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

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

Ivan Donaldson

, C. David Marsden

, Susanne A. Schneider

, and Kailash P. Bhatia

DOI:
10.1093/med/9780192619112.003.0472
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date: 22 October 2020

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.

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