Pallid syncope successfully treated by permanent cardiac pacing

The aims of this study are to raise awareness among paediatricians, neurologists and paediatric neurologists of the potential benefits of permanent cardiac pacing in patients who have severe and disabling reflex anoxic seizures (RAS) associated with reflex asystole, sometimes called ‘pallid syncope’. Up to now pacing was generally held not to be required for RAS as it is self-limiting and safe. A 3-year-old child with disabling RAS (asystole up to 24 seconds and end-expiratory apnoea) has been paced (VVI, 70 bpm). Medical treatments failed and the post-ictal drowsiness and social effects of 1040 attacks a day were felt to warrant a trial of temporary pacing. This aborted an induced attack. Permanent pacing was therefore established. Two years later the patient remains free of RAS, off drugs and is attending school. The pacemaker was well tolerated and there have been no adverse effects. Family life has been normalized. A double-blind period of ambulatory monitoring, during which the pacemaker was either on or off, led to a typical RAS while the pacemaker was turned off. Permanent cardiac pacing can ameliorate RAS and return a family’s quality of life back to normal.