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[Diencephalic syndrome--report of three cases]
Insights
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.
Abstract:
Three cases of diencephalic syndrome, associated with brain tumors, are reported in this paper. Case 1. A 2-2/12-year old boy was initially admitted to our hospital because of failure to thrive which began at the age of three months. Physical examination revealed emaciation (weight, 7.8 kg), irritability and pallor without anemia. Horizontal nystagmus was seen. Laboratory studies were normal except for abnormally high plasma growth hormone (p-GH) which was incompletely suppressed by hyperglycemia (induced by glucose) and was not elevated by hypoglycemia (induced by insulin). A low grade astrocytoma of the optic nerve compression the hypothalamus was partially removed. After the operation followed by irradiation, p-GH returned to normal both in its basal level and in its reaction to insulin loading, then his gain of weight was accelerated. He was readmitted, however, at the age of 6 8/12 years with headache and vomiting. Since subtotal removal of the recurrent tumor and irradiation, preoperative symptoms have disappeared up to the present (7 9/12 years old). Case 2. A 3-9/12-year old girl was initially admitted because of failure to thrive since the age of 2 years. Examination on admission revealed emaciation (10.5 kg), irritability and right hemiparesis. Laboratory studies were normal except for high serum cholesterol (290 mg/dl). (p-HG was not measured) The patient had been well after the subtotal removal of the hypothalamic astrocytoma except occasional headache until the age of 6 years. She was readmitted at the age of 9 years with progressive emaciation.(ABSTRACT TRUNCATED AT 250 WORDS)