A. Członkowska

CL Chang

H Hayashi

K. Dziezyc

M. Gołębiowski

M. Karlinski

M. Skowrońska

N Kumar

P Dusek

SJA Bogaard Van den

T Ikeda

T Litwin

T. Litwin

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LETTER TO THE EDITORSMR image mimicking the ‘‘eye of the tiger’’ sign in Wilson’sdiseaseT. Litwin • M. Karlinski • M. Skowron´ska •K. Dziezyc • M. Gołe˛biowski • A. CzłonkowskaReceived: 14 December 2013 / Revised: 16 March 2014 / Accepted: 17 March 2014 / Published online: 26 March 2014 The Author(s) 2014. This article is published with open access at Springerlink.comDear Sirs,The eye of the tiger sign (EOT), seen on T2 sequences ofmagnetic resonance imaging (MRI), is considered patho-gnomonic for neurodegeneration with brain iron accumu-lation type 1 (NBIA1, previously known as Hallervorden–Spatz syndrome) [1–3]. This sign is described as low signalintensity in both globi pallidi (due to iron accumulation)that surrounds a central region of high signal intensity(caused by gliosis, edema, and neuronal loss with sub-sequent secondary vacuolization and cavitation) [1–3]. Ithas been occasionally reported in neurodegenerative dis-orders other than NBIA1, including neuroferritinopathy,progressive supranuclear palsy, multisystem atrophy, pureakinesia, and corticobasal degeneration [1–3]. Here, wedescribe the first case of EOT-like MRI appearance in a41-year-old man with Wilson’s disease.At the age of 24 years, the patient developed dysarthria,oromandibular dystonia, gait disturbances, and positionalhand tremor. One year later he was diagnosed with Wil-son’s disease, based on the presence of Kayser–Fleischerring and abnormal copper metabolism [i.e. decreasedserum ceruloplasmin (8.2 mg/dL, normal 25–45) andserum copper (30 lg/dL, normal 70–140 lg/dL) combinedwith increased copper urinary excretion (664 lg/24 h,normal 0–50)]. Genetic examination confirmed compoundheterozygosity, with ATP7B mutations in exon 13 (G988R)and in exon 7 (IVS7 ? 3A[G). After introduction of1000 mg/day d-penicillamine, the patient graduallyimproved. In 2013 his remaining symptoms were onlyslight dysarthria and oromandibular dystonia (occasionallygrimacing). The Kayser–Fleischer rings were no longerpresent. However, a routine control brain MRI revealedabnormalities very similar to EOT sign (Fig. 1).Fig. 1 The MR image mimicking the ‘‘eye of the tiger’’ sign in T2-weighted magnetic resonance sequences; bilateral hypointensity inboth putaminal regions and lateral parts of globi pallidi, with a centralhyperintensity (black arrows)T. Litwin (&)  M. Karlinski  M. Skowron´ska  K. Dziezyc A. CzłonkowskaSecond Department of Neurology, Institute of Psychiatry andNeurology, Sobieskiego 9, 02-957 Warsaw, Polande-mail: tomlit@medprakt.plM. Gołe˛biowskiDepartment of Radiology, Medical University, Warsaw, PolandA. CzłonkowskaDepartment of Experimental and Clinical Pharmacology,Medical University, Warsaw, Poland123J Neurol (2014) 261:1025–1027DOI 10.1007/s00415-014-7322-yThe difference was that hypointensity tended to localizein the lateral part of the globus pallidus and putamen insteadof the medial part of the globus pallidus. Subsequent MRspectroscopy showed that the mean N-acetyloaspartate/creatine ratio (TE-144mS) in the globus pallidus was 1.55,and the mean choline/creatine ratio (TE-144 mS) was 1.10(Fig. 2a). In the normal appearing control region, the valueswere 2.37 and 1.08, respectively (Fig. 2b), which mayreflect neuronal loss in the affected area. Noteworthy, thepatient has no co-morbidities, does not drink alcohol andhad no occupational risk factors for tissue metal accumu-lation. Normal levels of serum iron metabolism markers(ferritin, iron, total iron binding capacity) combined withthe early age of the disease onset, lack of anemia anddiabetes allowed to exclude aceruloplasminemia andneuroferritinopathy. The lack of acanthocytosis, pigmen-tary retinopathy, hypoprobetalipoproteinemia, and goodresponse to d-penicillamine with no subsequent progressionFig. 