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Parkinson Disease, Emergency Medicine

Basics

Description

- Gradual progressive neurologic disorder of middle or late life - Degeneration of dopaminergic neurons in the substantia nigra - Development of Lewy bodies in the residual dopaminergic neurons - Accelerated cortical atrophy - Can begin unilaterally, but generalizes to symmetric - Affects 1% of people >60 yr; 4% >80 yr - May have symptoms 20 yr prior to diagnosis - Nonspecific: - Fatigue - Constipation - Hyposomia

- Sporadic or idiopathic - Disorders presenting with parkinsonism: - Drug induced: - Parkinsonism-hyperpyrexia syndrome (dopaminergic drug withdrawal) - Amphotericin B - Chemotherapeutic drugs - Neuroleptic treatment induced

- Brain lesions: - Basal ganglia stroke - Midbrain lesions - Hydrocephalus

- Infections: - Mycoplasma - Viral encephalitis

- Motor symptoms: - "Pill-rolling " ¯ resting tremor - "Cog-wheel " ¯ rigidity due to increased muscular tone - Stooped posture and instability of posture - Bradykinesia: Extreme slowness in movement - "Masked face " ¯ appearance

- Treatment with antiparkinsonian medications can be initiated in the ED to alleviate symptoms - Consultation with neurology for recommended medication regimens and ongoing support and monitoring is prudent - For patients with mild disease, no medication may be required - For moderate disease, anticholinergic medications and dopaminergic medications should be used - Treat underlying infection, if present - Treat parkinsonism-hyperpyrexia syndrome: - Replace levodopa or bromocriptine - Supportive - Treat complications

- PD: - Amantadine: 100 mg BID - Stimulates dopamine release

- Benztropine: 0.5 " “1 mg TID - Anticholinergic - Limited use in tremor-dominant PD

- Entacapone: 200 mg PO BID " “QID - Adjunct therapy; should be administered concomitantly with carbidopa/levodopa - Increases CNS levadopa bioavailability

- Selegiline: 5 mg qam and noon - Rasagiline: 1 " “2 mg QD - Dopamine agonists: - Pramipexole: 0.5 " “1.5 mg PO TID - Ropinirole: 3 " “6 mg PO TID - Apomorphine: 0.2 " “0.6 mL SQ PRN

  • Gradual progressive neurologic disorder of middle or late life
  • Degeneration of dopaminergic neurons in the substantia nigra
  • Development of Lewy bodies in the residual dopaminergic neurons
  • Accelerated cortical atrophy
  • Can begin unilaterally, but generalizes to symmetric
  • Affects 1% of people >60 yr; 4% >80 yr
  • May have symptoms 20 yr prior to diagnosisNonspecific:FatigueConstipationHyposomia

Etiology

  • Sporadic or idiopathic
  • Disorders presenting with parkinsonism:Drug induced:Parkinsonism-hyperpyrexia syndrome (dopaminergic drug withdrawal)Amphotericin BChemotherapeutic drugsNeuroleptic treatment inducedToxins:Carbon monoxideMethanolCyanideOrganophosphate poisoning1-methyl-4-phenyl-1,2,3,6-tetrahydropyridineBrain lesions:Basal ganglia strokeMidbrain lesionsHydrocephalusInfections:MycoplasmaViral encephalitisOther:Central pontine myelinosisEncephalitis lethargica (autoantibodies against basal ganglia antigens)

Diagnosis

Signs and Symptoms

  • Nonmotor vs. motor symptoms:Nonmotor:Orthostatic hypotensionConstipationDelayed gastric emptyingDysphagiaPain sensory dysfunctionDepressionHallucinationsDementiaSleep disorders
  • Motor symptoms: "Pill-rolling " ¯ resting tremor "Cog-wheel " ¯ rigidity due to increased muscular toneStooped posture and instability of postureBradykinesia: Extreme slowness in movement "Masked face " ¯ appearance

History

  • Sudden change in baseline motor function or mental status:May be the only indication of systemic disease such as infection
  • Noncompliance (sudden withdrawal) of dopaminergic medications can lead to parkinsonism-hyperpyrexia syndrome:Rigidity, pyrexia, reduced consciousnessComplications:Acute renal failureVenothrombosisDisseminated intravascular coagulationRhabdomyolysisAutonomic instability

Physical Exam

  • Cog-wheel rigidity:Jerking movements when a muscle is passively stretched
  • Stooped posture
  • Pill-rolling tremor

Essential Workup

  • History is of primary importance:Diagnosis is made based on clinical findings
  • Important historical information includes:Onset of symptom, whether gradual or suddenHistory of potential causes of a Parkinson-like syndromePatients with established Parkinson disease (PD):Sudden change in baseline motor functionChange in mental statusShould prompt workup for infectious process

Diagnosis Tests & Interpretation

Lab

  • No specific or recommended lab studies necessary to confirm the diagnosis
  • Disorders presenting as PD may require directed lab studies as appropriate for suspected cause
  • Directed labs if suspect parkinsonism-hyperpyrexia syndrome

Imaging

  • CT and MRI are not required to diagnose PD but are often elements of evaluation for dementia
  • CXR may be indicated for any signs of respiratory tract infection

