J Korean Radiol Soc.  1998 Mar;38(3):539-545. 10.3348/jkrs.1998.38.3.539.

Leigh Syndrome: Serial MRI Findings

• Kwon JS
• Choi CG
• Ko TS
• Yoon CH
• Kim DE

• KMID: 2201417
• DOI: http://doi.org/10.3348/jkrs.1998.38.3.539

Abstract

PURPOSE: The purpose of this study was to evaluate the effect of the temporal changes in brain lesions onserial MR images during the course of Leigh syndrome.
MATERIALS AND METHODS
We retrospectively reviewed 11 MRimages in four patients diagnosed as suffering from Leigh syndrome on the basis of clinical features, MRIfindings, and biochemical data. Follow-up and earlier, MR images were compared and temporal changes in lesionswere analyzed, with particular attention to location, size, signal intensity, and contrast enhancement.
RESULTS
Initial MRI showed that in order of frequency, the following were involved : bilateral putamina (4/4), caudatenuclei (2/4), the brain stem (2/4), medial thalamic nuclei (1/4), and the cerebral cortex (1/4). In two patients,the size of acute putaminal lesions, as seen on follow-up MRI, decreased in the short term (within two weeks); inone patient, strong contrast enhancement of the lesions was observed twelve days after initial MRI. Long termfollow-up MRI, over 7 - 19 months, showed newly developed lesions (2/4) and atrophy (2/4) or obliteration ofprevious lesions (3/4) in the basal ganglia, thalami, and brain stem.
CONCLUSION
Serial MRI demonstratedtemporal changes in brain lesions during the course of Leigh syndrome. On follow-up MRI, the appearance ofbilateral lesions in basal ganglia and the brain stem, not present on initial MRI, may be helpful corroborativeevidence to support a diagnosis of this syndrome.

Keyword

Brain, MR; Brain, metabolism

MeSH Terms

Atrophy
Basal Ganglia
Brain
Brain Stem
Cerebral Cortex
Diagnosis
Follow-Up Studies
Humans
Leigh Disease*
Magnetic Resonance Imaging*
Retrospective Studies
Thalamic Nuclei

Figure

• 

  Fig. 1. A 7-month-old infant with seizure and ptosis. A, B. Axial T2-weighted images of initial MRI reveal bilateral symmetrical lesions in the putamina, medial thalamic nuclei, red nuclei and periac- queductal gray matter. C. Contrast enhanced Tl-weighted images of second MRI demonstrate strong enhancement of the lesions in the caudate nuclei and putamia. D. Axial Τ 2-weighted images of third MRI, obtained 7 month later from initial MRI, show small cleft like residual lesions in both putamina, atrophy of caudate heads, and resolution of midbrain lesions. Clinical symptoms are persistent without progression after recovery from acute attack.

• 

  Fig. 2. A 2-year-old boy with respiratory difficulty. A, B. Axial T2-weighted images of initial MRI show multiple small lesions in left putamina and bilateral symmetrical lesions in tegmentum of the medulla oblongata(arrows). C, D. Axial T2-weighted images of second MRI, obtained 1 year and 6 months later from initial MRI, show progression of putaminal lesions, newly developed lesions in both caudate nuclei, medial thalamic nuclei, and disappearance of medullary lesions. This patient showed progression of limb rigidity and quadri- plegia at the time of second MRI.

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