Reversible Diffusion Weighted Imaging Changes in Propionic Acidemia. (January 2013)

• Record Type: Journal Article
• Authors: Kandel, Amit; Amatya, Sirisa Kandel; Yeh, E. Ann
• Is Part Of: Journal of child neurology. Volume 28:Number 1(2013)
• Page Start: 128
• Page End: 131
• Publication Date: 2013-01
• Subjects: diffusion weighted abnormalities -- propionic acidemia -- metabolic encephalopathy; Nervous system -- Diseases -- Periodicals; 618.928
• DOI: 10.1177/0883073812441057
• Languages: English
• ISSNs: 0883-0738

Abstract

Propionic acidemia is an inborn error of metabolism with neurologic manifestations. We describe a 3-year-old boy with propionic acidemia presenting with a metabolic crisis including headache, vomiting, and altered mental status with metabolic acidosis. Electroencephalography showed focal slowing in right temporal region. Magnetic resonance imaging (MRI) of the brain showed restricted diffusion with apparent diffusion coefficient correlate in the right parietooccipital region. Correction of metabolic acidosis led to clinical improvement and normalization of MRI diffusion weighted imaging/apparent diffusion coefficient changes. This article suggests that restricted diffusion resulting from metabolic crises in propionic acidemia may be reversible in some cases.