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What is Transverse Myelitis?
Transverse myelitis is an inflammation of the spinal cord, the part of the central nervous system responsible for:
- Transmitting motor impulses from the brain to muscles and organs throughout the body
- Relaying sensory information from the body back to the brain
Understanding the Name
- Myelitis: Inflammation (-itis) of the spinal cord (myelo-, from the Greek for “marrow” or “cord”)
- Transverse: Refers to a characteristic band-like sensation that appears to cross the trunk of the body, below which specific sensory and motor changes are perceived, as if an invisible band were wrapped around the torso.
Overview of Clinical Presentation
Transverse myelitis typically produces a combination of:
- Pain: often back pain or pain radiating into the limbs
- Muscle weakness: in the legs and sometimes the arms
- Sensory disturbances: abnormal or reduced sensation below the affected spinal cord level
- Bladder and bowel dysfunction
Symptoms can develop suddenly (over hours) or more gradually (over days or weeks).
Who Is Affected?
Transverse myelitis can occur at any age, in any sex, and across all ethnic groups, with no known genetic predisposition. There appear to be two peaks of incidence: between 10 and 19 years of age, and between 30 and 39 years.
Prognosis
The recovery trajectory varies considerably between individuals:
- Some patients achieve complete or near-complete recovery.
- The majority experience at least partial recovery, typically within the first 3 months of the acute attack.
- Others are left with permanent neurological deficits that affect daily activities.
- Relapses (recurrent episodes) are possible, particularly when the underlying cause is an autoimmune condition such as multiple sclerosis or neuromyelitis optica.
There is no specific cure for transverse myelitis, but several treatments are available to reduce the severity of the acute episode and minimize permanent neurological disability.
Causes
The exact cause of transverse myelitis is not always identifiable. When no cause can be determined, the condition is classified as idiopathic transverse myelitis.
When a cause is identified, it falls into one of several broad categories:
Immune System Disorders
- Neuromyelitis optica (NMO / Devic’s disease): A serious inflammatory condition specifically targeting the optic nerves and spinal cord. Aquaporin-4 (AQP4) antibodies are the hallmark biomarker.
- Multiple sclerosis (MS): An autoimmune demyelinating disease of the central nervous system in which immune cells attack the protective myelin sheath covering the brain, optic nerves, and spinal cord.
- Post-infectious or post-vaccine autoimmune phenomenon: The immune system, while responding to an infection or very rarely a vaccine, erroneously attacks spinal cord tissue.
- Paraneoplastic syndrome: An immune response triggered by a tumor elsewhere in the body that causes collateral damage to the nervous system.
Viral Infections
Many viruses have been associated with transverse myelitis, including:
- Herpesviruses: Herpes zoster (causing chickenpox and shingles), herpes simplex, cytomegalovirus (CMV), Epstein-Barr virus (mononucleosis)
- Flaviviruses: West Nile virus, Zika virus
- Other viruses: Influenza, echovirus, hepatitis B virus (HBV), mumps virus (parotitis), paramyxovirus (measles), rubella virus (rubella)
Note: Distinguishing between direct viral invasion of the spinal cord and a post-infectious immune-mediated response is often clinically challenging.
Bacterial Infections
Bacterial causes of transverse myelitis include:
- Syphilis
- Tuberculosis
- Actinomycosis
- Whooping cough (pertussis)
- Tetanus
- Diphtheria
- Lyme disease
- Bacterial skin infections, middle ear infections, Campylobacter jejuni gastroenteritis, and bacterial pneumonia from Mycoplasma pneumoniae
Fungal, Parasitic, and Other Infections
- Fungal infections of the spinal cord (rare; typically in immunocompromised patients)
- Parasites: Toxoplasmosis, cysticercosis, schistosomiasis, strongyloidiasis
Systemic Inflammatory and Autoimmune Disorders
Several systemic diseases can cause spinal cord inflammation as part of their broader involvement:
- Sarcoidosis
- Systemic lupus erythematosus (SLE)
- Sjögren’s syndrome
- Mixed connective tissue disease
- Scleroderma
- Behçet’s syndrome
Vascular Disorders
Vascular abnormalities can cause ischemic or hemorrhagic spinal cord damage mimicking transverse myelitis:
- Arteriovenous malformations (AVM)
- Dural arteriovenous fistulas
- Intraspinal cavernous malformations
Partial vs. Complete Transverse Myelitis
A clinically important distinction:
- Partial transverse myelitis: Only a portion of the spinal cord’s cross-sectional area is inflamed; relatively more common in multiple sclerosis. Tends to be associated with better recovery.
- Complete transverse myelitis: Affects the full cross-section, causing severe bilateral deficits; more characteristic of neuromyelitis optica. Associated with more severe attacks and poorer recovery potential.
