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Neuro-ophthalmology

Last Updated: September 27, 2018

Table 1: Diplopia
​Differential list for diplopia
Neoplastic
  • Intraorbital meningioma
  • Pituitary adenoma (GH secreting, or apoplexy)
  • Metastasis
  • Carcinomatosis
Vascular
  • Giant cell arteritis
  • Giant intracranial aneurysm
  • Microvascular disease
Infectious
  • Meningitis
  • Sphenoid sinusitis (CN VI palsy)
  • Botulism
Metabolic
  • Wernicke’s encephalopathy
Inflammatory
  • Multiple sclerosis
  • Myasthenia gravis
  • GBS
  • Miller-Fisher syndrome
  • Orbital pseudotumor
  • Orbital myositis
Miscellaneous
  • Traumatic neuropathy (orbital apex fracture, blowout fracture of orbital wall)
  • Increased intracranial pressure (CN III and VI palsy)
  • INO (lesion of MLF)
  • Graves’ disease (thyroid orbitopathy)
  • Corneal disease
  • Cataracts
  • Idiopathic intracranial hypertension (CN VI palsy)
Table 2: Ptosis
Differential list for true ptosis
Neoplastic
  • Infantile rhabdomyosarcoma
  • Hemangioma
  • Optic glioma
  • Dermoid cyst
  • Metastatic neuroblastoma
Neurologic
  • Ophthalmoplegia
  • Unilateral ptosis
  • Mydriasis
  • Cranial nerve nuclear lesions
Endocrine
  • Thyroid orbitopathy
Congenital
  • Congenital Horner’s syndrome
  • Marcus Gunn phenomenon
  • Jaw Winking syndrome
  • Blepharophemosis syndrome
  • Neonatal myasthenia
Inflammatory
  • Myasthenia gravis
  • Orbital pseudotumor
  • Tolosa-Hunt syndrome
  • Polymyositis
Miscellaneous
  • Trauma (post-surgical)
  • Third Cranial Nerve Palsy
  • Chronic topical steroid drops
Table 3: Monocular Blindness
Differential list for acute or chronic monocular blindness
Neoplastic
  • Intraorbital neoplasm
  • Tuberculum sella meningioma
Vascular
  • Amaurosis fugax (carotid bifurcation, great vessel, or cardiogenic thromboembolism)
  • Carotid cavernous aneurysm rupture
  • AION
  • Central or branch retinal artery occlusion
  • Central retinal vein occlusion
  • Takayasu’s arteritis
  • Temporal arteritis
  • Hypoperfusion (hypovolemia, hypotension, hyperviscosity)
Inflammatory
  • Optic neuritis
  • Retrobulbar neuritis
  • Multiple sclerosis
Miscellaneous
  • Trauma
  • Ocular migraine
  • Retinal detachment
  • Papilledema
  • Vitreous hemorrhage
Table 4: Binocular Blindness
Differential list for binocular blindness. The distinction of cortical blindness from a pathway lesion is the preservation of the pupillary light reflex in the setting of cortical blindness.
Neoplastic
  • Multifocal malignant glioma
  • Occipital lobe metastases
Vascular
  • Anoxia (cortical infarction, hemorrhage)
  • AION
Infectious
  • Multifocal abscess
Metabolic
  • Hypovitaminosis (B1, folic acid, B12)
Toxic
  • Methanol (acute loss)
  • Ethanol (progressive)
Inflammatory
  • Optic neuritis
Miscellaneous
  • Traumatic neuropathy
Table 5: Transient Blindness
Differential list for causes of transient blindness. Cerebrovascular occlusion often involves either a branch or the central retinal artery.
Vascular
  • Cerebrovascular embolism (carotid bifurcation, cardiogenic, calcific, atrial myxoma, air, adipose, amniotic fluid)
  • Hypoperfusion
  • Hemorrhage
  • Central retinal vein occlusion
  • AION
  • Takayasu’s arteritis
Inflammatory
  • Multiple sclerosis
  • Optic neuritis
  • Papilledema
Miscellaneous
  • Typical migraine
  • Psychogenic
Table 6: Argyll Robertson Pupil
Differential list for the Argyll Robertson Pupil, which can be defined as bilateral miotic pupils that constrict to accommodation but are non-reactive to light.
Neoplastic
  • Pinealoma
Infectious
  • Meningovascular syphilis
  • Brainstem encephalitis
Endocrine
  • Diabetes Mellitus
Toxic
  • Alcoholism
Table 7: Marcus Gunn Pupil (Afferent defect)
