INFANTILE SPASMS ARE AN EMERGENCY

Infantile spasms are one of the most severe infantile epilepsy.

These are more commonly called infantile spasms (IS) since they are seen most often in the first year of life. It is also called West Syndrome. West Syndrome/IS is characterized by epileptic spasms, developmental delay, and a specific EEG pattern called hypsarrhythmia. The onset is usually in the first year of life, typically between 4-8 months.

SEIZURE TYPE: The seizures often look like a sudden bending forward of the head,trunk, and both upper and lower limbs lasting for 1-2 seconds; some children have sudden extension of their head,arms and legs . Sometimes it can be as subtle as just head nods. Spasms tend to occur upon awakening and often occur in multiple clusters ,varying from few to many seizures per day. Most children,but not all, will have EEG readings of hypsarrhythmia.

SPASMS ARE EMERGENCY: Infantile spasms are an emergency because there will be developmental stagnation(means child doesn’t learn new things) or it causes regression in milestones(means child forgets the learnt behavior and social interaction) if not treated early.

ITS MISSED & MISDIAGNOSED: They are often misdiagnosed as colic,reflux or exaggerated startle.
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,called cryptogenic west syndrome.

INVESTIGATION: All children will need MRI brain. Further testing,if needed, for genetic and metabolic cause differs from case to case basis.

TREATMENT: Standard first-line therapies include several forms of hormonal therapy (including adrenocorticotrophic hormone [ACTH] or prednisolone) or the anti-seizure medication vigabatrin. These treatments are effective but have serious side effects and should be administered in consultation with a pediatric neurologist. The goal of treatment for infantile spasms is for the seizures to stop and the EEG to improve (hypsarrhythmia should resolve).

FINAL MESSAGE: All babies with head nods in cluster or sudden forward or backward head,trunk and limb jerks esp after awakening needs EEG to look for hypsarrythmia. Premature babies and babies with developmental delay have more chances to have spasms. It is a disorder not to be missed.