Diplopia

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Approach

  1. Monocular or binocular?
    • Monocular: Double vision when 1 eye ist covered: almost always ocular, use pinhole
    • Binocular: Double vision disappears when either eye is covered
  2. If binocular: Constant or temporary double vision?
  3. If constant double vision: Paretic or restrictive?
  4. If paretic:
  5. Eye misalignments (Cover-/Uncover tests)?

Monocular Double Vision

  • Eye diseases
    • dry eye
    • High astigmatism
    • Iridotomy/iridodialysis
    • Cataract
    • Decentered intraocular lens
    • Incorrect glasses correction
    • Corneal diseases, e.g., keratoconus
    • Macular edema

Binocular Double Vision

  • Muscle and orbital diseases
    • Restrictive myopathies
      • Endocrine orbitopathy
      • Myositis (restrictive or paretic)
      • Mitochondrial myopathy, e.g. chronic progressive external ophthalmoplegia (CPEO).
    • Paretic myopathies
      • Myositis
      • Myotoxicity after retrobulbar or peribulbar anesthesia
    • Orbital diseases
      • Orbital cellulitis (bacterial, fungal, viral)
      • Vascular-related orbitopathies: carotid-cavernous fistula, giant cell arteritis, cavernous sinus thrombosis, orbital ischemic syndrome
      • Autoimmune-related inflammations, e.g., granulomatosis with polyangiitis, sarcoidosis, orbital pseudotumor
      • Trauma
      • Orbital tumors, including lymphoma
  • Neuromuscular junction disease
    • Myasthenia gravis, others: idiopathic, drug-induced (e.g. penicillamine, aminoglycosides, betablockers, chlorpromazine etc.)
  • Nerve Diseases
    • N. III palsy
      • Compressive: Aneurysm (in 1/3 of patients, often at the junction between the internal carotid artery and the posterior communicating artery), tumours (e.g., pituitary tumour, sphenoid wing meningioma), increased intracranial pressure with uncal herniation (non-reactive wide pupils in unconscious patients)
      • Ischemic: Atherosclerosis, diabetes mellitus, hypertension, giant cell arteritis
      • Inflammatory: Multiple sclerosis, viral, postviral
      • Trauma
    • N. IV palsy
      • Congenital (unilateral, frequent)
      • Traumatic (bilateral or unilateral)
      • Ischemic (unilateral!): Atherosclerosis, diabetes mellitus, hypertension, giant cell arteritis
      • Compressive: Tumours in the midbrain, pineal region, or cavernous sinus
      • Inflammatory (rare): Infectious or post-infectious neuritis
    • N. VI palsy
      • Ischemic (common, unilateral): Atherosclerosis, diabetes mellitus, giant cell arteritis
      • Compressive (common, unilateral or bilateral): Sphenoid wing meningioma, internal carotid artery aneurysm in the cavernous sinus, pituitary tumors, nasopharyngeal carcinomas, metastases with invasion of the cavernous sinus
      • Inflammatory (rare, unilateral): Sarcoidosis, viral, meningitis, mastoiditis, sphenoid sinusitis
      • Increased intracranial pressure of any etiology
      • Trauma (unilateral or bilateral)
    • Unilateral Multiple Nerve Palsies
      • Cavernous sinus syndrome: caused by pituitary apoplexy, cavernous sinus thrombosis, carotid-cavernous fistula, aneurysm of the internal carotid artery, infection, inflammation, tumour
      • Orbital apex syndrome: same causes and symptoms as cavernous sinus syndrome but additionally optic neuropathy
    • Bilateral Multiple Nerve Palsies
      • Bilateral cavernous sinus syndrome
      • Bilateral apex syndrome
      • Meningitis
      • Guillain-Barré syndrome/Miller Fisher syndrome (presumed post-infectious autoimmune demyelinating polyneuropathy, mainly affecting cranial nerves and peripheral motor nerves)
      • Wernicke encephalopathy (Thiamine = Vitamin B1 deficiency, leading to degeneration of the oculomotor cranial nerves and vestibular nuclei in the brainstem -> ataxia, confusion, ophthalmoplegia)
  • Brain Disease
    • Supranuclear eye movement disorders (result in gaze palsies characterized by the absence of diplopia and a normal vestibuloocular reflex)
      • Horizontal gaze palsy due to lesions in the pons
        • PPRF lesion (= pontine paramedian reticular formation: connection to the ipsilateral abducens nerve. Lesion of the PPRF: ipsilateral horizontal gaze palsy).
        • MLF lesion = Internuclear ophthalmoplegia (MLF = medial longitudinal fasciculus) -> reduced ipsilateral adduction when looking towards the opposite side and abducens nystagmus of the contralateral eye; adduction is better/normal with convergence!); possible skew deviation, vertical diplopia, direction- and upbeat-nystagmus. Exclude Myasthenia gravis!
          • Posterior lesion (pons): preserved convergence
          • Anterior lesion (midbrain): WEBINO = wall-eyed bilateral INO: impaired convergence
          • Brainstem (midbrain and pons) infarction, tumour, inflammation, infection, or Multiple Sclerosis
          • Wernicke encephalopathy
          • Pernicious anemia (Vitamin B12 deficiency)
        • Ipsilateral combined PPRF and MLF lesion (or ipsilateral abducens and MLF lesion) = one-and-a-half syndrome = Fischer syndrome (only abduction of the contralateral eye is possible, which also has an ataxic nystagmus; vertical eye movements intact).
          • Infarction (most common cause)
          • Multiple Sclerosis
          • Basilar artery occlusion
          • Pontine metastases
      • Vertical gaze palsy due to midbrain lesions (lesions of the rostral interstitial nucleus of the MLF, which is immediately dorsal to the red nucleus in the midbrain).
        • Progressive supranuclear palsy (PSP = progressive supranuclear palsy) = Steele-Richardson-Olszewski syndrome -> a severe degenerative disease = generalized brainstem degeneration with destruction of supranuclear connections to the oculomotor nerve nuclei. Findings: supranuclear gaze palsy, initially primarily affecting downward gaze (and the appearance of vertical saccades) -> “dirty tie” syndrome because patients soil themselves while eating due to not seeing their food (DDs for saccade disorders inferiorly: PSP, Whipple’s disease, Niemann-Pick); with the progression of the disease, upward gaze is also affected; horizontal movements are eventually impaired, and a general gaze palsy develops; extrapyramidal rigor, gait ataxia, and dementia; convergence paralysis.
        • Dorsal Parinaud midbrain syndrome (= pretectal syndrome)-> Parallel position in primary position, supranuclear paresis of upward gaze, disturbed convergence, large pupils with light-near dissociation, lid retraction (Collier’s sign), convergence-retraction nystagmus with attempted upward gaze, skew deviation.
          • Etiology in 90%: Pineal tumor; others: MS, infarction, trauma.
        • Skew deviation (vertical bilateral divergence, concomitant or incomitant, without cyclodeviation, due to an imbalance of prenuclear inputs, i.e., a brainstem lesion, whose cause is not nuclear or fascicular N. III or N.IV palsy).
          • Brainstem (midbrain, pons, or medulla) infarction, tumour, infection, inflammation, or MS.

