scholarly journals Neurodegeneration with Brain Iron Accumulation in an Eleven-Year-Old Jamaican Male

2014 ◽  
Vol 2014 ◽  
pp. 1-2 ◽  
Author(s):  
Peter Johnson ◽  
Roxanne Melbourne-Chambers ◽  
Nilesh Desai ◽  
Emma Greenaway

We present a case of an eleven-year-old boy presenting with progressive extrapyramidal signs and dementia. His imaging findings demonstrated the classic eye-of-the-tiger sign on T2W magnetic resonance imaging. He was diagnosed with pantothenate kinase-associated neurodegeneration (PKAN). This is a rare autosomal recessive inborn error of coenzyme A metabolism, caused by mutations inPANK2. This is the first reported case of PKAN from the Caribbean.

Author(s):  
Prajnya Ranganath ◽  
Mallikarjun Patil

AbstractThe “eye-of-the-tiger” sign in brain magnetic resonance imaging (MRI) is typically associated with neurodegeneration with brain iron accumulation disorders, especially pantothenate kinase-associated neurodegeneration. However, very similar neuroimaging findings may be seen in other neurodegenerative disorders involving the basal ganglia. We report here a patient with fucosidosis who had MRI brain findings closely resembling the “eye-of-the-tiger” sign.


2014 ◽  
Vol 38 (2) ◽  
pp. 212-214 ◽  
Author(s):  
Mehmet Beyazal ◽  
Necip Pirinççi ◽  
Alpaslan Yavuz ◽  
Sercan Özkaçmaz ◽  
Gülay Bulut

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