Syndromes with neurodegeneration with brain iron accumulation (NBIA) are a group of
neurodegenerative disorders associated with abnormalities in brain iron metabolism and …

Introduction Pantothenate kinase-associated neurodegeneration (PKAN) is a rare
autosomal recessive genetic disease that leads to extrapyramidal symptoms, such as …

Background X-linked dystonia-parkinsonism (XDP) is an inherited neurodegenerative adult-
onset basal ganglia model disease associated with severe striatal atrophy. Anatomical …

Introduction Neurodegeneration with brain iron accumulation (NBIA) comprises a
heterogeneous group of disorders with accumulation of iron in the brain, mostly basal …

Background Pantothenate kinase (PANK) is the first and rate-controlling enzymatic step in
the only pathway for cellular coenzyme A (CoA) biosynthesis. PANK-associated …

BACKGROUND AND PURPOSE: A detailed delineation of the MR imaging changes in the
globus pallidus in pantothenate kinase–associated neurodegeneration will be helpful for …

Background To examine structural connectivity of white matter tracts in patients with
Pantothenate Kinase-Associated Neurodegeneration (PKAN) dystonia and identify those …

Purpose To demonstrate deviations of functional connectivity within the motor system in
dystonic patients suffering from Pantothenate Kinase Associated Neurodegeneration, a …

Objective To determine the volume changes in gray and white matter during a long-term
follow-up in patients suffering from pantothenate kinase-associated neurodegeneration …

In this chapter, we will discuss the contributions of structural and functional imaging to the
diagnosis and management of genetic and degenerative diseases that lead to the …