What is the management of Parkinson hyperpyrexia syndrome?

Management of Parkinson Hyperpyrexia Syndrome

Parkinson hyperpyrexia syndrome (PHS) is a life-threatening emergency requiring immediate dopaminergic medication replacement, supportive care, and treatment of complications to prevent mortality and long-term sequelae.

Definition and Clinical Presentation

PHS is a rare but potentially fatal complication seen in Parkinson's disease patients that resembles neuroleptic malignant syndrome (NMS). The clinical presentation includes:

• Hyperthermia/fever
• Severe muscle rigidity
• Altered mental status/confusion
• Autonomic instability (tachycardia, hypertension, tachypnea)
• Elevated serum creatine kinase (CK)

Common Triggers

The most common triggers for PHS include:

• Sudden withdrawal or reduction of anti-parkinsonian medications (especially levodopa) 1, 2
• Deep brain stimulator (DBS) battery depletion or malfunction 3, 2, 4
• DBS surgery when antiparkinsonian medications are discontinued 5

Immediate Management

1. Urgent dopaminergic medication replacement

   • Restore previous antiparkinsonian medication regimen immediately
   • If oral intake is compromised, administer medications via nasogastric tube 5
   • For patients with DBS, urgent battery replacement if depletion is the cause 3, 4

2. Supportive care

   • Aggressive intravenous fluid resuscitation to prevent dehydration and renal failure
   • Temperature management for hyperthermia
   • Cardiovascular support for autonomic instability
   • Close monitoring in an intensive care setting

3. Specific pharmacological interventions

   • Dantrolene may be considered for severe rigidity 3
   • Bromocriptine (dopamine agonist) can be used as adjunctive therapy 6
   • Avoid benzodiazepines as they may worsen cognitive symptoms and increase fall risk 7

Management of Complications

Monitor for and aggressively treat common complications:

• Acute renal failure (due to rhabdomyolysis)
• Aspiration pneumonia
• Deep venous thrombosis/pulmonary embolism
• Disseminated intravascular coagulation (DIC)
• Electrolyte abnormalities

Diagnostic Workup

• Serum creatine kinase (CK) levels - typically elevated
• Complete blood count - to assess for leukocytosis
• Renal function tests - to monitor for acute kidney injury
• Coagulation studies - to rule out DIC
• Blood cultures - to rule out sepsis (important differential diagnosis)
• Urinalysis - for myoglobinuria

Differential Diagnosis

PHS can mimic several conditions, most importantly:

• Sepsis/infection
• Neuroleptic malignant syndrome (NMS)
• Serotonin syndrome
• Malignant hyperthermia
• Central anticholinergic toxicity
• Heat stroke

Prevention Strategies

• Patient and caregiver education about the dangers of abrupt medication discontinuation
• Careful planning for surgical procedures requiring medication adjustments
• Regular monitoring of DBS battery status
• Gradual rather than abrupt medication changes when necessary

Prognosis

With prompt recognition and appropriate management, outcomes can be improved, but:

• Mortality rates of up to 4% have been reported 1
• Approximately one-third of patients may have permanent sequelae 1

Common Pitfalls to Avoid

• Misdiagnosing PHS as sepsis, delaying appropriate treatment
• Failing to recognize DBS battery depletion as a potential cause
• Not restoring dopaminergic medications quickly enough
• Inadequate fluid resuscitation
• Overlooking complications such as rhabdomyolysis and renal failure

PHS should always be considered in any patient with Parkinson's disease who presents with fever, rigidity, altered mental status, and autonomic instability, especially following recent changes in medication regimen or DBS function.