Abstract
Background: Ectopic Neurohypophysis is a rare congenital anomaly of the pituitary gland associated with growth hormone and other anterior pituitary hormone deficiencies.
Aims: To report a case of hypopituitarism due to ectopic neurohypophysis presenting with hypoglycemic seizures.
Case report: A 7-year-old Romanian boy was admitted to our hospital with a generalized tonicclonic seizure associated with severe hypoglycemia (25mg/dL). His past medical history was remarkable for a perinatal history of asphyxia; congenital left eye strabismus; and three previous episods of hypoglicemic seizures with inconclusive metabolic and neurologic investigations. He was under treatment with carbamazepine for 3 months.
According to his mother he had also language delay and growth retardation.
The physical examination revealed a boy with a hoarse tone voice, dry skin, brittle hair, cold extremities and left eye convergent strabismus. His height was in the 3rd percentile (target height between 75th- 85th percentile).
Laboratory studies showed hypercholesterolemia, normal values of ACTH, cortisol and TSH but abnormal low values of free T4 and IGF-1. Antithyroglobulin and antiperoxidase antibodies were negative.
Abdominal and thyroid ultrasounds were normal. Left wrist X-ray revealed a bone age of 4 years.
The brain MRI showed an ectopic neurohypophysis. The pituitary stalk was not seen.
The patient started thyroid hormone replacement therapy and has been proposed for recombinant human growth hormone.
Conclusion: This case emphasizes the need for a careful clinical and imagiologic investigation in children presenting with signs of pituitary hormone deficiency. Early diagnosis can prevent further evolution and complications of hypopituitarism.
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Ferraria, N., Afonso, P., Castro, S. et al. 1110 Ectopic Neurohypophysis - Case Report. Pediatr Res 68 (Suppl 1), 550–551 (2010). https://doi.org/10.1203/00006450-201011001-01110
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DOI: https://doi.org/10.1203/00006450-201011001-01110