Symptoms
Onset Patterns
Transverse myelitis can develop in two temporal patterns:
- Acute: Symptoms appear and progress within hours to days
- Subacute: Slower progression over 1 to 4 weeks
Level-Dependent Symptoms
The level of the spinal cord affected by inflammation determines which body regions develop symptoms. As a general rule:
- Symptoms appear at and below the affected spinal level
- Both sides of the body are generally involved (bilateral)
- Higher spinal cord lesions (cervical region) also affect the arms; lower lesions primarily affect the legs
Core Symptoms
Muscle Weakness
- Weakness typically begins in the legs (and arms if the lesion involves the cervical spinal cord).
- Weakness can progress to paraparesis (partial paralysis of the legs) and, in severe cases, to paraplegia (complete paralysis of the lower extremities), potentially requiring wheelchair use.
Pain
- Early symptoms often include lower back pain and/or sharp, shooting pains radiating into the legs, arms, or around the torso.
- Pain may be the initial or most prominent symptom in some patients.
Sensory Disturbances
- Paresthesias: Abnormal sensations including burning, tingling, pins-and-needles, numbness, cold sensations, or tingling/formication in the legs and trunk.
- Sensory loss: Reduced or absent sensation below the lesion level.
- Abnormal sensations in the trunk region and genitals are particularly common.
- The characteristic “band-like” sensation around the torso described as tightness or pressure at a specific spinal level is a classic symptom.
Bladder and Bowel Dysfunction
- Urinary urgency or increased frequency
- Urinary incontinence
- Fecal incontinence
- Constipation
- Urinary retention (inability to pass urine)
Additional Symptoms
Many patients also report:
- Muscle spasms
- General sense of discomfort and malaise
- Headache
- Fever
- Loss of appetite
- Respiratory difficulties (if upper spinal cord levels are involved)
- Sexual dysfunction
Psychological Impact
Depression and anxiety are common secondary consequences of transverse myelitis, developing as reactions to the forced lifestyle changes, chronic pain, and stress associated with this condition. These should be actively identified and addressed as part of holistic management.
Complications
The clinical course of transverse myelitis and its ultimate recovery are highly variable and difficult to predict. Several factors influence prognosis:
Prognostic Indicators
| Factor | Associated Outcome |
|---|---|
| Rapid, severe symptom onset | Generally indicates worse prognosis |
| Gradual onset, milder symptoms | Often associated with better recovery |
| Negative antibody tests (e.g., AQP4) | May suggest a more favorable prognosis |
| Partial vs. complete lesion | Partial lesions have better recovery potential |
| Underlying cause | Idiopathic or post-infectious forms tend to recover better than NMO-associated forms |
Timeline of Recovery
- The most substantial neurological improvements typically occur within the first 3 months after the acute attack.
- Full recovery, when it occurs, may require months to years.
- Many patients achieve only partial recovery and are left with residual deficits.
Permanent Neurological Complications
Potential long-term neurological sequelae include:
- Chronic pain (neuropathic and musculoskeletal)
- Spasticity: Muscle stiffness and spasms that can be disabling
- Paraparesis or paraplegia: Permanent lower limb weakness or paralysis
- Bladder and bowel dysfunction: Permanent incontinence or retention
- Sexual dysfunction
- Fatigue
- Psychological complications: Depression, anxiety, adjustment disorders
Diagnosis
The diagnosis of transverse myelitis is established through a systematic multi-step evaluation:
1. Medical History and Neurological Examination
A detailed medical history explores:
- The onset, progression, and character of symptoms
- Recent infections, vaccinations, or other potential triggering events
- Personal or family history of autoimmune or neurological conditions
A careful neurological examination assesses:
- Motor strength and tone
- Sensory level (identifying the dermatome below which sensation is altered)
- Reflexes
- Bladder and bowel function
- Coordination
2. Neuroimaging
- MRI (Magnetic Resonance Imaging): The gold standard for diagnosing transverse myelitis. MRI with and without contrast (gadolinium) of the entire spine and brain provides detailed images revealing:
- The location, extent, and characteristics of spinal cord lesions
- Whether brain lesions are present (which may suggest MS or NMO)
- The signal characteristics of lesions (helpful in distinguishing causes)
- CT scan: Can be used to detect localized inflammatory changes or rule out structural causes, but is less informative than MRI for spinal cord evaluation.
