Clinical Review & Education Images in Neurology Hemidystonia With One Eye-of-the-Tiger Sign Sanjay Pandey, MD, DM; Debra L. Byler, MD; Mark Hallett, MD A woman in her late teens presented with dystonic posturing of her left hand for 2 years and left foot for 3 months. Dystonic posturing of her hand had started when she was 8 months pregnant, and it slowly worsened to involve her left lower limb, leading to difficulty in walking (Video). She was evaluated for left hemidystoVideo at jamaneurology.com nia. Fluid-attenuated inversion recovery (FLAIR) magnetic resonance images (MRIs) of the brain gave the appearance of the eyeof-the-tiger sign in the right basal ganglia similar to what would be seen with a neuroferritinopathy, but susceptibility-weighted imaging revealed evidence of hemosiderin indicative of an old hemorrhagic infarct (Figure). The results of magnetic resonance angiography for evaluation of intracranial vessels were normal. Mutation analysis for pantothenate kinase 2 was negative. Antinuclear antibody and anti–double-stranded DNA were negative. Figure. Magnetic Resonance Images (MRIs) of the Patient FLAIR MRI B A Susceptibility-weighted imaging Discussion The woman likely had a pregnancy-related stroke leading to hemidystonia. Poststroke hemidystonia may progress over time. The time course for the appearance of movement disorders after a stroke may vary from the day of onset of the stroke to many years. The duration also depends on the type of movement disorder, which is short for chorea and long for dystonia.1 The neuroimaging finding observed on FLAIR MRI in our patient was suggestive of the eye-of-the-tiger sign on one side. This sign is characterized by low signal-intensity rings, which surround the central high signalintensity region on the anteromedial aspect of the globus pallidus on FLAIR and T2-weighted MRI sequences. The central hyperintensity represents gliosis, and the surrounding hypointensity is due to excess iron accumulation. The eye-of-the-tiger sign has been considered to be specific for neuroferritinopathy but has also been reported2 in multiple system atrophy, pure akinesia, corticobasal degeneration, and organophosphate poisoning. Pseudo eyeof-the-tiger sign has been reported3 in atypical parkinsonism with a T2-weighted MRI showing no iron accumulation. To our knowledge, unilateral eye-of-the-tiger sign has not been previously described in the literature. An eye-of-the-tiger sign should not ARTICLE INFORMATION Author Affiliations: Human Motor Control Section, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland (Pandey, Hallett); Department of Neurology, Govind Ballabh Pant Hospital, New Delhi, India (Pandey); Department of Neurology, Penn State College of Medicine, Hershey, Pennsylvania (Byler); Department of Pediatrics, Penn State College of Medicine, Hershey, Pennsylvania (Byler). Corresponding Author: Mark Hallett, MD, Human Motor Control Section, National Institute of 1574 C D Fluid-attenuated inversion recovery (FLAIR) MRI (A and B) gave the appearance of the eye-of-the-tiger sign in the right basal ganglia, but susceptibilityweighted imaging (C and D) demonstrated an old hemorrhagic infarct in the right putamen and caudate region. be interpreted in isolation, and further investigations may be required before a final diagnosis is determined. Published Online: October 27, 2014. doi:10.1001/jamaneurol.2014.1077. Acquisition, analysis, or interpretation of data: All authors. Drafting of the manuscript: Pandey. Critical revision of the manuscript for important intellectual content: All authors. Administrative, technical, or material support: Pandey, Hallett. Study supervision: Hallett. Author Contributions: Dr Hallett had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis. Study concept and design: Pandey. Conflict of Interest Disclosures: Dr Pandey was supported by a fellowship from the Indo-US Science Technology Forum. Dr Hallett serves as chair of the Medical Advisory Board for and receives honoraria and funding for travel from the Neurotoxin Neurological Disorders and Stroke, National Institutes of Health, 10 Center Dr, MSC 1428, Bldg 10, Room 7D37, Bethesda, MD 20892 (hallettm @ninds.nih.gov). Accepted for Publication: April 8, 2014. JAMA Neurology December 2014 Volume 71, Number 12 Copyright 2014 American Medical Association. All rights reserved. Downloaded From: http://archneur.jamanetwork.com/ by a New York University User on 05/22/2015 jamaneurology.com Images in Neurology Clinical Review & Education Institute. He may accrue revenue on patents for Immunotoxin for the treatment of focal movement disorders and for a coil for magnetic stimulation (ie, H-coil), and he has received license fee payments from the National Institutes of Health (NIH) for licensing of the H-coil patent by Brainsway. He is a member of the editorial boards of 20 journals and has received royalties from publishing. He has received honoraria for lecturing from Columbia University and the Parkinson and Aging Research Foundation. Dr Hallett’s research at the NIH is largely supported by the NIH Intramural Program. Supplemental research funds came from the Kinetics Foundation for studies of instrumental methods to monitor Parkinson disease and from BCN Peptides, S.A., for treatment studies of blepharospasm. No other disclosures were reported. Funding/Support: The work was supported by the National Institute of Neurological Disorders and Stroke (NINDS) Intramural Program. Role of the Funder/Sponsor: The NINDS Intramural Program had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication. jamaneurology.com REFERENCES 1. Siniscalchi A, Gallelli L, Labate A, Malferrari G, Palleria C, Sarro GD. Post-stroke movement disorders: clinical manifestations and pharmacological management. Curr Neuropharmacol. 2012;10(3):254-262. 2. Chang CL, Lin CM. Eye-of-the-tiger sign is not pathognomonic of pantothenate kinase–associated neurodegeneration in adult cases. Brain Behav. 2011;1(1):55-56. 3. Ikeda T, Matsuo Y, Ueda A, Hirano T. Pseudo eye of the tiger sign in atypical parkinsonism. Neurol Sci. 2013;34(5):777-778. JAMA Neurology December 2014 Volume 71, Number 12 Copyright 2014 American Medical Association. All rights reserved. Downloaded From: http://archneur.jamanetwork.com/ by a New York University User on 05/22/2015 1575