Objective: To report the case of a PD patient treated with bilateral deep brain stimulation of subthalamic nucleus (STN-DBS) who developed DHS after the sudden interruption of stimulation.

Background: DHS is a rare complication of Parkinson’s disease (PD): it represents a movement disorder emergency, characterized by severe dyskinesias, hyperthermia, rhabdomyolisis and alteration of mental status. To date, DHS has been described only in a few case reports.

Methods: A 79-year-old woman with advanced PD, treated with bilateral STN-DBS and achieving a good control of motor fluctuations, was admitted to hospital in May 2017 because of a cutaneous infection at the implantable pulse generator (IPG) site. IPG was surgically removed; cultural examinations were negative. After the IPG explantation, oral dopaminergic therapy was increased to maintain a good control of PD symptoms: the patient immediately presented severe uncontrolled dyskinesias, fever (39°C), confusional state and respiratory failure. Blood tests revealed increase of creatinine kinase (CK) and leucocytes; bacteriological blood and urine cultures were negative. Antiparkinsonian drugs were progressively reduced until the suspension; parenteral fluids, benzodiazepines and antipyretics were administered.

Results: After 2 days, dyskinesias disappeared and the patient presented rest tremor, rigidity, normal mental status and no fever. CK levels and leucocytes became normal. After 3 days, dopaminergic therapy was readministered and slowly increased, without side effects.

Conclusions: Pathogenesis of DHS is still not well understood: some characteristics are similar to parkinsonism hyperpyrexia syndrome but, in contrast, DHS is probably the consequence of an excessive dopaminergic replacement in basal ganglia and hypothalamus, influencing the origin of dyskinetic status, confusion and altered body temperature regulation. PD patients treated with STN-DBS can rarely face a sudden interruption of stimulation: an increase of antiparkinsonian drugs is often required to avoid the acute worsening of PD symptoms. We suggest a careful monitoring of these patients for the possible risk of DHS onset.

« Back to 2018 International Congress

MDS Abstracts - https://www.mdsabstracts.org/abstract/dyskinesia-hyperpyrexia-syndrome-dhs-after-sudden-interruption-of-deep-brain-stimulation-and-increase-of-oral-dopaminergic-therapy-a-case-report/