Autoimmune Neurology Flashcards
What is the pathophysiology of Multiple Sclerosis
Altered immune response in CNS leads to demyelination of CNS nerves
What are the 4 patterns of Multiple Sclerosis
- Relapsing Remitting
- Secondary Progressive
- Relapsing Progressive
- Primary Progressive
Describe Relapsing Remitting MS
- Episode of monophasic neurological disturbance
- Return to normal function between attacks
Describe Secondary Progressive MS
- Starts with Relapsing Remitting picture
- Later on has background progression
- I.e relapses without full return to baseline
Describe Relapsing Progressive MS
- Relapses on top of background progression
- No remission between relapses
Describe Primary Progressive MS
- Relentless progression from onset
- Worst prognosis
- Associated with: Male, late onset, paucity of findings on imaging
What are some possible clinical features of MS
- Optic neuritis
- Cerebellar ataxia
- Spastic paraparesis
- Transverse myelitis
- Internuclear Ophthalmoplegia
- Patchy sensory disturbance
Describe Optic Neuritis
- Blurry vision/profound vision loss
- Colour vision sometimes affected
- Can lead to optic atrophy
How do you diagnose Multiple sclerosis
MRI scan
- needs evidence of distribution in time + space
- 2 lesions, in 2 different places, from 2 episodes
Acute management of MS
High dose steroids
- reduces severity + duration of relapses
- doesn’t affect progression of disease
- become less effective each time
Prevention of relapses in MS
Biologic agents
- don’t affect overall progression
General management of MS
- MDT approach
- Psychological support
- Advice about work/life adaptations
What is myasthenia gravis
Autoimmune synaptopathy affecting the neuromuscular junction
What are the causes of Myasthenia gravis
- Anti-acetylcholine receptor antibodies (majority)
- Anti-MUSK antibodies (minority)
Myasthenia Gravis - Clinical features
Symmetrical proximal muscle weakness
Extra-occular muscle involvement
- Diplopia + Ptosis
Bulbar involvement
- esp. Anti-MUSK
- Dysarthria, dysphagia
- risk of aspiration
Associated with Thymoma development
Myasthenic crisis
What is a Myasthenic crisis
- Rapid evolution of neuromuscular weakness
- Requires emergency ventilation
Myasthenia Gravis - Investigations
- Autoantibody screen
- Single fibre EMG: high sensitivity
- CT thorax: rule out thymoma
Myasthenia Gravis - Management
Acetylcholinesterase inhibitors
- e.g. pyridostigmine
Immunomodulators
- steroids, azathioprine, plasma exchange
Thymectomy
What is Guillain-Barre Syndrome
Autoimmune-mediated inflammatory peripheral neuropathy
Guillain-Barre Syndrome - Clinical Features
- Typically 1-3wks after infection
- Ascending Motor weakness
- Symmetrical face + neck weakness
- Respiratory failure (requires ventilation)
What infections does Guillain-Barre typically follow
Cytomegalovirus or Campylobacter
Guillain-Barre Syndrome - Management
- Spontaneously resolves
- Supportive
- Gamma globulin/plasma exchange helps
