On April 1st, 2014 we began the process of opening our brand new West Syndrome Center of Excellence. Due to the very generous donation by the West Syndrome Foundation we are able to have a comprehensive center where patients from all over the world can come and receive care for West Syndrome and infantile spasms.
Gift of $1 million establishes Center of Excellence for West Syndrome Research which is expected to open this Spring.
The Center will focus on searching for the causes of and developing new treatments for West Syndrome and related pediatric epilepsy disorders, as well as increasing awareness and disseminating education about the disease.
Ruth Brewer, co-founder of the West Syndrome Foundation, said of the Foundation’s decision to establish the Center at UTHealth. “Where else would we go but right back to the only other person I know who understands the importance and the impact such a Center could have not only on children diagnosed with West Syndrome, but children with an incredible variety of profound neurological disorders.”
West Syndrome, a disorder of the developing nervous system, is characterized by infantile spasms that result in clusters of flexion jerks of the body. The jerks generally last only one to two seconds and can be as mild as slight bobs of the head, which parents might mistake for simple startle reflexes. The onset occurs typically when the child is 4 to 8 months old. “These clusters of spasms are like a hurricane that comes in and stirs everything up and goes away, but leaves damage,” said Butler, the Adriana Blood Professor of Pediatrics and co-director of the Center.
Diagnostic testing at Children’s Memorial Hermann Hospital includes a detailed metabolic workup, spinal tap and high-resolution imaging to look for structural defects. In particular, neurologists will look for a specific pattern on electroencephalograpy (EEG) called hypsarrhythmia, or chaotic brain waves. Physicians will also ask whether there was an intrauterine infection, perinatal trauma or hypoxia, because these conditions may also result in the syndrome. Children diagnosed with tuberous sclerosis often suffer from infantile spasms.
“The sooner the diagnostic workup is done, the sooner treatment is started and the sooner we can stop the developmental decline,” said Gretchen Von Allmen, M.D., co-director of the Center and assistant professor of pediatric neurology at the McGovern Medical School. “We are hoping that having a place that specializes in the cause, diagnosis and treatment of West Syndrome will improve outcomes.” Von Allmen is chief of pediatric epilepsy for the Texas Comprehensive Epilepsy Program at the Mischer Neuroscience Institute and medical director of the Children’s Memorial Hermann Hospital Pediatric Epilepsy Monitoring Unit.