Efficacy of long-term adrenocorticotropic hormone therapy for West syndrome: A retrospective multicenter case series.

Epilepsia Open
Shimpei BabaAyataka Fujimoto

Abstract

Long-term adrenocorticotropic therapy (LT-ACTH), which consisted of 2-4 weeks of daily injections of adrenocorticotropic hormone (ACTH) and subsequent months of weekly injections, was tried for relapsed West syndrome (WS) or other intractable epilepsies in small case reports. Our aim was to explore the efficacy of LT-ACTH for preventing WS relapse, as well as the prevalence of its adverse events. This is a retrospective, nationwide, multicenter case series of patients with WS who underwent LT-ACTH. Clinical information of the patients and protocol of LT-ACTH were collected from participating institutes in this study. We defined clinical response to ACTH as achievement of hypsarrhythmia and epileptic spasms resolution. Patients who responded to daily ACTH injections were identified and assessed whether they experienced WS relapse during/after the weekly ACTH injection period. The outcome was measured by the nonrelapse rate at 24 months after daily ACTH injections using the Kaplan-Meier method. Clinical information of 16 children with WS was analyzed. The median age at LT-ACTH initiation was 14.5 months (range: 7-68 months). Thirteen (81%) patients had previously undergone conventional ACTH treatment. The LT-ACTH regimens compris...Continue Reading

References

Oct 1, 1991·Journal of Child Neurology·L D Cowan, L S Hudson
Mar 1, 1980·The Journal of Pediatrics·W D SingerJ S Haller
Sep 1, 1980·Archives of Disease in Childhood·R Riikonen, M Donner
Feb 1, 1981·Developmental Medicine and Child Neurology·A MatsumotoS Miyazaki
Apr 1, 1983·Epilepsia·R Riikonen
Oct 1, 1983·The Journal of Pediatrics·R A HrachovyT E Zion
Jun 15, 1999·Epilepsia·E TrevathanM Yeargin-Allsopp
Oct 16, 1999·Brain & Development·S YanagakiM Osawa
Jun 27, 2000·Journal of Child Neurology·M ItoY Takuma
Nov 10, 2001·Brain & Development·R Riikonen
Nov 10, 2001·Brain & Development·R Riikonen
Jun 4, 2002·International Review of Neurobiology·K L BrunsonT Z Baram
Jan 5, 2008·Journal of Child Neurology·Arthur Partikian, Wendy G Mitchell
May 20, 2008·Pediatric Neurology·Tohru OkanishiHajime Togari
Jul 9, 2010·Epilepsia·John M PellockJames W Wheless
Jun 19, 2014·Seizure : the Journal of the British Epilepsy Association·Keitaro YamadaYasuhiro Suzuki
Aug 26, 2014·Brain & Development·Takehiko InuiKazuhiro Haginoya
Sep 13, 2016·Epilepsia·Kelly G KnuppUNKNOWN Pediatric Epilepsy Research Consortium

❮ Previous
Next ❯

Software Mentioned

R

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.