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)
- Sensory deficits (pain, paresthesia, numbness, weakness)
- Motor deficits (decreased tone and deep tendon reflexes, progresses to increased tone and hyperreflexia)
- 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 neurology, 12(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