Winner of CoW 28 August 2016
Pantothenate kinase-associated neurodegeneration (PKAN)/Hallervorden-Spatz syndrome
Findings
T2-weighted hypointense globus pallidus with a central anteromedial region of T2 hyperintensity - the characteristic "eye-of-the-tiger" appearance. SWI shows corresponding areas of blooming.
Discussion
PKAN is the most common type of Neurodegeneration with brain iron accumulation (NBIA) , caused by excessive iron deposition in the globus pallidus and substantia nigra .Imaging findings reflect the anatomic distribution of the excessive iron accumulation.T2WI demonstrates marked hypointensity in the GP and SN. A small focus of central hyperintensity in the medial aspect of the very hypointense GP (the classic “eye of the tiger” sign) is caused by tissue gliosis and vacuolization.PKAN does not enhance on T1 C, nor does it demonstrate restricted diffusion. DTI demonstrates significantly increased fractional anisotropy in both the GP and SN
Findings
T2-weighted hypointense globus pallidus with a central anteromedial region of T2 hyperintensity - the characteristic "eye-of-the-tiger" appearance. SWI shows corresponding areas of blooming.
Discussion
PKAN is the most common type of Neurodegeneration with brain iron accumulation (NBIA) , caused by excessive iron deposition in the globus pallidus and substantia nigra .Imaging findings reflect the anatomic distribution of the excessive iron accumulation.T2WI demonstrates marked hypointensity in the GP and SN. A small focus of central hyperintensity in the medial aspect of the very hypointense GP (the classic “eye of the tiger” sign) is caused by tissue gliosis and vacuolization.PKAN does not enhance on T1 C, nor does it demonstrate restricted diffusion. DTI demonstrates significantly increased fractional anisotropy in both the GP and SN
Note:
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
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The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!