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[Diencephalic syndrome--report of three cases]
No Shinkei Geka. Neurological Surgery
|February 1, 1984
Summary
Diencephalic syndrome in children, often linked to brain tumors, can cause failure to thrive and hormonal imbalances. Early diagnosis and treatment, including surgery and irradiation, can improve outcomes and growth.
Area of Science:
- Pediatric Endocrinology
- Pediatric Oncology
- Pediatric Neurology
Background:
- Diencephalic syndrome is a rare disorder characterized by failure to thrive, often associated with hypothalamic or optic pathway tumors.
- It can present with hormonal dysregulation, including abnormal growth hormone (GH) levels.
Observation:
- This paper reports three cases of diencephalic syndrome in children, all associated with brain tumors.
- Case 1 involved a boy with failure to thrive, emaciation, and elevated plasma growth hormone (p-GH) due to an astrocytoma compressing the hypothalamus.
- Case 2 presented a girl with failure to thrive and emaciation, also linked to a hypothalamic astrocytoma.
Findings:
- Treatment with partial tumor removal and irradiation in Case 1 normalized p-GH levels and accelerated weight gain.
- The patient in Case 1 experienced symptom resolution after recurrent tumor treatment.
- Case 2 showed initial improvement after surgery, but later presented with progressive emaciation.
Implications:
- Surgical and radiological interventions can effectively manage diencephalic syndrome and associated hormonal disturbances.
- Prompt diagnosis and multidisciplinary treatment are crucial for improving the prognosis of children with diencephalic syndrome.
- Further research is needed to fully understand the long-term effects and optimal management strategies for this condition.