The “eye-of-the-tiger” sign refers to a specific pattern of hyperintensity within a hypointense globus pallidus that is seen on T2-weighted magnetic resonance (MR) imaging. The central hyperintensity is possibly due to tissue necrosis and edema, and the surrounding hypointensity, due to iron deposition. This appearance has been considered to be pathognomonic of Hallervorden-Spatz syndrome.1 The proposed new nomenclature suggests that the group of disorders with high iron in the basal ganglia be called neurodegeneration with brain iron accumulation (NBIA).2 The most prevalent form of NBIA is due to mutations in the pantothenate kinase 2 (PANK2) gene (chromosomal locus 20p13-p12.3) and is called pantothenate kinase–associated neurodegeneration (PKAN). Within this group the recognized forms include an early-onset, rapidly progressive, extrapyramidal syndrome often associated with pigmentary retinopathy; a later-onset syndrome with prominent behavioral difficulties and slower progression (atypical PKAN); and HARP (hypoprebetalipoproteinemia, acanthocytosis, retinitis pigmentosa, and pallidal degeneration). Neuroferritinopathy and aceruloplasminemia are 2 disorders without the PANK2 mutation that are part of the NBIA spectrum. We herein describe 2 patients with a clinical picture of atypical PKAN who had the eye-of-the tiger sign on MR imaging but lacked the PANK2 mutation.
Axial T2-weighted (A and D), axial proton density (B and E), coronal fluid-attenuated inversion recovery (C), and coronal T2-weighted (F) magnetic resonance images in patients 1 (A-C) and 2 (D-F) showing the “eye-of-the-tiger” sign. A-C, Patient 1 shows the increased central signal in the globus pallidus with surrounding hypointensity (arrows). D-F, Patient 2 shows a similar hypointensity with a subtly increased central signal (arrows). Patient 2 also shows generalized cerebral atrophy.
Thank you for submitting a comment on this article. It will be reviewed by JAMA Neurology editors. You will be notified when your comment has been published. Comments should not exceed 500 words of text and 10 references.
Do not submit personal medical questions or information that could identify a specific patient, questions about a particular case, or general inquiries to an author. Only content that has not been published, posted, or submitted elsewhere should be submitted. By submitting this Comment, you and any coauthors transfer copyright to the journal if your Comment is posted.
* = Required Field
Disclosure of Any Conflicts of Interest*
Indicate all relevant conflicts of interest of each author below, including all relevant financial interests, activities, and relationships within the past 3 years including, but not limited to, employment, affiliation, grants or funding, consultancies, honoraria or payment, speakers’ bureaus, stock ownership or options, expert testimony, royalties, donation of medical equipment, or patents planned, pending, or issued. If all authors have none, check "No potential conflicts or relevant financial interests" in the box below. Please also indicate any funding received in support of this work. The information will be posted with your response.
Some tools below are only available to our subscribers or users with an online account.
Download citation file:
Web of Science® Times Cited: 18
Customize your page view by dragging & repositioning the boxes below.
More Listings atJAMACareerCenter.com >
Enter your username and email address. We'll send you a link to reset your password.
Enter your username and email address. We'll send instructions on how to reset your password to the email address we have on record.
Athens and Shibboleth are access management services that provide single sign-on to protected resources. They replace the multiple user names and passwords necessary to access subscription-based content with a single user name and password that can be entered once per session. It operates independently of a user's location or IP address. If your institution uses Athens or Shibboleth authentication, please contact your site administrator to receive your user name and password.