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. Treatment usually involves surgery to remove as much of the tumor as possible, radiation therapy, and chemotherapy.
What are the alternative names for Diencephalic Syndrome?
- Diencephalic cachexia
- Diencephalic syndrome of childhood
- Diencephalic syndrome of emaciation
- Russell diencephalic cachexia
- Russell syndrome
What is the outlook (prognosis) for Diencephalic Syndrome?
The prognosis for individuals with diencephalic syndrome differs depending on the type of tumor associated and grade of the tumor. If left untreated, most with this condition will pass away within 12 months. With treatment, the long-term survival is greatly improved; however, even with treatment, the tumor may continue to grow slowly and there may be lasting symptoms related to issues with the hypothalamus and vision.