Serious Seizure Syndromes in Infants

When Epilepsy Affects a Child's Development or Lifespan

Mother holding newborn baby
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When a child is born, parents usually have all kinds of hopes and dreams for the future. Adjusting to the news of any kind of medical disorder is heart-wrenching, but especially so if the child will not think or develop normally. While many seizure disorders can have an ultimately good outcome, some pediatric epilepsies are more serious and associated with learning disabilities or early death. 

Here are some basic facts about a few serious seizure disorders seen in children. 

Early Myoclonic Encephalopathy

Early myoclonic encephalopathy is associated with myoclonus, a fast muscle twitch that comes on in early infancy. An electroencephalogram (EEG) is very abnormal in these cases. Sadly, the infants remain totally dependent on others, and over half are likely to die within the first year of life.

Early Infantile Epileptic Encephalopathy

Early infantile epileptic encephalopathy, also known as Ohtahara syndrome, affects children when they are only a few weeks or months old. Seizures are frequent and often intractable. Ohtahara syndrome has a typical EEG pattern that helps in diagnosis. Those who survive the disorder may be profoundly handicapped.

West Syndrome

West Syndrome is named after the English physician William James West, who described the disease in 1841. West Syndrome is associated with a classic triad of infantile spasms, an abnormal EEG pattern called hypsarrhythmia, and developmental regression. West syndrome occurs in about one per 1900 to 1 per 3900 infants. Medications are available that can treat the disorder, including vigabatrin or corticotropin. Even these drugs usually don't have a large effect, though, and the prognosis usually remains very poor unless a specific and reversible cause of the syndrome is found.

Malignant Migrating Partial Seizures in Infancy

This seizure syndrome usually occurs in the first seven months of life. The seizures are typically rare to begin with, then increase to the point where there may be up to 50 a day. Similarly, the seizures will increase in duration, going from seconds in length to minutes. An EEG will seem to show seizures starting in different areas of the brain, rather than always starting in the same fashion. The seizures are usually very difficult or impossible to control, and child development is impaired.

Dravet Syndrome

Dravet Syndrome begins in the first year of life in an infant who had previously been well. The first seizure is often triggered by a fever. As time passes, the child has many types of seizures, including general seizures, partial seizures, and myoclonic jerks. The initial EEG is normal but slows over time. Between the ages of 1 and 4, the child loses previously learned skills. Seizures become refractory to any kind of treatment, and 16 to 18 percent of children die, often due to status epilepticus, drowning, or sudden unexplained death in epilepsy (SUDEP).

Coping With Serious Infantile Epilepsy

If your child has a serious form of epilepsy like one of the above, you may feel helpless. Most parents would rather suffer anything themselves than watch their child go through something like epilepsy with a developmental delay. It's important to remember that, just because a disease may not have a cure, that doesn't mean there is no help available to you.

  • Talk to your pediatrician and educate yourself about your child's illness. This can help alleviate some of the fear of unfamiliarity, and help you plan for what your child needs.
  • Educate friends and family members so they know what to do when your child has a seizure.
  • Take care of yourself, relax, and think positively. Children take their cues from their parents. If you are relaxed, your child will pick up on that and be more likely to be relaxed and happy as well.
  • Talk to other people who've gone through something similar. Consider joining a support group. Beyond just talking about your feelings, you may gain valuable tips and advice.

Having a child with a serious developmental disability is uniquely challenging and can be very emotional, but it's not without rewards as well. You still have your own baby, completely unique, who needs you just as much as any other baby would — and maybe more. Nobody would wish their child to have such a disease. But most parents with whom I've spoken feel that the gratitude they have for knowing their baby outweighs even the burdens of developmental delay or early death.

DISCLAIMER: The information in this site is for educational purposes only. It should not be used as a substitute for personal care by a licensed physician. Please see your doctor for diagnosis and treatment of any concerning symptoms or medical condition.

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Article Sources
  • Elaine Wirrell, Katherine C. Nickels. Continuum: Epilepsy, Volume 16, Number 3, June 2010.
  • Gerald M Finichel. Clinical Pediatric Neurology. 6th Edition. Sanders-Elsevier, 2009.