Hess-Weiss of a Patient with Skew Deviation due to Posterior Thalamic Infarction

CAVE:

  • In supranuclear brain disorders (PSP, Parinaud syndrome), eye motility during the vestibuloocular reflex (VOR) is less restricted than during intentional eye movements
  • In ophthalmoplegias of other etiologies (e.g., CPEO = chronic progressive external ophthalmoplegia), the VOR is equally limited as intentional eye movements

Other Causes of Double Vision

  • Decompensated strabismus
  • Decompensated phorias
  • Convergence insufficiency (primary or secondary; double vision only up close)
  • Convergence spasm (primary or secondary; double vision worse in the distance than up close, miosis, increased accommodation)
  • Divergence insufficiency (double vision only in the distance)
    • Unilateral or bilateral abducens nerve palsy due to a tumour
    • Myasthenia gravis (bilateral medial rectus palsy)
  • Cerebral diplopia or polyopia: each eye sees the same two (or more) images, and it makes no difference whether the right, left eye is covered or binocular viewing occurs; the double vision persists even when looking through a pinhole. Cause: parieto-occipital tumours, infarction, or migraine.

Sources

  • EyeWiki Basic Approach to Diplopia
  • The Wills Eye Manual: Office and Emergency Room Diagnosis and Treatment of Eye Disease; Nika Bagheri MD, Brynn Wajda MD, et al; Lippincott Williams&Wilkins; 7. Auflage (2016)
  • Kanski’s Clinical Ophthalmology: A Systematic Approach; Jack J. Kanski MD, Brad Bowling MD; Saunders Ltd.; 8. Auflage (2015)
  • Clinical Pathways in Neuro-Ophthalmology An Evidence-Based Approach: Stacy Smith; Andrew G. Lee; Paul W. Brazis; Thieme; 3. Auflage (2018)
  • The Neuro-Ophthalmology Survival Guide: Anthony Pane; Neil R Miller; Michael Burdon; Elsevier; 2. Auflage (2017)