RT Journal Article
SR Electronic
T1 Thiamine Deficiency in Infants: MR Findings in the Brain
JF American Journal of Neuroradiology
JO Am. J. Neuroradiol.
FD American Society of Neuroradiology
SP 1668
OP 1674
VO 26
IS 7
A1 Kornreich, Liora
A1 Bron-Harlev, Efrat
A1 Hoffmann, Chen
A1 Schwarz, Michael
A1 Konen, Osnat
A1 Schoenfeld, Tommy
A1 Straussberg, Rachel
A1 Nahum, Elhanan
A1 Ibrahim, Abu-Kishk
A1 Eshel, Gideon
A1 Horev, Gadi
YR 2005
UL http://www.ajnr.org/content/26/7/1668.abstract
AB BACKGROUND AND PURPOSE: Thiamine deficiency is extremely rare in infants in developed countries. To our knowledge, its MR findings in the brain have not been reported. The purpose of this study was to investigate the brain MR findings in infants with encephalopathy due to thiamine deficiency.METHODS: The study group included six infants aged 2–10 months with encephalopathy who had been fed with solely soy-based formula devoid of thiamine from birth. All underwent MR evaluation at admission and follow-up (total of 14 examinations). In one patient, MR spectroscopy (MRS) was performed.RESULTS: In five patients T2-weighted, fluid-attenuated inversion recovery, or proton-attenuated sequences showed bilateral and symmetric hyperintensity in the periaqueductal area, basal ganglia and thalami. Five had lesions in the mammillary bodies, and three, in the brain stem. In all six patients, the frontal region (cortex and white matter) was clearly involved. At presentation, MRS of the periaqueductal area showed a lactate doublet. On long-term follow-up, three of four patients had severe frontal damage; in two, this occurred as part of diffuse parenchymal loss, and in one, it was accompanied by atrophy of the basal ganglia and thalami.CONCLUSION: Thiamine deficiency in infants is characterized by involvement of the frontal lobes and basal ganglia, in addition to the lesions in the periaqueductal region, thalami, and the mammillary bodies described in adults. MRS demonstrates a characteristic lactate peak.