3. Blood Tests
Blood tests are essential to:
- Exclude treatable systemic conditions:
- HIV infection
- Vitamin B12 deficiency (can cause subacute combined degeneration of the spinal cord, mimicking myelitis)
- Detect autoantibodies associated with specific conditions:
- Aquaporin-4 (AQP4) antibodies: Highly specific for neuromyelitis optica
- MOG (myelin oligodendrocyte glycoprotein) antibodies: Associated with MOG-antibody disease
- Antinuclear antibodies (ANA), anti-dsDNA: For lupus
- Anti-Ro/La: For Sjögren’s syndrome
- Paraneoplastic antibodies: When tumor-related NMO is suspected
- Screen for infectious causes: Viral and bacterial serology as clinically indicated
4. Cerebrospinal Fluid (CSF) Analysis
Lumbar puncture (spinal tap) and analysis of the cerebrospinal fluid provides valuable information:
- Elevated white blood cells (pleocytosis): Suggests active inflammation or infection
- Elevated protein: Non-specific but common in inflammatory spinal cord disease
- Oligoclonal bands: Characteristic of MS
- PCR for infectious agents: Detects viral or bacterial DNA/RNA in CSF
- Culture and sensitivity: For bacterial or fungal infections
Treatment
Treatment of transverse myelitis aims to:
- Treat any underlying infection
- Reduce spinal cord inflammation
- Suppress the abnormal immune response
- Relieve symptoms and prevent complications
Acute Phase Treatment
Intravenous Corticosteroids
High-dose intravenous corticosteroids (typically methylprednisolone, 1g/day for 3–5 days) are the first-line acute treatment. They act to:
- Reduce spinal cord edema (swelling)
- Suppress inflammatory activity
- Decrease immune system activity
Corticosteroids may also be used to reduce the frequency and severity of subsequent attacks in relapsing conditions.
Plasmapheresis (Plasma Exchange)
For patients who do not respond adequately to corticosteroids, plasmapheresis is an important second-line intervention. This procedure:
- Removes the patient’s plasma (the fluid component of blood containing antibodies and white blood cells)
- Replaces it with substitute fluids
- Reduces circulating pathological autoantibodies and inflammatory mediators
Multiple sessions over 1–2 weeks are typically required.
Intravenous Immunoglobulins (IVIg)
An infusion of pooled immunoglobulins from healthy donors that can:
- Bind to and neutralize pathological autoantibodies
- Modulate the immune response through multiple mechanisms
Often used in combination with or as an alternative to corticosteroids and plasmapheresis.
Pain Management
Nociceptive (Musculoskeletal) Pain
- NSAIDs (anti-inflammatory drugs): Ibuprofen, ketoprofen, naproxen
- Paracetamol (acetaminophen)
Neuropathic Pain
Neuropathic pain, caused by damage to the spinal cord itself, requires specific treatments:
- Antidepressants: SNRIs such as duloxetine, or tricyclics such as amitriptyline
- Anticonvulsants: Gabapentin or pregabalin, first-line agents for neuropathic pain
- Muscle relaxants / antispastics: Baclofen, tizanidine, or cyclobenzaprine for spasticity and muscle spasms
Antiviral Therapy
When viral infection of the spinal cord is confirmed or strongly suspected (e.g., herpes simplex or varicella-zoster virus), antiviral agents (e.g., intravenous acyclovir) are administered as a matter of urgency.
Management of Specific Complications
Depending on the clinical picture, additional treatments may include:
- Bladder dysfunction: Catheterization, anticholinergic medications, or bladder training
- Bowel dysfunction: Bowel management programs, laxatives, dietary modifications
- Spasticity: Oral baclofen, tizanidine, or in severe cases intrathecal baclofen pump or botulinum toxin injections
- Sexual dysfunction: Specialist counseling and targeted pharmacotherapy
- Depression and anxiety: Psychotherapy and pharmacological treatment when indicated
Long-Term Immunotherapy for Underlying Conditions
Patients diagnosed with multiple sclerosis or neuromyelitis optica require long-term disease-modifying therapy to reduce the frequency and severity of future attacks. These may include:
- Immunomodulatory drugs
- Immunosuppressive agents
- Monoclonal antibodies (e.g., rituximab, eculizumab, inebilizumab, satralizumab, several of which are specifically approved for NMO)
Rehabilitation and Long-Term Therapy
For patients with permanent or residual neurological deficits, multidisciplinary rehabilitation is an essential component of care. While rehabilitation cannot repair the underlying nerve damage, it can significantly improve function, independence, and quality of life.
A comprehensive rehabilitation team may include:
- Physiatrists (rehabilitation medicine physicians)
- Physical therapists
- Occupational therapists
- Speech-language therapists (if swallowing or speech affected)
- Psychologists
- Social workers
- Vocational rehabilitation counselors
Physical Therapy
Physical therapy aims to:
- Maintain and improve muscle strength and flexibility
- Improve coordination and balance
- Reduce spasticity
- Enhance bladder and bowel control through pelvic floor training
- Improve joint range of motion
- Provide gait training and training with assistive devices (canes, walkers, wheelchair)
Occupational Therapy
Occupational therapists teach adaptive strategies and use of assistive devices to help patients regain independence in daily activities, including:
- Bathing and personal hygiene
- Dressing
- Meal preparation
- Household management
- Driving adaptations
Vocational Rehabilitation
Vocational rehabilitation supports patients in:
- Identifying and developing transferable work skills
- Exploring employment opportunities compatible with their abilities
- Navigating workplace accommodations
Psychotherapy and Psychological Support
Psychotherapy provides:
- Strategies for coping with chronic illness and disability
- Tools for managing depression, anxiety, and grief
- Support for adjustment to life with a chronic neurological condition
- Techniques for managing chronic pain (e.g., cognitive behavioral therapy for pain)
