Acta Neuropathologica

, Volume 56, Issue 4, pp 303–306 | Cite as

Neuropathology of citrullinaemia

  • J. J. Martin
  • J. P. Farriaux
  • P. De Jonghe
Short Original Communications


The examination of the central nervous system (CNS) of an 11-month-old citrullinaemic patient revealed an ulegyria with a dense fibrillary gliosis and large amounts of lipid phagocytes. Alzheimer type 2 glial cells were present in intact cortical areas. While such cells were related to the insufficiently controlled hyperammonaemia, the ulegyria resulted from impaired oxygenation or impaired blood circulation, possibly influenced by the hyperammonaemia and citrullinaemia.

Key words

Citrullinaemia Hyperammonaemia Argininosuccinate synthetase deficiency Alzheimer type 2 glial cells Ulegyria 


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Copyright information

© Springer-Verlag 1982

Authors and Affiliations

  • J. J. Martin
    • 1
  • J. P. Farriaux
    • 2
  • P. De Jonghe
    • 1
  1. 1.Born-Bunge FoundationUniversitaire Instelling AntwerpenWilrijkBelgium
  2. 2.Clinique de Pédiatrie, Service de Génétique et Maladies métaboliquesHôpital Régional, CHULilleFrance

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