Transverse Myelitis: Symptoms, Diagnosis, and Management

Transverse myelitis is a rare acquired, auto-immune, spinal cord disorder. In TM inflammation of the spinal cord occurs, which is characterized by rapid onset of weakness, sensory alterations, and bladder or bowel dysfunction.

It can be cured, and most patients recover completely if treated well. The maximum recovery occurs in 3 months and some patients have residual symptoms.

ETIOLOGY

• Transverse myelitis occurs after post-infection, post-vaccination, and musculoskeletal-associated conditions.
• It could be the continuum of Acute disseminated encephalomyelitis (ADEM), Multiple sclerosis (MS), Myelin oligodendrocyte glycoprotein antibody disease (MOGAD), Neuromyelitis optica spectrum disorder (NMOSD), and Acute flaccid myelitis (AFM).
• The systemic infection by viruses, enterovirus, varicella-zoster virus, herpes, listeria monocytogenes.
• The direct exposure to the central nervous system.

IMMUNOPATHOGENESIS

• Perivascular infiltration of monocytes and lymphocytes.
• Axonal degeneration.
• Pathological heterogeneity is present and both grey and white matter is affected.
• It is not a pure demyelinating disorder rather it is a mixed inflammatory disorder that affects neurons, axons, oligodendrocytes, and myelin.
• Lymphocytic infiltration with demyelination and axonal loss.
• In cases of post-vaccination, the onset of disease to be found 5-28 days after vaccination.
• The molecular mimicry (enterovirus vermicularis) and super antigen-mediated disease have also been described as potential mechanisms of auto-immunity.

CLINICAL FEATURES

1. Autonomic dysfunction (Bowel and bladder dysfunction): There is an increase in frequency or urge, due to incontinence and constipation.
2. Sensory loss: The loss of sensation horizontally, below the area of sensation leading to paraesthesia.
3. Weakness in the legs, initially paraparesis occurs and later results in paraplegia if not treated, or residual permanent disability occurs after treatment.
4. Pain in the lower back occurs, the pain is characterized as sharp shooting pain. The pain can be radiating up to the torso.
5. Autonomic dysreflexia: It occurs in a lesion above T6, where mass reflexes are produced. The increase in BP & HR, and headache and anxiety occur.
6. Pyramidal weakness: It occurs in upper motor neuron lesions (UMNL). There is sparring of antigravity muscles, the extensors in the upper limb, and flexors in the lower limb.
7. Unilateral posterior cord syndrome: It leads to loss of proprioception and wide steppage gait.
8. Contralateral spinothalamic loss
   1. Spinal shock: The immediate loss of reflexes (areflexia) below the level of injury.
9. Loss of vision
10. Spasticity: The abnormal muscular tightness due to prolonged muscle contraction.
11. Tremors: The uncontrollable repetitive movement.
12. Fatigue
13. Psychological distress: Depression, anxiety, suicidal tendency.

EVALUATION

Investigation

• CBC
• Blood culture: To identify the causative organism
• Blood analysis: HIV, Vit. B12 deficiency
• ESR & CRP
• Detection of autoantibodies.
• CT scan: To determine the extent of inflammation.
• MRI: Either the cross-sectional view or 3D view has been done. To locate the lesion in the brain (underlying cause, especially MS) and spinal cord (site of the lesion).
• Lumbar puncture
• CSF culture
• CSF examination: There is an increase in protein content and WBC

Additional testing may be performed in the appropriate clinical setting.

• Neuro-ophthalmologic evaluation
• Paraneoplastic evaluation
• Infectious serologic and CSF studies
• Nasopharyngeal swab for enteroviral PCR
• Serum copper and ceruloplasmin (copper deficiency may mimic TM)
• Serum vitamin B12 and vitamin E levels
• Spinal angiogram
• Prothrombotic evaluation
• Salivary gland biopsy

PHYSICAL EXAMINATION

• Pain assessment: Visual analog scale (VAS) or numeric pain rating scale (NRS)
• Fatigue assessment
• Muscle tone and strength
• Active and passive ROM
• Grade of spasticity – modified Ashworth scale
• Sensory ataxia
• Reflexes: Babinski, bulbocavernosus, plantar and cremasteric
• Dermatome and Myotome
• Sensory assessment
• Functional assessment – functional independence measure (FIM)
• Functional reach tests
• Gait assessment

MANAGEMENT MEDICAL

• The first-line therapy for the treatment of transverse myelitis is intravenous glucocorticoids. High-dose intravenous glucocorticoids should be initiated as soon as possible. There are few contraindications to glucocorticoid therapy.
• The regimens would include methylprednisolone or dexamethasone for 3 to 5 days. Further duration of therapy should be directed as the clinical case progresses.
• Plasma exchange may be efficacious for acute central nervous system demyelinating disease, which fails to respond to glucocorticoid therapy.
• Additional immunomodulatory therapy such as cyclophosphamide, mycophenolate, or rituximab might offer benefits in chronic recurrent TM or resistant acute TM.
• Treatment modalities that should also be utilized in the management of TM include pain management, intravenous immunoglobulin (IVIG), and antivirals.

PHYSIOTHERAPY MANAGEMENT

Goals:

• To retain muscle strength and flexibility.
• To reduce pain, muscle tone, spasm, and spasticity.
• To improve coordination.
• To regain bladder and bowel functions.
• To maintain and improve range of motion (ROM)
• To improve functional status.
• To make the patient functionally independent.
• Gait training
• To educate the patient.

Points to keep in mind before planning the rehab:

• While planning rehab, keep in mind about patient’s fatigue level.
• Teach the patient about energy conservation techniques.
• Always remember every patient is different.
• Anticipating and minimizing secondary complications.

