Go Back Brain/18. More than one of above/Metabolic, Endocrine, Toxic/18.5 - PKAN - Neurodegeneration with brain iron accumulation (NBIA)/ Go to Index

18 yom presenting with facial and mouth dystonia with progressive involvement.

PKAN is a form of NBIA - neurodegeneration with brain iron accumulation which was formerly known as Hallervorden-Spatz Disease.

NBIA is an eclectic group of diseases with neurodegeneration and basal ganglia iron accumulation.

PKAN (Pantothenate Kinase-associated neurodegeneration) is the most common of these diseases and presents with the "eye of the tiger" sign which is a central bright area within the iron accumulation.

PKAN has two forms - early and late onset. Both appear similar on MRI.

This patient has late onset disease which usually presents prior to age 20.