Infantile spasms: symptoms, causes and treatment

Infantile spasms represent a rare form of seizure disorder that affects very young children. First diagnosed in 1841 infantile spasms’ average age of presentation is four months of age. However, there have been cases where symptoms appear in a child as young as one month of age, and a few up to the age of 2 years.

Most people confuse infantile spasms with epilepsy. But that is a falsehood. Infantile spasms are not a symptom of epilepsy. In most cases, children outgrow these symptoms by the time they reach 4 years of age.

Symptoms on infantile spasms

The symptoms of infantile spasms are very subtle. In many cases, parents fail to recognize the symptoms. If not recognized and treated right away infantile spasms can lead to further complications. 

Most people think of seizure disorders in terms of someone falling down and having full-body convulsions. In infantile spasms disorder this is not the case. Its symptoms may appear like - 

Bobbings

This involves the complete head heaving forward towards the knees and then relaxing into the normal straight position.

These bowings and relaxings occur repeatedly in rows with few second intervals. They can last about 2-3 minutes.

These clusters of spasms can occur several times a day. An EEG throws up hypsarrhythmia between and during the spasms. These children may also have underlying neurological issues and developmental delays .

Head drops

In very young children, infantile spasms may present as little head drops that do not appear too serious.

Even the pediatrician may not be able to pick it up as they resemble the normal startle reflex

Apart from these symptoms, parents may also notice other changes in their little one. These are:

●     Loss of previously acquired developmental milestones (sitting, crawling, babbling, etc.)

●     Increased silence or fussiness

●     Loss of social interactions

●     Lesser smiles

Other names for infantile spasms

Due to the abnormal positioning of the body during spasms, infantile spasms have been variously described as- 

●     Salaam seizures

●     Nodding spasms

●     Jack-knife spasms

●     West syndrome

●     Infantile spastic epilepsy

Causes of infantile spasms

Understanding or recognizing the causes of infantile spasms can help determine its treatment too. Some of the causes are:

●     Brain abnormality (tumors, cysts, etc.)

●     Brain injury

●     Genes

●     Prevailing disorders like down syndrome, cerebral palsy, etc.

What happens if infantile spasms are not treated?

Leaving infantile spasms untreated increases the risk of developmental disabilities and autism. There are over 200 disorders associated with infantile spasms. Some of these include brain tumors or cysts, metabolic conditions, tuberous sclerosis, genetic abnormalities, brain malformations and brain injuries due to a variety of causes including brain infections, trauma or low oxygen at birth.

Diagnosing infantile spasms

An EEG is most often prescribed to diagnose infantile spasms. The EEG pattern will typically show a characteristic random high-voltage slow waves and spikes that are almost continuous and known as hypsarrhythmia.

With progress in neuroimaging technology (MRI and CT scans), the proportion of confirmed cases of infantile spasms has increased over the years. Studies have shown that one in every 2,200-4,500 children born, have infantile spasms and boys are more prone to developing this disorder than girls.

Treating infantile spasms

Although infantile spasms are difficult to recognize and diagnose, starting treatment at the earliest is critical. And how can we do that? By familiarizing ourselves with the symptoms (mentioned above), and visiting a developmental pediatrician if required.

Treatment is usually successful with anticonvulsant medications and associated injections of ACTH hormone. It takes about 1-2 weeks before you’ll start noticing improvement in your little one. So, we urge you to be patient and diligent with the treatment.

However, if there is another underlying cause for the infantile spasms, for example, the presence of brain tumors or cysts, then these need to be surgically removed first.

S.T.O.P.

When in doubt, STOP and -

S-ee the signs (uncontrolled movements, bobbing, head drops)

T-ake a video of the symptoms and consult your doctor asap

O-btain diagnosis with an EEG

P-rioritize treating your little one’s infantile spasms straight away

Infantile spasms typically disappear by the age of 3-4 years, though it may reappear as a part of epilepsy syndrome in later years in a few people.