Movement Disorders in Children: Recognition and Management in the Emergency Department

Table of Contents

 

About This Issue

This issue reviews 3 less-common neurologic conditions that commonly impact movement: acute cerebellar ataxia (ACA), anti-N-methyl-D-aspartate receptor encephalitis (NMDARE), and acute disseminated encephalomyelitis (ADEM). The common presentations, evaluation, and management of these conditions in the emergency department are discussed. In this issue, you will learn:

Common causes of ACA, NMDARE, and ADEM

The differential diagnosis of abnormal movements in children, including those with hyperkinetic movements, hypokinetic movements, and ataxic movements

Key historical and physical examination findings that will help narrow the differential diagnosis

Which diagnostic studies are preferred and when they are indicated

Recommendations for treatment of NMDARE and ADEM in the emergency department

Which patients can be discharged with appropriate follow-up, which should be transferred, and which need to be admitted to the intensive care unit

1. About This Issue
2. Abstract
3. Case Presentations
4. Introduction
5. Critical Appraisal of the Literature
6. Etiology and Pathophysiology
   1. Acute Cerebellar Ataxia
   2. Anti-N-Methyl-D-Aspartate Receptor Encephalitis
   3. Acute Disseminated Encephalomyelitis
7. Differential Diagnosis
   1. Hyperkinetic Movements
   2. Hypokinetic Movements
   3. Ataxic Movements
   4. Causes of Movement Problems
8. Prehospital Care
   1. Acute Cerebellar Ataxia
   2. Anti-N-Methyl-D-Aspartate Receptor Encephalitis
   3. Acute Disseminated Encephalomyelitis
9. Emergency Department Evaluation
   1. History
      1. Acute Cerebellar Ataxia
      2. Anti-N-Methyl-D-Aspartate Receptor Encephalitis
      3. Acute Disseminated Encephalomyelitis
   2. Physical Examination
      1. Acute Cerebellar Ataxia
      2. Anti-N-Methyl-D-Aspartate Receptor Encephalitis
      3. Acute Disseminated Encephalomyelitis
10. Diagnostic Studies
    1. Acute Cerebellar Ataxia
    2. Anti-N-Methyl-D-Aspartate Receptor Encephalitis
    3. Acute Disseminated Encephalomyelitis
11. Treatment
    1. Acute Cerebellar Ataxia
    2. Anti-N-Methyl-D-Aspartate Receptor Encephalitis
    3. Acute Disseminated Encephalomyelitis
12. Special Considerations
    1. Acute Cerebellar Ataxia
    2. Anti-N-Methyl-D-Aspartate Receptor Encephalitis
13. Controversies and Cutting Edge
    1. COVID-19
    2. Anti-N-Methyl-D-Aspartate Receptor Encephalitis
    3. Acute Disseminated Encephalomyelitis
14. Disposition
    1. Acute Cerebellar Ataxia
    2. Anti-N-Methyl-D-Aspartate Receptor Encephalitis
    3. Acute Disseminated Encephalomyelitis
15. Summary
16. 5 Things That Will Change Your Practice
17. Risk Management Pitfalls to Avoid in Children With Movement Disorders
18. Cost-Effective Strategies
19. Case Conclusions
20. Clinical Pathway for Management of Children With Movement Disorders
21. Tables
22. References

Abstract

The differential diagnosis for pediatric patients presenting to the emergency department with movement-based neurological complaints is wide. Clinicians must ensure these children receive an appropriate evaluation to recognize rare diseases. Early recognition of these diagnoses helps facilitate coordination with specialists, guides imaging, and ensures proper management and disposition. This issue reviews 3 less-common movement disorders: acute cerebellar ataxia, anti-N-methyl-D-aspartate receptor encephalitis, and acute disseminated encephalomyelitis. The common presentations, evaluation, and management of these conditions in the emergency department are discussed.

Case Presentations

A previously healthy 6-year-old boy presents via EMS with concern for possible seizure-like activity…

• According to the boy’s mother, he had been complaining of headache for the past 2 days. This morning, he complained that his “stomach hurt,” he vomited once, and then shortly after stared off to the side with right-sided facial twitching. The mother states that a week and a half ago he had a fever and cold symptoms, and he was diagnosed with a viral infection by his primary physician. She says his symptoms resolved within a few days, and he seemed to have fully recovered.
• Upon arrival, the boy’s vital signs are within normal limits for age, and he is afebrile. He is somnolent and not answering your questions, but he is able to follow commands. You are concerned that there may be a very slight facial droop on the right side. The boy’s pupils are equal and reactive, and his extraocular movements are intact. The boy demonstrates 5/5 strength of the extremities on the left, and 4/5 strength of the extremities on the right. His cardiovascular, pulmonary, HEENT, and abdominal examinations are benign.
• What tests or treatments should be initiated immediately? Which type of imaging is most appropriate for this patient? What is the correct disposition?

