Learn About Diencephalic Syndrome

What is the definition of Diencephalic Syndrome?
Diencephalic syndrome is a condition caused by a tumor located in a portion of the brain above the brainstem called the diencephalon. The diencephalon includes the hypothalamus and the thalamus. This condition is usually found in infants and young children and may result in symptoms including failure to gain weight and grow normally (failure to thrive), progressive thinness and weakness (emaciation), and hyperactivity and restlessness (hyperkinesia). Other symptoms may include abnormal eye movements and vision issues, vomiting, and hydrocephalus. Diencephalic syndrome is most commonly caused by astrocytomas; however, other types of tumors may be associated including ependymoma, dysgerminoma, and ganglioma. Given their location, these tumors may cause symptoms by leading to abnormal functioning of the hypothalamus and optic nerve.
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What are the alternative names for Diencephalic Syndrome?
  • Diencephalic syndrome
  • Diencephalic cachexia
  • Diencephalic syndrome of childhood
  • Diencephalic syndrome of emaciation
  • Russell diencephalic cachexia
  • Russell syndrome
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What are the latest Diencephalic Syndrome Clinical Trials?
GROWing Up With Rare GENEtic Syndromes ….When Children With Complex Genetic Syndromes Reach Adult Age

Summary: Introduction Rare complex syndromes Patients with complex genetic syndromes, by definition, have combined medical problems affecting multiple organ systems, and intellectual disability is often part of the syndrome. During childhood, patients with rare genetic syndromes receive multidisciplinary and specialized medical care; they usually receive medical care from 3-4 medical specialists. Increased...

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Clinical Investigation to Evaluate the CONVIVO System for Discrimination of Normal From Abnormal Tissue During Brain Tumor Resection

Summary: The primary objective of this study is to evaluate the diagnostic performance of the CONVIVO confocal endomicroscope in discriminating between normal and abnormal tissue in vivo during brain tumor surgery. The interpretation of intraoperative images obtained in situ will be tested against conventional histologic evaluation of targeted biopsies from imaged tissue. The study team hypothesize that th...

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Published Date: May 02, 2022
Published By: Genetic and Rare Diseases Informnation Center

What are the Latest Advances for Diencephalic Syndrome?
Comparison of Surgical Outcomes in Individuals With Hypothalamic Hamartoma Alone or With Other Potentially Epileptogenic Focal Lesions.
Dextroamphetamine Treatment in Children With Hypothalamic Obesity.
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Hepatopulmonary syndrome: a rare manifestation of cirrhosis in patient with diencephalic obesity and nonalcoholic fatty liver disease after surgery for craniopharyngioma.