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Research ArticlePEDIATRICS

Leigh Syndrome in a 3-Year-Old Boy with Unusual Brain MR Imaging and Pathologic Findings

Meral Topçu, Isil Saatci, R. Anil Apak, Figen Söylemezoglu and Zuhal Akçören
American Journal of Neuroradiology January 2000, 21 (1) 224-227;
Meral Topçu
aFrom Hacettepe University, Faculty of Medicine, Departments of Child Neurology (M.T., R.A.A.), Radiology (I.S.), Pathology (F.S.) and Pediatric Pathology (Z.A.), Ankara, Turkey.
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Isil Saatci
aFrom Hacettepe University, Faculty of Medicine, Departments of Child Neurology (M.T., R.A.A.), Radiology (I.S.), Pathology (F.S.) and Pediatric Pathology (Z.A.), Ankara, Turkey.
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R. Anil Apak
aFrom Hacettepe University, Faculty of Medicine, Departments of Child Neurology (M.T., R.A.A.), Radiology (I.S.), Pathology (F.S.) and Pediatric Pathology (Z.A.), Ankara, Turkey.
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Figen Söylemezoglu
aFrom Hacettepe University, Faculty of Medicine, Departments of Child Neurology (M.T., R.A.A.), Radiology (I.S.), Pathology (F.S.) and Pediatric Pathology (Z.A.), Ankara, Turkey.
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Zuhal Akçören
aFrom Hacettepe University, Faculty of Medicine, Departments of Child Neurology (M.T., R.A.A.), Radiology (I.S.), Pathology (F.S.) and Pediatric Pathology (Z.A.), Ankara, Turkey.
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    fig 1.

    Initial MR examination.

    A and B, T2-weighted (3500/98 [TR/TE]) axial turbo spin-echo images showing involvement of the posterior periventricular white matter, symmetrical bilaterally (A and B), with the abnormal signal intensity in the genu and splenium of the corpus callosum (white arrows, A). The hyperintensity extends to the posterior crus of the internal capsule (A). There are well-marginated cystic areas within the abnormal white matter (black arrows). Note the basal ganglia are not affected.

    C, Contrast-enhanced T1-weighted (600/14 [TR/TE]) axial image, corresponding to figure A, showing the cystic nature of the periventricular white matter with foci of contrast enhancement mostly at the periphery of the abnormal area (black arrows).

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    fig 2.

    Repeat MR examination at 3 months

    A, T2-weighted (2340/90 [TR/TE]) axial turbo spin-echo image through the same level as 1A, showing the progression of periventricular white matter abnormality. Corpus callosum appear swollen and hyperintensity extends further through the internal capsule as well as in the external capsule. Note that the U-fibers are spared.

    B, Unenhanced T1-weighted (550/15 [TR/TE]) midsagital image showing diffuse involvement of the corpus callosum, with decreased signal intensity (arrows).

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    fig 3.

    Electron microscopic findings of the brain tissue showing foam cells within the cerebral white matter

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American Journal of Neuroradiology
Vol. 21, Issue 1
1 Jan 2000
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Cite this article
Meral Topçu, Isil Saatci, R. Anil Apak, Figen Söylemezoglu, Zuhal Akçören
Leigh Syndrome in a 3-Year-Old Boy with Unusual Brain MR Imaging and Pathologic Findings
American Journal of Neuroradiology Jan 2000, 21 (1) 224-227;

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Leigh Syndrome in a 3-Year-Old Boy with Unusual Brain MR Imaging and Pathologic Findings
Meral Topçu, Isil Saatci, R. Anil Apak, Figen Söylemezoglu, Zuhal Akçören
American Journal of Neuroradiology Jan 2000, 21 (1) 224-227;
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