Pyridostigmine has been proposed for the treatment of
postural orthostatic tachycardia syndrome in adults at a dose of 60 mg twice daily, but no dosing recommendation exists for children. With the approval of our local ethics board, we tested the pharmacokinetics of
pyridostigmine in 6 children with myasthenia and a pediatric index patient with severe
postural orthostatic tachycardia syndrome whose condition failed all conventional
therapy and who had developed significant postural
hypertension.
Pyridostigmine was quantified by using a validated, semiautomated, and specific high-performance liquid chromatography/tandem mass spectrometry assay in combination with online column-switching extraction and turbo electrospray ionization. The patient with
postural orthostatic tachycardia syndrome showed a dose-dependent favorable response to oral
pyridostigmine. Pharmacokinetic evaluation revealed a short half-life of 2.29 hours, similar to the 2.0 +/- 0.63 hours in the patients with myasthenia. The patient with
postural orthostatic tachycardia syndrome has subsequently been treated at a dose of 45 mg in the morning, 30 mg at lunchtime, and 15 mg at bedtime; after 9 months, there has been persistent positive effect and without additional blood pressure medication. No major adverse effects occurred.
Pyridostigmine has been a safe and effective treatment modality for this child with
postural orthostatic tachycardia syndrome. The short half-life suggests that dosing 3 times per day is preferable.