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Brainstem Dysfunction in Infants Responsive to Thiamine Disulfide: Preliminary Studies in Four Patients

Introduction A preliminary report has been published concerning our experience with brainstem auditory evoked potential (BAEP) testing of infants 1 and treatment of two of them with thlamlne." In this communication we report four infants whose BAEP tests indicated abnormal responses in brainstem function. The most dangerous symptom in all four patients was recurrent, unpredictable life threatening apnea, but all of them demonstrated other symptomatology which appeared to be centrally mediated. All were treated with thiamine tetrahydrofurfuryl disulfide (TTFD) and symptoms gradually improved. Serial BAEP tests correlated with the clinical improvement. Although the subsequent clinical course indicated residual damage in at least one of these infants, the other three appear to be progressing well. Long term follow-up care will be necessary to evaluate ultimate sequelae in these children.