Definition

Alpers' disease is an early-onset, progressive neurological degenerative disease that severely affects the brain and liver. In the familial (inherited) form of the disorder, it is transmitted as a recessive condition, which means that parents are unaffected, but both are carriers. Carrier parents have a 25% risk of having their biological child affected with Alpers' disease.

Description

Alpers' disease was first described by the late neurologist Alfons Maria Jakob (1884–1931). The disease was characterized and published by Bernard Jacob Alpers, Erna Christensen, and Knud Haraldsen Krabbe; thus, Alpers' disease is also known as Christensen's disease or Christensen-Krabbe disease. Additionally, the disease is known as progressive sclerosing poliodystrophy. Alpers' disease afflicts children and is eventually fatal....