West syndrome: early remission is not assurance of normal final outcome

Child: Care, Health and Development
A P de Queiroz Campos AraújoL Pires

Abstract

West syndrome (WS), an age-specific epilepsy of infancy and early childhood, considered of poor prognosis, may have remission. With the objective of describing the clinical features, treatment and outcome of the children with WS followed in a Latin American country, we devised a retrospective study. Infants with recurrent spasms, and typical or atypical hypsarrhythmia on electroencephalography (EEG) were included, and their medical report reviewed with particular interest on therapy (anti-epileptic drug, steroids) and outcome. Short-term (remission of spasms up to 6 months after the beginning) and long-term outcome (development and seizure status over the age of 2 years) were focused. Of the 37 selected infants, most (29/37, 78.3%) were classified as symptomatic, and neonatal history of hypoxia (10/29) was the most frequent etiology. The majority had a typical hypsarrhythmia EEG pattern. Most used a combination of anti-epileptic drugs and steroids. A favourable short-term outcome occurred in 20 infants, but only three had normal development and were seizure free after the age of 2 years. Early seizure remission is not assurance of normal final outcome in WS.

References

Jul 8, 2000·Epilepsia·W D Shields
Oct 24, 2001·Neurology·R D EltermanUNKNOWN US Infantile Spasms Vigabatrin Study Group
Jul 31, 2003·Epilepsia·Giuseppe CapovillaGiangennero Coppola
Feb 6, 2004·Arquivos de neuro-psiquiatria·Newra Tellechea RottaRudimar Riesgo
Aug 2, 2005·Arquivos de neuro-psiquiatria·Maria Helena P MoraesMarilisa M Guerreiro
Sep 27, 2005·Seizure : the Journal of the British Epilepsy Association·Matloob AzamJai Krishin
Feb 20, 2007·The Journal of Pediatrics·Shin-ichiro HamanoYoshikatsu Eto
Dec 8, 2007·European Journal of Paediatric Neurology : EJPN : Official Journal of the European Paediatric Neurology Society·Francesco GuzzettaFrancesca Tinelli
Apr 8, 2009·European Journal of Neurology : the Official Journal of the European Federation of Neurological Societies·S Cohen-SadanH Goldberg-Stern

❮ Previous
Next ❯

Citations

Feb 7, 2016·Acta neurologica Belgica·Sanem YilmazSarenur Gokben
Feb 1, 2017·The International Journal of Neuroscience·Gulen Gul MertIlker Unal

❮ 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.