Objective: To report two cases of parkinsonism-hyperpyrexia secondary to failure of the deep brain stimulator (DBS) of the subthalamic nucleus (STN) in patients with Parkinson’s disease (PD).

Background: Parkinsonism-hyperpyrexia syndrome (PHS) is a neurological emergency that resembles the semiology of a neuroleptic malignant syndrome. It is usually secondary to a sudden deprivation of dopaminergic treatment, although similar cases have been described due to failure of the neurostimulator.

Method: Description of two cases.

Results: First case: A 67-year-old male diagnosed with PD and with STN DBS implantation in 2016 suddenly developed febrile syndrome, increased rigidity and tremor. After reviewing the neurostimulator, the discharge of its battery was assessed. After proceeding to his recharge, no clinical improvement was observed and he was admitted with a diagnosis of fever of unknown origin. Analytically, increased levels of creatine kinase (CK) and renal failure (RF) were observed and fever did not respond to broad-spectrum antibiotic therapy, therefore PHS was suspected. In order to manage the PHS, the dose of dopaminergic drugs and the amplitude of the neurostimulation were increased, and dantrolene was administered. As no clinical improvement was observed, apomorphine infusion was indicated, resulting in a favorable response during the first 24-48 hours, with decreased rigidity, fever and CK levels. However, the patient suffered several infectious complications and paralytic ileus, this along with not being admitted to the Intensive Care Unit (ICU), resulting in death.
Second case: A 73-year-old male diagnosed with PD, that required STN DBS implantation in 2005, abruptly presented with increased body rigidity, high-grade fever (38.9ºC) and abundant respiratory secretions. There was no response to antibiotic treatment, so PHS was suspected due to neurostimulator failure and sudden withdrawal of dopaminergic treatment. He required  ICU admittance due to respiratory distress, dysautonomic symptoms and RF due to elevated CK. After stabilization, the neurostimulator was replaced without immediate complications. The oral dopaminergic treatment was optimized and the rehabilitation treatment was intensified. Patient was discharged in a good baseline state.

Conclusion: The infrequency of PHS entails the lack of precise guidelines for the clinical management of these patients.

References: [1] Azar J, Elinav H, Safadi R, Soliman M. Malignant deep brain stimulator withdrawal syndrome. BMJ Case Rep CP. 1 de mayo de 2019;12(5):e229122.
[2] Simonet C, Tolosa E, Camara A, Valldeoriola F. Emergencies and critical issues in Parkinson’s disease. Pract Neurol. febrero de 2020;20(1):15.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/parkinsonism-hyperpyrexia-as-a-rare-consequence-of-deep-brain-stimulator-malfunction-in-patients-with-parkinsons-disease/