title: The "eye of the tiger" sign in pure akinesia with gait freezing.
abstract: The "eye of the tiger" is a neuroradiologic sign due to iron deposition in the globus pallidus: it appears as diffuse low signal intensity with a central area of high signal intensity confined to the globus pallidus. The "eye of the tiger" sign has been associated with neurodegeneration with brain iron accumulation type 1 (NBIA1), a condition caused by mutations in the gene encoding pantothenate kinase 2 (PANK2). However, the specificity of this neuroradiologic sign has been already challenged and it has been described in other neurodegenerative diseases. Here, we report the first case of a patient suffering from pure akinesia with gait freezing with the "eye of the tiger" sign in T2-weighted MRI sequences. All clinical, laboratory and radiologic data excluded other diagnosis and genetic testing excluded PANK2 mutations suggesting that the "eye of the tiger" is not specific for NBIA1 and may also occur in other movement disorders.
authors.affiliation: Department of Neurological Sciences, University of Naples Federico II, Naples, Italy.
authors.forename: Roberto
authors.initials: R
authors.lastname: Erro
authors.forename: Marianna
authors.initials: M
authors.lastname: Amboni
authors.forename: Carmine
authors.initials: C
authors.lastname: Vitale
authors.forename: Katia
authors.initials: K
authors.lastname: Longo
authors.forename: Mariangela
authors.initials: M
authors.lastname: Rocco
authors.forename: Carmela
authors.initials: C
authors.lastname: Russo
authors.forename: Sabina
authors.initials: S
authors.lastname: Pappatà
authors.forename: Arturo
authors.initials: A
authors.lastname: Brunetti
authors.forename: Paolo
authors.initials: P
authors.lastname: Barone
mesh_terms: D001921:Brain / Q000473:pathology*; D020820:Dyskinesias / Q000473:pathology*; D019788:Fluorodeoxyglucose F18; D020233:Gait Disorders, Neurologic / Q000473:pathology*; D005917:Globus Pallidus / Q000473:pathology; D008279:Magnetic Resonance Imaging; D049268:Positron-Emission Tomography; D019275:Radiopharmaceuticals
chemical_list: D019275:Radiopharmaceuticals; D019788:Fluorodeoxyglucose F18