Makindo Medical Notes

Parkinson Hyperpyrexia Syndrome (PHS) is a rare but life-threatening condition that can occur in individuals with Parkinson’s disease (PD), often after the abrupt withdrawal or reduction of dopaminergic medications (e.g., levodopa) or due to severe illness, infection, or trauma.

Key Features

• High Fever (Hyperpyrexia): Sudden onset of a very high fever, typically above 38°C (100.4°F).
• Rigidity: Severe muscular rigidity, sometimes worse than typical PD symptoms.
• Autonomic Dysfunction: Can present as sweating, tachycardia (rapid heart rate), unstable blood pressure, and dehydration.
• Altered Mental Status: Confusion, agitation, or delirium; in severe cases, stupor or coma.
• Rhabdomyolysis and Acute Renal Failure: Breakdown of muscle tissue, which can lead to acute kidney injury.

Causes

• Abrupt reduction or discontinuation of dopaminergic medications.
• Intercurrent illness or infection (e.g., pneumonia, urinary tract infection).
• Trauma or surgery.
• Stressful medical conditions or dehydration.

Diagnosis

• Sudden fever, rigidity, altered mental status
• Stupor, dysarthria and dysphagia, autonomic impairment
• In context of recent medication changes or illness.

Laboratory tests may reveal

• Elevated creatine kinase (CK) due to muscle breakdown.
• Abnormal liver or kidney function.
• High WCC.
• Hypernatraemia and AKI due to dehydration or electrolyte imbalance.

Differential Diagnosis

• Neuroleptic Malignant Syndrome (NMS): Similar to PHS but typically associated with antipsychotic drugs.
• Sepsis: Rule out infection as the cause of fever and altered mental status.
• Malignant Hyperthermia: Typically associated with anesthesia exposure.
• Heat Stroke: Consider in the context of environmental heat exposure.

Complications

• Acute kidney injury
• Aspiration pneumonia and respiratory failure
• Sepsis
• DIC
• Thromboembolism
• Death

Management

• Restart Dopaminergic Medications: Resume levodopa usual dose or other dopaminergic agents promptly at the previous dose if possible. These should be given orally or if not possible via NG tube. If this is not possible then a Rotigotine patch can be considered at 2-4 mg/24 hrs. Where available Apomorphine 1.0-2.0 mg/hr can be used
• Hydration and Electrolyte Balance: Aggressive IV fluid administration to prevent dehydration and rhabdomyolysis-induced kidney failure.
• Cooling Measures: Use antipyretics (fever-reducing medications) and physical cooling methods (e.g., cooling blankets) to manage hyperpyrexia.
• ICU Admission: Severe cases may require intensive care, especially if complications such as acute renal failure occur. Some may need mechanical ventilation and haemodialysis.
• Muscle Relaxants: In severe cases of rigidity, medications such as Dantrolene 10 mg/kg per day in 4 divided doses may be considered.
• Palliation: may be appropriate after a trial of treatment in the setting of end stage disease.