There is no cure for West syndrome , so unfortunately, the only treatment is to reduce symptoms. West syndrome treatments usually include a course of prednisolone and/or an anti-epileptic medication. In a few individuals, surgery in the brain may help reduce symptoms.
What is the treatment for West syndrome?
Steroids and anti-seizure medication are the most common treatments. The steroid may have adrenocorticotropic hormone (ACTH) and be injected into the muscles. Steroids might also be given in pill form. For some children a specific diet may help their condition.
Is West syndrome a rare disease?
West syndrome is a rare neurological syndrome that can affect males and females. The X-linked form of West syndrome affects males more often than females. West syndrome has been estimated to affect . 31 per 1000 live births in the United States.
Can children grow out of infantile spasms?
Infantile spasms usually stop by age five , but may be replaced by other seizure types. Many underlying disorders, such as birth injury, metabolic disorders, and genetic disorders can give rise to IS, making it important to identify the underlying cause. In some children, no cause can be found.
Does West syndrome go away?
West syndrome (also called infantile spasms) should go away by the time your child is 4 years old . But most people will develop another kind of epilepsy or seizure condition in childhood or as an adult.
Can you live a normal life 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 do you test for West syndrome?
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. The EEG is always abnormal in children with West syndrome but sometimes the abnormality is seen only during sleep.
What happens if infantile spasms are left untreated?
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.
Is myoclonus a seizure?
Myoclonic epilepsy causes the muscles in the body to contract . This type of seizure causes quick jerking movements. Myoclonic seizures often happen in everyday life. This includes hiccups and a sudden jerk while falling asleep.
What does infantile spasms look like?
Infantile spasms often look like a sudden, brief stiffening of a baby’s muscles . Symptoms may include: a cluster of spasms that may be associated with waking from sleep. jackknife seizures, where the body bends forward, the knees are pulled up, and the arms are thrown out to the side.
Is West syndrome inherited?
Genetic Predisposition
This form of the disorder is called X-linked West syndrome because these genes are present on the X chromosome. West syndrome due to a mutated ARX gene is inherited in an X-linked recessive manner .
Do infantile spasms cause mental retardation?
Infantile spasms is one of the “catastrophic childhood epilepsies” because of the difficulty in controlling seizures and the association with mental retardation .
What percentage of epilepsy is genetic?
About 30 to 40 percent of epilepsy is caused by genetic predisposition. First-degree relatives of people with inherited epilepsy have a two- to four-fold increased risk for epilepsy.
What are the signs to look for in neurological symptoms in infants?
- Fussiness.
- Decreased level of consciousness.
- Abnormal movements.
- Feeding difficulty.
- Changes in body temperature.
- Rapid changes in head size and tense soft spot.
- Changes in muscle tone (either high or low)
How do you know if your baby has infantile spasms?
Symptoms of Infantile Spasms (IS)
Stiffen their legs or “tuck them into the belly ,” as if having stomach pain. Suddenly bend at the waist. Drop or bob their heads briefly. Roll their eyes back suddenly with subtle head nodding.
Do infantile spasms cause autism?
Press Release. BALTIMORE – A severe form of epilepsy known as infantile spasms may increase the risk of children developing autism spectrum disorder (ASD) due to the condition’s unique seizures and resulting chaotic brainwaves, suggests research being presented at the American Epilepsy Society Annual Meeting.
