Pediatric Acute Demyelinating Syndromes: Identification and Management in the Emergency Department -

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Publication Date: March 2021 (Volume 18, Number 03)

CME Credits: 4 AMA PRA Category 1 Credits™, 4 ACEP Category I Credits, 4 AAP Prescribed Credits, 4 AOA Category 2-A or 2-B Credits. CME expires 03/01/2024.

Author

Peer Reviewers

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?