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Endocrine dysfunction in the diencephalic syndrome of emaciation in infancy
Insights
This study describes a child with diencephalic syndrome caused by a brain tumor. Radiation therapy successfully treated the emaciation and normalized growth hormone levels.
Area of Science:
- Pediatric Endocrinology
- Pediatric Oncology
- Neuro-oncology
Background:
- Diencephalic syndrome of emaciation is a rare condition in infants.
- Suprasellar tumors, such as spongioblastomas, can cause hormonal imbalances.
- Growth hormone (GH) dysregulation is a key feature, impacting development.
Observation:
- A 10-month-old boy presented with diencephalic syndrome of emaciation.
- The patient exhibited high basal growth hormone (GH) levels and elevated plasma testosterone.
- Elevated luteinizing hormone (LH) response to gonadotropin-releasing hormone (GnRH) was also noted.
Findings:
- Radiation therapy led to normalized basal GH levels.
- Post-treatment, GH responses to insulin-induced hypoglycemia and propranolol-glucagon stimulation were adequate.
- Clinical remission of emaciation correlated with normalized GH regulation.
Implications:
- Radiation therapy is an effective treatment for suprasellar spongioblastoma causing diencephalic syndrome.
- Successful treatment restores hormonal balance, specifically GH regulation.
- Early diagnosis and intervention are crucial for favorable outcomes in pediatric brain tumors.
Abstract:
A 10-month-old boy with the clinical features of the diencephalic syndrome of emaciation due to a suprasellar spongioblastoma is described. The patient showed high basal levels of growth hormone (GH greater than 80 muU/ml on several occasions). In addition, elevated concentration of plasma testosterone (125.5 ng/100ml) was combined with a relatively high LH-increase to LHRH (45.6 mU/ml). After completion of irradiation basal GH-levels had been normalized, and GH responses to insulin induced hypoglycemia (IIH) and propranolol-glucagon (PG) were adequate. Complete clinical remission of emaciation occurred soon after radiation therapy and went parallel with the normalization of GH-regulation.