Differential list for a Marcus Gunn pupil, which can be defined as a pupil which demonstrates greater pupillary sphincter contraction consensually than by direct exposure. The etiology of this finding is a prechiasmal disruption of the optic pathway.
Neoplastic
  • Olfactory groove meningioma
  • Optic glioma
Vascular
  • Hemorrhage (vitreous)
Inflammatory
  • Multiple sclerosis
  • Optic neuritis
Miscellaneous
  • Retinal detachment
  • Macular degeneration
Table 8: Adie Tonic Pupil
Differential list for Adie tonic pupil, which can be defined as a pupil that is tonically dilated and slowly reactive to light exposure. Tonic accommodation is commonly observed. Etiology of this condition relates to neurodegeneration within the ciliary ganglion.
Neoplastic
  • Midbrain tegmental tumors
Vascular
  • Ischemia
  • Amyloidosis
Infectious
  • Meningovascular syphilis
  • Herpes zoster
  • Measles
  • Influenza
  • Cellulitis
  • Other viral agents
Toxic
  • Alcohol
Congenital
  • Familial dysautonomia
  • CMT disease
Inflammatory
  • Sarcoidosis
  • Rheumatoid arthritis
  • GBS
  • Miller-Fisher syndrome
  • Sjogren syndrome
  • Shy-Drager syndrome
  • Orbital vasculitis
Miscellaneous
  • Trauma
  • Ocular migraine
  • Post-orbital surgery
  • Paraneoplastic syndrome
  • Ross syndrome
  • Oculomotor nerve palsy
Table 9: Hippus
Differential list for Hippus, which can be defined as alternating miosis and mydriasis of the pupil under a consistent light source.
Vascular
  • Infarction (contralateral)
Infectious
  • Meningitis
Inflammatory
  • Multiple sclerosis
Miscellaneous
  • Idiopathic
  • Cataracts (during initial stages)
  • Oculomotor palsy recovery
  • Hysteria
Table 10: Miosis
Differential list for miosis. Those causes that induce a unilateral miosis are specifically noted.
Vascular
  • Hemorrhage (pontine)
Infectious
  • Neurosyphilis (rarely unilateral)
Toxic & Pharmacologic
  • Cannabinoids
  • MAOI
  • Guanethidine
  • Opioids
  • Phentolamine
  • Cholinergics
  • Cholinesterase inhibitors
  • Barbiturates
  • Reserpine
Miscellaneous
  • Horner’s syndrome (unilateral)
  • Argyll Robertson pupil
  • Advanced age
Table 11: Mydriasis
Differential list for mydriasis. Those causes that induce a unilateral mydriasis are noted.
Neoplastic
  • Supratentorial mass induced rostrocaudal deterioration
  • Tectal mass inducing Parinaud’s syndrome
Vascular
  • Aneurysm (PCA, P-comm, SCA)
Infectious
  • Acute ciliary ganglionitis
Endocrine
  • Thyrotoxicosis
Toxic & Pharmacologic
  • Epinephrine
  • Phenylephrine
  • Cocaine
  • Amphetamines
  • LSD
  • TCA
  • Anticholinergics
  • Cyclopentolate
  • Antihistamines
  • Thiopental
Miscellaneous
  • Cluster headaches (unilateral)
  • Uncal herniation (unilateral)
  • Adie pupil syndrome (unilateral)
  • Pseudodilation (from a contralateral constriction syndrome)
  • Epilepsy (post-ictal)
  • Hyperemotional state
Table 12: Internuclear Ophthalmoplegia
Differential list for internuclear ophthalmoplegia, which is a syndrome resulting from a lesion of the medial longitudinal fasciculus between the 3rd and 6th cranial nerves nuclei. This disorder manifests as diplopia on lateral gaze and disconjugate eye movements.
Neoplastic
  • Intra-axial brainstem tumors
  • Extra-axial brainstem tumors
  • 4th ventricular tumors
  • Metastasis
Vascular
  • Brainstem infarction
Infectious
  • Brainstem encephalitis
  • Tertiary syphilis
Metabolic
  • Hepatic encephalopathy
  • Maple syrup urine disease
Toxic
  • TCA
  • Barbiturates
  • Phenothiazines
  • Phenytoin
  • Wernicke’s encephalopathy (pINO)
Congenital
  • Chiari II (syringobulbia associated)
  • Chiari III (syringobulbia associated)
Inflammatory
  • Multiple sclerosis
  • SLE
  • Myasthenia gravis (pINO)
  • GBS (pINO)
  • Miller-Fisher syndrome (pINO)
Miscellaneous
  • Trauma
  • Exotropia (pINO)
  • Progressive supranuclear palsy
Table 13: Vertical Gaze Palsy
Differential list for vertical gaze palsy
Neoplastic

