Neuro-ophthalmology

Blepharospasm

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Differentiate essential blepharospasm with secondary blepharospasm due to

  • Blepharitis
  • Trichiasis
  • Dry eyes
  • Chronic lid or ocular surface disease
  • Glaucoma
  • Uveitis

Causes of apraxia of eyelid opening

  • Parkinson’s
  • Progressive supranuclear palsy (secondary parkinsonism syndromes)
  • Huntington's disease

Benign essential blepharospasm

Clinical features

  • Bilateral involuntary lid closure (although may be asymmetrical)
  • Increased frequency of blinking
  • Remainder of face not involved
  • Relieved by relaxation/distraction
  • Not present during sleep

Management

  • Botox injection to lids: very effective in the short term
    • Botulinum toxin A prevents the release of acetylcholine from the presynaptic neuron into the neuromuscular junction
  • Management of primary causes (see above)

Meige syndrome

  • Blepharospasm with lower facial involvement
  • Spastic contractions of lower facial muscles (facial dystonia)
  • Unknown cause
  • Treatment: botox injection

Hemifacial spasm

  • Unilateral tonic-clonic blepharospasm and facial dystonia
  • Indicative of a compressive lesion irritating the VII nerve: typically a ectatic vertebral artery or anterior inferior cerebellar artery
    • Compression is at the nerve root’s exit at the level of the brainstem
  • Typically persists while asleep
  • Imaging required to exclude tumour (eg. pontine glioma and cerebellopontine angle)
  • Management: botox injections or surgery

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