Table of Contents

 

Acute demyelinating disorders can present with vague complaints and subtle abnormalities of the neurological examination. A thorough history and physical examination are important for narrowing the differential diagnosis and determining which diagnostic studies are indicated. This issue focuses on 2 of the more common pediatric demyelinating disorders that primarily affect the spinal cord: Guillain-Barré syndrome (GBS) and acute transverse myelitis (ATM). Evidence-based recommendations are provided for workup in the emergency department, treatment, and disposition. You will learn:

Typical and atypical presentations of GBS and ATM

Factors that can help differentiate between GBS and ATM

Key threats to watch out for, including respiratory distress and autonomic dysregulation

Which studies can help diagnose GBS, and which studies can help rule out other pathologies in order to make the diagnosis of ATM

The best methods for monitoring pulmonary function

Evidence-based treatment recommendations, such as when to treat with intravenous immunoglobulin, systemic corticosteroids, or plasmapheresis

How to determine when mechanical ventilation or intensive care unit admission is warranted

1. Abstract
2. Case Presentations
3. Introduction
4. Critical Appraisal of the Literature
5. Etiology and Pathophysiology
   1. Guillain-Barré Syndrome
   2. Acute Transverse Myelitis
6. Differential Diagnosis
7. Prehospital Care
   1. Guillain-Barré Syndrome
   2. Acute Transverse Myelitis
8. Emergency Department Evaluation
   1. History
      1. Guillain-Barré Syndrome
      2. Acute Transverse Myelitis
   2. Physical Examination
9. Diagnostic Studies
   1. Guillain-Barré Syndrome
   2. Acute Transverse Myelitis
10. Treatment
    1. Guillain-Barré Syndrome
    2. Acute Transverse Myelitis
11. Special Populations
12. Controversies and Cutting Edge
13. Disposition
14. Summary
15. Risk Management Pitfalls for Pediatric Patients With Acute Demyelinating Syndromes
16. Case Conclusions
17. Clinical Pathway for Management of Pediatric Patients With Acute Demyelinating Syndromes
18. Table
    1. Table 1. Distinguishing Factors in Guillain-Barré Syndrome and Acute Transverse Myelitis
19. References

Abstract

Acute demyelinating disorders can present with vague complaints and subtle abnormalities of the neurological examination. A thorough history and physical examination are important for narrowing the differential diagnosis and determining which diagnostic studies are indicated. This issue focuses on the most common acute demyelinating disorders in children: Guillain-Barré syndrome and acute transverse myelitis. Common presenting signs and symptoms of these conditions are reviewed, and evidence-based recommendations are provided for the initial assessment and management of Guillain-Barré syndrome and acute transverse myelitis in the emergency department.

Case Presentations

A 5-year-old boy presents to the ED with limping…

• The patient’s mother describes him as a healthy child who is up-to-date on vaccinations and has never required hospitalization. This morning, he was not as active as usual and stayed on the couch to watch TV. After lunch, she encouraged him to go outside and play, but he appeared to be dragging his feet and stumbling. She says that she had to assist him from the car to a wheelchair in the ED parking lot. When asked, the patient describes shooting, stabbing pain in his legs and says he is unable to walk. He denies trauma. He says the symptoms started this morning when he got out of bed, and that he has never felt this way before. The patient denies having a bowel movement today but does report normal morning urination.
• The patient is well-appearing, with normal mental status. The examination is significant for muscle strength 2/5 in the large muscle groups of his bilateral lower extremities, and the patient is not able to walk. He has absent deep tendon reflexes at the patellar and Achilles tendons, but sensation is intact. When asked about previous illnesses, the patient’s mother states that he was sick approximately 2 weeks ago with a “stomach bug.”
• What tests are indicated immediately? What interventions to prevent disease progression should be initiated in the ED? What is the appropriate disposition for this patient?

A 15-year-old girl presents to the ED with a chief complaint of back pain…

• She arrives with her parents, who say that she has been otherwise healthy apart from having “the flu” approximately 3 weeks ago. For the past several hours, the girl has had intolerable back pain that is made worse by walking. The pain was so severe that her father had to carry her to the car and into the ED today. She also noted increasing pressure-like pain in her abdomen.
• On examination, the patient is noted to have 1/5 strength in the bilateral lower extremities and diminished sensation to light touch to her umbilicus and below. She has a distended bladder, and decompression by Foley catheter reveals a volume of 840 mL and results in resolution of her abdominal pain. Noncontrast MRI of the cervical, thoracic, and lumbar spine reveals no compressive mass lesion in the spinal canal but does demonstrate abnormal enhancement.
• What is the most likely diagnosis? What treatments may help improve the patient's symptoms? What is the appropriate disposition for this patient?

Clinical Pathway for Management of Pediatric Patients With Acute Demyelinating Syndromes

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Table

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Key References

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

9. * Lehmann HC, Hartung HP, Kieseier BC, et al. Guillain-Barré syndrome after exposure to influenza virus. Lancet Infect Dis. 2010;10(9):643-651. (Review article) DOI: 10.1016/S1473-3099(10)70140-7

35. * Agrawal S, Peake D, Whitehouse WP. Management of children with Guillain-Barré syndrome. Arch Dis Child Educ Pract Ed. 2007;92(6):161-168. (Review article) DOI: 10.1136/adc.2004.065706

36. * Patwa HS, Chaudhry V, Katzberg H, et al. Evidence-based guideline: intravenous immunoglobulin in the treatment of neuromuscular disorders: report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology. 2012;78(13):1009-1015. (Literature review) DOI: 10.1212/WNL.0b013e31824de293

40. * Korinthenberg R, Monting JS. Natural history and treatment effects in Guillain-Barré syndrome: a multicentre study. Arch Dis Child. 1996;74(4):281-287. (Multicenter retrospective chart review; 175 patients) DOI: 10.1136/adc.74.4.281

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Keywords: acute demyelinating syndromes, demyelinating syndromes, demyelinating conditions, Guillain-Barré syndrome, GBS, acute transverse myelitis, ATM, acute flaccid paralysis, acute inflammatory demyelinating polyradiculopathy, acute motor axonal neuropathy, Miller Fisher syndrome, weakness, back pain, deep tendon reflexes, respiratory distress, forced vital capacity, albuminocytologic dissociation, intravenous immunoglobulin, IVIG, corticosteroids, plasmapheresis, plasma exchange