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.
Similar content being viewed by others
References
Hayflick SJ, Westaway SK, Levinson B, Zhou B, Johnson MA, Ching KH, Gitschier J (2003) Genetic, clinical and radiographic delineation of Hallervorde-Spatz syndrome. N Engl J Med 348(1):33–40
McNeill A, Birchall D, Hayflick SJ, Gregory A, Schenk JF, Zimmerman EA, Shang H, Miyajima H, Chinnery PF (2008) T2* and FSE MRI distinguishes four subtypes of neurodegeneration with brain iron accumulation. Neurology 70(18):1614–1619
Kumar N, Boes CJ, Bobovic-Vuksanovic D, Boeve BF (2006) The “eye-of-the-tiger” sign is not pathognomonic of the PANK2 mutation. Arch Neurol 63(2):292–293
Molinuevo JL, Munoz E, Valldeoriola F, Tolosa E (1999) The eye of the tiger sign in cortical-basal ganglionic degeneration. Mov Disord 14:169–171
Strecker K, Hesse S, Sabri O, Schwarz J, Schneider JP (2007) Eye of tiger in multiple system atrophy. Eur J Neur 14:e1–e2
Davie CA, Baker GJ, Machado C, Miller DH, Lees AJ (1997) Proton magnetic spectroscopy in Steele Richardson Olszewski syndrome. Mov Disord 12:767–771
Quattrone A, Nicoletti G, Messina D, Fera F, Condino F, Pugliese P, Lanza P, Barone P, Morgante L, Zappia M, Aguglia U, Gallo O et al (2008) MR imaging index for differentiation of progressive supranuclear palsy from Parkinson disease and Parkinson variant of multiple system atrophy. Radiology 246:214–221
Williams DR, Holton JL, Revesz T, Lees AJ (2007) Pure akinesia with gait freezing: a third clinical phenotype of progressive supranuclear palsy. Mov Disord 22(15):2235–2241
Stewart A, Factor DO (2008) The clinical spectrum of FOG in atypical Parkinsonism. Mov Disord 23(suppl 2):S431–S438
Park HK, Kim JS, Im KC, Oh SJ, Kim MJ, Lee JH, Chung SJ, Lee MC (2009) Functional brain imaging in pure akinesia with gait freezing: 18F-FDG PET and 18F-FP-CIT PET analyses. Mov Disord 24:237–245
Molinuevo JL, Martí MJ, Blesa R, Tolosa E (2003) Pure akinesia: an unusual phenotype of Hallervorden-Spatz syndrome. Mov Disord 18(11):1351–1353
Acknowledgment
We thank Jean Ann Gilder, Scientific Communication srl, for text editing.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Erro, R., Amboni, M., Vitale, C. et al. The “eye of the tiger” sign in pure akinesia with gait freezing. Neurol Sci 32, 703–705 (2011). https://doi.org/10.1007/s10072-011-0589-1
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10072-011-0589-1