Parkinsonism-Hyperpyrexia Syndrome and Dyskinesia-Hyperpyrexia Syndrome in Parkinson’s Disease: Two Cases and Literature Review

Abstract

Parkinsonism-hyperpyrexia syndrome (PHS) and dyskinesia-hyperpyrexia syndrome (DHS) are rare but exhibit life-threatening complications in Parkinson’s disease (PD). We herein presented two cases of PD patients and performed a comprehensive and comparative literature review for these two syndromes. The first case was diagnosed as PHS with cerebral salt wasting syndrome caused by abrupt withdrawal of antiparkinsonian medication. Her symptoms were gradually remitted with reinstitution of the medication. The second one was an early-stage PD patient diagnosed as DHS in association with abuse of antiparkinsonian drugs. Her symptoms were gradually remitted with reduced dosage of dopaminergic drugs. Results of literature reviews revealed a total of 56 and 13 cases of PHS and DHS, respectively, and they were more likely to occur in elderly and long-term PD patients. These two syndromes showed different female-to-male ratio, similar mortality, and different recovery time. There were stark differences between PHS and DHS, including triggers (abrupt drug stoppage versus drug abuse), symptoms (worsened tremor and rigidity versus continuous dyskinesia), and treatment (drug reinstitution versus drug reduction). In summary, our reports and the review provide new insights into PHS and DHS in association with PD and may facilitate rapid discrimination of the syndromes for timely and proper treatment to reduce mortality.

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“…Parkinsonism hyperpyrexia syndrome (PHS) is a rare but serious reaction 20 . Istradefylline is an antagonist of adenosine A2a receptors, which often coexist with dopamine D2 receptors on many neurons.…”

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“…It is often caused by the abuse of antiparkinsonian drugs. Several other triggering factors for DHS include infection, hot weather, dehydration, and trauma ( 2 , 4 , 5 ). Only a few cases have been reported thus far ( 4 , 6 – 12 ) and few have described the neuroimaging features of DHS ( 4 , 9 ).…”

“…It is characterized by the acute onset of persistent systemic dyskinesia associated with increased creatine kinase (CK) levels, hyperpyrexia, and an altered mental state, and affects patients with advanced PD ( 2 , 3 ). It typically occurs in patients with long disease durations undergoing high daily dopaminergic doses and can be triggered by changes in dopaminergic therapy, infections, hot weather, dehydration, or trauma ( 2 , 4 , 5 ). Although this rare acute complication has been reported in recent years, neuroimaging of DHS has been underreported.…”

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“…Based on the reported cases, DHS has a mortality rate of 15.4%. The pathogenesis of DHS remains unclear, though a number of factors including antiparkinsonian drug abuse/change, infection, heatwave, gastrointestinal dysmotility, and trauma have been reported as potential triggers [5].…”

“…The remission of DHS mainly relies on treatment of triggers, reduction of dopaminergic drugs, vital function support, and antipyretic measures [5]. DHS may recur in some PD patients, and it seems di cult to prevent the episodes [6,7].…”