International Journal of Research in Medical Sciences | January-March 2014 | Vol 2 | Issue 1 Page 370 International Journal of Research in Medical Sciences Mondal S et al. Int J Res Med Sci. 2014 Feb;2(1):370-372 www.msjonline.org pISSN 2320-6071 | eISSN 2320-6012 Case Report Eye of the tiger sign in neurodegeneration with brain iron accumulation: a case report Sumantro Mondal 1 *, Debasish Dey 2 , Tony Ete 1 , Debanjali Sinha 1 , Arijit Nag 1 , Atanu Chakraborty 1 , Amod Prasad 3 INTRODUCTION The classic form of NBIA is manifested as extra pyramidal symptoms and retinal degeneration. This condition encompasses a variety of disorders. The clinical phenotype of Wilson disease, neuroacanthocytosis shares some similarity with NBIA and offers diagnostic challenges to the physician. MRI of brain is particularly helpful in this scenario to differentiate NBIA from its mimickers. Our patients presented with early onset progressive extra pyramidal features and final diagnosis of NBIA was secured from characterized MRI findings. CASE REPORT A nine years old boy presented with intermittent abnormal flexor posturing of face, both upper limbs and involuntary jerky movements along with increased stiffness of all four limbs for last eight months. The symptoms hampered his walking and other daily normal activities. The disease course was progressive. He also had mild coarse tremor. Abnormal posturing, movements and tremor were relieved during sleep. There was no history of fever, convulsion, and jaundice preceding or during his current illness. His birth history was uneventful and his elder brother was having normal health. Examination showed normal higher mental functions, increased tone in all four limbs, exaggerated deep tendon reflexes with bilateral extensor planter response. Coarse tremor of limbs was present at rest. Sensory, cerebellar and bladder bowel functions were unremarkable. Examinations of the other systems were normal. Ophthalmoscopic examination was normal. Routine blood investigations, liver and renal function tests were normal. Serum ceruloplasmin and ferritin level were within normal limits with a normal 24 hour urinary copper excretion value (48 microgram/day). Peripheral blood smear did not reveal any acanthocyte. Slit lamp examination of eye was negative for Kayser-Fleischer ring. Magnetic resonance imaging (MRI) of brain revealed marked hypo intensity in bilateral globus pallidus surrounding an area of hyperintensity at its ABSTRACT Neurodegeneration with brain iron accumulation (NBIA) is a rare autosomal recessive disorder characterized by abnormal accumulation of ferritin in globus pallidus of brain. Magnetic resonance imaging (MRI) of brain demonstrates a characteristic ‘eye-of-the-tiger’ sign. We describe a case of NBIA in a child with classical clinical and MRI of brain features. Keywords: Neurodegeneration with brain iron accumulation, MRI, Eye of the tiger 1 Department of Medicine, Institute of Post Graduate Medical Education and Research, Kolkata, West Bengal, India 2 Department of Radiology, Bangur Institute of Neurosciences, Kolkata, West Bengal, India 3 Department of Medicine, R. G. Kar Medical College and Hospital, Kolkata, West Bengal, India Received: 23 December 2013 Accepted: 7 January 2014 *Correspondence: Dr. Sumantro Mondal, E-mail: drmsumantro@gmail.com © 2014 Mondal S et al. This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. DOI: 10.5455/2320-6012.ijrms20140277