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, mostly 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.

Visual representation of spine after Transverse Myelitis

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

  1. Stretching programme – to prevent contractures.
  2. Joint care
  3. Strengthening: lower limb muscle and core
  4. Gait training: provision of equipment, orthoses, and wheelchairs.
  5. Wheelchair training: teach skills to use a wheelchair,
  6. Transfers: teach transfers (getting in and out of a wheelchair, bed, car, shower/bath, and onto and off a toilet)
  7. 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.
  8. Aid control of spasticity
  9. Hydrotherapy treatment
  10. Breathing exercises and chest clearance techniques
  11. Pain reduction/management – TENS, heat, or cold therapy.
  12. Teach caring and handling techniques to patient and caregivers.
  13. 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

Beginning Stance/Activity: Heel strike

Ending Stance/Activity: Swing through to forefoot stance

Location: Parallel Bars

Beginning Stance/Activity: Heel strike emphasized through the full gait cycle

Ending Stance/Activity: Multiple steps through gait cycle to the end of parallel bars

30 feet in the gym with hand-held assistance

Location: Parallel Bars

Beginning Stance/Activity: Full gait cycle (emphasis on heel strike)

Ending Stance/Activity: 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:

  1. Multiple Sclerosis
  2. Guillain Barre Syndrome
  3. Disc herniation
  4. Parainfectious myelitis
  5. Neuromyelitis optica (Devic’s disease)
  6. Myelitis related to systemic diseases, such as systemic lupus erythematosus
  7. Compression of the spinal cord caused by trauma e.g., vertebral fractures
  8. Epidural or subdural hematoma
  9. Epidural and/or paraspinal abscess
  10. 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.

FAQs

Q: What is Transverse Myelitis?

Transverse Myelitis is a rare autoimmune spinal cord disorder characterized by inflammation of the spinal cord, leading to a range of neurological symptoms such as weakness, sensory alterations, and bladder dysfunction.”

Q: What causes Transverse Myelitis?

Transverse Myelitis can be triggered by various factors, including infections (viral and bacterial), autoimmune processes, and associations with conditions like Acute Disseminated Encephalomyelitis (ADEM) and Multiple Sclerosis (MS).

Q: What are the symptoms of Transverse Myelitis?

The symptoms of Transverse Myelitis include autonomic dysfunction (bowel and bladder issues), sensory loss, progressive weakness, pain, autonomic dysreflexia, pyramidal weakness, vision loss, tremors, fatigue, and psychological distress.

Q: How is Transverse Myelitis diagnosed?

Transverse Myelitis is diagnosed through a combination of blood tests, imaging techniques (MRI and CT scans), cerebrospinal fluid (CSF) analysis, neurological evaluations (reflexes, dermatomes, and myotomes), and additional tests based on specific clinical scenarios.

Q: What is the treatment for Transverse Myelitis?

Treatment for Transverse Myelitis may involve medical interventions such as intravenous glucocorticoids, plasma exchange for non-responsive cases, and immunomodulatory therapies like cyclophosphamide, mycophenolate, and rituximab. Rehabilitation and psychological support also play a crucial role in managing the condition.

Q: How can Transverse Myelitis be managed?

Managing Transverse Myelitis involves a multidisciplinary approach, including medical interventions, rehabilitation (physical and occupational therapies), psychological support, and long-term strategies for chronic cases. Personalized treatment plans aim to enhance functional recovery and quality of life.

Q: What are the long-term effects of Transverse Myelitis?

Long-term effects of Transverse Myelitis can vary and may include residual symptoms, physical disabilities, and psychological distress. Rehabilitation and comprehensive management strategies aim to mitigate these effects and promote optimal well-being.

Q: Can Transverse Myelitis be cured?

While Transverse Myelitis can have a significant impact, many patients experience significant recovery, especially with early diagnosis and appropriate treatment. Some individuals may have residual symptoms, but personalized management plans aim to optimize quality of life.

Q: What is the role of physical therapy in Transverse Myelitis?

Physical therapy plays a pivotal role in the management of Transverse Myelitis by promoting functional recovery, improving muscle strength, enhancing mobility, and aiding in adaptation to physical challenges. It is an essential component of comprehensive patient care.

Q: When should I seek medical attention for Transverse Myelitis symptoms?

If you experience sudden weakness, sensory alterations, pain, or autonomic dysfunction, it’s crucial to seek immediate medical attention. Early diagnosis and intervention are key to optimizing outcomes and managing the condition effectively.

Q: What are the risk factors for Transverse Myelitis?

Transverse Myelitis can occur in individuals of all ages, but certain risk factors may increase susceptibility. These include prior infections, autoimmune conditions, and associations with other neurological disorders like Multiple Sclerosis (MS) and Acute Disseminated Encephalomyelitis (ADEM).

Q: Is Transverse Myelitis hereditary?

Transverse Myelitis is not typically considered a hereditary condition. It is primarily associated with autoimmune and infectious triggers rather than genetic factors. However, individuals with a family history of autoimmune disorders may have a slightly elevated risk.

Q: Can children get Transverse Myelitis?

Yes, children can develop Transverse Myelitis, although it is relatively rare. Pediatric cases of Transverse Myelitis may present with similar symptoms as in adults, including weakness, sensory changes, and autonomic dysfunction. Prompt medical attention and proper management are crucial for pediatric patients.

Q: What are complications associated with Transverse Myelitis?

Transverse Myelitis can lead to various complications, such as physical disabilities, chronic pain, psychological distress, and challenges with daily activities. Comprehensive management strategies, including medical interventions and rehabilitation, aim to minimize these complications and enhance overall well-being.

Q: What research is being done on Transverse Myelitis?

Ongoing research focuses on better understanding the underlying mechanisms of Transverse Myelitis, exploring advanced treatment options, and identifying potential biomarkers for early diagnosis. Advances in immunology, neurology, and technology contribute to the evolving landscape of Transverse Myelitis research.

Leave a Comment