Pantothenate Kinase-Associated neurodegeneration (Hallervorden–Spatz syndrome)

Research output: Chapter in Book/Report/Conference proceedingChapter


Specific Imaging Findings. In pantothenate kinase-associated neurodegeneration (PKAN, formerly known as Hallervorden–Spatz syndrome), MRI shows markedly hypointense globi pallidi on T2-weighted images, with a small hyperintense central or anteromedial area. This finding has been labelled the “eye-of-the-tiger” sign and is highly characteristic of PKAN; it is visible on both axial and coronal images. Gradient-echo T2*-weighted images show more profound hypointensity owing to paramagnetic effects. T1-weighted images may show a corresponding high signal intensity of the pallida. There is no contrast enhancement. CT may reveal symmmetrically increased attenuation, primarily in the anteromedial globus pallidus. Pertinent Clinical Information. This rare autosomal recessive disorder is a part of a group of diseases called “neurodegeneration with brain iron accumulation” (NBIA) which also includes aceruloplasminemia and neuroferritinopathy. PKAN typically presents in older children or adolescents with oromandibular dystonia, mental deterioration, pyramidal signs, and retinal degeneration. Most patients die within 10 years of the clinical onset, although longer survival into early adulthood is possible. Differential Diagnosis. HARP Syndrome (hypopre-β-lipoproteinemia, acanthocytosis, retinitis pigmentosa, and pallidal degeneration. • may be indistinguishable. Other Forms of NBIA. • “eye-of-the-tiger” sign absent. Toxic Encephalopathies (CO poisoning) (3)• globus pallidus T2 hyperintensity without hypointense portion Kernicterus • globus pallidus T2 hyperintensity without hypointense portion. Methylmalonic Acidemia. • globus pallidus T2 hyperintensity without hypointense portion. Normal Iron Deposition. • iron starts accumulating in the pallidi during later childhood and adolescence and is usually seen on MRI from approximately 25 years of age onwards

Original languageEnglish
Title of host publicationBrain Imaging with MRI and CT: An Image Pattern Approach
PublisherCambridge University Press
Number of pages2
ISBN (Print)9781139030854, 9780521119443
Publication statusPublished - Jan 1 2010

ASJC Scopus subject areas

  • Medicine(all)


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