Syringomyelia and Transverse Myelitis

Question 1

What is Syringomyelia?

Answer 1

Formation of a collection of CSF (called a syrinx) within the spinal cord causing neurological symptoms.

Question 2

What causes syringomyelia?

Answer 2

Congenital – Arnold-Chiari malformation increases your risk of syringomyelia, This is when the cerebellum protrudes slightly from its normal location
Acquired – cysts occurring as a result of trauma, haemorrhage, tumour or arachnoiditis.

Question 3

How does syringomyelia present?

Answer 3

Sensation loss with absent pain and temperature sensation but preserved light touch, vibration and joint sensation due to pressure from the syrinx on the decussating spinothalamic pathway. This is seen in a cape like (neck and arms) root distribution reflecting location of the syrinx, typically over trunk and arms.

UMN arms signs > UMN leg signs presenting as spastic weakness
Paraesthesia
Neuropathic pain
Upgoing plantars and bowel and bladder dysfunction
Scoliosis occurs over a matter of years if not treated
Horner’s syndrome if compression of sympathetic chain bit this is rare
Body asymmetry

Question 4

How does syringobulbia present?

Answer 4

Syringobulbia – brainstem involvement causing nystagmus, tongue atrophy, dysphagia, pharyngeal/palatal weakness, and Vth nerve sensory loss

Question 5

How should suspected syringobulbia be investigated?

Answer 5

Full spine MRI with contrast to exclude tumour

Brain MRI to exclude Chiari malformation

Question 6

How is syringobulbia managed?

Answer 6

Treat the cause of the syrinx

If persistent or symptomatic then a shunt can be places into the syrinx

Question 7

What is transverse myelitis?

Answer 7

inflammation of the spinal cord. Transverse implies it extends across the entire width of the spinal cord. This inflammation results in progressive loss of myelin sheath for unknown reasons.

Question 8

What causes transverse myelitis?

Answer 8

Multiple Sclerosis

Infection (HIV, herpes simplex, herpes zoster, cytomegalovirus and Epstein-Barr)

Question 9

How does transverse myelitis present?

Answer 9

Weakness and numbness of the limbs
Both upper and lower motor neuron signs 
Loss of continence 
Autonomic nervous system dysfunction
Progresses over hours or days 
Respiratory failure risk if cervical spin involved

Question 10

How is suspected transverse myelitis investigated?

Answer 10

MRI an CT scan are useful
Lumbar puncture
Routine bloods

Question 11

How is transverse myelitis managed?

Answer 11

Corticosteroids to reduce inflammation