Treatment Overview
For parents, receiving a diagnosis of West syndrome also known as infantile spasms can be a terrifying and confusing experience. The condition typically appears in the first year of life, characterized by a specific type of seizure that looks like a sudden stiffening or “jackknife” movement of the body. These spasms often occur in clusters and can happen dozens of times a day, disrupting a baby’s sleep, development, and interaction with the world.
Treatment is considered a medical urgency. The immediate goal is not just to reduce the number of seizures but to stop them completely and correct the chaotic brain wave patterns (hypsarrhythmia) seen on an EEG. Rapid treatment is essential to protect the developing brain and minimize the risk of long-term cognitive or developmental delays. Because the underlying cause of West syndrome varies, ranging from genetic mutations to brain malformations, treatment plans are tailored to the specific needs of the infant (National Institute of Neurological Disorders and Stroke, 2023).
Overview of treatment options for West Syndrome
The treatment approach for West syndrome is distinct from typical epilepsy management. While standard anti-seizure medications are used for many conditions, West syndrome often requires aggressive, short-term courses of high-potency medications to “reset” the brain’s electrical activity.
The primary objective is to achieve “spasm freedom” and normalize the EEG reading. Doctors typically categorize treatment into two main pillars: hormonal therapy and specific anticonvulsant medication. Clinical experience suggests that the best developmental outcomes are associated with the shortest time between the onset of spasms and the start of effective treatment. While dietary therapies (like the ketogenic diet) or epilepsy surgery may be considered later for drug-resistant cases, pharmacological intervention is the standard first step.
Medications used for West Syndrome
There are two primary first-line treatments widely accepted by pediatric neurologists. The choice between them often depends on the underlying cause of the spasms.
The first category is hormonal therapy. This typically involves Adrenocorticotropic Hormone (ACTH), given as an injection into the muscle. Alternatively, high-dose oral corticosteroids, such as prednisolone, may be prescribed. These hormonal treatments are often used for a few weeks to abruptly stop the spasms. Studies show that hormonal therapy is highly effective for many infants, particularly those where no specific brain lesion is identified.
The second major option is vigabatrin, an oral anti-seizure medication. This is the preferred first-line treatment specifically for infants whose spasms are caused by Tuberous Sclerosis Complex (TSC). It is also used when hormonal therapy is ineffective or not a viable option.
If these first-line treatments do not resolve the spasms, doctors may try second-line anticonvulsants such as topiramate, zonisamide, valproic acid, or benzodiazepines like clobazam. However, these are generally considered less effective for the initial control of infantile spasms compared to ACTH or vigabatrin (Epilepsy Foundation, 2022).
How these medications work
Hormonal therapies (ACTH and steroids) are thought to work by suppressing corticotropin-releasing hormone (CRH). By stimulating or directly providing steroids, they reduce neuroinflammation and excitability, thereby calming the chaotic electrical bursts of hypsarrhythmia.
Vigabatrin works chemically by inhibiting the enzyme that breaks down GABA, a calming neurotransmitter. Increasing GABA levels strengthens the brain’s “brakes,” stopping the excessive electrical firing that causes spasms.
Side effects and safety considerations
West syndrome treatments are potent and need close, often in-hospital, monitoring. Hormonal therapies cause significant side effects: irritability (often called “roid rage”), increased appetite, weight gain, and facial swelling. Since steroids suppress the immune system, infants face a higher infection risk and must not receive live vaccines during treatment. High blood pressure and elevated blood sugar also require monitoring.
Vigabatrin carries the specific, permanent risk of peripheral vision loss (retinal toxicity). While hard to test in infants, this risk is weighed against uncontrolled seizure damage. Drowsiness and muscle weakness are common. Parents must seek immediate medical care if their child develops fever, difficulty breathing, or signs of infection while on these medications (Mayo Clinic, 2021).
Since everyone’s experience with the condition and its treatments can vary, working closely with a qualified healthcare provider helps ensure safe and effective care.
References
- National Institute of Neurological Disorders and Stroke. https://www.ninds.nih.gov
- Epilepsy Foundation. https://www.epilepsy.com
- Mayo Clinic. https://www.mayoclinic.org
- National Organization for Rare Disorders. https://rarediseases.org
Medications for West Syndrome
These are drugs that have been approved by the US Food and Drug Administration (FDA), meaning they have been determined to be safe and effective for use in West Syndrome.