Chapter

Pantothenate kinase-associated neurodegeneration (PKAN), previously also known as Hallervorden–Spatz disease and related disorders of neurodegeneration with brain iron accumulation

Ivan Donaldson, C. David Marsden, Susanne A. Schneider and Kailash P. Bhatia

in Marsden's Book of Movement Disorders

Published on behalf of Oxford University Press

ISBN: 9780192619112
Published online January 2012 | e-ISBN: 9780199698103 | DOI: http://dx.doi.org/10.1093/med/9780192619112.003.0472
Pantothenate kinase-associated neurodegeneration (PKAN), previously also known as Hallervorden–Spatz disease and related disorders of neurodegeneration with brain iron accumulation

Show Summary Details

Preview

In this chapter we will discuss the syndromes of Neurodegeneration with Brain Iron Accumulation (NBIAs). We will focus on the most common form Pantothenate Kinase-Associated Neurodegeneration (PKAN), previously also known as Hallervorden–Spatz disease. In line with the other chapters, we will begin with historical remarks, followed by pathological and clinical aspects. In the latter part of the chapter, other NBIA disorders will be discussed. Similar to other disorders it is difficult to say in how far cases reported in the literature in the pre-genetic era may have belonged to one or the other group. Because of this we use the terms, the original term Hallervorden-Spatz disease (HSD) and more recent term PKAN.

Chapter.  13246 words.  Illustrated.

Subjects: Neurology

Full text: subscription required

How to subscribe Recommend to my Librarian

Buy this work at Oxford University Press »

Users without a subscription are not able to see the full content. Please, subscribe or login to access all content.