Parkinsonism-hyperpyrexia syndrome following globus pallidus internus deep brain stimulation withdrawal: a report of two cases and literature review

Research overview

The text below is the source article’s original abstract under a verified reuse license. cc by

Abstract Background Parkinsonism–hyperpyrexia syndrome (PHS) is a life-threatening neurologic emergency. Deep brain stimulation (DBS) withdrawal is a recognized precipitant, yet most reported cases involve the subthalamic nucleus (STN). In contrast, detailed descriptions of PHS following globus pallidus internus (GPi) DBS withdrawal remain scarce, particularly regarding the temporal relationship between the resolution of hyperthermia and the normalization of serum biochemical markers (creatine kinase) after device reactivation. Case presentation: We describe two cases of GPi-DBS withdrawal PHS triggered by accidental implantable pulse generator shutdown. Case 1 involved a 67-year-old woman with fulminant PHS (peak temperature 39.5°C; creatine kinase 2486 U/L) and case 2 involved a 71-year-old man with prodromal PHS (temperature 38.2°C; creatine kinase 770 U/L). In both cases, device reactivation resulted in rapid resolution of hyperthermia within 2–4 hours and creatine kinase levels normalized only after 5–9 days of intensive intravenous hydration. This temporal dissociation between clinical improvement and biochemical recovery has not been well understood in GPi-DBS withdrawal PHS. Conclusions These cases demonstrate that in GPi-DBS withdrawal PHS, body temperature can normalize rapidly despite persistently elevated serum creatine kinase. Resolving hyperthermia in the setting of ongoing rhabdomyolysis creates a deceptive clinical impression that can lead to the premature de-escalation of supportive care. Our findings underscore the importance of the “Device-first” principle, and emphasize that continuous biochemical monitoring remains crucial even after apparent clinical stabilization.

Related topics