Transverse Myelitis

1. Clinical Overview

Transverse myelitis (TM) is an acute inflammatory disorder of the spinal cord characterized by bilateral motor, sensory, and autonomic dysfunction below the level of the lesion. [1,2] It represents a heterogeneous group of conditions that may occur as an isolated, monophasic illness (idiopathic TM) or as the first presentation of multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), or other systemic inflammatory diseases. [1,3,4]

The term "transverse" refers to inflammation spanning the full or partial width of the spinal cord, disrupting ascending and descending neural pathways. [1] Prompt recognition and treatment are critical, as early intervention with high-dose corticosteroids can significantly improve outcomes. [5] The most important initial step is to exclude compressive myelopathy, which requires emergency surgical intervention.

Key Facts

Clinical Pearls

Pearl 1: Exclude Compression First: THE FIRST STEP IS ALWAYS TO EXCLUDE CORD COMPRESSION. Compressive myelopathy from disc herniation, epidural abscess, tumour, or haematoma requires emergency surgical decompression. Get an urgent whole spine MRI before attributing symptoms to TM. Delay in recognizing compression can result in permanent paralysis.

Pearl 2: Partial vs Complete TM Matters: Partial TM (asymmetric, mild, affecting less than 2/3 of cord width on MRI) is associated with MS and has a better prognosis. Complete TM (symmetric, severe, full-width involvement) is more often idiopathic or associated with NMOSD and has worse prognosis. [8,10] This distinction guides both aetiological workup and prognostic counselling.

Pearl 3: Brain MRI is Mandatory: Always request MRI Brain in addition to MRI Spine. If brain lesions are present, the likelihood of developing clinically definite MS is significantly higher—up to 90% at 14 years with 2 or more brain lesions. [9] Brain imaging changes management and follow-up planning.

Pearl 4: LETM = Think NMOSD: Longitudinally extensive transverse myelitis (LETM—lesion spanning 3 or more vertebral segments) is the hallmark of NMOSD. [4,11] LETM should prompt immediate testing for AQP4-IgG and MOG-IgG antibodies. NMOSD requires different long-term management (rituximab, eculizumab, or inebilizumab) compared to MS.

Pearl 5: Bladder Dysfunction is Nearly Universal: Bladder dysfunction occurs in over 90% of patients with TM. [1,8] Urinary retention in the acute phase requires catheterization. Post-void residual volume should be monitored. Long-term bladder dysfunction is a major source of morbidity and requires specialist urological input.

Pearl 6: Sensory Level Assessment is Critical: The sensory level on examination localizes the lesion and guides imaging. Use a systematic approach: start testing from the sacral region and work upward using light touch and pinprick. Document the dermatome level precisely (e.g., T10 sensory level). The sensory level typically corresponds to the upper extent of the MRI lesion. [1]

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2. Epidemiology

Incidence and Prevalence

Transverse myelitis is a rare condition with an estimated annual incidence of 1.34 to 4.6 per million population. [6] The true incidence may be underestimated due to diagnostic challenges and misclassification. Acute myelopathies from all causes (including compression, ischaemia, and inflammation) have a combined incidence of approximately 24.6 per million per year. [6]

Demographics

Aetiological Breakdown

The aetiology of TM is heterogeneous. A significant proportion remains idiopathic despite extensive investigation.

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3. Pathophysiology

Molecular and Cellular Mechanisms

Transverse myelitis results from immune-mediated inflammation of the spinal cord, leading to demyelination, oedema, axonal damage, and in severe cases, necrosis. The pathophysiology varies depending on the underlying aetiology.

Idiopathic and Post-Infectious TM

Step 1: Triggering Event

• Preceding viral or bacterial infection in 30-60% of cases [1,13]
• Common triggers: respiratory viruses (influenza, enterovirus), EBV, CMV, VZV, Mycoplasma pneumoniae [13]
• COVID-19 has been associated with post-infectious myelitis [14]
• Vaccination may rarely precede TM (temporal association, not proven causation) [16]

Step 2: Molecular Mimicry and Immune Activation

• Cross-reactivity between pathogen antigens and myelin or axonal proteins
• Activation of autoreactive T cells and B cells
• Production of pro-inflammatory cytokines (IL-6, TNF-α, IFN-γ)
• Breakdown of the blood-spinal cord barrier [13]

Step 3: Inflammatory Demyelination

• Infiltration of lymphocytes (CD4+ and CD8+ T cells) and macrophages into spinal cord parenchyma
• Myelin sheath destruction by macrophages
• Oedema and swelling of affected cord segments
• "Transverse" pattern: inflammation spans full or partial width of cord
• Cytokine-mediated injury to oligodendrocytes and axons [1,13]

Step 4: Neurological Dysfunction

• Disruption of ascending sensory tracts (spinothalamic, dorsal columns) → sensory level and paraesthesia
• Disruption of descending motor tracts (corticospinal tracts) → weakness or paralysis
• Disruption of autonomic pathways → bladder, bowel, and sexual dysfunction
• Acute phase: spinal shock with flaccid paralysis and areflexia [1]

