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Improved Turnaround Times | Median time to first decision: 12 days

Research ArticlePediatric Neuroimaging

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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References

  1. ↵
    DiMaurio S, Servidei S, Zeivani M, et al. Cytochrome c oxidase deficiency in Leigh syndrome. Ann Neurol 1987;22:498-506
    CrossRefPubMedWeb of Science
  2. Santorelli FM, Mak SC, Vazquez-Memije ME, et al. Clinical heterogenity associated with the mitochondrial DNA T8993C mutation. Pediatr Res 1996;39:914-917
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    Medina L, Chi TL, DeVivo DC, Hilal SK. MR findings in patients with subacute necrotizing encephalomyelopathy (Leigh disease): correlation with biochemical defect. AJNR Am J Neuroradiol 1990;11:379-384
    Abstract/FREE Full Text
  4. ↵
    Zafeiriou DI, Koletzko B, Mueller-Felber W, Paetzke I, Kueffer G, Jensen M. Deficiency in complex IV (cytochrome c oxidase) of the respiratory chain, presenting as a leukodystrophy in two siblings with Leigh syndrome. Brain Dev 1995;17:117-121
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    Walter G, Brucher J, Martin J, Ceuterick C, Pilz P, Freund M. Leigh disease-several nosologic entities with an identical histopathological complex. Neuropathol Appl Neurobiol 1986;12:95-107
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  6. Leigh D. Subacute necrotizing encephalomyelopathy in an infant. J Neurol Neurosurg Psychiatry 1951;14:216-221
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  7. Willems JL, Monnens LAH, Trijbels JMF, et al. Leigh's encephalomyelopathy in a patient with cytochrome c oxidase deficiency. J Inher Metab Dis 1983;6:121-122
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    Koch TK, Yee MHC, Hutchinson HT, Berg BO. Magnetic resonance imaging in subacute necrotizing encephalomyelopathy (Leigh disease). Ann Neurol 1986;19:605-607
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  9. Greenberg SB, Faerber EN, Riviello JJ, De Leon G, Capitanio MA. Subacute necrotizing encephalomyelopathy (Leigh disease): CT and MRI appearances. Pediatr Radiol 1990;21:5-8
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    Valanne L, Ketonen L, Majander A, Suomalainen A, Pihko H. Neuroradiological findings in children with mitochondrial disorders. AJNR Am J Neuroradiol 1998;19:369-377
    Abstract
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    Barkovich AJ, Good WV, Koch TK, Berg BO. Mitochondrial disorders: analysis of their clinical and imaging characteristics. AJNR Am J Neuroradiol 1993;14:1119-1137
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    Harpey JP, Heron D, Prudent M, et al. Diffuse leukodystrophy in an infant with cytochrome-c oxidase deficiency. J Inher Metab Dis 1998;21:748-752
    CrossRefPubMed
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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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