Long-term otucome of West syndrome: a study of adults with a history of infantile spasms

Epilepsia
R Riikonen

Abstract

To our knowledge, ours is the first study to evaluate the outcome of infantile spasms (IS) in adult patients. We analyzed 214 children born between 1960 and 1976 who had been followed for 20-35 years or until death at 3 months to 30 years of age. Mortality was 31% (67 of 214 patients). Thirty-six of the surviving patients (24%) had normal (25 patients) or only slightly impaired (11 patients) intelligence as assessed by their educational abilities. Four had academic occupations. Eight were married or living unmarried with a partner. Five had healthy children. At follow-up, the EEGs of the 25 normal persons were either normal or slightly abnormal, demonstrated focal findings in 9 (36%), and had unspecific changes in 1. Focal abnormalities were not more common in patients with less good outcomes (37%). In patients with normal neurological outcomes, IS had been classified as cryptogenic only in 9 of 25 (36%) cases. Therefore, some patients with IS apparently have normal intelligence and socioeconomic status as adults, including patients whose spasms were either symptomatic or associated with focal EEG findings.

References

Jan 1, 1992·Epilepsia·W D ShieldsW J Peacock
Mar 1, 1990·Developmental Medicine and Child Neurology·R Riikonen, O Simell
Jul 1, 1987·Pediatric Neurology·V K WongR Hanson
Jun 1, 1973·Epilepsia·P M JeavonsM Dimitrakoudi
Jun 1, 1984·European Journal of Pediatrics·D LangG von Bernuth
Sep 1, 1980·Archives of Disease in Childhood·R Riikonen, M Donner
Aug 1, 1983·Neurology·O C SneadG J Myers
Feb 1, 1981·European Journal of Pediatrics·A MatsumotoS Miyazaki
Oct 1, 1994·Journal of Child Neurology·G B Bobele, J B Bodensteiner
Mar 1, 1995·Developmental Medicine and Child Neurology·R Riikonen
Oct 1, 1993·Neuropediatrics·R Riikonen

❮ Previous
Next ❯

Citations

Apr 3, 2012·Neurotherapeutics : the Journal of the American Society for Experimental NeuroTherapeutics·Stéphane AuvinAndrey Mazarati
Mar 29, 2001·Pediatric Neurology·M Wong, E Trevathan
Sep 2, 2003·Epilepsy Research·Monika Maria EisermannC Chiron
Mar 21, 2002·Epilepsia·Zvonka Rener PrimecDavid Neubauer
Jul 31, 2003·Epilepsia·Giuseppe CapovillaGiangennero Coppola
Mar 12, 2010·The Neurologist·Eric H Kossoff
Apr 8, 2009·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·S Cohen-SadanH Goldberg-Stern
Jun 15, 1999·Epilepsia·E TrevathanM Yeargin-Allsopp
Jul 8, 2000·Epilepsia·W D Shields
Sep 20, 2008·Epilepsia·David A MuzykewiczElizabeth A Thiele
Jul 9, 2010·Epilepsia·John M PellockJames W Wheless
May 28, 2013·BMC Neurology·Salvatore ManganoAntonina Fontana
Jun 1, 2010·Continuum : Lifelong Learning in Neurology·Elaine Wirrell, Katherine C Nickels
Jun 27, 2009·Neuropsychiatric Disease and Treatment·Chang-Yong Tsao
Aug 16, 2012·European Journal of Pediatrics·Stéphane AuvinLuigi Titomanlio
Sep 2, 2014·Pediatric Neurology·Drew Thodeson, Yoshimi Sogawa
Apr 9, 1998·Brain & Development·K Watanabe
Nov 10, 2001·Brain & Development·O Dulac
Nov 10, 2001·Brain & Development·C Young, UNKNOWN Taiwan Child Neurology Society
Nov 10, 2001·Brain & Development·R Riikonen
Nov 10, 2001·Brain & Development·R E Appleton
Jun 10, 2011·Expert Review of Neurotherapeutics·Matti Sillanpää, Dieter Schmidt
Aug 15, 2012·Brain & Development·Akiko FujiiMakiko Osawa
Jan 31, 2012·Pediatric Clinics of North America·Wendy G Silver, Isabelle Rapin
Jul 9, 2010·Pediatric Neurology·Eveline V PerretMaja Steinlin
Apr 14, 2009·European Journal of Paediatric Neurology : EJPN : Official Journal of the European Paediatric Neurology Society·Raili S Riikonen
Jun 9, 2007·Brain & Development·Fumika EndohYoko Ohtsuka
Feb 20, 2007·The Journal of Pediatrics·Shin-ichiro HamanoYoshikatsu Eto
Dec 2, 2006·Pediatric Neurology·Ayako HattoriHajime Togari
Jun 10, 2005·Pediatric Allergy and Immunology : Official Publication of the European Society of Pediatric Allergy and Immunology·Masashi KondoNaomi Kondo
Aug 14, 2012·Pediatrics International : Official Journal of the Japan Pediatric Society·Takeo MureMasafumi Matsuo
May 29, 2012·Acta Neurologica Scandinavica·M K Heim, B E Gidal
Mar 5, 2016·Expert Opinion on Pharmacotherapy·Catherine Chiron
Aug 31, 2006·Pediatric Neurology·Zvonka Rener-PrimecDavid Neubauer
Feb 14, 2006·Lancet·Renzo Guerrini

❮ Previous
Next ❯

Related Concepts

Trending Feeds

COVID-19

Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.

Blastomycosis

Blastomycosis fungal infections spread through inhaling Blastomyces dermatitidis spores. Discover the latest research on blastomycosis fungal infections here.

Nuclear Pore Complex in ALS/FTD

Alterations in nucleocytoplasmic transport, controlled by the nuclear pore complex, may be involved in the pathomechanism underlying multiple neurodegenerative diseases including Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Here is the latest research on the nuclear pore complex in ALS and FTD.

Applications of Molecular Barcoding

The concept of molecular barcoding is that each original DNA or RNA molecule is attached to a unique sequence barcode. Sequence reads having different barcodes represent different original molecules, while sequence reads having the same barcode are results of PCR duplication from one original molecule. Discover the latest research on molecular barcoding here.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Position Effect Variegation

Position Effect Variagation occurs when a gene is inactivated due to its positioning near heterochromatic regions within a chromosome. Discover the latest research on Position Effect Variagation here.

STING Receptor Agonists

Stimulator of IFN genes (STING) are a group of transmembrane proteins that are involved in the induction of type I interferon that is important in the innate immune response. The stimulation of STING has been an active area of research in the treatment of cancer and infectious diseases. Here is the latest research on STING receptor agonists.

Microbicide

Microbicides are products that can be applied to vaginal or rectal mucosal surfaces with the goal of preventing, or at least significantly reducing, the transmission of sexually transmitted infections. Here is the latest research on microbicides.

Related Papers

European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies
S Cohen-SadanH Goldberg-Stern
Epilepsy Research and Treatment
Joe Yuezhou Yu, Phillip L Pearl
© 2021 Meta ULC. All rights reserved