Parkinsonism-Hyperpyrexia Syndrome — SCE Neurology MCQ

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HardMovement DisordersParkinsonism-Hyperpyrexia SyndromeSCE Neurology

A 72-year-old woman with advanced Parkinson's disease presents with sudden onset of severe rigidity, hyperthermia (39.5°C), tachycardia, diaphoresis, and altered consciousness. Her co-careldopa was abruptly stopped 48 hours ago by a hospital team unfamiliar with her medications. CK is 8,500 IU/L. What is the diagnosis?

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Correct answer: C — Parkinsonism-hyperpyrexia syndrome (neuroleptic malignant-like syndrome)

Parkinsonism-hyperpyrexia syndrome (also called neuroleptic malignant-like syndrome or dopaminergic withdrawal syndrome) occurs when dopaminergic medication is abruptly withdrawn in Parkinson's disease. It presents identically to NMS (rigidity, hyperthermia, autonomic instability, raised CK, altered consciousness) and is a medical emergency. Treatment is immediate reinstatement of dopaminergic therapy, IV fluids, dantrolene, and cooling. A: Serotonin syndrome has clonus and hyperreflexia as distinguishing features. B: NMS is caused by dopamine antagonists, not dopaminergic withdrawal, though the pathophysiology overlaps. D: Malignant hyperthermia is anaesthesia-related. E: Sepsis is possible but the temporal relationship to medication withdrawal is diagnostic.

Reference: NICE NG71 Parkinson's Disease (2017); Newman et al. Movement Disorders (2009)