Department of Neurology

West syndrome and complex partial seizures

Q: Hi...

I was wondering if you could send us some info on these two topics. My god-daughter was diagnosed with West's Syndrome last year and we were told yesterday that they have "become" partial complex seizures. She is in her second week of treatment on 150mg a day of tregretol. ACTH was used in the past, and while it was successful in temporarily stopping the seizures, she has recently begun to have them again. We have exhausted our libraries in search for material, and the hospital and staff don't have much information to provide us with.

Thank you for your attention in this matter.
Hamilton, Ontario
Canada


A: West syndrome is an age-specific epilepsy that occurs during the first years of life. It consists of 1) spasms, that result in sudden flexing of the body or thrusting of the arms and legs 2) a specific EEG (brain wave) pattern called hypsarrhytmia and 3) developmental problems. It is often associated with an underlying brain problem, although in some cases no specific problems can be found. They are often sensitive to ACTH, which is a hormone secreted by the brain. The role of ACTH in supressing these seizures is not known; its usual function is to raise cortisol (like a natural cortisone) in the body, but it's anti-seizure effects are probably not due to this, since administration of cortisone-like drugs are not as effective. One scientist (Dr. Baram, Univ of Cal Irvine) has recently hypothesized that ACTH may reduce the production of another brain hormone that could injure the brain.

As the child with West Syndrome becomes older, the seizures change into a different form. Complex partial seizures mean that the child has seizures that seem to start in one part of the brain and then cloud consciousness. This is one of the most common seizure types, and the affected person will often have staring spells, with semi- or non-purposeful movements.

For more information I would suggest checking a library at a hospital or health-related school. The Epilepsy Foundation of America also has information that may be obtained. (www.efa.org) I do not know the address of the epilepsy association in Canada, but I presume there is one.

Best wishes

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