Transverse myelitis - only Nelson
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Transverse Myelitis
Nelson Textbook of Pediatrics, 2024 - Chapter 640.3
Definition
Transverse myelitis (TM) is a condition characterized by the rapid development of both motor and sensory deficits of the spinal cord. It presents acutely as either partial or complete cord involvement at any level with bilateral neurologic signs; in adults and older children, there will usually be a clear sensory level.
Evidence of spinal cord inflammation can be demonstrated by:
- Enhancing lesion on MRI
- CSF pleocytosis (>10 cells)
- Increased IgG index
Time to maximal disability typically occurs 5-6 days after symptom onset.
Etiology / Classification
TM has multiple causes and can be:
- Secondary to immune-mediated condition (postinfectious or antibody-driven)
- Result of direct infection (infectious myelitis)
- Idiopathic
Table 640.5 - Reported Causes of TM:
| Category | Examples |
|---|---|
| Acquired demyelinating disorders | MS, NMO, ADEM, MOGAD |
| Systemic inflammatory/autoimmune | SLE, Sjogren syndrome, antiphospholipid syndrome, Behçet, ankylosing spondylitis, mixed CTD |
| Neurosarcoidosis | - |
| Parainfectious (viral) | Hepatitis A/B/C/E, measles, mumps, varicella zoster, EBV, CMV, HSV, influenza A/B, HIV, enterovirus 71, coxsackievirus, West Nile, dengue, parvovirus B19, COVID-19 |
| Parainfectious (bacterial) | Mycoplasma pneumoniae, Campylobacter jejuni, Borrelia burgdorferi, TB, Treponema pallidum, Brucellosis |
| Parainfectious (fungal) | Actinomyces, Blastomyces, Aspergillus, Cryptococcus |
| Parainfectious (parasitic) | Toxocara, Schistosoma, Toxoplasma, Taenia solium |
| Paraneoplastic | Anti-Ri, CRMP-5-IgG, anti-amphiphysin, anti-GAD65, NMDAR antibody |
| Atopic myelitis | - |
| Drugs/toxins | TNF-alpha inhibitors, sulfasalazine, epidural anesthesia, gemcitabine, cytarabine, heroin, benzene |
| Idiopathic TM | - |
Epidemiology
- TM is more common in adults but affects approximately 2 children/million per year
- Bimodal age distribution: children <5 years and >10 years
Age-based differences:
Children <5 years:
- Spinal cord dysfunction develops over hours to a few days
- Often preceded by infectious disease (viral or mycoplasma)
- Motor dysfunction is often severe, approaching complete loss of function
- Recovery is slow (weeks to months) and usually incomplete
- Most common residual: bowel and bladder dysfunction (15-50%)
- Pathology: perivascular infiltration with mononuclear cells; overt necrosis of the spinal cord may occur
Older children (>10 years):
- Onset is also rapid, but recovery is faster and more likely to be complete
- Necrosis/irreversible injury may occur in a small but important number
- Associated etiologies include SLE, AQP4-Ab or MOG-Ab-associated NMOSD, mycoplasma, enterovirus, or idiopathic
Clinical Manifestations
TM is often preceded within the previous 1-3 weeks by a mild non-specific illness or minimal trauma.
Progression:
- Discomfort or overt pain in the neck or back is common
- Progresses to: numbness, anesthesia, ataxia, areflexia, motor weakness (truncal and appendicular musculature at or distal to the lesion)
- Paralysis begins as flaccidity (paraparesis, tetraparesis)
- Spasticity develops over weeks, accompanied by hyperreflexia and clonus
Specific features:
- Weakness is usually bilateral; unilateral weakness suggests a hemicord lesion (most often associated with MS)
- Urinary retention is a common early symptom; incontinence occurs later
- Early sensory findings may be isolated to the posterior column - evaluate vibratory sensation
- Progressive sensory loss: anesthesia, paresthesia, or allodynia
- Other findings: priapism, respiratory compromise, spinal shock with subsequent autonomic dysreflexia
- Rarely, overlap syndrome with Guillain-Barré syndrome may occur
Diagnostic Evaluation
TM is a diagnosis of exclusion. Differential diagnoses include:
- Guillain-Barré syndrome
- Demyelinating disorders
- Systemic rheumatologic conditions
- Meningitis / infectious myelitis
- Spinal cord infarction
- Arteriovenous malformations
- Trauma, mass lesions, abscess, tumors of the spine/spinal cord
- Bony and intervertebral disk distortion
MRI (with and without contrast) - Essential:
| Finding | Significance |
|---|---|
| T1: may be normal or show cord distension | - |
| Infantile form: T2 high signal over multiple segments | - |
| Adolescent form: T2 high signal centrally, involves gray + white matter | May be 1-2 segments or more extensive |
| Gadolinium enhancement (limited degree) | Expected, indicates inflammation |
