Acute Transverse Myelitis in Children

Acute Transverse Myelitis in Children

Ooooo the infamous chief complaint of “weakness” ……. We just love those don’t we? Although neurological complaints may not be a crowd’s favorite, it is one we must become comfortable with. Timely recognition and treatment can be life-improving and life-saving! So, with that goal in mind, let us digest another delicious morsel on weakness – Acute Transverse Myelitis in Children:

Acute Transverse Myelitis in Children: Basics

  • Rare, non-compressive, inflammatory condition of the spinal cord. (Tavasoli 2018, Wolf 2012)
  • More common in adults; children comprise 20% of total cases (Pidcock 2007, Wang 2019)
  • Bimodal age distribution (Absoud 2016)
    • Children < 5 years
    • Children > 10 years  
  • Symptoms evolve over 2-4 days and peak at 5-6 days (Absoud 2016) 
  • ATM may occur alone OR as part of another disorders (e.g. Neuromyelitis Optica Spectrum Disorder (NMOSD), Multiple Sclerosis (MS), Autoimmune Rheumatologic Disorder (Absoud 2016)
  • It is a DIAGNOSIS of Exclusion 

Acute Transverse Myelitis in Children: Presentation

Complete (bilateral symptoms) vs Partial TM (asymmetric symptoms) (Absoud 2016, Wang 2019) 

  1. Sensory deficits (pain, paresthesia, numbness, weakness)
  2. Motor deficits (decreased tone and deep tendon reflexes, progresses to increased tone and hyperreflexia)
  3. Autonomic dysfunction (temperature irregularity, unstable RR, HR, and rhythm, bowel/bladder dysfunction) 

Prodromal illness is seen in 66% of patients presenting with ATM. (Absoud 2016) 

Acute Transverse Myelitis in Children: DDx  

  • Demyelinating Disorders (Absoud 2016, Jacob 2008)
    • Multiple Sclerosis (MS)
    • Neuromyelitis optica spectrum disorder (NMOSD) 
    • Acute disseminated encephalomyelitis (ADEM)
    • Myelin oligodendrocyte glycoprotein-antibody-associated disease (MOGAD)
    • Guillain-Barre syndrome 
  • Infection 
    • Infectious myelitis (bacterial, viral, fungal)
  • Trauma
  • Vascular Disorders (Jacob 2008)
    • Acute vascular occlusion 
    • AVF/vascular malformation 
  • Neoplastic/paraneoplastic
    • Spinal tumor/extramedullary tumor 
  • Inflammatory disorders (Absoud 2016, Jacob 2008)
    • Systemic Lupus Erythematous (SLE)
    • Neurosarcoidosis
    • Sjogren
    • Behcets
  • Metabolic myelopathies (e.g. B12, cooper, vitamin E) (Wang 2019)

Acute Transverse Myelitis in Children: Evaluation 

Primary diagnostic tools (Wang 2019)

  • Spinal MRI
    • Findings: abnormal T2/fluid-attenuated inversion recovery (FLAIR) hyperintensities affecting one or more cord segment. 
  • CSF Studies
    • Findings: elevation of cell count and differential, protein, and glucose analysis. CSF is abnormal in 50% of transverse myelitis cases, glucose typically normal. 
    • Positive oligoclonal bands and increased immunoglobulin G suggest autoimmune myelitis (e.g. MS).  
  • Other
    • Viral pathogen testing (enterovirus, west nile virus, arbovirus, HIV, varicella-zoster virus). (Wang 2019)
    • Mycoplasma, borrelia, syphilis, and listeria monocytogenes have been reported in association with TM. (Wang 2019)
    • Serum lab testing for anti-AQP4 and MOG antibodies testing should be considered in children with myelitis. (Wang 2019)
      • A positive result warrants ongoing surveillance and management for other diagnosis including NMDO and MS. 

Diagnostic Criterion for ATM 

(Tavasoli 2018, Wang 2019)

  • Bilateral sensory, motor, and autonomic dysfunction localized to one OR more spinal segment 
  • No evidence of compressive cord lesion 
  • Enhancing lesion on MRI 
  • Pleocytosis or elevated immunoglobulin type G (IgG) index 
  • Transverse myelitis consortium working group can be applied to children
    • May be challenging in younger children unable to report a sensory level (Absoud 2016)

Acute Transverse Myelitis in Children: Treatment 

(Absoud 2016, Wang 2019)

  • Current treatment is based on adult data, case series, and expert opinion,
    • No randomized controlled trials in pediatric population 
  • IV corticosteroids daily, 3-5 days 
  • Plasma exchange 
  • IVIG
  • Cyclophosphamide (for myelitis related to systemic inflammatory and connective tissue disorders)

Acute Transverse Myelitis in Children: Prognosis

  • Children generally have better outcome than adults (Absoud 2016). 
  • Recovery by 2 years 
  • 40% have residual deficits (Wolf 2012)
  • A small percentage may later be diagnosed with other demyelinating disease (Wolf 2012)
    • NMDOS
    • Multiple sclerosis 

Moral of the Morsel 

  • REMEMBER, ATM is a diagnosis of exclusion, ask yourself what else could be going on?
  • ALWAYS rule out a compressive lesion, this is a medical emergency!
  • REMEMBER, ATM can come with other baggage (i.e. first sign of MS). These patients will require ongoing surveillance and management. 

References

  • Absoud, Michael et al. “Pediatric transverse myelitis.” Neurology vol. 87,9 Suppl 2 (2016): S46-52. doi:10.1212/WNL.0000000000002820
  • Jacob, Anu, and Brian G Weinshenker. “An approach to the diagnosis of acute transverse myelitis.” Seminars in neurology vol. 28,1 (2008): 105-20. doi:10.1055/s-2007-1019132
  • Pidcock FS, Krishnan C, Crawford TO, Salorio CF, Trovato M, Kerr DA. Acute transverse myelitis in childhood: center-based analysis of 47 cases. Neurology 2007; 68:1474–1480.
  • Tavasoli, A., & Tabrizi, A. (2018). Acute Transverse Myelitis in Children, Literature Review. Iranian journal of child neurology12(2), 7–16.
  • Wang, Cynthia, and Benjamin Greenberg. “Clinical Approach to Pediatric Transverse Myelitis, Neuromyelitis Optica Spectrum Disorder and Acute Flaccid Myelitis.” Children (Basel, Switzerland) vol. 6,5 70. 17 May. 2019, doi:10.3390/children6050070
  • Wolf, Varina L et al. “Pediatric acute transverse myelitis overview and differential diagnosis.” Journal of child neurology vol. 27,11 (2012): 1426-36. doi:10.1177/0883073812452916

Author

Maureen Nsofor
Maureen Nsofor
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