meaning of Alexander disease

Alexander disease meaning in Medical Dictionary

A slowly modern and in the end fatal brain disorder that a lot of frequently takes place in children. The infantile as a type of the condition is described as megalencephaly (an abnormally large head), seizures, spasticity and developmental retardation. It causes death usually inside the first ten years. Patients because of the juvenile and person forms of Alexander infection usually experience ataxia and spasticity and a far more slowly modern training course. The classic hallmark of all of the forms of Alexander disease is the presence of Rosenthal materials, abnormal inclusions in astrocytes that have the advanced filament protein GFAP. Mutations when you look at the gene for GFAP (glial fibrillary acid protein) cause Alexander condition, 1st recognized example of a primary genetic condition of astrocytes, one of several major cell types when you look at the vertebrate CNS. The disease was initially described by W. Stewart Alexander, a fresh Zealand pathologist, in 1949.