What is West baby syndrome?
West syndrome is a constellation of symptoms characterized by epileptic/infantile spasms, abnormal brain wave patterns called hypsarrhythmia and intellectual disability.
How is hypsarrhythmia treated?
Children with IS typically exhibit epileptic spasms along with the electroencephalographic (EEG) pattern known as hypsarrhythmia. The major options for treatment of IS include hormonal therapy, mainly corticotropin (ACTH), and antiseizure medication, mainly vigabatrin.
What is the life expectancy of a child with West syndrome?
Life expectancy for West syndrome is variable. About five in every 100 infants and children with West syndrome do not survive beyond five years of age. A study of 214 Finnish children over 25 years of age showed about 61% dying at or before age 10 years.
Is West syndrome fatal?
West syndrome is generally not life-threatening but it occurs more often in children with life-threatening conditions. Furthermore, the treatments used for West syndrome can (rarely) cause death. As a result, only 5 out of every 100 children diagnosed with West syndrome will not survive beyond the age of five years.
Why does ACTH work in infantile spasms?
ACTH stimulates part of the brain to release other hormones. In infantile spasms, the ACTH is injected into a muscle with an IV needle. Moderate evidence shows short-term use of ACTH may help treat infantile spasms. Moderate evidence shows that a low dose of ACTH can be as effective as higher doses.
What is the difference between West syndrome and infantile spasms?
(See Presentation and Workup.) West syndrome is an age-dependent expression of a damaged brain, and most patients with infantile spasms have some degree of developmental delay. The term infantile spasm has been used to describe the seizure type, the epilepsy syndrome, or both.
Is West syndrome hereditary?
West syndrome due to a mutated ARX gene is inherited in an X-linked recessive manner.
What is Gervais syndrome?
Dravet syndrome is a rare, drug-resistant epilepsy that begins in the first year of life in an otherwise healthy infant. It is lifelong. It usually presents with a prolonged seizure with fever that affects one side of the body. Most cases are due to severe SCN1A gene mutations.
What is the medical definition of hypsarrhythmia?
Medical Definition of hypsarrhythmia. : an abnormal encephalogram that is characterized by slow waves of high voltage and a disorganized arrangement of spikes, occurs especially in infants, and is indicative of a condition that leads to severe intellectual disability if left untreated.
What is hypsarrhythmia in PEHO syndrome?
Hypsarrhythmia with little spike or sharp activity. The “H” in PEHO syndrome stands for hypsarrhythmia. Together with developmental regression and infantile spasms, hypsarrhythmia is one of the diagnostic criteria for West syndrome.
What does hypsarrhythmia mean ON EEG?
Hypsarrhythmia. It is an abnormal interictal pattern, consisting of high amplitude and irregular waves and spikes in a background of chaotic and disorganized activity seen on electroencephalogram (EEG), and frequently encountered in an infant diagnosed with infantile spasms, although it can be found in other conditions.
What is hypsarrhythmia in West syndrome?
Hypsarrhythmia is the characteristic interictal elecroencephalography (EEG) pattern of West syndrome, a severe infantile epileptic encephalopathy ( Gibbs & Gibbs, 1952; Hrachovy et al., 1984; Lux & Osborne, 2004a ).