Step 5: Resolution or Progression

• In monophasic idiopathic TM: inflammation resolves over weeks; remyelination and partial functional recovery occur in most cases [8]
• Residual disability depends on extent of axonal loss
• In MS-associated TM: risk of further relapses at different CNS sites [9]
• In NMOSD: high relapse risk without long-term immunosuppression [4,11]

NMOSD Pathophysiology

Neuromyelitis optica spectrum disorder (NMOSD) has a distinct pathophysiology centered on antibody-mediated astrocyte injury. [4,11]

Approximately 10-20% of NMOSD patients are AQP4-IgG negative; some of these patients are positive for MOG-IgG (myelin oligodendrocyte glycoprotein antibodies), which defines a separate disease entity (MOG-antibody disease). [15]

MS-Associated TM

In MS, TM represents a focal demyelinating attack within the spinal cord. [9] The pathophysiology involves:

• T-cell mediated autoimmune attack on myelin
• Oligodendrocyte injury and myelin breakdown
• Typically short-segment lesions (less than 2 vertebral segments)
• Partial cord involvement (asymmetric, dorsolateral)
• Less severe inflammation compared to NMOSD

Classification

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4. Clinical Presentation

Classic Triad

The classic presentation of TM consists of three core features:

All three features are typically present, although the degree may vary. [1,8]

Symptom Frequency

Onset and Progression

The Transverse Myelitis Consortium Working Group (TMCWG) diagnostic criteria specify that symptoms must progress to nadir (maximum deficit) between 4 hours and 21 days from onset. [1]

Clinical Features by Spinal Level

The level of the spinal cord lesion determines the clinical presentation.

Detailed Sensory Level Determination

Accurate sensory level assessment is critical for lesion localization and guiding imaging.

Systematic Approach to Sensory Examination:

1. Preparation: Explain the procedure to the patient. Use a cotton wisp for light touch and a disposable pin for pinprick.

2. Start Distally: Begin testing at the feet or sacral region (S4-S5) and work upward.

3. Test Modalities:

   • Light touch: Use cotton wisp; test symmetrically on both sides
   • Pinprick (pain): Use disposable pin; ask "sharp or dull?"
   • Temperature: Use tuning fork or cold object (tests spinothalamic tract)
   • Vibration: Use 128 Hz tuning fork on bony prominences (tests dorsal columns)
   • Proprioception: Move toe or finger up/down with eyes closed (tests dorsal columns)

4. Identify the Level: The sensory level is the most caudal dermatome with normal sensation. Mark the level where sensation changes from abnormal (below) to normal (above).

5. Document Precisely: Record the dermatome level (e.g., T6 sensory level to light touch and pinprick).

6. Consider Posterior Column Sparing: Some patients have preserved vibration and proprioception (posterior columns intact) but impaired pain and temperature (spinothalamic tracts affected).

Key Dermatome Landmarks:

Red Flags

Recognition of red flags is essential for timely diagnosis and management.

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5. Clinical Examination

General Inspection

• Respiratory distress: High cervical lesions (C3-C5 involvement) can cause diaphragmatic weakness and respiratory compromise [1]
• Posture: Patient may be bedbound or wheelchair-dependent
• Urinary catheter: Indicates urinary retention (very common in TM)
• Muscle wasting: Suggests subacute or chronic presentation

Motor Examination

The motor examination findings change over time due to spinal shock.

Power Grading (MRC Scale):

• 0 = No movement
• 1 = Flicker of contraction
• 2 = Movement with gravity eliminated
• 3 = Movement against gravity
• 4 = Movement against resistance (weak)
• 5 = Normal power

Document power in all major muscle groups (hip flexion, knee extension, ankle dorsiflexion, etc.).

Sensory Examination

See detailed section above ("Detailed Sensory Level Determination"). Key points:

• Test all modalities (light touch, pinprick, vibration, proprioception)
• Identify and document the sensory level precisely
• Posterior column sparing may occur (preserved vibration/proprioception)

Autonomic Examination

Spinal Level-Specific Signs

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6. Investigations

The diagnostic workup aims to confirm the diagnosis of TM, exclude compressive and other structural causes, identify the underlying aetiology, and assess prognosis.

Emergency Investigation: MRI Spine

Indication: ALL patients with acute myelopathy require urgent MRI of the entire spine (cervical, thoracic, lumbar) with and without gadolinium contrast. [1,7]

Purpose:

• Exclude compressive myelopathy (disc herniation, tumour, abscess, haematoma)
• Visualize intramedullary inflammation
• Determine lesion extent (short-segment vs LETM)
• Assess for enhancement (indicates blood-spinal cord barrier breakdown)

Timing: Within 24 hours of presentation; emergent if suspicion of compression [1,7]

MRI Features of Transverse Myelitis

Advanced MRI Interpretation

Axial Location Patterns and Clinical Correlation

The location of the lesion on axial MRI images correlates with clinical phenotype and aetiology.

Enhancement Patterns

Enhancement on T1-weighted post-gadolinium images provides information about blood-spinal cord barrier integrity and inflammation activity.

