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Diencephalic syndrome: a rare and easily overlooked cause of failure to thrive

Prapai Dejkhamron1, Surachai Likasitwattankul, Kevalee Unachak

  • 1Department of Pediatrics, Faculty of Medicine, Chiang Mai University, Thailand. pdejkham@mail.med.cmu.ac.th

Journal of the Medical Association of Thailand = Chotmaihet Thangphaet
|October 9, 2004
PubMed

Insights

Diencephalic syndrome (DS) causes failure to thrive in children, marked by emaciation and euphoria. Early diagnosis through careful examination and imaging is key for managing hypothalamic-optic chiasma tumors.

Area of Science:

  • Pediatric Endocrinology
  • Pediatric Neurology
  • Pediatric Oncology

Background:

  • Diencephalic syndrome (DS) is a rare condition causing failure to thrive in young children.
  • Key symptoms include emaciation, hyperkinesia, and euphoria.
  • Tumors in the hypothalamic-optic chiasma region are the most common cause.

Observation:

  • Two pediatric cases of DS are presented, aged 14 months and 5 years 9 months.
  • Both presented with classic DS features and had suprasellar masses identified as pilocytic astrocytomas.
  • One child showed papilledema; neurological exams were otherwise normal.

Findings:

  • Pilocytic astrocytomas in the suprasellar region were diagnosed via brain imaging.
  • One patient underwent tumor resection and radiotherapy, showing improved weight and height gain.
  • DS requires consideration in children with unexplained emaciation and euphoria.

Implications:

  • Highlights the importance of considering DS in the differential diagnosis of pediatric failure to thrive.
  • Emphasizes the need for thorough clinical evaluation, including neurological and fundoscopic exams.
  • Underscores the role of appropriate neuroimaging in diagnosing suprasellar masses causing DS.
Abstract

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