Transverse Myelitis

Inflammatory disorder in which injury to spinal cord results in rapid onset of neurological symptoms such as weakness, sensory loss and autonomic dysfunction.

Introduction

  • Inflammatory disorder in which injury to spinal cord results in rapid onset of neurological symptoms such as weakness, sensory loss and autonomic dysfunction.

Epidemiology

  • The annual incidence of transverse myelitis ranges from 1.34 to 4.60 cases per million.
  • Transverse myelitis has bimodal peak at:
    • 10-19 years of age
    • More predominant in female
    • 30-39 years of age
  • Older patients(more than 50) are more prone to develop spinal cord infarction.
It is associated with:
  • Spinal cord infarct

Etiology

  • Trauma to spinal cord
  • Vaccinations in children
  • Viral Infections
  • Immunocompromised State
  • Carcinoma
  • Autoimmune disease
  • Systemic Lupus Erytheromatosis
  • Sjogren Syndrome
  • Anti phospholipid Syndrome
  • Sarcoidosis
  • Bechet Disease
  • Multiple Sclerosis
  • Devic Syndrome
  • Idiopathic(15-30%)

Clinical Presentation

  • It has insidious progressive course. It depends upon area of spinal cord involvement.
  • Detailed in the following chapter

History

  • Development of sensory, motor, or autonomic dysfunction attributable to spinal cord
  • Bilateral signs and/or symptoms
  • Weakness of the limbs
  • Stiffness of the limbs
  • Sensory Impairments
  • Numbness of limb
  • Tingling of limb
  • Bowel dysfunction (constipation)
  • Bladder Dysfunction (urinary incontinence)
  • Pain

Examination

  • Clearly defined sensory level
  • Increased reflexes
  • Decrease muscle strength
  • Spastic gait
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Hypo-reflexia can occur with transverse myelitis in the setting of spinal shock; can be easily confused with peripheral disorders. Hence acute presentations should follow spine imaging to rule out acute myelopathy like transverse myelitis.

Table 1: Clinical Signs and Reflexes

Table 1: Clinical Signs and Reflexes
Reflexes / sign
Lesion Level of Spinal Cord
Description
C7, C8, T1)
Flexion and adduction of the thumb and flexion of the index finger with snapping of middle finger; usually more specific if unilateral or bilateral with hyperreflexia
T10-T12
Umblicus move up during sitting up from supine position
At T10 or below
Upper and middle superficial abdominal reflexes are present
Below T12
All superficial abdominal reflexes are present
Lesion at or below L2
Absent

Investigations

Radiological Investigations

  • Magnetic Resonance Imaging(MRI) is investigation of choice.
    • Gadolinium enhancement
    • If none of the inflammatory criteria is met at symptom onset (including CSF finding and MRI finding), repeat MRI and LP evaluation between 2-7 days following symptom onset

Laboratory Investigations

  • Usual blood Counts( CBC, ESR, LFT, RFT, Serum Electrolytes)
  • CSF pleocytosis or elevated IgG index
  • Anti nuclear antibodies
  • Anti double stranded DNA antibodies
  • Anti Sm antibodies
  • Serum B12, methylmalonic acid
  • VDRL

Conduction Studies

They are used to differentiate transverse myelitis from nervous pathology like Guillain-Barre Syndrome.
  • EMG/NCS: absent H-reflex and prolonged latency or absent F-waves in early GBS

