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Leigh Syndrome: Serial MR Imaging and Clinical Follow-up

Junko Ariia and Yuzo Tanabea

a From the Division of Neurology, Chiba Children's Hospital, 579-1 Heta-cho, Midori-ku, Chiba 266-0007, Japan. Address reprint requests to Junko Arii, MD.



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  		FIG 1. Patient 1: group BG lesion pattern. Axial T2-weighted image (2000/80/2) at the age of 7 months during an acute strokelike episode shows a hyperintense lesion in the right parietotemporal region (arrowheads)



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  		FIG 2. Patient 2: group BG lesion pattern.

A, Axial T2-weighted image (2000/80/2) at the age of 3 years shows increased signal intensity in the bilateral putamina and caudate heads, but no detectable brain stem lesion. Clinical findings included mental retardation, muscle weakness, and failure to thrive.

B and C, Axial (B) and sagittal (C) T2-weighted images (2000/80/2) at the age of 9 years show static and atrophic lesions in the bilateral putamina and progressive lesions in the cerebral white matter, corpus callosum, and medial medulla oblongata (arrow, C). Clinical findings included irregular breathing, lethargy, and inability to feed.



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  		FIG 3. Patient 6: group BS lesion pattern.

A–C, Axial T2-weighted images (2000/80/2) at the age of 11 months show lesions in the substantia nigra (arrows, B) and periaqueductal region (arrowheads, B), and subtle increased signal intensity within the medullary reticular formation (arrow, C) but not in the basal ganglia. Clinically, the patient had episodic unexplained tachypnea.



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  		FIG 4. Patient 8: group WM lesion pattern.

A and B, Coronal (A) and axial (B) T2-weighted images (2000/80/2) at the age of 12 years show symmetrical hyperintensity in the cerebral white matter (arrows, A), thalamus, basal ganglia, and medullary tegmentum (arrowheads, B). Clinically, the patient had acute respiratory failure.



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  		FIG 5. Brain stem lesions on MR images and abnormal respiration. Patient numbers correspond to those in Table 1. L indicates lower brain stem lesion; U, upper brain stem lesion; BG, basal ganglia lesion; WM, cerebral white matter lesion; solid line, duration of the disease; solid circle, acute respiratory failure; dotted line, permanent mechanical ventilation; cross, death





Copyright © 2009 by the American Society of Neuroradiology. Print ISSN: 0195-6108 Online ISSN: 1936-959X