The Rasmussen Encephalitis (RE) Children's Project, a foundation that supports scientific research to find a cure for this devastating neurological disease, has donated $111,000 to researchers in the departments of neurosurgery and pathology/lab medicine (neuropathology) at Ronald Reagan UCLA Medical Center.
"We need to do more to accelerate our understanding of Rasmussen Encephalitis," said Seth H. Wohlberg, founder of the RE Children's Project, and whose teenage daughter suffers from the condition. "I am hopeful that by providing extra resources to UCLA's top notch team of talented and dedicated individuals, we can increase our knowledge of this disease and find a cure."
Rasmussen Encephalitis is a rare neurological disease that causes intractable seizures, cognitive deficits and paralysis of half of the body. RE typically affects previously normal children between the ages of two and ten years old. The disease process typically runs its course over a one to two year period during which time one half of the body is rendered useless and epileptic seizures continue unabated. An unusual feature of the disease is that it is usually confined to one hemisphere of the brain and is resistant to standard anti-seizure medicines. The only known "cure" is a surgical hemispherectomy — the removal or disconnection of the affected side of the brain. Recent progress in understanding of the disease, and the emergence of therapies that slow disease progression and help control symptoms, has led some researchers to believe that more targeted and effective medical treatments are potentially within reach.
The gift will be used to support the research of Dr. Gary Mathern, professor of pediatric neurosurgery and director of the UCLA Pediatric Epilepsy Program at Mattel Children's Hospital; Dr. Carol Kruse, professor of neurosurgery; and Dr. Harry Vinters, professor of neuropathology. The researchers' goal is to narrow down the cause(s) of the disease by identifying whether immune cells or infectious agents such as bacteria or virus play a major role. By developing an animal model using cells from diseased brain tissue from patients, they hope to determine what components of the human brain leads to a Rasmussen-like disease. This research involves the collaboration of experts in brain immunology and neuropathology working together to create a mouse model of RE.
In the summer of 2008, the Wohlberg's 10-year-daughter Grace started to experience epileptic seizures. After months of testing, her parents learned that she had the extremely rare neurological disorder. Grace underwent an initial hemispherectomy surgery in February 2009. However, her seizures recurred so her parents then brought Grace to UCLA to complete the hemispherectomy which was performed by Mathern in March 2010. Today, Grace is back in school readjusting to her new life with the assistance of a full-time aid. While the surgery has stopped the seizures, Grace faces lifelong disabilities that resulted from the surgery including partial blindness, cognitive issues and learning how to walk again.
Wohlberg and his wife, Deb, created the RE Children's Project in 2010. Since then, the foundation has sponsored cross-disciplinary research conferences and funded leading edge research around the globe focused on finding the cause and an eventual cure for RE. The organization also supports research dedicated toward the recovery process following hemispherectomy surgery.
"On behalf of the UCLA team, we thank the Wohlbergs for their confidence in our research proposal and hope to continue to partner with the RE Children's Project to solve this disorder and to help more children and their families," said Mathern.