RESULTS: The male/female ratio was 65/44 (59.6%/40.4%). The mean age at onset of infantile spasm (IS) was 6 ± 6 (1-36) months. With regard to neuro-developmental and social conditions during the final evaluation, 29.4% of the patients were socially dependent on caregivers, 61.8% needed assistance, and 8.8% were normal. Among the patients, 5.9% were free of epilepsy and antiepileptic drugs (AED) for at least 2 years, 49.0% had no seizures with AEDs, and 45.1% had uncontrollable seizures. Parameters with significant negative effects on the long-term outcomes included symptomatic etiology, presence of developmental retardation before the onset of IS, persistence of active epilepsy, and male gender. CONCLUSION: In this study, 37 (33.9%) patients had severe consequences as a result of WS. The majority of the rest could cope with daily life with varying degrees of assistance. Eight percent of the patients had a normal development. These results draw attention to the two-thirds of patients with WS who have the chance of an acceptable quality of life (QoL) with early diagnosis and therapeutic measures.
Authors: Scott T Demarest; Renée A Shellhaas; William D Gaillard; Cynthia Keator; Katherine C Nickels; Shaun A Hussain; Tobias Loddenkemper; Anup D Patel; Russell P Saneto; Elaine Wirrell; Iván Sánchez Fernández; Catherine J Chu; Zachary Grinspan; Courtney J Wusthoff; Sucheta Joshi; Ismail S Mohamed; Carl E Stafstrom; Cynthia V Stack; Elissa Yozawitz; Judith S Bluvstein; Rani K Singh; Kelly G Knupp Journal: Epilepsia Date: 2017-11-03 Impact factor: 5.864
Authors: Maria Eulina Quilião; Fabio Antonio Venancio; Lisany Krug Mareto; Sahra de Almeida Metzker; Ana Isabel do Nascimento; Daniele Cristina Vitorelli-Venancio; Cláudia Du Bocage Santos-Pinto; Everton Falcão de Oliveira Journal: Viruses Date: 2020-09-25 Impact factor: 5.048