Differential Diagnoses

Table 1: Differential Diagnosis

Table 1: Differential Diagnosis
Diseases
Typical Location
Length of segments involved
Onset
MRI features
Other Comments
Cervical or thoracic spine along with lesions in brain
Short peripheral lesions in dorsal/lateral columns
Acute/subacute with contrast enhancement and chronic/progressive/fluctuating with T2 and T1 changes
Post contrast pattern: Ring like, homogeneous. T2 white matter hyperintensity <2 segments and <50% diameter.
Dissemination in space and time; meeting McDonald Criteria
Thoracic
Long cord lesion
Chronic/progressive
Symmetric T2 hyperintensity in the posterior and lateral columns; no contrast enhancement
Gastric bypass, malabsorption
Cervical or thoracic
Long segment
Hyperacute/acute
Linear strip, owl eye
Vascular Risk Factors
Cervical or thoracic
Variable
Variable
Almost Universal: Linear dorsal subpial/trident enhancement
Lung/eyes/skin involvement; biopsy to confirm
Cervical or thoracic
tract-specific lesion, especially gadolinium enhancing
Variable
usually lateral tracts
Antineuronal antibody testing
Cervical more common
Long or short T2 lesion
Faint or no enhancement; conus involvement may suggest MOG-Ab T1 hypo- and T2 hyperintense
Optic neuritis; brainstem symptoms
Thoracic level more common
Longitudinally extensive T2 lesion (≥3 vertebral segments)
Variable (hyperacute in AQP4-Ig positive cases only)
Central lesion with grey matter or holocord involvement
optic neuritis and area postrema involvement
*
Coexisting deep gray brain lesions
Commonly diffuse lesions
Altered mental status, more common in children
Variable
Variable
Acute
Leptomeningeal enhancement
Recent tick bite/rash; IV drug use
Variable
Variable
Acute/subacute
Predominantly central grey matter
Zoster rash, genital herpes, endemic region for enterovirus
*
*
Acute/subacute
White matter nodules or target lesions on axial cuts
Marked pleocytosis and raised protein on CSF

Diagnostic criterion

Inclusion Criterion

  • Neurologic impairment attributable to the spinal cord
  • Bilateral symptoms
  • Clearly defined sensory level
  • Exclusion of extra-axial compressive etiology by neuroimaging(MRI)
  • Evidence of inflammation in the spinal cord (CSF cells or IgG index, or MRI gadolinium enhancement) seen at onset or within 7 day
  • Progression to nadir between 4 hour and 21 day following the onset of symptoms (if patient awakens with symptoms, symptoms must become more pronounced from point of awakening)

Exclusion Criterion

  • History of radiation to the spine within 10 year
  • Clear arterial distribution clinical deficit consistent with thrombosis of the anterior spinal artery
  • Abnormal flow voids on the spinal cord
  • Serologic or clinical evidence of systemic autoimmune disease
  • CNS manifestations of infectious etiology (eg, syphilis, Lyme, HIV, HTLV-1, Mycoplasma)
  • Brain MRI lesions suggestive of multiple scleroma
  • History of Optic Neuritis

Prognostic factors

1/3 of patients recover with little to no sequelae, 1/3 are left with moderate degree of permanent disability, and 1/3 have severe disabilities
Following are poor prognostic Factors:
  • Time to maximal deficit of <24 h
  • Back pain
  • Requirement of respiratory Support
  • Spinal shock
  • A higher anatomical spinal lesion
  • Complete paraplegia

Management

First monitoring of vital signs, respiratory rate is done then we can use following:

Pharmacological Management

  • High dose IV methyl prednisolone 2.5–3g for 3-5 days
  • Followed by oral prednisolone starting at 1 mg/kg/day.
  • Taper steroid over 1-3 months
  • Plasmapheresis for severe attack
  • IV cyclophosphamide
  • Azathioprine prevent relapses

Rehabilitation

Physical, mental, and vocational rehabilitation can be achieved through multi-disciplinary approach:
  • Strengthening muscle technique
  • Ankle foot orthoses
  • Functional Electrical Stimulator device

Disease Complications

Following are complications due to spinal cord dysfunction:
  • Spasticity
  • Constipation
  • Ileus
  • Impaired thermoregulation
  • Gaseous Distension
  • Neuropathic pain
  • Bladder Dysfunction
  • Sexual Dysfunction
  • Anxiety and Depression

Conclusion

  • It needs immediate treatment.
  • Transverse Myelitis is a serious spinal cord injury and has catastrophic implications.
  • There are multiple triggers of transverse myelitis such as infections, auto-immune Diseases and vaccinations

Further Reading

Frohman, E. M., & Wingerchuk, D. M. (2010). Transverse myelitis. New England Journal of Medicine363(6), 564-572.

Bibliography

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