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Research ArticlePEDIATRICS

Leigh Syndrome: Serial MR Imaging and Clinical Follow-up

Junko Arii and Yuzo Tanabe
American Journal of Neuroradiology September 2000, 21 (8) 1502-1509;
Junko Arii
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Yuzo Tanabe
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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

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    TABLE 1:

    Clinical features and results of investigations in Leigh syndrome

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    TABLE 2:

    MR imaging findings in 41 patients with Leigh syndrome reported in the literature

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American Journal of Neuroradiology
Vol. 21, Issue 8
1 Sep 2000
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Junko Arii, Yuzo Tanabe
Leigh Syndrome: Serial MR Imaging and Clinical Follow-up
American Journal of Neuroradiology Sep 2000, 21 (8) 1502-1509;

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Leigh Syndrome: Serial MR Imaging and Clinical Follow-up
Junko Arii, Yuzo Tanabe
American Journal of Neuroradiology Sep 2000, 21 (8) 1502-1509;
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