Case report: MR spectroscopy in pantothenate kinase-2 associated neurodegeneration.
We report a case of a 13-year-old girl with Hallervorden-Spatz disease (HSD) or pantothenate kinase-2 associated neurodegeneration (PKAN). HSD is a rare neurodegenerative disorder, which is characterized by a rapidly progressive extrapyramidal syndrome, dementia with optic atrophy, and retinal degeneration. It is associated with accumulation of cysteine-iron complex in the globus pallidi and substantia nigra. The MRI "eye of the tiger" sign is the characteristic. MRI spectroscopy is also characteristic. It shows markedly decreased NAA/Cr values in the globus pallidi and substantia nigra with increased mI/Cr values that suggest of gliosis.
Department of Radio Diagnosis, C.S.M. Medical University, (Upgraded K.G. Medical University) Lucknow, UP, India.
Umesh C
UC
Parashari
Pallavi
P
Aga
Anit
A
Parihar
Ragini
R
Singh
Vindhya
V
Joshi