Spinal Cord Swelling

Cord swelling (expansion) is common in acute TM but absence does not exclude diagnosis.

Prognostic Value of MRI:

• Short-segment lesion (less than 3 segments): Better prognosis; often MS-associated [9]
• LETM (3+ segments): Worse prognosis; NMOSD-associated [4,11]
• T1 hypointensity ("black holes"): Suggests axonal loss and necrosis; poor recovery [8]
• Extensive cord swelling: Severe inflammation; may have worse outcome
• Brain lesions: High risk of MS (90% at 14 years if 2+ lesions) [9]

MRI: Transverse Myelitis vs Compressive Myelopathy

Short-Segment TM vs LETM

This distinction is critical for aetiological diagnosis.

MRI Brain

Indication: ALL patients with TM should have MRI brain to assess for demyelinating lesions suggestive of MS. [1,9]

Purpose:

• Identify periventricular, juxtacortical, infratentorial, or spinal cord lesions (McDonald criteria for MS)
• Predict risk of developing clinically definite MS [9]

Prognostic Value: Presence of 2 or more brain lesions on initial MRI increases the risk of MS to approximately 90% at 14 years. [9]

Lumbar Puncture and CSF Analysis

Lumbar puncture (LP) must be performed AFTER MRI to exclude cord compression and raised intracranial pressure.

Timing: Within 48-72 hours of presentation [1,7]

CSF Parameters in TM:

CSF Analysis: Advanced Interpretation

Cell Count Patterns

Protein Levels

Oligoclonal Bands (OCBs)

OCBs indicate intrathecal immunoglobulin synthesis and suggest chronic inflammatory CNS disease.

Clinical Significance in TM:

• OCBs present (Type 2): Increases likelihood of MS; 50-70% of MS patients have OCBs at first attack [9]
• OCBs absent: Does not exclude MS (30-50% of early MS are OCB-negative); idiopathic TM or NMOSD more likely

IgG Index

IgG Index = (CSF IgG / Serum IgG) / (CSF Albumin / Serum Albumin)

Additional CSF Tests in Specific Scenarios

Important: A completely normal CSF does NOT exclude TM. The TMCWG criteria state that either CSF pleocytosis, elevated IgG index, OR gadolinium enhancement on MRI is sufficient to demonstrate inflammation. [1]

Serum Antibody Testing

Antibody testing is critical for distinguishing NMOSD and MOG-antibody disease from idiopathic TM and MS.

Critical Point: AQP4-IgG and MOG-IgG testing MUST be performed using cell-based assays (CBA), which are more sensitive and specific than ELISA. [11,15]

Additional Investigations

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7. Diagnostic Criteria

Transverse Myelitis Consortium Working Group (TMCWG) Criteria (2002)

The TMCWG criteria remain the gold standard for diagnosing idiopathic acute transverse myelitis. [1]

Inclusion Criteria (ALL must be met):

1. Bilateral motor, sensory, or autonomic dysfunction attributable to the spinal cord
2. Clearly defined sensory level
3. Inflammation demonstrated by at least ONE of:
   • CSF pleocytosis (elevated white cell count)
   • Elevated CSF IgG index
   • Gadolinium enhancement on MRI
4. Progression to nadir between 4 hours and 21 days from symptom onset
5. MRI spine excludes compressive lesion

Exclusion Criteria:

• History of previous radiation to the spine
• Clear arterial distribution (suggests spinal cord infarction)
• Definite diagnosis of MS, NMOSD, or other systemic inflammatory disease (unless acute TM is the presenting feature)

