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Selective involvement of the globus pallidus and dentate nucleus in succinic semialdehyde dehydrogenase deficiency

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Pediatric Radiology Aims and scope Submit manuscript

Abstract.

MRI findings in a 12-year-old boy with succinic semialdehyde dehydrogenase (SSADH) deficiency are described. SSADH deficiency is a rare neurometabolic disorder of GABA catabolism. The clinical diagnosis is difficult and the disease is underdiagnosed. MRI showed an unusual pattern with hyperintense signal in the globus pallidus and cerebellar dentate nucleus in T2-weighted images. The remaining basal ganglia and white matter were normal. This is the second report showing this particular pattern of pallidal-dentate nucleus involvement, which might be suggestive for SSADH deficiency.

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Authors and Affiliations

  1. Section of Neuroradiology, Department of Neurosurgery, University of Freiburg, Germany

    Sargon Ziyeh, Ansgar Berlis, Joachim Spreer & Martin Schumacher

  2. Department of Neuropaediatrics, University of Freiburg, Germany

    Rudolf Korinthenberg

Authors
  1. Sargon Ziyeh
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  2. Ansgar Berlis
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  3. Rudolf Korinthenberg
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  4. Joachim Spreer
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  5. Martin Schumacher
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Ziyeh, S., Berlis, A., Korinthenberg, R. et al. Selective involvement of the globus pallidus and dentate nucleus in succinic semialdehyde dehydrogenase deficiency. Ped Radiol 32, 598–600 (2002). https://doi.org/10.1007/s00247-002-0717-4

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  • DOI: https://doi.org/10.1007/s00247-002-0717-4

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