Emergency Department Management of Patients With Complications of Chronic Neurologic Disease: Parkinson Disease, Myasthenia Gravis, and Multiple Sclerosis (Pharmacology CME)

Table of Contents

 

About This Issue

The chronic neurologic diseases, Parkinson, myasthenia gravis, and multiple sclerosis (MS), can cause or complicate ED patient presentations, requiring swift recognition and targeted management. This issue summarizes the etiology, complications, and management of patients with these diseases. In this issue, you will learn:

The most common mimics of Parkinson disease and the importance of maintaining consistent carbidopa/levodopa drug delivery during a patient’s ED stay.

The various precipitants of myasthenic crisis, including infection and medications.

How to identify impending respiratory failure in myasthenia gravis patients, and when typical ventilation assessment parameters are insufficient.

Safe ventilation and intubation strategies for patients who present with myasthenic crisis.

The stages of MS, with ED presentations that are common, repeat, or inconsistent, whether disease is known or unknown.

Evidence-based diagnostic strategies for MS patients, including imaging and lumbar puncture.

When outpatient imaging and diagnostic studies for MS patients may be appropriate, and when they can be completed in the ED.

How pregnancy affects the course of MS, and specific management strategies for these patients.

1. About This Issue
2. Abstract
3. PART 1: Parkinson Disease
   1. Case Presentation
   2. Introduction
   3. Critical Appraisal of the Literature
   4. Etiology and Pathophysiology
   5. Prehospital Care
   6. Emergency Department Evaluation
      1. History
      2. Physical Examination
   7. Differential Diagnosis
   8. Diagnostic Testing
   9. Treatment
   10. Controversies and Cutting Edge
   11. Disposition
   12. Summary
   13. Case Conclusion
   14. Time- and Cost-Effective Strategies
4. PART 2: Myasthenia Gravis
   1. Case Presentation
   2. Introduction
   3. Critical Appraisal of the Literature
   4. Etiology and Pathophysiology
   5. Prehospital Care
   6. Emergency Department Evaluation
      1. History
      2. Physical Examination
         1. Negative Inspiratory Force Testing
   7. Differential Diagnosis
   8. Diagnostic Studies
   9. Treatment
   10. Disposition
   11. Controversies and Cutting Edge
   12. Time- and Cost-Effective Strategies
   13. Summary
   14. Case Conclusion
   15. Clinical Pathway for Emergency Department Management of Patients With Myasthenia Gravis
5. PART 3: Multiple Sclerosis
   1. Case Presentation
   2. Introduction
   3. Critical Appraisal of the Literature
   4. Etiology and Pathophysiology
      1. Stages of Disease
         1. Radiologically Isolated Syndrome
         2. Clinically Isolated Syndrome
         3. Relapsing-Remitting Multiple Sclerosis
         4. Primary Progressive Multiple Sclerosis
         5. Secondary Progressive Multiple Sclerosis
   5. Differential Diagnosis
   6. Prehospital Care
   7. Emergency Department Evaluation
      1. History and Physical Examination
      2. Common Presentations
      3. Inconsistent Presentations
      4. Repeat Presentations
   8. Diagnostic Studies
      1. Laboratory Testing
      2. Imaging
         1. Ultrasound
      3. Magnetic Resonance Imaging
      4. Lumbar Puncture
      5. Framework for Diagnostic Testing Decisions
   9. Treatment
      1. Chronic Medications and Side Effects
         1. Platform Therapies
         2. Higher-Efficacy Disease-Modifying Therapies
   10. Special Considerations
       1. Pediatric Patients
       2. Pregnant Patients
       3. Complications of Multiple Sclerosis
          1. Infections
          2. Spasticity
   11. Disposition
       1. Palliative Care
   12. Summary
   13. Case Conclusion
6. Risk Management Pitfalls for Parkinson Disease, Myasthenia Gravis, and Multiple Sclerosis
7. 5 Things That Will Change Your Practice
8. Tables and Figures
9. References

Abstract

This issue reviews 3 neurological conditions that can cause or complicate a patient‘s emergency department presentation: Parkinson disease, myasthenia gravis, and multiple sclerosis. Parkinson disease is a common comorbidity, and can be exacerbated in the ED if not managed expeditiously. Myasthenia gravis carries the potential for significant respiratory compromise and requires swift recognition and emergency care. Multiple sclerosis has many manifestions, stages, and treatments that emergency clinicians need to understand to best manage flares. This review presents a summary of the presentations, the common pitfalls, and the roles of various therapeutics in managing patients with chronic neurologic disease.