Differential Diagnosis

  • Benign familial tremor
  • Major depression
  • Wilson disease
  • Huntington disease
  • Alzheimer disease
  • Creutzfeldt " “Jakob disease
  • Carbon monoxide poisoning
  • B12 deficiency
  • Hydrocephalus
  • Multi-infarct dementia
  • Essential tremor disorders
  • Hypothyroidism
  • Dementia with Lewy bodies

Treatment

Ed Treatment/Procedures

  • Treatment with antiparkinsonian medications can be initiated in the ED to alleviate symptoms
  • Consultation with neurology for recommended medication regimens and ongoing support and monitoring is prudent
  • For patients with mild disease, no medication may be required
  • For moderate disease, anticholinergic medications and dopaminergic medications should be used
  • Treat underlying infection, if present
  • Treat parkinsonism-hyperpyrexia syndrome:Replace levodopa or bromocriptineSupportiveTreat complications

Medication

  • PD:Amantadine: 100 mg BIDStimulates dopamine releaseBenztropine: 0.5 " “1 mg TIDAnticholinergicLimited use in tremor-dominant PDCarbidopa/levodopa: 25/100 mg TIDCarbidopa lessens peripheral side effects and increased levodopa CNS bioavailabilityLevadopa is direct precursor to dopamineEntacapone: 200 mg PO BID " “QIDAdjunct therapy; should be administered concomitantly with carbidopa/levodopaIncreases CNS levadopa bioavailabilityMAO inhibitorsMay be used in mild disease as first-line therapySelegiline: 5 mg qam and noonRasagiline: 1 " “2 mg QDDopamine agonists:Pramipexole: 0.5 " “1.5 mg PO TIDRopinirole: 3 " “6 mg PO TIDApomorphine: 0.2 " “0.6 mL SQ PRN
  • Parkinsonism-hyperpyrexia syndrome:Levodopa: 50 " “100 mg IV over 3 hrBromocriptine: 7.5 " “15 mg PO TID

First Line

Carbidopa/levodopa ‚

Follow-Up

Disposition

Admission Criteria

  • Patients with previously diagnosed Parkinson with infections, trauma, cardiovascular emergencies, cerebrovascular emergencies, GI emergencies, electrolyte disturbances, altered mental status, or other medical problems
  • Depression with intent to do self-harm
  • Confirm diagnosis and levodopa responsiveness
  • Medication complications (parkinsonism-hyperpyrexia syndrome)
  • Management of motor fluctuations and dyskinesias
  • Inability to go home secondary to elder abuse
  • Complications from deep brain stimulation devices (e.g., headache, infection, mental status change)
  • Failure to thrive

Discharge Criteria

  • Mild to moderate disease without medications
  • Moderate to severe disease with medications and urgent neurologic outpatient follow-up

Followup Recommendations

Discuss prevention strategies in disease management ‚

Pearls and Pitfalls

  • Diagnosis is often difficult; keep in mind other conditions commonly misdiagnosed as PD
  • Sudden withdrawal of dopaminergic medications can result in parkinsonism-hyperpyrexia syndrome, a medical emergency

Additional Reading

  • Chou ‚ KL. In the clinic. Parkinson disease. Ann Intern Med. 2012,157:ITC5-1 " “ITC5-16.
  • Gazewood ‚ JD, Richards ‚ DR, Clebak ‚ K. Parkinson disease: An update. Am Fam Physician. 2013;15:267 " “273.
  • Grinberg ‚ LT, Rueb ‚ U, Alho ‚ AT, et al. Brainstem pathology and non-motor symptoms in PD. J Neurol Sci. 2010;289:81 " “88.
  • Kipps ‚ CM, Fung ‚ VSC, Grattan-Smith ‚ P, et al. Movement disorder emergencies. Mov Disord. 2005;20:322 " “334.
  • Newman ‚ EJ, Grosset ‚ DG, Kennedy ‚ PG. The parkinsonism-hyperpyrexia syndrome. Neurocrit Care. 2009;10:136 " “140.
  • Worth ‚ PF. How to treat Parkinsons disease in 2013. Clin Med. 2013;13:93 " “96.

Codes

ICD9

  • 332.0 Paralysis agitans
  • 332.1 Secondary parkinsonism
  • 333.0 Other degenerative diseases of the basal ganglia
  • 332 Parkinsons disease

ICD10

  • G20 Parkinsons disease
  • G21.9 Secondary parkinsonism, unspecified
  • G21.19 Other drug induced secondary parkinsonism
  • G90.3 Multi-system degeneration of the autonomic nervous system
  • G21.11 Neuroleptic induced parkinsonism
  • G21.2 Secondary parkinsonism due to other external agents
  • G21.3 Postencephalitic parkinsonism
  • G21.8 Other secondary parkinsonism

SNOMED

  • 49049000 Parkinsons disease (disorder)
  • 230292008 Secondary parkinsonism (disorder)
  • 4223005 Parkinsonism due to drug (disorder)
  • 192835007 Parkinsonism with orthostatic hypotension (disorder)
  • 72820004 neuroleptic-induced Parkinsonism (disorder)