TREATMENT

• Stretching program – to prevent contractures.
• Joint care
• Strengthening: lower limb muscle and core
• Gait training: provision of equipment, orthoses, and wheelchairs.
• Wheelchair training: teach skills to use a wheelchair,
• Transfers: teach transfers (getting in and out of a wheelchair, bed, car, shower/bath, and onto and off a toilet)
• Reduce the risk of pressure ulcers: Positioning, daily inspections, keeping the pressure-prone areas dry, avoiding wear and tear of skin, use pressure relieving techniques.
• Aid control of spasticity
• Hydrotherapy treatment
• Breathing exercises and chest clearance techniques
• Pain reduction/management – TENS, heat, or cold therapy.
• Teach caring and handling techniques to patients and caregivers.
• Teaching you how to use specialized exercise equipment and equipment for ADLS.

For bladder and bowel dysfunction

Goals:

• To normalize abdominal and pelvic floor muscle tone/activity, motor control, and coordination.
• Pelvic floor muscle contractions (commonly known as Kegels) will be incorporated once the involved musculature is at its optimal length and neuromuscular control is present.
• Education regarding proper bladder/bowel habits, postural needs, and ergonomics may positively influence rehabilitative outcomes.

Interventions:

• External and internal soft tissue mobilization, myofascial and trigger point release, connective tissue manipulation, visceral mobilization
• Neuromuscular re-education using electromyographic and tactile biofeedback for down training and/or uptraining the pelvic floor musculature
• Timed voiding mechanics, Valsalva maneuver, and intermittent catheterization.
• Instruction regarding normal defecation mechanics (coordination of abdominal muscle contraction for a gradual increase in intra-abdominal pressure combined with pelvic floor muscle relaxation)
• Behavioral training, bladder/bowel education, postural training, and self-management techniques

Home exercise program (HEP)

Phase 1

• Energy conservation education
• Seated heel raises
• Seated toe raises
• Long arc quads
• Supine abdominal (TA) marching

*All exercises to tolerance

Phase 2

• Energy conservation education
• Seated heel raises
• Seated toe raises
• Long arc quads
• Supine abdominal (TA) marching

*All exercises to tolerance

Phase 3

• Energy conservation education
• Seated heel raises
• Seated toe raises
• Long arc quads
• Supine abdominal (TA) marching
• Ankle alphabet in supine x1 bilaterally
• Supine hip abduction x5 bilaterally
• (+) Hip, knee, and ankle PROM flexion/extension x5 bilaterally during breaks

* All exercises to tolerance

Except:

• Ankle alphabet
• Supine hip abduction
• PROM

Phase 4

• Seated heel raises
• Seated toe raises
• Long arc quads
• Supine abdominal (TA) marching
• Ankle alphabet x1 bilaterally
• (+) Supine hip abduction x8 bilaterally
• Hip, knee, and ankle PROM flexion/extension x5 bilaterally during breaks
• (+) the tibiofemoral joint distraction of the right knee

* All exercises to tolerance

Except:

• Ankle alphabet
• Supine hip abduction
• PROM

Joint mobilization was performed only as necessary to address joint ROM deficits present.

(Outcome measures performed this date, limiting further additions to HEP due to fatigue)

Phase 5

• Seated heel raises
• Seated toe raises
• (+) Long arc quads with 0.5# ankle weights
• Supine abdominal (TA) marching
• Ankle alphabet x1 bilaterally
• Supine hip abduction x8 bilaterally
• Hip, knee, and ankle PROM flexion/extension x5 bilaterally during breaks
• (+) Ankle circles (AROM) clockwise and counterclockwise x5 each on the BAPS board at level 2
• (+) Gait training in parallel bars:
  • Heel → toe rocking on the right foot to address foot Drop
  • Progress to heel → toe with a swing through of left foot
  • Progress to walking in parallel bars with emphasis on heel → toe rockers
  • Progress to hand-held assistance outside of parallel bars x20 feet
  • Education on core strength in relation to gait and addressing residual back pain

* All exercises to tolerance

Except:

• Ankle alphabet
• Supine hip abduction
• PROM
• Ankle circles on BAPS

**Note: (+) indicates an addition to HEP or progression of exercise within HEP.

Gait Training Progression

Location: Parallel Bars B
Beginning Stanc: Heel strike
Ending Stance: Swing through to forefoot stance

Location: Parallel Bars
Beginning Stance: Heel strike emphasized through the full gait cycle
Ending Stance: Multiple steps through the gait cycle to the end of parallel bars 30 feet in the gym with hand-held assistance

Location: Parallel Bars
Beginning Stance: Full gait cycle (emphasis on heel strike)
Ending Stance: Full gait cycle (emphasis on heel strike)

* Always reassess the patient for progression of phase after every 5-7 initially and later 2-3 weeks days. *

DIFFERENTIAL DIAGNOSIS

The most common aetiologies to be distinguished from idiopathic acute transverse myelitis are:

• Multiple Sclerosis
• Guillain Barre Syndrome
• Disc herniation
• Parainfectious myelitis
• Neuromyelitis optica (Devic’s disease)
• Myelitis related to systemic diseases, such as systemic lupus erythematosus
• Compression of the spinal cord caused by trauma e.g., vertebral fractures
• Epidural or subdural hematoma
• Epidural and/or paraspinal abscess
• Tumour

Key points

• TM is an acquired autoimmune disorder causing inflammation of the spinal cord.
• Symptoms include weakness, sensory alterations, and bladder/bowel dysfunction.
• Prompt treatment can lead to significant recovery, with most improvement occurring within 3 months.

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