ArticleAJNR. American journal of neuroradiology2018
Looking Deep into the Eye-of-the-Tiger in Pantothenate Kinase-Associated Neurodegeneration.
Article in AJNR. American journal of neuroradiology, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.
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Who cites it
12 citing papers in PubMed, 19 citations in OpenAlex.
- Article
- Therapeutic Advances in Major NBIA Disorders: Current Strategies and Translational Challenges.Neurology international · 2026Review
- Article
- 소아 신경영상에서 보이는 비선천성 원인의 영상 소견: 임상 화보.Journal of the Korean Society of Radiology · 2026Review
- Pathology and treatment methods in pantothenate kinase-associated neurodegeneration.Postepy psychiatrii neurologii · 2024Review
- Anterior pallidal hyperintensity mimicking the eye of the tiger sign in spastic paraplegia type 7.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2024Article
- Heterozygous Nonsense Variants in the Ferritin Heavy Chain GenemedRxiv : the preprint server for health sciences · 2023Article
- Pallidal degenerations and related disorders: an update.Journal of neural transmission (Vienna, Austria : 1996) · 2022Review
- Cerebral Iron Deposition in Neurodegeneration.Biomolecules · 2022Review
- Cerebral blood flow in dystonia due to pantothenate kinase-associated neurodegeneration.The neuroradiology journal · 2020Article
- Brain MRI Pattern Recognition in Neurodegeneration With Brain Iron Accumulation.Frontiers in neurology · 2020Review
- Novel mutations in KMT2B offer pathophysiological insights into childhood-onset progressive dystonia.Journal of human genetics · 2019Article
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Authors and funding
5 authors at 2 institutions in 5 countries.
Funding
Abstract
background and purposeA detailed delineation of the MR imaging changes in the globus pallidus in pantothenate kinase-associated neurodegeneration will be helpful for diagnosis and monitoring of patients. The aim of this study was to determine the morphologic spectrum of the "eye-of-the-tiger" sign and the topographic pattern of iron deposition in a group of patients with pantothenate kinase-associated neurodegeneration. MATERIALS AND
methodsSeventy-four MR imaging scans from 54 individuals with
resultsThe linear hyperintensity alongside the medial border of the globus pallidus was the earliest T2 signal change. This finding was evident before SWI changes from iron deposition became visible. T2WI performed in early childhood mostly showed isolated hyperintense signal. In adult patients, marked signal reduction within an earlier hyperintense center resulting from iron accumulation led to the loss of signal difference between the central and surrounding areas. Signal hypointensity on SWI progressed from the medial to the lateral portion of the globus pallidus with increasing age. The fiber connections between the medial globus pallidus and the anteromedial aspect of the substantia nigra and subthalamic nucleus were markedly hypointense on SWI.
conclusionsIn pantothenate kinase-associated neurodegeneration, the globus pallidus MR imaging changes using SWI develop as region-specific and age-dependent phenomena. Signal inhomogeneity was observed across the globus pallidus in pantothenate kinase-associated neurodegeneration and should be considered when determining the concentration of iron.
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