Makindo Medical Notes

Related Subjects: |Neurological History taking |Motor Neuron Disease (MND-ALS) |Miller-Fisher syndrome |Guillain Barre Syndrome |Multifocal Motor Neuropathy with Conduction block | Inclusion Body Myositis | Multiple Sclerosis (MS) Demyelination | Transverse myelitis | Acute Disseminated Encephalomyelitis |Cervical spondylosis |Spinal Cord Anatomy |Acute Disc Prolapse |Spinal Cord Compression |Spinal Cord Haematoma |Foix-Alajouanine syndrome |Cauda Equina |Conus Medullaris syndrome |Anterior Spinal Cord syndrome |Central Spinal Cord syndrome |Brown-Sequard Spinal Cord syndrome

About

• Transverse Myelitis is an inflammatory disorder affecting one or multiple levels of the spinal cord, leading to neurological dysfunction. It has a bimodal age distribution, most common in individuals aged 10-20 and those over 40.
• The condition presents with a wide range of symptoms, reflecting the extent and location of spinal cord involvement.

Aetiology

• Transverse myelitis can involve both grey and white matter of the spinal cord, causing sensory, motor, and autonomic dysfunction.
• The degree of spinal cord involvement varies, ranging from a partial lesion affecting one side to a complete lesion impacting the entire cord.

Causes

• Autoimmune conditions: Multiple sclerosis (MS), Acute Disseminated Encephalomyelitis (ADEM), Neuromyelitis Optica Spectrum Disorders (NMOSD).
• Systemic autoimmune diseases: Systemic Lupus Erythematosus (SLE), Sjögren's syndrome, vasculitis, sarcoidosis.
• Infections: Viral infections (e.g., Coxsackievirus, Echovirus, HIV, EBV, Zika), bacterial infections (e.g., Mycoplasma pneumoniae), and rare fungal or parasitic infections.
• Paraneoplastic syndromes: Associated with underlying malignancies, especially small cell lung cancer and other tumors.
• Vaccination-associated: Rare cases post-vaccination (e.g., flu vaccine), though causal association remains uncertain.

Risk Factors

• History of autoimmune disease or recent infection.
• Genetic predisposition to autoimmune or demyelinating disorders.
• Recent immunizations or vaccinations (in rare cases).

Clinical Presentation

• Onset: Symptoms may develop rapidly (within hours to days) or gradually over weeks.
• Initial symptoms: Fever, myalgia, back pain, and band-like paraesthesia (tightening or squeezing sensation) around the trunk.
• Pain: Acute pain localized to the spine, often radiating to arms or legs, which may mimic radiculopathy.
• Weakness: Progressive spastic weakness in the legs, arms, or both, leading to difficulty with walking or using the hands.
• Sensory symptoms: Loss of sensation to position, pain, and temperature, typically below a defined spinal level.
• Autonomic dysfunction: Urinary and bowel symptoms such as urgency, retention, incontinence, and constipation.

MRI

Red Flags

• Sudden and severe progression of symptoms.
• Respiratory muscle weakness (suggesting higher-level spinal cord involvement).
• Evidence of systemic infection or associated malignancy (e.g., fever, weight loss).

Investigations

• MRI with Gadolinium: Essential to exclude compressive lesions and often reveals T2 hyperintensities in affected spinal cord segments.
• CSF Analysis: To assess for inflammation (pleocytosis), elevated protein, and oligoclonal bands, indicative of inflammatory or autoimmune pathology.
• Blood tests: Autoimmune panel (ANA, ANCA), infectious serologies (HIV, EBV, Zika), and screening for paraneoplastic antibodies.
• Evoked Potentials: Can help detect electrical conduction deficits in the spinal cord, especially in demyelinating diseases.

Differential Diagnosis

• Spinal cord compression (e.g., tumor, abscess, or herniated disc).
• Multiple sclerosis and other demyelinating diseases.
• Spinal cord infarction or ischaemia.
• Neuromyelitis optica spectrum disorders (NMOSD).
• Autoimmune myelopathies (e.g., from SLE or Sjögren’s syndrome).

Management

• Acute phase:
  • High-dose corticosteroids: IV methylprednisolone for 3-5 days to reduce inflammation.
  • Plasma exchange (PLEX): Considered for severe or steroid-refractory cases, particularly in NMOSD.
  • IV Immunoglobulins (IVIG): An option in cases unresponsive to steroids or in certain autoimmune etiologies.
• Supportive care: Includes physical therapy, pain management, and occupational therapy to aid mobility and function.
• Long-term management: Monitoring for relapse, rehabilitation, and potentially disease-modifying therapies for autoimmune conditions (e.g., MS, NMOSD).

Prognosis

• Prognosis varies based on etiology, severity, and response to treatment.
• Recovery may be partial or full, taking weeks to months; some cases may lead to long-term disability.
• Early intervention with steroids or immunosuppression is associated with better outcomes, especially in inflammatory causes.

Note: Rapid initiation of treatment for autoimmune or inflammatory causes can improve outcomes and reduce long-term disability in transverse myelitis.