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8. Management

Management Algorithm

     ACUTE MYELOPATHY PRESENTATION
     (Weakness + Sensory Level + Bladder Dysfunction)
                        ↓
┌─────────────────────────────────────────────────────┐
│        STEP 1: EXCLUDE COMPRESSION                  │
│  EMERGENCY MRI WHOLE SPINE + CONTRAST               │
│  → If compression: EMERGENCY NEUROSURGERY           │
│  → If no compression: proceed to TM workup          │
└─────────────────────────────────────────────────────┘
                        ↓
┌─────────────────────────────────────────────────────┐
│        STEP 2: CONFIRM TRANSVERSE MYELITIS          │
│  - MRI shows intramedullary T2 hyperintensity       │
│  - ± Cord swelling [1,18]                           │
│  - ± Contrast enhancement [1,18]                    │
│  - No evidence of external compression              │
└─────────────────────────────────────────────────────┘
                        ↓
┌─────────────────────────────────────────────────────┐
│        STEP 3: MRI BRAIN + LUMBAR PUNCTURE          │
│  MRI Brain: Look for MS lesions [9]                 │
│  LP: Cell count, protein, glucose, OCBs, cytology   │
│  Bloods: AQP4-IgG, MOG-IgG (especially if LETM)     │
│         [4,11,15]                                   │
└─────────────────────────────────────────────────────┘
                        ↓
┌─────────────────────────────────────────────────────┐
│        STEP 4: ACUTE TREATMENT                      │
│  IV Methylprednisolone 1000 mg daily x 3-5 days     │
│  [5,7]                                              │
│                                                     │
│  If no response OR severe/LETM:                     │
│  → Plasma exchange (PLEX) 5-7 sessions [5,19]      │
│  → IVIG if PLEX unavailable [7]                     │
└─────────────────────────────────────────────────────┘
                        ↓
┌─────────────────────────────────────────────────────┐
│        STEP 5: SUPPORTIVE CARE                      │
│  - DVT prophylaxis (enoxaparin 40 mg SC daily) [7]  │
│  - Urinary catheterization if retention [1]         │
│  - Bowel care (stool softeners)                     │
│  - Pressure area care (turning, specialist mattress)│
│  - Early physiotherapy [7]                          │
│  - Pain management (gabapentin, pregabalin) [7]     │
└─────────────────────────────────────────────────────┘
                        ↓
┌─────────────────────────────────────────────────────┐
│        STEP 6: DETERMINE AETIOLOGY                  │
├─────────────────────────────────────────────────────┤
│  MS (brain lesions, OCB+, short-segment) [9]        │
│  → Disease-modifying therapy (discuss with neuro)   │
├─────────────────────────────────────────────────────┤
│  NMOSD (AQP4+, LETM, optic neuritis) [4,11]         │
│  → Long-term immunosuppression (rituximab,          │
│    eculizumab, inebilizumab, satralizumab) [20]     │
├─────────────────────────────────────────────────────┤
│  MOG-antibody disease (MOG-IgG+) [15]               │
│  → Consider long-term immunosuppression if relapsing│
├─────────────────────────────────────────────────────┤
│  Idiopathic (no MS/NMOSD features) [1]              │
│  → Monitor for relapse; no maintenance treatment    │
└─────────────────────────────────────────────────────┘
                        ↓
┌─────────────────────────────────────────────────────┐
│            REHABILITATION                           │
│  - Inpatient neurorehabilitation [7]                │
│  - Physiotherapy (mobilization, strength)           │
│  - Occupational therapy (ADLs, adaptations)         │
│  - Long-term bladder management (urology referral)  │
│  - Psychological support [7]                        │
└─────────────────────────────────────────────────────┘

Acute Pharmacological Treatment

High-Dose Corticosteroids (First-Line)

Protocol: Intravenous methylprednisolone 1000 mg daily for 3-5 days [5,7]

Evidence: High-dose corticosteroids are the standard of care for acute TM, although RCT evidence is limited. Observational studies and extrapolation from MS optic neuritis trials support early use. [5,7] The Optic Neuritis Treatment Trial (ONTT) demonstrated that IV methylprednisolone (1 g daily for 3 days) accelerated recovery from optic neuritis, and this regimen has been adopted for TM. [5]

Mechanism: Reduces inflammation, stabilizes blood-spinal cord barrier, suppresses immune cell infiltration

Administration:

• Dilute 1000 mg methylprednisolone in 100-250 mL normal saline
• Infuse over 60 minutes
• Monitor glucose (hyperglycaemia common), blood pressure, mood

Oral Taper: Some centers follow IV methylprednisolone with an oral prednisolone taper (e.g., 60 mg daily for 1 week, then taper over 2-4 weeks). Evidence for benefit is limited. [7]

Plasma Exchange (Second-Line)

Indication: Severe TM, LETM, no response to corticosteroids within 5-7 days, or AQP4-IgG positive NMOSD [5,19]

Protocol: 5-7 plasma exchange sessions on alternate days (total volume exchanged: 1-1.5 plasma volumes per session) [19]

Evidence: The Greenberg study (2007) showed that plasma exchange (PLEX) led to moderate or marked improvement in 42% of patients with steroid-refractory acute CNS demyelination (including TM). [19] A later study by Llufriu et al. (2009) confirmed benefit, particularly when PLEX was initiated early. [5]

Mechanism: Removes circulating pathogenic antibodies (especially AQP4-IgG in NMOSD), immune complexes, and inflammatory mediators

Contraindications: Haemodynamic instability, active infection, vascular access difficulties

Intravenous Immunoglobulin (IVIG)

Indication: Alternative to PLEX if PLEX is unavailable or contraindicated [7]

Protocol: IVIG 0.4 g/kg/day for 5 days (total dose 2 g/kg)

Evidence: Limited; case series suggest potential benefit. Less evidence than for PLEX. [7]

Supportive Care

Supportive care is critical to prevent complications and optimize recovery.

Long-Term Disease-Modifying Therapy

Long-term management depends on the underlying aetiology.

