title: Eye-of-the-tiger-sign in a 48 year healthy adult.
abstract: We report a healthy adult male, who underwent, as a control subject, part of a Huntington's disease study, extensive testing during three visits in a two year follow-up, including clinical examination and 3.0 T MRI scans. The T2-weighted MRI sequences revealed the "eye-of-the-tiger-sign". No clinical abnormalities in either motor, cognitive or behavioural domains were observed. PKAN2 and FTL gene mutation analysis were negative. This finding implies that an eye-of-the-tiger sign, which is considered a pathognomonic feature of neurodegeneration with brain iron accumulation (NBIA), can occur without any clinical symptoms.
authors.affiliation: Department of Neurology, Leiden University Medical Centre, Leiden, The Netherlands. Electronic address: s.j.a.van_den_bogaard@lumc.nl.
authors.forename: Simon J A
authors.initials: SJ
authors.lastname: van den Bogaard
authors.affiliation: Department of Radiology, Leiden University Medical Centre, Leiden, The Netherlands.
authors.forename: Mark C
authors.initials: MC
authors.lastname: Kruit
authors.affiliation: Department of Neurology, Leiden University Medical Centre, Leiden, The Netherlands.
authors.forename: Eve M
authors.initials: EM
authors.lastname: Dumas
authors.affiliation: Department of Neurology, Leiden University Medical Centre, Leiden, The Netherlands.
authors.forename: Raymund A C
authors.initials: RA
authors.lastname: Roos
mesh_terms: D005917:Globus Pallidus / Q000473:pathology*; D006305:Health Status Indicators; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged
keywords: Eye-of-the-tiger sign; Iron; Movement disorder; NBIA; Neuroferritinopathy; PKAN