West Syndrome or Infantile Spasms
West Syndrome also known as Epileptic or Infantile Spasms
As a nurse practitioner specializing in Epilepsy with the Comprehensive Epilepsy Program at Dell Children’s Medical Center, I see things in infants that most people do not, including a condition called West Syndrome, also known as Epileptic or Infantile Spasms. This can be pretty scary for parents during pregnancy, but the babies that I care for on a daily basis represent a very small minority; that is, the zebras of the world, not the horses. It is normal to feel nervous about pregnancy, but most babies are born without any complications at all.
November is National Epilepsy Awareness Month, and in an effort to raise awareness, we would like to introduce a specific type of Epilepsy, West Syndrome, to help new parents spot the symptoms. Epilepsy is a term used for recurrent seizures, not provoked by fever, infection or an acute cause. Seizures are caused by abnormal electrical activity in the brain. Many people think of seizures as dramatic “Hollywood seizures” often seen on television with whole-body shaking known as tonic-clonic seizures. In contrast, infantile spasms are difficult for parents (or even medical providers) to recognize, because the seizures may actually be very subtle. Although infantile spasms may not look dangerous, they may be more serious than tonic-clonic seizures.
What are infantile spasms?
Infantile spasms occur in 2-5 cases per 10,000 live births. They usually begin between 3-8 months of age and commonly stop by 2-4 years, but may go on longer. Spasms are typically a few seconds of sudden stiffening of the body, arms, and legs described as extensor, flexor, or mixed movement. During extensor spasms, arms suddenly spread out, head moves backward and legs may extend out resembling a Moro reflex. With flexor spasms, body flexes or crunches forward, head elevates off the bed or falls forward when stiffening and legs may pull up. Events may also be more subtle with brief head nodding and eye rolling. Spasms typically occur in clusters when drowsy or upon awakening. Infantile spasms can mimic less dangerous things such as cramps from colic, reflux (known as Sandifer Syndrome), or even a normal startle reflex. A child may be irritable or cry after the event.
What can parents do?
If your infant experiences events concerning for seizures, record the events on your phone if possible, and notify your primary care provider. It is extremely helpful for providers to observe these events on video. Keeping a journal of how often the events occur is also helpful. The sooner treatment can be started, the lower the chance of developmental problems. Therefore, parents should not wait to seek advice if they suspect their baby may be experiencing spasms.
How is West Syndrome diagnosed?
If a provider suspects that the infant may have West Syndrome, they will urgently refer the patient to a neurology or epilepsy clinic where the child will have an electroencephalogram (EEG). During this test, small electrodes with thin wires are placed on the scalp. These are connected to a computer, which can detect abnormal brain waves. If the provider is unable to capture a spasm on EEG, they may also look for a chaotic pattern of brain waves known as hypsarrhythmia. This is a common finding in children with spasms. If the 60-minute EEG does not capture sufficient information to diagnose spasms, the child may need an overnight stay in the hospital for a longer recording. If infantile spasms are diagnosed, the child may undergo further investigative studies such as an MRI of the brain, labs, or a lumbar puncture to try to find a cause for infantile spasms.
How are spasms treated?
Identifying an underlying cause is important because this can guide treatment, which can then improve the developmental outcome of the child. A cause can be identified in about 70% of patients. Structural brain abnormalities are common causes. Other causes include tumors, infection, and metabolic or genetic disorders. The goal of treatment is to completely control spasms. First line treatment is adrenocorticotropic hormone therapy (ACTH), which must be given via injection 1-2 times a day for a few weeks. Relapse of spasms after initial response may occur, in which case a second course may be tried. An oral medication called vigabatrin is another effective treatment for spasms, particularly in patients with tuberous sclerosis. Oral steroids have also been used; however there is insufficient evidence whether this should be used as first line therapy. Second line therapies include ketogenic diet, or other antiepileptic medications. Pyridoxine (vitamin B6) may be extremely helpful in a few children. Therefore, if there is not a known cause it should always be tried. If spasms are resistant to first line therapies, a surgical focal resection — surgery where an abnormal part of the brain thought to be causing seizures is removed — may be an option in some patients.
What is the prognosis?
The outlook of babies with spasms depends on the underlying cause and how quickly treatment is initiated. Most children with infantile spasms have some kind of learning disabilities. Children with certain types of underlying brain disorders may lead to severe developmental delays and may even become progressively worse. Even if infantile spasms are completely stopped, children may develop other types of seizures in the future. There is a higher chance for infants to develop normally if they were developmentally appropriate before the spasms started, if cause is unknown, or if there is a treatable cause. Therefore, it is important for children to receive treatment promptly, especially in those who have a chance at developing normally.
For all new parents, it’s so important to have an awareness about infantile spasms so that the signs and symptoms do not go unnoticed. Black with white stripes, white with black stripes, or no stripes at all, I am blessed to help these wonderful and wonderfully unique infants every day.
For more information, please visit: Comprehensive Epilepsy Program at Dell Children’s Medical Center
Miriam Jackson received her Bachelor of Science in Nursing degree in 2008 from The University of Texas at Austin and has several years of experience working on a busy general pediatric unit at Children’s Medical Center in Dallas, Texas. In 2012, she earned her Master of Science in Nursing from the University of Texas at Arlington with dual certification in acute and primary care. Miriam is an Advanced Practice Provider who joined the Comprehensive Epilepsy Program in 2013 and provides care at Dell Children’s Medical Center in the epilepsy monitoring unit. She is passionate about providing holistic care and is involved in epilepsy research. Miriam enjoys trips to the beach with her husband Erick, and spending time with family and friends.
Miriam Jackson, CPNP-PC/AC
Dell Children’s Medical Center
Comprehensive Epilepsy Program
Austin, TX
Facebook: @dellchildrensmedicalcenter
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