E. Systemic, Neurologic, and Ophthalmic Disorders Commonly Associated with Neuro-Ophthalmic Manisfestations

I. Developmental and congenital anomalies with neuro-ophthalmologic consequences


1.Know how to recognize and evaluate congenital neuro-ophthalmologic syndromes.
2.Recognize and manage neuro-ophthalmic complications of in-utero or birth injuries
3.Recognize neuro-ophthalmic complications of child abuse


1. Visual maturation(1)
2. Complications of prematurity(3)
3. Cerebral palsy(3)
4. Complications of birth injuries(2)
5. Congenital hydrocephalus(1)
6. Cranial dysostoses (craniosynostosis)(3)
7. Amblyopia(1)
8. Congenital optic nerve anomalies(1)

  1. Bergmeister papilla
  2. Optic nerve dysplasia and aplasia
    1. Septo-optic dysplasia 
  3. Optic nerve coloboma
  4. Optic nerve pit
  5. Morning glory syndrome
  6. Optic nerve drusen
  7. Tilted disc
  8. Myelinated nerve fibers
  9. Staphyloma

9. Common malformations of the eye and orbit(3)
10. Skull base malformations. Chiari malformation(1) \
11. Fibrous dysplasia(3)
12. Ocular manifestations of child abuse(1)

II. Systemic disorders commonly associated with neuro-ophthalmologic manisfestations


1. Know how to recognize and diagnose various systemic disorders commonly associated with neuro-ophthalmic symptoms and signs.
2. Recognize the neuro-ophthalmic complications of systemic disorders.


1. Specific hereditary ocular and neurologic diseases with neuro-ophthalmic presentation(3)
2. Neurocutaneous syndromes

  1. Neurofibromatosis(2) 
    1. NF-1
    2. NF-2
  2. Tuberous sclerosis(2)
  3. von Hippel-Lindau(2)
  4. Sturge-Weber(2)
  5. Ataxia telangiectasia(3)
  6. Wyburn-Mason(3)

3. Vascular disease

  1. Vascular risk factors(2)
  2. Prevention of vascular disease(3)
  3. Neuro-ophthalmic manifestations of vascular diseases(1)
  4. Hypercoagulable states(3)

4. Systemic hypertension(2)
5. Metabolic diseases including diabetes mellitus(2)
6. Autoimmune diseases, classification of vasculitides(3)

  1. Specific vasculitides with ocular and neurologic manifestations 
    1. Giant cell arteritis(1) 
    2. Sarcoidosis(2) 
    3. Wegener granulomatosis(2)

7. Complications of cancers, paraneoplastic syndromes; principles of treatment(2)
    Neuro-ophthalmic complications of chemotherapy, and radiation therapies(1)
8. Complications of infections(2)
    Specific infections with common neuro-ophthalmic complications (AIDS for example)

III. Neurologic disorders commonly associated with neuro-ophthalmologic manifestations


1. Recognize and evaluate neuro-ophthalmologic complications of head injury (acute and chronic).
2. Know the neuro-ophthalmic complications of raised intracranial pressure.
3. Diagnose and manage increased intracranial pressure.
4. Understand the pathophysiology and management of acute and chronic intracranial hypertension.
5. Recognize and evaluate neuro-ophthalmologic complications of cerebrovascular disease.
6. Know the classification of stroke and the basics of the vascular evaluation.
7. Know the general principles of stroke management.
8. Know how to recognize and manage ocular vascular diseases
9. Recognize and evaluate neuro-ophthalmologic manifestations and complications of seizures.
10. Diagnose and evaluate patients with headache and facial pain.
11. Know the basis of management of primary benign headaches and facial pain such as migraine, cluster headache and trigeminal neuralgia.
12. Recognize and diagnose various neurologic disorders commonly associated with neuro-ophthalmic symptoms and signs.
13. Recognize the neuro-ophthalmic complications of neurologic disorders.


