Eye Of The Tiger Sign Mri Brain

10 initial claims of perfect sensitivity and specificity of this sign for the pank2 mutation have recently been challenged with the description of a disappearing eye of the tiger in a. A genetic panel for nbia specifically pkan was ordered for this patient including pantothenate kinase 2 pank2 the genetic marker for pantothenate kinase enzyme. This mri finding is not seen in other forms of nbia.

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The Eye Of The Tiger Sign Refers To Abnormal Low T2 Signal On Mri

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He Eye Of The Tiger Sign Refers To Abnormal Low T2 Signal On Mri

Pantothenate Kinase Associated Neurodegeneration Radiology

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This sign describes characteristic low signal surrounding a central region of high signal in the anteromedial globus pallidus on t2.

Eye of the tiger sign mri brain. The eye of the tiger sign is most classically associated with pantothenate kinase associated. On t2 weighted mri virtually all cases with pank2 mutations described to date are said to have the eye of the tiger sign reflecting a central area of hyperintensity possibly due to tissue necrosis and oedema within the medial globus pallidus which is hypointense due to iron deposition fig 3. Brain mris show an eye of the tiger sign with a specific t 2 weighted pattern of hyperintensity within the medial globus pallidus and the substantia nigra pars reticulata. For instance in one study 100 of the examined pkan cases had an eye of the tiger sign on brain mri.

The eye of the tiger sign refers to abnormal low t2 signal on mri due to abnormal accumulation of iron in the globus pallidus with a longitudinal stripe of high signal due to gliosis and spongiosis. It is low signal intensity rings surrounding the central high signal intensity regions in the medial aspect of bilateral globus pallidus on t2 weighted mri fig. Neuroferritinopathy a brain iron accumulation and neurodegenerative disorder. Pkan is commonly associated with the eye of the tiger sign.

Sethi et al 2 described the eye of the tiger sign in 1988 in 2 patients with what was then called hallervorden spatz syndrome now appropriately named pantothenate kinase associated neurodegeneration or neurodegeneration with brain iron accumulation type 1. Eye of the tiger sign in a 48year healthy adult journal of the neurological sciences vol. Eye of the tiger sign is seen in hallervorden spatz syndrome also known as pantothenate kinase associated neurodegeneration pkan. Pkan is typically suspected when the characteristic brain mri finding called the eye of the tiger sign which is a dark area indicating accumulation of iron with a bright spot in the center is observed on t2 weighted mri.

In the transverse plane through the basal ganglia this appears as a tiger like image with prominent eyes. The eye of the tiger sign is known as a radiological sign that refers to abnormal low signal intensity in the globus pallidus with a central longitudinal zone of high signal as seen on t2 weighted mri images. An eye of the tiger sign is a specific magnetic resonance imaging mri pattern a key diagnostic feature of pantothenate kinase associated neurodegeneration pkan.