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Parkinsonism-Hyperpyrexia Syndrome in Parkinson’s Disease

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Movement Disorder Emergencies

Part of the book series: Current Clinical Neurology ((CCNEU))

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Abstract

In this chapter, we cover three related emergency syndromes seen in the background of Parkinson’s disease. The primary focus is parkinsonism-hyperpyrexia syndrome (PHS), a potentially life-threatening complication. PHS results from a hypodopaminergic state, generally caused by abrupt interruption of treatment or in relationship to anything that changes the serum and brain levels such as motor fluctuations, diet, medical illness, even when the patient is treated with deep brain stimulation. The clinical presentation of PHS is similar to neuroleptic malignant syndrome and includes the clinical features and lab results of hyperpyrexia, severe muscle rigidity, altered consciousness, dysautonomia, leukocytosis, and elevated creatine kinase. The mainstay of PHS treatment is supportive therapy and rapid replacement of effective anti-parkinsonian therapy. The second syndrome is the deep brain stimulation withdrawal syndrome (DBS-WDS). This occurs when DBS is abruptly turned off and most commonly occurs with battery failure. The symptoms are similar to PHS but replacing dopaminergic drugs is not always helpful, suggesting a different mechanism. Finally, the rarest of these syndromes is dyskinesia-hyperpyrexia syndrome (DHS). This disorder is the result of a hyperdopaminergic state and is characterized by severe dyskinesia, fever, muscle breakdown, and renal failure. Early recognition is the key to treating all these potentially fatal syndromes. Prevention is the best possible approach for all whenever possible.

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Authors and Affiliations

  1. Department of Neurology, Emory University School of Medicine, Atlanta, GA, USA

    Daniel E. Huddleston

  2. Movement Disorders Program, Vance Lanier Chair for Neurology, Emory University School of Medicine, Atlanta, GA, USA

    Stewart A. Factor

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  1. Daniel E. Huddleston
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  2. Stewart A. Factor
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Correspondence to Stewart A. Factor .

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  1. Department of Neurology, NYU Langone Health, New York, NY, USA

    Steven J. Frucht

Electronic Supplementary Material

A young man with familial Lewy-body parkinsonism was maintained on a regimen of levodopa taken every 2 hours throughout the day. He developed a viral respiratory infection, and missed taking his medications for 36 hours. Soon thereafter he was admitted to the intensive care unit after developing fever, obtundation, and an elevated creatine kinase of 2,000. Prominent rigidity and lower extremity movements (likely myoclonus) are seen. He was diagnosed with the parkinsonism-hyperpyrexia syndrome. Hourly levodopa was administered via the nasogastric tube along with his other Parkinson medications. He began to improve and his CK fell; however, enteral feedings interfered with the absorption of the levodopa and he temporarily worsened. Shifting the feeding to the night, with levodopa administered during the day, solved this problem. Several months later he returned for follow-up, with mild residual parkinsonism and reduced arm swing. (MP4 118639 kb)

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Huddleston, D.E., Factor, S.A. (2022). Parkinsonism-Hyperpyrexia Syndrome in Parkinson’s Disease. In: Frucht, S.J. (eds) Movement Disorder Emergencies. Current Clinical Neurology. Humana, Cham. https://doi.org/10.1007/978-3-030-75898-1_5

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  • DOI: https://doi.org/10.1007/978-3-030-75898-1_5

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