An otherwise healthy 3-year-old girl presents with an unsteady gait…

• The girl’s father reports that for the past 2 days she has been stumbling when she walks and seems to be walking “like she is drunk.” She has also seemed to have a harder time playing with her toys, but is in good spirits and does not seem ill. The father tells you the girl and her brother were sick 2 to 3 weeks ago with congestion, cough, and low-grade fever; however, both have been well since that time. There was no head trauma.
• The girl is well-appearing, smiling, and playful, but she tips over to the side when not supporting herself on the bed railings. The HEENT examination is benign. The girl’s pupils are equal, round, and reactive, with nystagmus noted with extraocular movements. Her strength is 5/5 in all extremities, reflexes are present in upper and lower extremities, and sensation is intact. When the girl reaches for your stethoscope, she misses by an inch or two with both hands. She walks with a broad-based gait, tilting to the side.
• What further history is important to obtain? What testing is indicated?

A 15-year-old girl presents with her parents after a seizure episode...

• Her parents report that over the last week the girl had been acting differently, and they were worried “she might be on drugs.” A couple of days ago, the girl started to complain about some body aches and low-grade fevers. Today, the girl had a 2-minute episode of generalized shaking of her upper and lower extremities, associated with urinary incontinence and upward eye rolling. After the episode, she was quite drowsy but interactive. This episode prompted her parents to bring her to the ED. They deny any prior episodes of seizure or family history of any seizure disorders.
• On arrival, the girl is awake and aggressively lashing out with verbal abuse at her parents and clinicians. She has no obvious signs of trauma. Her pupils are equal, round, and reactive, without nystagmus. Her mouth continually twitches during her interview, and she repeatedly sticks her tongue out. The remainder of her neurological examination appears nonfocal, though she is not very cooperative.
• Is this a common first-time seizure or is there more to be worried about? Should you consider potential drug ingestions?

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Clinical Pathway for Management of Children With Movement Disorders

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Tables

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Key References

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

6. * Thakkar K, Maricich SM, Alper G. Acute ataxia in childhood: 11-year experience at a major pediatric neurology referral center. J Child Neurol. 2016;31(9):1156-1160. (Retrospective; 120 patients) DOI: 10.1177/0883073816643407

12. * Huang Q, Xie Y, Hu Z, et al. Anti-N-methyl-D-aspartate receptor encephalitis: a review of pathogenic mechanisms, treatment, prognosis. Brain Res. 2020;1727:146549. (Review) DOI: 10.1016/j.brainres.2019.146549

19. * Pohl D, Alper G, Van Haren K, et al. Acute disseminated encephalomyelitis: updates on an inflammatory CNS syndrome. Neurology. 2016;87(9 Suppl 2):S38-S45. (Review) DOI: 10.1212/wnl.0000000000002825

26. * Overby P, Kapklein M, Jacobson RI. Acute ataxia in children. Pediatr Rev. 2019;40(7):332-343. (Review) DOI: 10.1542/pir.2017-0223

28. * Gurrera RJ. Recognizing psychiatric presentations of anti-NMDA receptor encephalitis in children and adolescents: a synthesis of published reports. Psychiatry Clin Neurosci. 2019;73(5):262-268. (Systematic review; 167 cases) DOI: 10.1111/pcn.12821

32. * Titulaer MJ, McCracken L, Gabilondo I, et al. Treatment and prognostic factors for long-term outcome in patients with anti-NMDA receptor encephalitis: an observational cohort study. Lancet Neurol. 2013;12(2):157-165. (Observational cohort study; 577 patients) DOI: 10.1016/S1474-4422(12)70310-1

33. * Abe KK, Koli RL, Yamamoto LG. Emergency department presentations of anti-N-methyl-D-aspartate receptor encephalitis. Pediatr Emerg Care. 2016;32(2):107-112. (Review) DOI: 10.1097/pec.0000000000000713

35. * George T, Basin A, Avva U, et al. Early recognition and treatment of acute disseminated encephalomyelitis in pediatrics: a case series. Pediatr Emerg Care. April 9, 2019. (Case series; 7 cases) DOI: 10.1097/pec.0000000000001771

38. * Ashizawa T, Xia G. Ataxia. Continuum (Minneap Minn). 2016;22(4 Movement Disorders):1208-1226. (Review) DOI: 10.1212/CON.0000000000000362

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Keywords: movement disorder, abnormal movement, neurological complaint, acute cerebellar ataxia, ACA, anti-N-methyl-D-aspartate receptor encephalitis, NMDARE, NMDA, NMDA encephalitis, NMDAR encephalitis, acute disseminated encephalomyelitis, ADEM, hyperkinetic movements, hypokinetic movements, ataxic movements