Home > Encyclopedia of Neurological Disorders > Pantothenate Kinase-associated Neurodegeneration

Definition

Pantothenate kinase-associated neurodegeneration (PKAN), long known as Hallervorden-Spatz syndrome (HSS), is a very rare childhood neurodegenerative disorder that is associated with the accumulation of iron in the brain, which causes progressively worsening abnormal movements and dementia.

Description

In addition to its original name, Hallervorden-Spatz syndrome, pantothenate kinase-associated neurodegeneration has also been called neurodegeneration with brain iron accumulation (NBIA). The name Hallervorden-Spatz is rapidly being discontinued by those who study and treat the disease, both because the new names indicate the nature of the underlying disorder, and because Julius Hallervorden, who described the syndrome, was involved in a "selective euthanasia" program in Nazi Germany to kill retarded children.