Open AccessMagnetic resonance imaging in pantothenate kinase-2-associated neurodegeneration
Author(s) -
Paramdeep Singh,
Kavita Saggar,
Maneet Kaur,
Davinder Singh Pannu
Publication year - 2012
Publication title -
journal of pediatric neurosciences
Language(s) - English
Resource type - Journals
eISSN - 1998-3948
pISSN - 1817-1745
DOI - 10.4103/1817-1745.97618
Subject(s) - globus pallidus , medicine , magnetic resonance imaging , neurodegeneration , dystonia , neuroscience , pathology , basal ganglia , central nervous system , disease , psychology , radiology , psychiatry
Pantothenate kinase-2-associated neurodegeneration (PKAN) is a rare autosomal recessive pediatric neurodegenerative disorder characterized by rigidity, dystonia, impaired postural reflexes, and progressive dementia. On T2-weighted magnetic resonance imaging images, marked low signal intensity is seen in the globus pallidus. This low signal intensity surrounds a central region of high signal intensity in the anteromedial globus pallidus, giving an eye-of-the-tiger appearance.