Demyelinating disease in children is not rare, though some etiologies are far more common than others. While presenting signs and symptoms can overlap, some distinguishing features can help guide acute therapies. We compare and contrast three unique cases of pediatric demyelinating disease presenting with rapid neurologic deterioration and respiratory failure.

Case series

13-year-old male presenting with 3 days of headache, progressive somnolence and focal weakness progressing to flaccid quadriplegia and respiratory failure. Had neutrophilic pleocytosis and extensive patchy T2 FLAIR hyperintensities throughout brain and spinal cord, ultimately diagnosed with acute disseminated encephalomyelitis (ADEM).

 9-year-old female presented with fever, vomiting, unresponsiveness, seizures progressing to respiratory failure. Had lymphocytic pleocytosis, positive MOG antibody (1:100), and multifocal cortical T2 hyperintensities with restricted diffusion and edema, ultimately diagnosed with FLAIR-hyperintense lesions in anti-MOG associated encephalitis with seizures (FLAMES) variant of myelin oligodendrocyte glycoprotein antibody associated disease (MOGAD). 

 15-year-old female with right leg paresthesia and weakness initially responsive to high dose steroids and IVIG, followed by a rapid progression to quadriplegia and respiratory failure. Had mild lymphocytic pleocytosis, positive oligoclonal bands and Kappa free light chain in CSF, and numerous, large T2 FLAIR hyperintensities with outer concentric ring of restricted diffusion and enhancement, ultimately diagnosed with Balo’s concentric sclerosis (BCS) variant of multiple sclerosis (MS).

Through these cases, we compare and contrast the distinct etiologies of each case, namely ADEM, MOGAD, and BCS, and describe the effective treatments.