Diencephalic Syndrome

National Organization for Rare Disorders, Inc.

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It is possible that the main title of the report Diencephalic Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.


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

Disorder Subdivisions

  • None

General Discussion

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.


National Hydrocephalus Foundation
12413 Centralia Rd.
Lakewood, CA 90715-1653
Tel: (562)924-6666
Fax: (562)924-6666
Tel: (888)857-3434
Email: nhf@earthlink.net
Internet: http://www.nhfonline.org

Hydrocephalus Association
4340 East West Highway Ste 950
Bethesda, MD 20814
Tel: (301)202-3811
Fax: (301)202-3813
Tel: (888)598-3789
Email: info@hydroassoc.org
Internet: http://www.hydroassoc.org

American Cancer Society, Inc.
250 Williams NW St
Ste 6000
Atlanta, GA 30303
Tel: (404)320-3333
Tel: (800)227-2345
TDD: (866)228-4327
Internet: http://www.cancer.org

National Cancer Institute
6116 Executive Blvd Suite 300
Bethesda, MD 20892-8322
Tel: (301)435-3848
Tel: (800)422-6237
TDD: (800)332-8615
Email: cancergovstaff@mail.nih.gov
Internet: http://www.cancer.gov

Children's Brain Tumor Foundation
274 Madison Avenue, Suite 1004
New York, NY 10016
United States
Tel: (212)448-1595
Fax: (212)448-1022
Tel: (866)228-4673
Email: info@cbtf.org
Internet: http://www.cbtf.org

Brain Tumor Foundation for Children, Inc.
6065 Roswell Road Suite 505
Atlanta, GA 30328-4015
Tel: (404)252-4107
Fax: (404)252-4108
Email: info@braintumorkids.org
Internet: http://www.braintumorkids.org

Rare Cancer Alliance
1649 North Pacana Way
Green Valley, AZ 85614
Internet: http://www.rare-cancer.org

Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg, MD 20898-8126
Tel: (301)251-4925
Fax: (301)251-4911
Tel: (888)205-2311
TDD: (888)205-3223
Internet: http://rarediseases.info.nih.gov/GARD/

Pediatric Brain Tumor Foundation
302 Ridgefield Court
Asheville, NC 28806
Tel: (828)665-6891
Fax: (828)665-6894
Tel: (800)253-6530
Email: pbtfus@pbtfus.org
Internet: http://www.pbtfus.org

PLGA Foundation
98 Random Farms Drive
Chappaqua, NY 10514
Email: contact@fightplga.org
Internet: http://www.fightplga.org/

Friends of Cancer Research
1800 M Street NW
Suite 1050 South
Washington, DC 22202
Tel: (202)944-6700
Email: info@focr.org
Internet: http://www.focr.org

National Brain Tumor Society
55 Chapel Street
Suite 200
Newton, MA 02458
Tel: (617)924-9997
Fax: (617)924-9998
Tel: (800) 770-8287
Email: info@braintumor.org
Internet: http://www.braintumor.org

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This is an abstract of a report from the National Organization for Rare Disorders (NORD). A copy of the complete report can be downloaded free from the NORD website for registered users. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational therapies (if available), and references from medical literature. For a full-text version of this topic, go to www.rarediseases.org and click on Rare Disease Database under "Rare Disease Information".

The information provided in this report is not intended for diagnostic purposes. It is provided for informational purposes only. NORD recommends that affected individuals seek the advice or counsel of their own personal physicians.

It is possible that the title of this topic is not the name you selected. Please check the Synonyms listing to find the alternate name(s) and Disorder Subdivision(s) covered by this report

This disease entry is based upon medical information available through the date at the end of the topic. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder.

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Last Updated:  10/25/2013
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