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Epileptic spasms - 175 years on: Trying to teach an old dog new tricks

Wilmshurst, JM; Ibekwe, RC; O'Callaghan, FJK; (2017) Epileptic spasms - 175 years on: Trying to teach an old dog new tricks. Seizure , 44 pp. 81-86. 10.1016/j.seizure.2016.11.021. Green open access

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Abstract

PURPOSE: This text provides an overview of how the condition "infantile spasms" has evolved in the last 175 years. METHOD: Key references are summarised to assimilate this review. Results: Infantile spasms, first described by Dr West in 1841, has undergone extensive investigation to understand the pathogenesis, aetiologies, optimal intervention and most likely prognosis for the affected child. The terminology has recently evolved such that the preferred term for the condition is now “epileptic spasms” in recognition of the fact that cases can present outside infancy. The aetiologies are diverse and can be structural, genetic, metabolic or acquired. Increasing numbers of presumed causative genetic mutations are now being identified. The condition is an epileptic encephalopathy such that without adequate control of the clinical seizures and correction of the abnormal EEG, ongoing neurological damage occurs. In some cases neuroregression is inevitable despite intervention. First-line treatments are either hormonal therapies, adrenocortcotrophic hormone or prednisolone, or vigabatrin. In the sub-group of patients with tuberous sclerosis complex, vigabatrin is the preferred treatment. High dose prednisolone may be a more viable option in resource limited settings. Recent research has suggested that combining hormonal therapies with vigabatrin will result in more patients achieving spasm cessation. CONCLUSIONS: Despite extensive study, the pathogenic mechanisms remain an area of debate and in need of further exploration. The enigma, however, may be explained as the role of resting state and dysfunctional brain networks are elucidated further.

Type: Article
Title: Epileptic spasms - 175 years on: Trying to teach an old dog new tricks
Open access status: An open access version is available from UCL Discovery
DOI: 10.1016/j.seizure.2016.11.021
Publisher version: http://doi.org/10.1016/j.seizure.2016.11.021
Language: English
Additional information: Crown Copyright © 2016 Published by Elsevier Ltd on behalf of British Epilepsy Association. All rights reserved. This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: Science & Technology, Life Sciences & Biomedicine, Clinical Neurology, Neurosciences, Neurosciences & Neurology, Infantile spasms, Late-onset epileptic spasms, ACTH, Oral corticosteroids, Prednisolone, Vigabatrin, KINGDOM INFANTILE SPASMS, MULTICENTER RANDOMIZED-TRIAL, AGE-OF-ONSET, KETOGENIC DIET, WEST-SYNDROME, TUBEROUS SCLEROSIS, COMPARING VIGABATRIN, PROGNOSTIC-FACTORS, ILAE COMMISSION, CHILDREN
UCL classification: UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Pop Health Sciences > UCL GOS Institute of Child Health
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Pop Health Sciences > UCL GOS Institute of Child Health > ICH Developmental Neurosciences Prog
URI: http://discovery.ucl.ac.uk/id/eprint/1536777
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