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Pediatric Radiology

, Volume 32, Issue 8, pp 598–600 | Cite as

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

  • Sargon Ziyeh
  • Ansgar Berlis
  • Rudolf Korinthenberg
  • Joachim Spreer
  • Martin Schumacher
Case Report

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.

MRI Globus pallidus Dentate nucleus Succinic semialdehyde dehydrogenase deficiency 

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Copyright information

© Springer-Verlag 2002

Authors and Affiliations

  • Sargon Ziyeh
    • 1
  • Ansgar Berlis
    • 1
  • Rudolf Korinthenberg
    • 2
  • Joachim Spreer
    • 1
  • Martin Schumacher
    • 1
  1. 1.Section of Neuroradiology, Department of Neurosurgery, University of FreiburgGermany
  2. 2.Department of Neuropaediatrics, University of FreiburgGermany

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