MS (Clinically Isolated Syndrome or Relapsing-Remitting MS)

If MRI brain shows 2 or more demyelinating lesions (meeting McDonald criteria), discuss disease-modifying therapy (DMT) with neurology. [9,21]

Options:

• Injectable therapies: Interferon-β (Avonex, Rebif), glatiramer acetate (Copaxone)
• Oral therapies: Dimethyl fumarate (Tecfidera), teriflunomide (Aubagio), fingolimod (Gilenya)
• High-efficacy therapies (for aggressive disease): Natalizumab (Tysabri), ocrelizumab (Ocrevus), alemtuzumab (Lemtrada)

Evidence: The CHAMPS trial showed that early interferon-β treatment after a clinically isolated syndrome reduced the risk of a second attack (conversion to MS) by 44%. [21]

NMOSD (AQP4-IgG Positive)

NMOSD has a high relapse risk (50-60% within 1 year without treatment). [4] Long-term immunosuppression is mandatory.

First-Line Options:

• Rituximab (anti-CD20 monoclonal antibody): 1000 mg IV at weeks 0 and 2, then every 6 months; off-label but widely used [20]
• Eculizumab (anti-C5 monoclonal antibody): Licensed for AQP4-IgG positive NMOSD; reduces relapse risk by 94% [20,22]
• Inebilizumab (anti-CD19 monoclonal antibody): Licensed for AQP4-IgG positive NMOSD; reduces relapse risk by 73% [20]
• Satralizumab (anti-IL-6 receptor monoclonal antibody): Licensed for NMOSD; subcutaneous administration [20]

Second-Line:

• Azathioprine (2-3 mg/kg/day)
• Mycophenolate mofetil (1000-1500 mg BD)

Evidence: The PREVENT trial (eculizumab) and N-MOmentum trial (inebilizumab) demonstrated significant relapse reduction in AQP4-IgG positive NMOSD. [20,22]

MOG-Antibody Disease

MOG-antibody disease may be monophasic or relapsing. [15] If relapsing, consider long-term immunosuppression (rituximab, azathioprine, mycophenolate). Evidence is still emerging.

Idiopathic TM

If no features of MS or NMOSD, no long-term immunosuppression is required. [1] Monitor for relapse. Risk of future MS is approximately 10-20%. [9]

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9. Complications

Acute Complications

Long-Term Complications

Relapse Risk

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10. Prognosis and Outcomes

Natural History

The prognosis of TM is highly variable and depends on severity, aetiology, and rapidity of treatment.

Recovery Timeline

Prognostic Factors

Long-Term Outcomes

Data from the Scott et al. study (1998) of 91 patients with acute TM: [8]

Factors predicting poor outcome: rapid onset (nadir less than 24 hours), complete paralysis at nadir, back pain at onset, advanced age. [8]

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11. Differential Diagnosis

It is critical to distinguish TM from other causes of acute myelopathy.

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12. Evidence and Guidelines

Major Guidelines

Diagnostic Criteria (TMCWG 2002) [1]

See section 7 above.

Key Studies

Evidence Levels for Interventions

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13. Patient Explanation

Simple Explanation

What is transverse myelitis? Transverse myelitis is a condition where part of your spinal cord becomes inflamed and swollen. The spinal cord is the bundle of nerves that runs down your back inside your spine. It carries messages between your brain and your body. When it becomes inflamed, these messages are disrupted, causing weakness, numbness, and problems with bladder and bowel control.

What causes it? In many cases, it happens after a viral infection—the immune system becomes confused and attacks the spinal cord by mistake (like "friendly fire"). In some people, it can be the first sign of another condition like multiple sclerosis (MS) or a condition called neuromyelitis optica (NMO). Sometimes we don't find a specific cause at all.

What are the symptoms?

• Weakness in your legs (and sometimes arms)—this can range from mild to complete paralysis
• Numbness or tingling, often with a "level" on your body below which sensation is changed
• Problems with bladder and bowel control (very common)
• Back pain
• Tight "band-like" feeling around your chest or abdomen

What tests will I need?

• MRI scan of your spine (and brain): This shows the inflammation in your spinal cord
• Lumbar puncture (spinal tap): A small sample of fluid from around your spinal cord to look for signs of inflammation
• Blood tests: To look for specific antibodies and rule out other causes

How is it treated?

• Steroids: High-dose steroid injections through a drip for 3-5 days to reduce inflammation
• Plasma exchange: If steroids don't work well enough, we may do a treatment that filters your blood to remove harmful antibodies
• Supportive care: We'll help manage bladder problems, prevent blood clots, and start physiotherapy early
• Rehabilitation: You'll work with physiotherapists and occupational therapists to regain strength and function

What is the outcome? Recovery varies a lot between people. About one-third of people recover well, one-third have some lasting symptoms but can still walk and do most things, and one-third have more significant disability. Recovery usually happens over weeks to months. Some symptoms may be permanent, but most people continue to improve over the first 6-12 months.

Will it happen again? It depends on the underlying cause. If it's a one-off (idiopathic), it usually doesn't come back. If it's related to MS or NMO, you may need long-term treatment to prevent further attacks. Your doctors will do tests to work out which type you have.