2 The magnetic brainspectroscopy from: a unaffectedstructures in the right temporallobe; b central, hyperintensivepart of ‘‘eye of the tiger’’ signshowing markedly decreased N-acetyloaspartate in the globuspallidus as a results of reactivegliosis1026 J Neurol (2014) 261:1025–1027123over 15 years allowed us to exclude NBIA1. The patient’sbrother was diagnosed with hepatic Wilson’s diseasewithout any neurological signs at 28 years of age.Wilson’s disease is a treatable, inherited disorder lead-ing to pathological copper accumulation that damagesaffected organs, especially the liver and brain. It has beensuggested that disturbances of iron metabolism occur in theliver of Wilson’s disease patients, as copper-dependentenzymes are also involved in its metabolic pathways [4]. Ina recent post-mortem study we demonstrated that ironaccumulates also in the brain [5]. As the EOT sign suggestsaccumulation of iron [2, 3, 6, 7], its increased concentrationin the basal ganglia of the reported patient appeared to beparticularly pronounced. However, the exact role of iron-metabolism disturbances in Wilson’s disease is stillunknown and further studies are needed. Considering thatpatients with EOT sign may occasionally have conditionsother than NBIA1, it is advisable to carry out careful dif-ferential diagnosis in order not to miss a condition that canbe effectively treated.Conflicts of interest The authors declare no conflict of interest. Allfinancial involvements (e.g., employment, consultancies, honoraria,stock ownership or options, grants or patents receiving or pending,royalties) with any organization or entity with a financial interest in orfinancial conflict with the subject matter or materials discussed in theSubmitted Publication material are completely disclosed. We have norelevant financial interest in the Submitted Publication material.Ethical standard Informed constent was obtained from the patient.Open Access This article is distributed under the terms of theCreative Commons Attribution License which permits any use, dis-tribution, and reproduction in any medium, provided the originalauthor(s) and the source are credited.References1. Chang CL, Lin CM (2011) Eye-of-the tiger sign is not pathogno-monic of pantothenate kinase-associated neurodegeneration inadult case. Brain Behav 1:55–562. Dusek P, Jankovic J, Le W (2012) Iron dysregulation in movementdisorders. Neurobiol Dis 46:1–183. Ikeda T, Matsuo Y, Ueda A, Hirano T (2013) Pseudo eye of thetiger sign in atypical Parkinsonism. Neurol Sci 34:777–7784. Hayashi H, Motoyoshi Y, Yoshikazu F, Wakusawa S (2006)Compound overload of copper and iron in patients with Wilson’sdisease. Med Mol Morphol 39:121–1265. Litwin T, Gromadzka G, Szpak GM, Jablonka-Salach E, Bulska A,Czlonkowska A (2013) Brain metal accumulation in Wilson’sdisease. J Neurol Sci 329:55–586. Van den Bogaard SJA, Kruit MC, Dumas E, Roos RAC (2014)Eye-of-the-tiger-sign in a 48 year healthy adult. J Neurol Sci336:254–2567. Kumar N, Boes JC, Babovic-Vuksanovic D, Boeve BF (2006) The‘‘eye-of-the-tiger’’ sign is not pathognomonic of the PANK2mutation. Arch Neurol 63:292J Neurol (2014) 261:1025–1027 1027123

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MR image mimicking the “eye of the tiger” sign in Wilson’s disease

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MR image mimicking the “eye of the tiger” sign in Wilson’s disease

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