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
Etiology
- Sporadic or idiopathic
- Disorders presenting with parkinsonism:
- Drug induced:
- Parkinsonism-hyperpyrexia syndrome (dopaminergic drug withdrawal)
- Amphotericin B
- Chemotherapeutic drugs
- Neuroleptic treatment induced
- Toxins:
- Carbon monoxide
- Methanol
- Cyanide
- Organophosphate poisoning
- 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine
- Brain lesions:
- Basal ganglia stroke
- Midbrain lesions
- Hydrocephalus
- Infections:
- Mycoplasma
- Viral encephalitis
- Other:
- Central pontine myelinosis
- Encephalitis lethargica (autoantibodies against basal ganglia antigens)
Diagnosis
Signs and Symptoms
- Nonmotor vs. motor symptoms:
- Nonmotor:
- Orthostatic hypotension
- Constipation
- Delayed gastric emptying
- Dysphagia
- Pain sensory dysfunction
- Depression
- Hallucinations
- Dementia
- Sleep disorders
- 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
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 consciousness
- Complications:
- Acute renal failure
- Venothrombosis
- Disseminated intravascular coagulation
- Rhabdomyolysis
- Autonomic 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 sudden
- History of potential causes of a Parkinson-like syndrome
- Patients with established Parkinson disease (PD):
- Sudden change in baseline motor function
- Change in mental status
- Should 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 bromocriptine
- Supportive
- Treat complications
Medication
- PD:
- Amantadine: 100 mg BID
- Stimulates dopamine release
- Benztropine: 0.5 " “1 mg TID
- Anticholinergic
- Limited use in tremor-dominant PD
- Carbidopa/levodopa: 25/100 mg TID
- Carbidopa lessens peripheral side effects and increased levodopa CNS bioavailability
- Levadopa is direct precursor to dopamine
- Entacapone: 200 mg PO BID " “QID
- Adjunct therapy; should be administered concomitantly with carbidopa/levodopa
- Increases CNS levadopa bioavailability
- MAO inhibitors
- May be used in mild disease as first-line therapy
- 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
- Parkinsonism-hyperpyrexia syndrome:
- Levodopa: 50 " “100 mg IV over 3 hr
- Bromocriptine: 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)