Transverse Myelitis

Question 1

What is Transverse Myelitis?

Answer 1

Transverse Myelitis is an inflammatory condition of the spinal cord characterised by rapidly developing motor and sensory deficits. It can occur at any level of the spinal cord.

Reference: Nelson’s Paediatrics [20th Edition], p. 2958.

Question 2

How does Transverse Myelitis present?

Think about age groups

Answer 2

Transverse Myelitis presents in two different ways depending on age:

<3 years

• Development of symptoms in hours to days
• Preceding infection (usually viral) or immunisation
• Severe loss of function
• Slow recovery and likely incomplete
• Perivascular infiltration with mononuclear cells
• Spinal cord necrosis rarely

Older Children

• Peak symptoms in 2 days to 2 weeks
• More rapid recovery and likely complete
• Acute demyelination

Question 3

What are the symptoms in Transverse Myelitis?

Answer 3

• Discomfort or pain in neck or back related to level of lesion (most often thoracic)
• Numbness
• Anaesthesia
• Ataxia
• Areflexia
• Motor weakness in truncal musculature
• Flaccidity developing into paralysis and later spasticity
• Hyperreflexia
• Clonus
• Urinary retention and, later, incontinence
• Visual loss (in Neuromyelitis Optica)

Question 4

What are the differential diagnoses for Transverse Myelitis?

Answer 4

Demyelinating disorders

Meningitis

Spinal Cord Infarction

Mass lesions

Abscess

Spinal Cord Tumour

Question 5

How is Transverse Myelitis investigated?

Answer 5

MRI

With and without contrast enhancement. Demonstrates high signal intensity over multiple segments (infantile form), 1-2 segments (adolescent form), or >3 segments with optic neuritis (Neuromyelitis Optica).

Lumbar Puncture

Evidence of CSF pleocytosis (increased WCC) >10.

Increased IgG

High Protein

Bloods

Aquaporin-4 Antibodies (AQP4 IgG)

Diagnostic for Neuromyelitis Optica (NMO; Devic’s Disease)

• Anti-myelin oligodendrocyte*
• Vitamin B₁₂*

Subacute Degeneration of the Cord

• Paraneoplastic Antibodies*
• HIV*

Reference: Nelson’s Paediatrics [20th Edition], p. 2959; NINDS

Question 6

How does Transverse Myelitis differ from Guillain-Barré Syndrome?

Answer 6

Guillain-Barré Syndrome involves spinal nerve root inflammation.

Question 7

What is the function of Aquaporin-4?

Answer 7

Aquaporin-4 is a water channel protein found on astrocytes that surround the blood-brain barrier.

Reference: NMOUK

Question 8

Useful Links

Answer 8

NINDS

Includes helpful patient information

Question 9

How is Transverse Myelitis treated?

Answer 9

Transverse Myelitis is managed with immune response modulation, the mainstay treatment being:

High dose steroids (Methylprednisolone)

Other treatment options include:

Intravenous Immunoglobulin (IVIg)

Plasma Exchange

Rituximab

Cyclophosphamide