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14. Rehabilitation and Long-Term Management

Acute Phase Rehabilitation (0-4 Weeks)

Rehabilitation should commence within 24-48 hours of admission, even during the acute inflammatory phase. [7]

Goals:

• Prevent complications (contractures, pressure ulcers, DVT)
• Maintain range of motion
• Begin strengthening
• Assess and address functional deficits

Subacute Rehabilitation (4-12 Weeks)

Transfer to inpatient neurorehabilitation unit if medically stable but functionally dependent. [7]

Multidisciplinary Team:

• Rehabilitation physician
• Physiotherapist (mobility, transfers, gait)
• Occupational therapist (ADLs, adaptive strategies)
• Speech and language therapist (swallowing if bulbar involvement)
• Psychologist (adjustment, mood)
• Specialist nurse (continence, skin care)
• Social worker (discharge planning, support services)

Key Focus Areas:

Mobility and Gait Training

Bladder Management

Neurogenic bladder is one of the most significant long-term complications. [1,7]

Urological Monitoring:

• Post-void residual volume (PVR) monthly initially, then 3-6 monthly
• Urine culture if symptomatic UTI (fever, dysuria, haematuria, increased spasticity)
• Renal ultrasound annually (check for hydronephrosis)
• Urodynamic studies if management challenges

Bowel Management

Neurogenic bowel causes constipation and/or faecal incontinence. [7]

Protocol:

1. Dietary modification: High-fibre diet (25-30 g/day); adequate fluid intake (2-3 L/day)
2. Regular toileting: Scheduled bowel care (e.g., after breakfast to utilize gastrocolic reflex)
3. Stool softeners: Docusate 200-400 mg/day
4. Stimulant laxatives: Senna 15-30 mg at night; bisacodyl 10-20 mg
5. Rectal interventions: Glycerine suppositories or bisacodyl suppositories; digital stimulation if needed
6. Specialist input: Colorectal surgeon or gastroenterologist if refractory

Pain Management

Neuropathic pain is common (50-80% of patients) and can be severe and refractory. [7]

Pharmacological Management (Stepwise):

Non-Pharmacological Management:

• Transcutaneous electrical nerve stimulation (TENS)
• Acupuncture
• Cognitive behavioural therapy (CBT)
• Multidisciplinary pain clinic referral

Spasticity Management

Spasticity develops weeks after the acute phase and can interfere with function, cause pain, and impair sleep. [7]

Non-Pharmacological:

• Regular physiotherapy and stretching exercises (daily)
• Identify and treat triggers (UTI, constipation, pressure ulcers, pain)
• Positioning and splinting

Pharmacological:

Interventional:

• Botulinum toxin (focal spasticity): Injections into spastic muscles (e.g., adductors, hamstrings); lasts 3-4 months
• Intrathecal baclofen pump: For severe generalized spasticity unresponsive to oral agents; requires surgical implantation
• Tendon lengthening or release: For fixed contractures (surgical)

Long-Term Follow-Up (Beyond 12 Months)

Outpatient Monitoring:

Red Flags for Relapse:

• New or worsening weakness
• New sensory symptoms
• Worsening bladder/bowel function
• New visual symptoms (optic neuritis—suggests NMOSD or MS)
• Increased spasticity not explained by triggers

Action if Relapse Suspected:

• Urgent MRI spine and brain
• Neurology review
• Consider repeat LP
• High-dose IV methylprednisolone if confirmed relapse [5,7]

Psychological Support

Adjustment to disability and chronic illness is challenging. Depression occurs in 30-50% of patients. [7]

Psychological Interventions:

• Individual counselling or psychotherapy
• Cognitive behavioural therapy (CBT) for depression and chronic pain
• Support groups (e.g., Transverse Myelitis Society)
• Family therapy if needed

Pharmacological Treatment of Depression:

• SSRIs (sertraline 50-200 mg/day, citalopram 20-40 mg/day)
• SNRIs (duloxetine 60 mg/day—also helps neuropathic pain)

Returning to Work and Quality of Life

Approximately 50-70% of patients with good functional recovery return to work, often with modifications. [8]

Vocational Rehabilitation:

• Occupational health assessment
• Workplace adaptations (ergonomic adjustments, flexible hours)
• Disability employment services
• Graduated return to work

Driving:

• DVLA notification required (UK) if neurological disability
• Assess visual fields, limb function, reaction time, seizures
• Occupational therapy driving assessment if borderline
• May require vehicle adaptations (hand controls)

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15. Special Populations

Paediatric Transverse Myelitis

TM in children (less than 18 years) has distinct features. [12]

Epidemiology:

• Annual incidence: 1-2 per million children [12]
• Peak age: 4-8 years
• Often post-infectious (Mycoplasma, enteroviruses, VZV) [12,13]

Clinical Features:

• May present with systemic symptoms (fever, malaise) more commonly than adults
• Higher proportion are LETM in children
• MOG-antibody disease more common in children than adults [15]
• Better overall recovery than adults (70-80% good outcome) [12]

Investigations:

• Same as adults (MRI spine/brain, LP, AQP4-IgG, MOG-IgG)
• Consider Mycoplasma serology and PCR [13]

Management:

• IV methylprednisolone 30 mg/kg/day (max 1 g) for 3-5 days [12]
• PLEX or IVIG if steroid-refractory
• Multidisciplinary paediatric neurorehabilitation

Long-Term:

• Monitor for relapse (risk of MS or MOG-antibody disease)
• Ensure educational support and accommodations
• Paediatric neurology follow-up

Pregnancy and Transverse Myelitis

TM in pregnancy or postpartum is rare but presents unique challenges.

