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 degen...

Descripción completa

Detalles Bibliográficos
Autores principales: Parashari, Umesh C, Aga, Pallavi, Parihar, Anit, Singh, Ragini, Joshi, Vindhya
Formato: Texto
Lenguaje:English
Publicado: Medknow Publications 2010
Materias:
Neuroradiology
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2963751/
https://www.ncbi.nlm.nih.gov/pubmed/21042441
http://dx.doi.org/10.4103/0971-3026.69353
Descripción
Sumario: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.

Ejemplares similares