Chronic progressive autoimmune demyelinating CNS condition

  • Only attacks the oligodendrocytes in the CNS
  • Symptoms per episode progress over >24 hours and last days to weeks.
  • Lesions vary in location so symptoms change over time - disseminated in time and space

Causes/Factors

  • Age - 40-50 y/o
  • Genetic susceptibility - HLA-DRB1 + many more
  • EBV infection
  • Smoking
  • Obesity

Symptoms

Eye issues

Optic neuritis

  • Central sctoma
  • Pain with eye movement
  • Impaired colour vision
  • RAPD

Eye movement abnormalities

Lesions can affect any cranial nerves, so Cranial Nerve Exam is important.

Focal Neurology

Breaks down into focal weakness and sensory symptoms:

  • Incontinence

  • Horner’s Syndrome

  • Facial nerve palsy

  • Limb paralysis

  • Trigeminal neuralgia

  • Numbness

  • Paraesthesia

  • Lhermitte’s sign - electric shock sensation down the spine and into the limbs when flexing the neck

  • Ataxia (cerebellar lesion)

Investigations

Based on the clinical picture and symptoms suggesting lesions are changing over time. Investigations can support the diagnosis:

  • MRI - typical lesions & location based on symptoms
  • LP - oligoclonal bands in CSF

Management

MDT - neurologists, specialist nurse, physios & OTs

Disease-modifying therapies - big long fancy names of immunotherapy drugs

Relapses may be treated with steroids

  • 500mg orally for 5 days
  • 1g IV daily for 3-5 days where oral treatments fail or relapses are severe

Complications/red Flags