| Hemicord involvement | Suggests MS |
| Holocord with brain + optic nerve involvement | Suggests NMOSD |
| Predominant gray matter involvement | Suggests vasculitic or infectious process (SLE, enterovirus) |
| Nerve root enhancement | Mixed picture (central + peripheral demyelination) or anterior horn cell involvement |
- Up to 6% of cases do not show spinal cord lesions on MRI; repeat imaging at 7 days may reveal atrophy
- MRI brain is also indicated: evidence of other demyelination seen in at least 40% of patients; guides consideration for MS, ADEM, NMOSD, SLE, enterovirus-associated AFM
- Cervical and cervicothoracic lesions represent most acute TM lesions
CSF (after excluding mass on MRI):
- Analyze for: cells, protein, immunoglobulin index, OCBs, infectious pathogens
- Mononuclear cells usually elevated
- Protein may be elevated or normal
- Presence of CSF inflammatory cells is essential for diagnosis of TM
Antibody testing:
- Serum and CSF: AQP-4 antibodies and MOG antibodies in ALL patients (to rule out NMOSD)
- Older children: additional serum autoimmune studies, particularly for SLE
Mimics of TM (Table 640.6)
| Mimic | Key Features |
|---|---|
| Vitamin B12 deficiency | Posterior column impairment; "inverted V" or "inverted rabbit ear" sign on MRI |
| Vitamin E deficiency | Dorsal column syndrome + peripheral neuropathy |
| Copper deficiency | Myelopathy + optic neuropathy; indistinguishable from B12 deficiency |
| Nitrous oxide (N2O) toxicity | Inactivates B12; causes clinical TM in subclinically B12-deficient individuals |
| Neurolathyrism/neurocassivism | Grass pea or cassava ingestion; subacute paraparesis with UMN features |
| Intramedullary tumors | Ependymoma, astrocytoma; usually progressive, but hemorrhage may mimic acute TM |
| Primary CNS lymphoma | Corticosteroid-responsive; CSF: elevated protein, hypoglycorrhachia, absent OCBs |
| Intravascular lymphoma | Affects conus medullaris (unlike TM); no malignant cells in CSF |
| Radiation myelitis | Early (10-16 wk post-RT, mainly sensory, resolves); Delayed (months-years, subacute myelopathy) |
Treatment
Treatment has not been standardized in childhood TM. Available evidence suggests immune response modulation may be effective.
Acute phase:
- High-dose steroids - methylprednisolone (first-line)
- IVIG - for poor response to steroids
- Plasma exchange (PLEX) - for poor response to steroids
- Rituximab - may be considered if antibody-driven TM is suspected
Long-term:
- Prophylactic therapy recommended for children with relapsing disease or biomarkers indicating risk of recurrence
Prognosis
| Age Group | Outcome |
|---|---|
| Older children | Better than adults; ~50% make a good recovery by 2 years (may reflect higher prevalence of MOG-Ab-associated disorders) |
| Remaining 50% | Most common sequelae: sensory problems and bladder dysfunction |
| Younger children | Comparatively poor; recovery slow and usually incomplete |
| Independent ambulation (younger children) | ~40% |
Acute Flaccid Myelitis (AFM) - Related Condition
AFM is an idiopathic neurologic disorder presenting with acute weakness/paralysis in previously healthy children. It is predominantly infectious in etiology.
- Recent biennial outbreaks since 2014 likely caused by enterovirus-D68
- Abnormalities in lower motor neurons of the anterior horn gray matter
- Symptoms: asymmetric paralysis, no sensory deficit, possible cranial nerve involvement (facial weakness, dysarthria, dysphagia)
- In the US, AFP is used as an umbrella term that includes: paralytic poliomyelitis, TM, AFM, GBS, toxic neuropathy, and muscle disorders
Diagnostic criteria for AFM (Table 640.7) - "Definite" requires:
- H1: Acute onset of limb weakness (onset to nadir: hours to 10 days)
- H2: Prodromal fever or illness
- E1: Weakness involving one or more limbs, neck, face, or cranial nerves
- E2: Decreased muscle tone in at least one weak limb
- E3: Decreased/absent DTRs in at least one weak limb
- MRI: Spinal cord lesion with predominant gray matter involvement, with or without nerve root enhancement
- CSF: Pleocytosis (WBC >5 cells/L)
Factors against AFM:
- Encephalopathy not explained by fever
- Sensory deficits on examination
- Supratentorial white matter/cortical lesions
- Absence of CSF pleocytosis
- Positive AQP-4 antibody (excludes AFM)
- Positive MOG antibody (suggests MOGAD)
Source: Nelson Textbook of Pediatrics, 2024 - Chapter 640.3 (Perry M and Hemingway C), Part XXV: The Nervous System, pp. 3727-3731
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