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Diencephalic Syndrome

Abstract

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NORD is very grateful to Roger J. Packer, MD, Children's National Medical Center, Senior Vice President, Center for Neuroscience and Behavioral Medicine; Director, Brain Tumor Institute; Director, Daniel and Jennifer Gilbert Neurofibromatosis Institute; Principal Investigator, Center for Neuroscience Research, for assistance in the preparation of this report.

Synonyms of Diencephalic Syndrome

  • Diencephalic Syndrome of Childhood
  • Diencephalic Syndrome of Emaciation
  • Paramedian Diencephalic Syndrome
  • Russell's Diencephalic Cachexia
  • Russell's Syndrome

Disorder Subdivisions

  • No subdivisions found.

General Discussion

Summary
Diencephalic syndrome is a rare disorder caused by a tumor that is usually located in the diencephalon, a portion of the brain just above the brainstem. The diencephalon includes the hypothalamus and the thalamus. Affected infants and young children may develop symptoms that include the failure to gain weight and grow as would be expected based upon age and gender (failure to thrive) and abnormal progressive thinness and weakness (emaciation). Affected infants and children may behave in an alert, happy and outgoing manner, which is in contrast to their outward appearance. Additional symptoms such as vomiting, vision abnormalities, headaches, and pallor can also develop. Diencephalic syndrome can progress to cause severe, life-threatening complications. Diencephalic syndrome is treated by surgery, radiation, and/or chemotherapy. The reason for the development of the tumor that causes diencephalic syndrome is unknown. Diencephalic syndrome was first described in the medical literature in 1951 by Dr. Russell.

Organizations related to Diencephalic Syndrome

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