Disease Information for Infantile muscle spasms/Hypsarrhythmia

Clinical Manifestations
Signs & Symptoms
Abnormal movements/involuntary
Infant Seizures
Mental Deficiency Child
Myoclonus/Myoclonic jerks on exam
Newborn/Infant jittery/jitters
Shalom/symmetrical muscle spasms
Bizarre salaam position Head down Child
Nocturnal awakenings Events Phenomenon
Disease Progression
Demographics & Risk Factors
Established Disease Population
Patient/Tuberous sclerosis
Population Group
Population/Pediatrics population
Sex & Age Groups
Population/Child-Infant Only
Population/Infant male
Diagnostic Test Results
EEG/Bilateral burst pattern
EEG/Characteristic pattern abnormal
EEG/High voltage waves
EEG/Hypsarrhythmia pattern/Gibbs
Associated Diseases & Rule outs
Rule Outs
Convulsions (grand mal)
Seizure disorder (epilepsy)
Associated Disease & Complications
CNS disease/involvement
Convulsions (grand mal)
Encephalopathy/CNS effect
Infantile muscular spasms/Hypsarrhythmia
Mental retardation
Seizure disorder (epilepsy)
Disease Mechanism & Classification
CLASS/Pediatric disorders (ex)
Pathophysiology/Gene locus Chromosome X.
Pathophysiology/Gene Locus Identified/OMIM database
Pathophysiology/Gene locus Xp22.13
Pathophysiology/Genomic indentifiers (polymorphism/snip/mutations)
Attack Lightning, Attacks Lightning, eclampsia nutans, flexion spasm, greeting spasms, Hypsarrhythmia syndrome, Infant spasms hypsarrythmia, Infantile myoclonic epilepsy, infantile salaam, Infantile Spasm, INFANTILE SPASM SYNDROME X LINKED, Infantile spasms, Infantile spasms hypsarrythmia, Infantile spasms (disorder), infantile spasms hypsarrhythmia mental retardation syndrome, infantile spasms with mental retardation, INFANTILE SPASMS X LINKED, ISSX, jackknife spasm, Lightning Attack, Lightning Attacks, lightning spasm, Lightning spasms, nodding spasm, Seizure infant spasms, spasm infantile, spasm lightning, Spasms Infantile, Syndrome West, West, West syndrome, West syndrome (disorder), West syndrome (WS), WEST SYNDROME X LINKED, West's syndrome, X linked infantile spasms, Synonym/Generalized flexion seizure, Synonym/Gibbs hypsarrhythmia syndrome, Synonym/Hypsarrhythmia (subset), Synonym/Massive myoclonus seizures, Synonym/Myoclonus, massive seizure, Synonym/West syndrome
Drug Therapy - Indication
RX/Clonazepam (Klonopin)
RX/Valproic/divalproex (Depakote/Epival)
RX/Vigabatrin (Sabril)

West"s syndrome; Blitz-Nick-Salaam-Krämpfe (German), BNS-Epilepsiem (German), eclampsia nutans, flexion spasm, generalized flexion epilepsy syndrome, greeting spasms, infantile spasms, infantile spasm syndrome, infantile spasms with mental retardation, infantile spastic epilepsy, jackknife convulsion syndrome, jackknife spasm, massive myoclonia syndrome, nodding spasm, salaam attacks, salaam convulsions; A form of myoclonus epilepsy with onset in infancy or early childhood; It is characterized by seizures involving the muscles of the neck, trunk, and limbs, with nodding of the head and flexion and abduction of the arms as typical features; Mental retardation is associated in most cases; EEG shows hypsarrhythmia; There is a propensity to infections with a high mortality due to bronchopneumonia; Some cases are transmitted as an X-linked trait; [whonamedit_com 2005]

West Syndrome is a rare form of infantile spasm that occurs very early in the development of an infant; Unusual brain wave patterns occur (hypsarrhythmia) and possibly mental retardation; The spasms that occur may range from violent jackknife or "salaam" movements where the whole body bends in half, or they may be no more than a mild twitching of the nose or mouth; These spasms usually begin in the early months after birth and can often be helped with medication; There may be many different causes for the spasms; Neurological testing will be helpful in determining the cause;----------- [nord 2005 ]------


External Links Related to Infantile muscle spasms/Hypsarrhythmia
PubMed (National Library of Medicine)
NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)