The UTHealth Center of Excellence for West Syndrome Research and the Geissler Distinguished Chair in West Syndrome Research were recently established after receiving a gift of $1 million on behalf of the West Syndrome Foundation. The Center of Excellence will be located at The University of Texas Health Science Center at Houston.
Causes, diagnosis and treatment of infantile spasms will be the main research objective of the new Center. Promoting awareness and education about the disease will be focused upon as well.
Ian Butler, M.D., professor and director of the Division of Child and Adolescent Neurology in the Department of Pediatrics at the UTHealth Medical School, says that he and his colleagues “are excited to be given the opportunity to establish a center devoted to clinical care and research for patients with infantile spasms.” He adds that the Center “will have an important role in increasing our understanding of this condition,” beside “exemplary clinical management.”
Ruth Brewer, co-founder of the West Syndrome Foundation, after telling how “empathetic and patient” Dr. Ian Butler was, when Brewer’s son was diagnosed in 2003 concluded: “Where else would we go but right back to the only other person I know who understands the importance and the impact,” of this problem.
West Syndrome was identified in 1841, after surgeon W.J. West wrote a letter describing the convulsions of his own child. At that time, Mr. West described the syndrome as “a very rare and singular species of convulsions peculiar to young children.”
Nowadays, medical practitioners refer to West Syndrome as a syndrome of the nervous system, which leads to infantile spasms resulting in flexion jerks of the body. The symptoms usually appear between the third and the twelfth month, mostly between the fourth and the eighth months.
These clusters of spasms are characterized by Dr. Butler as “a hurricane that comes in and stirs everything up and goes away,” resulting in significant “damage.”
Children’s Memorial Hermann Hospital has developed diagnostic tests for the syndrome consisting of a comprehensive metabolic workup, spinal tap ,and high-resolution imaging, allowing for a visualization of structural defects. For example, neurologists will try to find specific electroencephalograpyl patterns, a type of dysfunctional brain wave. Additionally, physicians also look for an intrauterine infection, hypoxia, or perinatal trauma, which may also lead to the syndrome.
The treatments used for taking care of these symptoms often involve taking vitamins to correct eventual metabolic deficiencies, antiepileptic medicines, corticosteroids and, when facing neurological consequences, surgical intervention.
An early diagnosis of the syndrome helps to curtail “the developmental decline,” says Gretchen Von Allmen, co-director of the Center and assistant professor of pediatric neurology at the UTHealth Medical School and a member of the Epilepsy Phenome/Genome Project established by the National Institute of Neurologic Disorders and Stroke. Von Allmen is optimistic that “having a place” specialized “in the cause, diagnosis and treatment of West Syndrome,” may give a great improvement in the results.
Dr. Ian Butler has great faith in “the newest research in genetics” because he believes that understanding “the pathway” may eventually be used to treat the syndrome.