Management During Pregnancy:

• Acute TM: IV methylprednisolone is safe in pregnancy (Category C—benefits usually outweigh risks) [7]
• PLEX: Safe if needed
• MRI: Safe without gadolinium (gadolinium crosses placenta—use only if essential)
• Obstetric collaboration: Monitor fetal well-being; consider corticosteroid cover for labour

Breastfeeding:

• High-dose corticosteroids: small amounts excreted in breast milk; generally considered compatible
• DMTs for MS: check compatibility (many are contraindicated)

NMOSD in Pregnancy:

• High relapse risk postpartum [4]
• Rituximab: discontinue before conception (teratogenic)
• Azathioprine: relatively safe (continue if disease control essential)
• Consider prophylactic immunosuppression postpartum

Elderly Patients

TM in the elderly (more than 65 years) has poorer prognosis. [8]

Challenges:

• Higher comorbidity burden (cardiovascular disease, diabetes, renal impairment)
• Polypharmacy and drug interactions
• Higher risk of complications (UTI, pressure ulcers, DVT)
• Slower recovery; higher risk of permanent disability
• Cognitive impairment may complicate rehabilitation

Management Modifications:

• Careful medication selection (avoid anticholinergics if cognitive impairment)
• Early involvement of geriatrics or elderly care medicine
• Enhanced supportive care and pressure area management
• Social services input for discharge planning

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16. Emerging Therapies and Research

Novel Immunotherapies

Emerging therapies are being investigated for acute TM and relapse prevention in NMOSD.

Stem Cell Therapy

Autologous haematopoietic stem cell transplantation (AHSCT) has been explored for severe refractory MS but is not standard for TM. [21]

Biomarkers

Research is focused on identifying biomarkers to predict prognosis and guide therapy:

• Serum neurofilament light chain (NfL): Marker of axonal injury; elevated levels predict poor recovery [8]
• Glial fibrillary acidic protein (GFAP): Marker of astrocyte injury; may predict NMOSD relapse [4]

Clinical Trials

Patients with TM, especially those with NMOSD, should be informed about ongoing clinical trials. Resources:

• ClinicalTrials.gov
• Guthy-Jackson Charitable Foundation (NMOSD trials)
• Transverse Myelitis Association

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17. Examination Focus

Common Exam Questions

High-Yield Viva Points

Common Mistakes

Model Viva Answer

Q: A 28-year-old woman presents with bilateral leg weakness, numbness up to the umbilicus, and urinary retention developing over 2 days. How would you approach this patient?

A: "This presentation is concerning for an acute myelopathy. My first priority is to exclude cord compression, which is a neurosurgical emergency. I would arrange an emergency MRI of the whole spine with gadolinium contrast.

If the MRI shows intramedullary T2 hyperintensity with cord swelling but no external compression, this suggests transverse myelitis. I would also request MRI brain to look for demyelinating lesions suggestive of MS, as the presence of brain lesions significantly increases the risk of developing MS.

I would perform a lumbar puncture after imaging to look for CSF pleocytosis, elevated protein, and oligoclonal bands. I would send bloods for AQP4-IgG and MOG-IgG antibodies, particularly if the MRI shows longitudinally extensive transverse myelitis (3 or more segments), which is characteristic of NMOSD.

For acute treatment, I would start IV methylprednisolone 1000 mg daily for 3-5 days. If there is no response, or if the patient has LETM or severe disease, I would discuss plasma exchange with neurology.

Supportive care is critical: DVT prophylaxis with enoxaparin, urinary catheterization for retention, pressure area care, and early physiotherapy.

The prognosis depends on severity. Partial TM has a good prognosis (70-90% good recovery), whereas complete TM has a worse prognosis (30-50% good recovery). Long-term management depends on whether this is idiopathic, MS-associated, or NMOSD." [1,4,5,7,8,9,11]

Examination Cheat Sheet

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15. References

1. Transverse Myelitis Consortium Working Group. Proposed diagnostic criteria and nosology of acute transverse myelitis. Neurology. 2002;59(4):499-505. doi:10.1212/wnl.59.4.499. PMID: 12221161

2. West TW, Hess C, Cree BA. Acute transverse myelitis: demyelinating, inflammatory, and infectious myelopathies. Semin Neurol. 2012;32(2):97-113. doi:10.1055/s-0032-1322586. PMID: 22961186

3. Beh SC, Greenberg BM, Frohman T, Frohman EM. Transverse myelitis. Neurol Clin. 2013;31(1):79-138. doi:10.1016/j.ncl.2012.09.008. PMID: 23186897

4. Wingerchuk DM, Banwell B, Bennett JL, et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 2015;85(2):177-189. doi:10.1212/WNL.0000000000001729. PMID: 26092914

