Eye-of-the-tiger-sign in a 48 year healthy adult.

We report a healthy adult male, who underwent, as a control subject, part of a Huntington's disease study, extensive testing during three visits in a two year follow-up, including clinical examination and 3.0 T MRI scans. The T2-weighted MRI sequences revealed the "eye-of-the-tiger-sign". No clinical abnormalities in either motor, cognitive or behavioural domains were observed. PKAN2 and FTL gene mutation analysis were negative. This finding implies that an eye-of-the-tiger sign, which is considered a pathognomonic feature of neurodegeneration with brain iron accumulation (NBIA), can occur without any clinical symptoms.