01/19/11 Portland, Ore.
OHSU Doernbecher is recognized as national leader in diagnosis, treatment, research of this rare, currently fatal neurodegenerative disease
Oregon Health & Science University Doernbecher Children’s Hospital has been named a Batten Disease Center of Excellence by North America’s largest support and research organization for children diagnosed with a rare neurological disorder called neuronal ceroid lipofuscinosis (NCL), commonly referred to as Batten disease. OHSU Doernbecher now is one of four centers nationwide recognized by the Batten Disease Support and Research Association (BDSRA).
Batten disease is caused by genetic mutations, and children who inherit the defective gene are unable to produce enough of an enzyme, or protein, to prevent cellular waste build-up in the brain, which eventually causes the cells to cease functioning and die. Children with Batten disease appear healthy at birth, but as their brain cells die, they begin to suffer seizures and progressively lose motor skills, sight and mental capacity. Eventually, they become blind, bedridden and unable to communicate or function on their own.
The center of excellence at OHSU Doernbecher will provide a comprehensive multidisciplinary approach to children with Batten disease. Families and patients will experience a well-coordinated, organized team approach that will meet the specific needs of each child.
The complete OHSU media release is available online.