Cerebrospinal fluid somatostatin in West syndrome: changes in response to combined treatment with high-dose pyridoxal phosphate and low-dose corticotropin.

Eighteen children with West syndrome (5-11 months of age) were selected to receive an oral dose of pyridoxal phosphate, (20-50 mg/kg) for 14 d. Seizures disappeared in one patient. The remaining 17 patients were treated with 0.01 mg/kg synthesized corticotropin intramuscularly for 2 weeks as an additional therapy. Seizures disappeared in all 17 patients within a few days after initiation of the corticotropin. Levels of somatostatin in the cerebrospinal fluid were as follows: 61.0+/-10.7 pg/ml before therapy, 34.2+/-6.4 pg/ml during pyridoxal phosphate therapy, and 26.8+/-4.2 pg/ml after 2 weeks corticotropin therapy. Somatostatin levels in untreated patients were higher (p < 0.05) than those of age-matched controls (35.7+/-11.8 pg/ml) and decreased (p < 0.05) after pyridoxal phosphate treatment. Somatostatin is a hypothalamic tetradecapeptide with excitatory effects on neurons and pyridoxal phosphate might subclinically influence neuronal excitation.