5. Llufriu S, Castillo J, Blanco Y, et al. Plasma exchange for acute attacks of CNS demyelination: Predictors of improvement at 6 months. Neurology. 2009;73(12):949-953. doi:10.1212/WNL.0b013e3181b87965. PMID: 19770468

6. Berman M, Feldman S, Alter M, Zilber N, Kahana E. Acute transverse myelitis: incidence and etiologic considerations. Neurology. 1981;31(8):966-971. doi:10.1212/wnl.31.8.966. PMID: 6115335

7. Scott TF, Frohman EM, De Seze J, Gronseth GS, Weinshenker BG; Therapeutics and Technology Assessment Subcommittee of American Academy of Neurology. Evidence-based guideline: clinical evaluation and treatment of transverse myelitis: report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology. 2011;77(24):2128-2134. doi:10.1212/WNL.0b013e31823dc535. PMID: 22156988

8. Scott TF, Bhagavatula K, Snyder PJ, Chieffe C. Transverse myelitis. Comparison with spinal cord presentations of multiple sclerosis. Neurology. 1998;50(2):429-433. doi:10.1212/wnl.50.2.429. PMID: 9841711

9. Frohman EM, Wingerchuk DM. Clinical practice. Transverse myelitis. N Engl J Med. 2010;363(6):564-572. doi:10.1056/NEJMcp1001112. PMID: 20818891

10. Kitley J, Leite MI, Nakashima I, et al. Prognostic factors and disease course in aquaporin-4 antibody-positive patients with neuromyelitis optica spectrum disorder from the United Kingdom and Japan. Brain. 2012;135(Pt 6):1834-1849. doi:10.1093/brain/aws109. PMID: 22993290

11. Weinshenker BG, Wingerchuk DM, Vukusic S, et al. Neuromyelitis optica IgG predicts relapse after longitudinally extensive transverse myelitis. Ann Neurol. 2006;59(3):566-569. doi:10.1002/ana.20770. PMID: 16636238

12. Absoud M, Greenberg BM, Lim M, et al. Pediatric transverse myelitis. Neurology. 2016;87(9 Suppl 2):S46-S52. doi:10.1212/WNL.0000000000002820. PMID: 26888959

13. Kincaid O, Lipton HL. Viral myelitis: an update. Curr Neurol Neurosci Rep. 2006;6(6):469-474. doi:10.1007/s11910-006-0045-1. PMID: 17074281

14. Alexopoulos H, Magira E, Bitzogli K, et al. Anti-SARS-CoV-2 antibodies in the CSF, blood-brain barrier dysfunction, and neurological outcome: Studies in 8 stuporous and comatose patients. Neurol Neuroimmunol Neuroinflamm. 2020;7(6):e893. doi:10.1212/NXI.0000000000000893. PMID: 32938785

15. Jarius S, Ruprecht K, Kleiter I, et al. MOG-IgG in NMO and related disorders: a multicenter study of 50 patients. Part 2: Epidemiology, clinical presentation, radiological and laboratory features, treatment responses, and long-term outcome. J Neuroinflammation. 2016;13(1):280. doi:10.1186/s12974-016-0718-0. PMID: 27793206

16. Karussis D, Petrou P. The spectrum of post-vaccination inflammatory CNS demyelinating syndromes. Autoimmun Rev. 2014;13(3):215-224. doi:10.1016/j.autrev.2013.10.003. PMID: 24514081

17. Darouiche RO. Spinal epidural abscess. N Engl J Med. 2006;355(19):2012-2020. doi:10.1056/NEJMra055111. PMID: 17093252

18. Jacob A, Weinshenker BG. An approach to the diagnosis of acute transverse myelitis. Semin Neurol. 2008;28(1):105-120. doi:10.1055/s-2007-1019130. PMID: 18256989

19. Greenberg BM, Thomas KP, Krishnan C, Kaplin AI, Calabresi PA, Kerr DA. Idiopathic transverse myelitis: corticosteroids, plasma exchange, or cyclophosphamide. Neurology. 2007;68(19):1614-1617. doi:10.1212/01.wnl.0000260970.63493.c8. PMID: 17452576

20. Pittock SJ, Berthele A, Fujihara K, et al. Eculizumab in Aquaporin-4-Positive Neuromyelitis Optica Spectrum Disorder. N Engl J Med. 2019;381(7):614-625. doi:10.1056/NEJMoa1900866. PMID: 31050279

21. Jacobs LD, Beck RW, Simon JH, et al. Intramuscular interferon beta-1a therapy initiated during a first demyelinating event in multiple sclerosis. CHAMPS Study Group. N Engl J Med. 2000;343(13):898-904. doi:10.1056/NEJM200009283431301. PMID: 11006365

22. Cree BAC, Bennett JL, Kim HJ, et al. Inebilizumab for the treatment of neuromyelitis optica spectrum disorder (N-MOmentum): a double-blind, randomised placebo-controlled phase 2/3 trial. Lancet. 2019;394(10206):1352-1363. doi:10.1016/S0140-6736(19)31817-3. PMID: 31495497