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Case Reports
. 2010 Jan-Mar;6(1):e6.
doi: 10.2349/biij.6.1.e6. Epub 2010 Jan 1.

Leigh syndrome: MRI findings in two children

Al Kartikasalwah  1 , Ngu Lh
Affiliations
Case Reports

Leigh syndrome: MRI findings in two children

Al Kartikasalwah et al. Biomed Imaging Interv J. 2010 Jan-Mar.

Abstract

Leigh syndrome is a progressive neurodegenerative disorder of childhood. The symmetrical necrotic lesions in the basal ganglia and/or brainstem which appear as hyperintense lesions on T2-weighted MRI is characteristic and one of the essential diagnostic criteria. Recognising this MR imaging pattern in a child with neurological problems should prompt the clinician to investigate for Leigh syndrome. We present here two cases of Leigh syndrome due to different biochemical/genetic defects, and discuss the subtle differences in their MR neuroimaging features.

Keywords: Leigh syndrome; MRI; SURF1; mitochondrial.

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Figures

Figure 1
Figure 1
(A): Axial T2-weighted image through the basal ganglia shows symmetrical hyperintense lesions involved thalamic posteromedial ventral nuclei (thin arrow), globus pallidi (thick arrow) and putamina (arrowhead). (B-D): Axial T2-weighted images through the brainstem show symmetrical involvement of reticular formation of midbrain (thin arrow in B), subthalamic nuclei (thick arrow in B), substantia nigra (thick arrow in C), dorsal midbrain (thin arrow in C) central tegmental tracts (thin arrow in D) and cerebellar nuclei region (thick arrow in D). (E-F): Coronal FLAIR images show symmetrical involvement of head of caudate nuclei (thick arrow in E), putamina (thin arrow in E) and dentate nuclei (thin arrow in F).
Figure 2
Figure 2
(A-C): Axial T2-weighted images through the brainstem show symmetrical hyperintense lesions involving central tegmental tracts (thin arrow in A), substantia nigra (thin arrow in B and C). (D): Axial T2-weighted image through basal ganglia shows putamen, globus pallidus and thalamus were not involved.
See this image and copyright information in PMC

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