Left untreated, infantile spasms can lead to serious outcomes, including an estimated infant mortality rate of between 5% and 6%. The most significant concern, however, is that infantile spasms are associated with autism and intellectual deficits that permanently affect quality of life.
Infantile spasms is a complex and rare disorder that can have very serious consequences. It can lead to death in some babies, and cause intellectual disabilities and developmental problems in others. Even once the seizures are gone, the damaging brain effects can remain.
Furthermore, do infantile spasms happen every day? Spasms tend to occur upon awakening and often occur in multiple clusters and hundreds of seizures per day. Most children, but not all, will have EEG readings of hypsarrhythmia. Infantile spasms usually stop by age five, but may be replaced by other seizure types.
Just so, can infantile spasms go away?
Infantile spasms (IS) is a seizure disorder in babies. The seizures (or spasms) make muscles in the arms and legs stiff and bend the baby’s head forward. Although the spasms usually go away by the time a child is 4 years old, many babies with IS will have other kinds of epilepsy later in life.
What happens if epilepsy is left untreated?
There is often no warning and no clear reason why the seizures happen. If epilepsy is not treated, seizures may occur throughout a person’s life. Seizures can become more severe and happen more often over time. Epilepsy can be caused by tumors or improperly formed blood vessels.
What is the life expectancy of a child with West syndrome?
Unfortunately, up to about 90% of all children with West syndrome still suffer severe cognitive and physical impairments. 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.
How often do infantile spasms occur in a day?
Clinically, the spasms appear in clusters and are characterized by brief, sudden contractions of the axial musculature. The clusters may occur several times daily, with up to 100 spasms per day. They appear to be temporally related to sleep, tending to occur as the infant falls asleep or awakens.
Can vaccines cause infantile spasms?
The majority of seizures are associated with fever, but there is no increased risk of febrile seizures after vaccination evolving into epilepsy. Infantile spasms occur within a time period when infants receive many scheduled vaccines.
How is West Syndrome diagnosed?
The diagnosis of West syndrome is made on a combination of the clinical features together with a typical EEG. The EEG shows a very disorganised pattern, called hypsarrhythmia. All children with infantile spasms and West syndrome will also need a magnetic resonance imaging (MRI) brain scan.
Are infantile spasms hereditary?
Infantile spasms are an age-dependent epilepsy that are highly associated with cognitive impairment, autism, and movement disorders. Children with certain genetic syndromes are much more likely to have infantile spasms, and we review the literature to propose a genetic classification of these disorders.
At what age do infantile spasms start?
Infantile spasms is considered an age specific epilepsy that typically begin between 3 and 8 months of age. Almost all cases begin by 1 year of age and usually stop by the age of 2 to 4 years.
Are infantile spasms painful?
Symptoms of Infantile Spasms (IS) Raise their arms over their head or stick their arms straight out to the side. Stiffen their legs or “tuck them into the belly,” as if having stomach pain. Suddenly bend at the waist.
How can you tell if your infant is having a seizure?
Neonatal Seizures Signs and Symptoms Random or roving eye movements, eyelid blinking or fluttering, eyes rolling up, eye opening, staring. Sucking, smacking, chewing and protruding tongue. Unusual bicycling or pedalling movements of the legs. Thrashing or struggling movements. Long pauses in breathing (apnea)
How common is West syndrome?
Occurrence in the United States. Infantile spasm constitutes 2% of childhood epilepsies but 25% of epilepsy with onset in the first year of life. The rate of infantile spasm is estimated to be 2.5-6.0 cases per 10,000 live births. Its prevalence rate is 1.5-2.0 cases per 10,000 children aged 10 years or younger.
What do Infantile seizures look like?
The seizures often look like a sudden bending forward of the body with stiffening of the arms and legs lasting for 1-2 seconds; some children arch their backs as they extend their arms and legs. Infantile spasms usually stop by age five, but may be replaced by other seizure types.
What is the treatment for infantile spasms?
Treatment of infantile spasms has little class I data, but adrenocorticotropic hormone (ACTH), prednisolone and vigabatrin have the best evidence as first-line medications. Other therapies including the ketogenic diet and other anti-epileptics medications may also prove useful in the treatment of infantile spasms.
Is West syndrome genetic?
The most common disorder responsible for West syndrome is tuberous sclerosis complex. (TSC). TSC is an autosomal dominant genetic condition associated with seizures, eye, heart and kidney tumors and skin findings. X-linked West syndrome can be caused by a mutation in the CDKL5 gene or the ARX gene in the X chromosome.
How do I know if my baby has neurological problems?
Additional warning signs of a neurological disorder include: Abnormal muscle tone at birth. Seizures. Floppy baby. Subtle staring/unresponsive episodes. Slow language and/or motor skills.
How are infantile spasms diagnosed?
Infantile spasms are diagnosed by a pediatric neurologist (a doctor who specializes in brain, spine and nervous system problems). Testing may include: blood tests and urine tests (to look for infections or illnesses) EEG, or electroencephalography (to see brain waves/electrical activity in the brain).