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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))

Abstract

Parkinsonism-hyperpyrexia syndrome (PHS) is a rare but potentially life-threatening complication of Parkinson’s disease (PD). PHS was first described in 1981 and has been reported in dozens of cases since then. The clinical presentation of PHS includes hyperpyrexia, rigidity, altered consciousness, dysautonomia, leukocytosis, and elevated creatine kinase. Although PHS and NMS are phenotypically nearly identical, PHS is a distinct entity in that it is triggered by removal or effective loss of dopaminergic therapy in a parkinsonian patient. The mainstay of PHS treatment is rapid replacement of effective anti-parkinsonian therapy. While abrupt levodopa withdrawal is the classic trigger of PHS, a variety of other inciting scenarios have been described. The pathophysiology underlying PHS is generally accepted to be a hypo-dopaminergic state, and its clinical features can be explained as sequelae of central dopamine depletion. PHS is a neurological emergency, with significant morbidity and mortality. Early recognition and rapid reintroduction of anti-parkinsonian medications are keys to the successful management of this syndrome.

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Author information

Authors and Affiliations

  1. Department of Neurology and Emory Movement Disorders Clinic, Emory University School of Medicine, Atlanta, GA, USA

    Daniel E. Huddleston M.D.

  2. Department of Neurology, Emory University School of Medicine, 1841 Clifton Road NE, Atlanta, GA, 30329, USA

    Stewart A. Factor D.O.

Authors
  1. Daniel E. Huddleston M.D.
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  2. Stewart A. Factor D.O.
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Corresponding author

Correspondence to Stewart A. Factor D.O. .

Editor information

Editors and Affiliations

  1. , Neurology, Columbia University Medical Center, West 168th St. Suite 310 710, New York, 10032, USA

    Steven J. Frucht

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Huddleston, D.E., Factor, S.A. (2013). Parkinsonism-Hyperpyrexia Syndrome in Parkinson’s Disease. In: Frucht, S. (eds) Movement Disorder Emergencies. Current Clinical Neurology. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-60761-835-5_3

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  • DOI: https://doi.org/10.1007/978-1-60761-835-5_3

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  • Publisher Name: Humana Press, Totowa, NJ

  • Print ISBN: 978-1-60761-834-8

  • Online ISBN: 978-1-60761-835-5

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