Pineal tumors
3rd ventricular tumors
Intra-axial midbrain tumors

Infarction (dorsal midbrain)
Hemorrhage (dorsal midbrain)

Vascular

Infarction (dorsal midbrain)

Hemorrhage (dorsal midbrain)

Endocrine

Thyroid-associated orbitopathy (mimic)

Metabolic

Hypovitaminosis B1 or B12
Niemann-Pick disease, type C

Gaucher’s disease

Congenital

Congenital oculomotor apraxia

Congenital upward gaze limitation (mimic)

Inflammatory

Multiple sclerosis
GBS (mimic)

Miller-Fisher syndrome

Miscellaneous

Trauma

Hydrocephalus

Table 14: Acute Ophthalmoplegia
Differential list for acute ophthalmoplegia. Oculomotor nerve palsy is most commonly observed with the presence of an aneurysm at the interface between the P-Comm and ICA. Abducens nerve palsy is more commonly observed with aneurysms of AICA or the basilar artery.
Neoplastic
  • Pituitary adenoma
  • Brainstem glioma
  • Craniopharyngioma
  • Pineal tumors
  • Lymphoma
  • Nasopharyngeal carcinoma
  • Optic glioma
  • Orbital dermoid cyst
  • Orbital rhabdomyosarcoma
  • Cavernous hemangioma of the orbit
  • Metastatic neuroblastoma
Vascular
  • Aneurysm (AICA, basilar, P-Comm, ICA terminus)
  • Hemorrhage (brainstem)
  • Inferior petrosal sinus thrombosis
  • Cavernous sinus thrombosis
  • Carotid-cavernous sinus fistula
Infectious
  • Encephalitis
  • Botulism
  • Diphtheria
Endocrine
  • Thyrotoxicosis
Toxic
  • TCA
  • Antiepileptic drugs
Inflammatory
  • Myasthenia gravis
  • Orbital pseudotumor
  • Pseudotumor cerebri
  • Tolosa-Hunt syndrome
  • Sarcoidosis
Miscellaneous
  • Trauma
  • Idiopathic cranial nerve palsy
  • Ophthalmoplegic migraine
Table 15: Papilledema
Differential for papilledema
Neoplastic
  • Melanocytoma
  • Hemangioma
  • Hemangioblastoma
  • Metastatic (lung, breast, leukemia)
  • Meningeal carcinomatosis
  • Tumors that compress the intraorbital optic nerve
Vascular
  • Hematoma (epidural, subdural, parenchymal)
  • AION
Infectious
  • Cerebral abscess
  • Meningoencephalitis
  • Lyme disease
  • Neurosyphilis
Congenital
  • Congenital disk elevation (pseudopapilledema)
Endocrine
  • Juvenile diabetes (unilateral)
Inflammatory
  • Sarcoidosis
  • Papillitis (unilateral)
  • Arteritis (SLE)
Miscellaneous
  • Post-traumatic cerebral edema
  • Foster-Kennedy syndrome (unilateral)
  • Acute obstructive hydrocephalus
Table 16: Optic Nerve Enlargement
Differential for enlargement of the optic nerve
Neoplastic
  • Optic nerve sheath meningioma
  • Intracranial meningioma (extension to optic nerve)
  • Optic nerve glioma
  • Plexiform neuroma
  • Metastasis (breast, prostate, lung, Ewing’s sarcoma, leukemia, neuroblastoma)
Infectious
  • Lyme disease
  • Syphilis
  • Tuberculosis
  • Whipple’s disease
  • Toxoplasmosis
  • Histoplasmosis
  • Cryptococcus
  • Viral encephalitis
  • Poliomyelitis
  • Herpes zoster
  • Measles
  • Mumps
  • Rubella
  • Mononucleosis
Metabolic
  • Adrenoleukodystrophy
Inflammatory
  • Multiple sclerosis
  • Optic neuritis
  • Neuromyelitis optica
  • Sarcoidosis
  • Idiopathic inflammatory pseudotumor
  • Inflammatory bowel disease (CD, UC)
  • Reiter’s syndrome
Table 17: Horner’s Syndrome
Differential list for Horner’s syndrome. The specific neuron involved in the pathway (first, second, or third order) is listed along with each etiology as 1, 2, or 3, respectively. The first-order neuron connects the hypothalamus with the intermediolateral cell column in the upper thoracic cord. The second-order neuron connects the intermediolateral cell column to the superior cervical ganglion. The third-order neuron completes the pathway from the superior cervical ganglion to the orbit.
Neoplastic
  • Pontine glioma (1)
  • Upper cord glioma (1)
  • Upper cord ependymoma (1)
  • Upper cord neurofibroma (2)
  • Upper cord schwannoma (2)
  • Pancoast tumors of the lung (2)
  • Cervical paravertebral mass (2)
  • Axillary metastasis (2)
Vascular
  • Infarction (lateral medullary, dorsolateral pontine, hemisphere) (1)
  • Cavernous sinus lesion (3)
  • Carotid artery dissection (3)
  • Carotid artery trauma (3)
Infectious
  • Encephalitis
  • Bulbar poliomyelitis (1)
Congenital
  • Cervical rib syndrome
Inflammatory
  • Multiple sclerosis (1)
  • ALS (1)
Miscellaneous
  • Cervical cord trauma (1)
  • Lower brachial plexus trauma (2)
  • Cervical disk herniation (2)
  • Cluster headache (3)
  • Syringobulbia (1)
  • Superior orbital fissure lesions (3)
  • Iatrogenic surgical injury during ACDF, pharyngeal, laryngeal, or thyroid surgery (2)

DOI: https://doi.org/10.18791/nsatlas.v2.02.1.2

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