Parkinsonism-Hyperpyrexia Syndrome in Parkinson’s Disease
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.
KeywordsDeep Brain Stimulation Neuroleptic Malignant Syndrome Dopaminergic Medication Drug Holiday Dopaminergic Therapy
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