oa Central African Journal of Medicine - Diencephalic syndrome with long-term survival

Volume 37, Issue 7
  • ISSN : 0008-9176



Infants and young children with the diencephalic syndrome exhibit a normal activity level despite profound marasmus. Investigation reveals an intracranial tumour and evidence of endocrine dysfunction. The condition is easily missed in Third World countries with a high prevalence of nutritional marasmus. The advent of computer tomography and magnetic resonance imaging has simplified diagnosis. The syndrome was diagnosed in 1981 in a boy of 20 months who had a large hypothalamic tumour. Initial endocrine studies revealed very high growth hormone levels. After radiotherapy, these levels reverted to normal for a time but have subsequently declined in keeping with a state of hypopituitarism. The boy's long-term survival has afforded an opportunity for study over eight years.

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