A man in his fifties with increasing motor fluctuations, sleep impairment and altered mental status.

BACKGROUND/CASE PRESENTATION: A man in his fifties with advanced Parkinson´s disease was admitted with increasing motor fluctuations including dyskinesias, as well as hallucinations and delusions. After reduction of oral dopaminergic treatment, the dyskinesias improved, but the psychotic symptoms persisted. They were perceived as levodopa-induced, despite concurrent prominent bradykinetic-rigid symptoms. Dopaminergic treatment was therefore discontinued. He subsequently developed hyperthermia, severe generalised rigidity and akinesia, and autonomic instability. Parkinsonism-hyperpyrexia syndrome was diagnosed, and continuous intraduodenal levodopa/carbidopa infusion was initiated. Despite this, he had several episodes of respiratory distress requiring mechanical ventilation, as well as bradycardia and a single asystole. Although motor and autonomic dysfunction slowly improved, severe akinetic-rigid and neuropsychiatric symptoms persisted, with poor response to increased levodopa. On vital indication, electroconvulsive therapy was performed with clear improvement of mobility and mental state. A hip fracture requiring surgery necessitated discontinuation of ECT, which failed to show equivalent effect when resumed. His condition was considered terminal and all active treatment ceased, resulting in death a few weeks later.

INTERPRETATION: Parkinsonism-hyperpyrexia syndrome can develop if dopaminergic treatment is reduced abruptly and excessively. Coexistence of confusion and/or psychosis and clear bradykinetic-rigid symptoms should alarm the clinician. Dopaminergic treatment should not be discontinued, but given intraduodenally. ECT can be effective if started sufficiently early and administered frequently.