Differential diagnosis

  • Inflammatory
    • Multiple sclerosis
    • Giant cell arteritis
    • Mononeuritis multiplex
    • Miller-Fischer syndrome
  • Eye movement defects (CN III, IV or VI)
    • Orbital apex (III, IV, VI plus ipsilateral II and V1)
    • Cavernous sinus (III, IV, V1, V2, VI, sympathetic fibres to eye)
    • Base of skull (VI, IX)
    • Posterior communicating artery aneurysm (III)
    • Cerebellopontine angle (VII, VIII and VI if severe)
    • Midbrain (Weber’s syndrome – III and contralateral hemiparesis)
    • Pontine (Millard-Gubler syndrome – VI, ipsilateral VII and contralateral hemiparesis)
    • Pontine (inter-nuclear ophthalmoplegia or one-and-a-half syndrome)
    • Raised intracranial pressure (bilateral VI)
    • Leptomeningeal disease
  • Neuromuscular
    • Myasthenia gravis
    • Lambert-Eaton myasthenic syndrome
  • Orbital pathology
    • Thyroid eye disease
    • Orbital / peri-orbital cellulitis
    • Neoplastic infiltration
  • Myopathy
    • Chronic progressive external ophthalmoplegia (CPOE)
    • Kearns-Sayre syndrome (CPOE + retinitis pigmentosa + complete heart block, ± ataxia)
    • Myotonic dystrophy
  • Endocrine
    • Diabetes mellitus
    • Acromegaly
    • Graves’ disease
    • Pituitary tumour (lateral extension into cavernous sinus / hemi-field slide)
  • Progressive supranuclear palsy
  • Migraine with ophthalmoplegia
  • Trauma
  • Ocular
    • Retinal detachment
    • Diabetic retinopathy
    • Central retinal vein / artery occlusion
    • Vitreous haemorrhage
    • Cataract

Questions to ask

  • Does the double vision go away when you cover either eye?
    • Binocular diplopia: correction when either eye is covered
    • Monocular diplopia: correction when the affected eye is covered (=ocular pathology)
  • Is the double vision horizontal or vertical?
    • Horizontal diplopia: lateral / medial rectus (unilateral / bilateral VI or INO)
    • Vertical diplopia: superior / inferior rectus, superior / inferior oblique (III or IV)
  • Is the double vision worse on looking in any particular direction?
    • Diplopia is maximal in the direction of action of the paretic muscle
  • Myasthenia gravis
    • Does the severity of the double vision fluctuate?
      • Myasthenia gravis gets worse as the day goes on, and with activity, e.g. reading
    • Can be improved by resting the eye
    • Do you cough when you swallow?
    • Do you fall asleep easily in the day / have you ever fallen asleep when driving?
  • Has anyone told you that your eyes appear different or bulging?
    • Thyroid eye disease
    • Orbital tumour
    • Carotid-cavernous fistula
  • Do you have a headache?
    • Giant cell arteritis: jaw claudication, scalp tenderness
    • Migraine: hemicranial, throbbing, may have aura, nausea, hemiplegia
    • Meningitis: photophobia, neck stiffness
  • Demyelination / optic neuritis
    • Do colours appear as vibrant as usual? (optic neuritis)
    • Is there any eye pain or redness?
    • Have you noticed any weakness or numbness?
  • Symptoms of thyroid disease
    • Do you ever feel your heart is beating very quickly?
    • Have you noticed the weather being unusually hot lately?
    • Have you noticed your hands trembling?
    • Have your periods been irregular or more heavy than usual?
    • Have you been having any diarrhoea?
  • Osmotic symptoms
    • Do you wake up in the middle of the night to pass urine?
    • Do you feel you are drinking more water than usual?
    • Have you been losing weight?

Things to examine for

  • Inspection
    • Ptosis
    • Lid retraction
    • Proptosis / exophthalmos
    • Strabismus
    • Peri-orbital swelling
    • Goitre / thyroid surgery scars
    • Parkinsonism
  • Pulse – rate, regular / irregular (hyperthyroidism)
  • Eyes
    • Acuity
      • Finger-counting
      • Red desaturation
    • Pupils
      • Equal (Horner’s may produce unequal pupils)
      • Consensual reflex
      • Relative afferent pupillary defect
    • Extra-ocular movements (in which direction is the diplopia maximal)
      • Convergence if INO detected – should be normal
    • Fatigability
  • Pronator drift (brainstem syndromes)
  • Upper limb fatigability
  • Thyroid status
    • Pulse
    • Tremor
    • Goitre – smooth / diffuse / nodular
    • Displaced apex beat
    • Murmurs
    • Lung bases for pulmonary oedema
    • Legs for pre-tibial myxedema / peripheral oedema

Investigations

  • Blood tests
    • Thyroid function tests
    • Thyroid receptor antibody if Graves’ disease
    • Fasting blood glucose
    • Erythrocyte sedimentation rate (giant cell arteritis)
  • Myasthenia gravis
    • Antibodies against nicotinic acetylcholine receptors
      • If negative and high index of suspicion: muscle-specific kinase antibodies
    • Tensilon test
      • Improvement in weakness with edrophonium
    • Repetitive nerve stimulation
      • Measures neuronal response to repeated stimulation. Indicative of myasthenia if decrement in response observed.
  • Imaging (if cranial nerve palsy present)
    • Computed tomography / magnetic resonance imaging of the brain

Patient concerns

  • Will it get better?
    • Should get better with treatment of underlying cause
    • Can refer to ophthalmology colleague in the meantime to help with aids to minimize diplopia
  • Driving: should not drive until diplopia is corrected
  • Occupation
  • Inheritance

Management

  • Multidisciplinary
    • Visual aids: e.g. patches to cover either eye, prisms
    • Referral to therapists for adaptation to monocular vision while treating underlying cause
    • Referral to neuro-ophthalmologist
    • Referral to specialist in underlying condition
  • Treat underlying cause