PART 1: Parkinson Disease

Case Presentations

A 68-year-old man with a history of Parkinson disease presents by EMS to the ED with chest pain…

• The patient was given an aspirin by EMS, but he said he missed his routine morning medications.
• His Parkinson disease is managed with carbidopa/levodopa 25 mg/100 mg orally 3 times daily. His initial vital signs are: temperature, 37°C; heart rate, 88 beats/min; blood pressure, 130/80 mm Hg; and oxygen saturation, 98% on room air.
• He has had 2 electrocardiograms, and both were normal sinus rhythm with no signs of ischemia.
• The patient has been waiting in the ED for 8 hours for the results of a second troponin test. He attempts to stand, using his cane, but he falls and sustains a subdural hematoma. Risk management contacts you and inquires whether this adverse event was preventable…

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Risk Management Pitfalls for Parkinson Disease, Myasthenia Gravis, and Multiple Sclerosis

1. “That Parkinson patient had a feeding tube that fell out. I thought it could be replaced in the morning.” The tube may not be a feeding tube; it may be used for a continuous enteral carbidopa/levodopa administration. Without it, the patient may not be receiving their medication, and it needs to be replaced immediately.

4. “The oxygen saturation for my myasthenia gravis patient was fine!” Myasthenia gravis is fundamentally a problem of ventilation rather than oxygenation. The first metabolic sign is hypercarbic respiratory acidosis, although even this may be compensated. A normal oxygen saturation does not indicate normal ventilation but rather, normal oxygenation.

9. “It's OK if the MS patient‘s baclofen pump stopped. We can give baclofen orally.” Oral baclofen doses do not achieve the same effects as IV or intrathecal dosing. Abrupt withdrawal is a true emergency and must be managed aggressively with high doses of IV GABA-ergic medications such as benzodiazepines and/or propofol. MRI may reset the pump settings, causing an abrupt, occult drop in dosage.

Tables and Figures

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Key References

Following are the most informative references cited in this paper, as determined by the authors.

11. * Cheron J, Deviere J, Supiot F, et al. The use of enteral access for continuous delivery of levodopa-carbidopa in patients with advanced Parkinson’s disease. United European Gastroenterol J. 2017;5(1):60-68. (Retrospective cohort; 27 patients) DOI: 10.1177/2050640616650804

12. * Sharma VD, Patel M, Miocinovic S. Surgical treatment of Parkinson’s disease: devices and lesion approaches. Neurotherapeutics. 2020;17(4):1525-1538. (Review) DOI: 10.1007/s13311-020-00939-x

13. * Dresser L, Wlodarski R, Rezania K, et al. Myasthenia gravis: epidemiology, pathophysiology and clinical manifestations. J Clin Med. 2021;10(11):2235. (Review) DOI: 10.3390/jcm10112235

15. * Gummi RR, Kukulka NA, Deroche CB, et al. Factors associated with acute exacerbations of myasthenia gravis. Muscle Nerve. 2019;60(6):693-699. (Retrospective chart review; 127 patients) DOI: 10.1002/mus.26689

21. * Sanders DB, Wolfe GI, Benatar M, et al. International consensus guidance for management of myasthenia gravis: executive summary. Neurology. 2016;87(4):419-425. (Consensus guidance) DOI: 10.1212/WNL.0000000000002790

25. * Elsheikh B, Arnold WD, Gharibshahi S, et al. Correlation of single-breath count test and neck flexor muscle strength with spirometry in myasthenia gravis. Muscle Nerve. 2016;53(1):134-136. (Prospective single blind study; 31 patients) DOI: 10.1002/mus.24929

38. * Kraker JA, Xu SC, Flanagan EP, et al. Ocular manifestations of multiple sclerosis: a population-based study. J Neuroophthalmol. 2024;44(2):157-161. (Population-based retrospective cohort study; 200 patients) DOI: 10.1097/WNO.0000000000002116

40. * Pelletier J, Sugar D, Koyfman A, et al. Multiple sclerosis: an emergency medicine-focused narrative review. J Emerg Med. 2024;66(4):e441-e456. (Narrative review) DOI: 10.1016/j.jemermed.2023.12.003

46. * Aletreby W, Alharthy A, Brindley PG, et al. Optic nerve sheath diameter ultrasound for raised intracranial pressure: a literature review and meta-analysis of its diagnostic accuracy. J Ultrasound Med. 2022;41(3):585-595. (Systematic review and meta-analysis; 18 studies) DOI: 10.1002/jum.15732

49. * Pakpoor J, Saylor D, Izbudak I, et al. Emergency department MRI scanning of patients with multiple sclerosis: worthwhile or wasteful? AJNR Am J Neuroradiol. 2017;38(1):12-17. (Retrospective cohort study; 142 patients) DOI: 10.3174/ajnr.A4953

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Keywords: Parkinson, myasthenia gravis, multiple sclerosis, tremor, dyskinesia, rigidity, carbidopa, levodopa, respiratory, beta blocker, neuromuscular, negative inspiratory force, BPAP, diplopia, pseudoflare, relapse, optic, Lhermitte, McDonald, MRI, lumbar puncture, corticosteroids, baclofen