1. Head and ocular injury

  1. Recognize and evaluate neurological complications of head injury(3)
  2. Neuro-ophthalmic complications of brain injury (acute and late) (1)
  3. Traumatic optic neuropathies (direct and indirect) (1)
  4. Diagnose and evaluate orbital and facial fractures(2)
  5. Recognized the complications of ocular trauma(3)
  6. Evaluate post-traumatic visual loss(1)
  7. Diagnosis of post-concussion syndrome(1)

2. Increased intracranial pressure

  1. Differential diagnosis and management of intracranial hypertension(1) 
    1. Intracranial mass, infection or bleed 
    2. Meningitis 
    3. Subarachnoid hemorrhage 
    4. Hydrocephalus 
    5. Cerebral venous thrombosis 
    6. Idiopathic intracranial hypertension.
  2. Neuro-ophthalmic manifestations and complications(1)

3. Vascular disease of the brain and the eye

  1. Classification of stroke(3)
  2. Mechanisms(3) 
    1. Vein vs artery 
    2. Hemorrhage vs ischemia 
    3. Embolism vs thrombosis vs hemodynamic
    4. Large artery vs small artery
  3. Diagnosis and evaluation of stroke(2) 
    1. Vascular evaluation 
    2. Cardiac evaluation 
    3. Hypercoagulable state 
  4. Basics of acute treatment and secondary prevention of stroke(3)
  5. Neuro-ophthalmologic manifestations of stroke(1)
  6. Ocular ischemia(1) 
    1. Transient visual loss 
    2. Central and branch retinal artery occlusions 
    3. Ocular ischemic syndrome 
    4. Central and branch vein occlusions
  7. Venous sinus thrombosis(1)
  8. Subarachnoid hemorrhage, neuro-ophthalmic manifestations(1)
  9. Intracranial vascular malformations, diagnosis and neuro-ophthalmologic manifestations(1) 
    1. Aneurysms 
    2. Arteriovenous malformations 
    3. Dural fistulas 
    4. Carotid cavernous fistulas (direct, indirect) 
    5. Cavernous hemangiomas

4. Seizures with neuro-ophthalmologic manifestations

  1. Occipital seizures(1)
  2. Pupillary changes, ocular movement changes during seizures(1)

5. Neoplasms:

  1. Intracranial tumors(2)
  2. Skull base tumors(2)
  3. Orbital tumors(1)
  4. Optic nerve tumors(1)
  5. Benign vs malignant neoplasm
  6. Primary vs secondary
  7. Pediatric vs adult tumors​

6. Demyelinating disease

  1. Multiple sclerosis(1) 
    1. Relationships between optic neuritis and multiple sclerosis(1)
  2. Devic's disease(1)
  3. Acute disseminated encephalomyelitis(2)

7. Infections (neuro-ophthalmic manifestations of localized and systemic infections)

  1. Intracranial infections(2) 
    1. Abscess 
    2. Cerebritis
    3.  Empyema 
    4. Meningitis 
    5. Meningo-encephalitis
  2. Whipple's disease(2)
  3. Orbital infections (cellulitis)(1)
  4. Optic nerve infection (infectious optic neuritis and neuroretinitis)(1)
  5. Primary vs secondary neoplasm 

8. Metabolic diseases(2)

  1. Wilson's disease
  2. Vitamin deficiencies (vitamin A, B1, B12, …)
  3. Metabolic storage diseases
  4. Amyloidosis

9. Neuro-degenerative diseases(3)

  1. Parkinson syndromes 
    1. Parkinson's disease 
    2. Progressive supranuclear palsy
  2. Alzheimer, Frontotemporal dementia
  3. Vascular dementias
  4. Creutzfeld-Jacob disease
  5. Amyotrophic lateral sclerosis

10. Hereditary ataxias(3)
11. Polyradiculopathies(2)

  1. Guillain-Barré(2)
  2. Miller Fisher variant(2)

12. Neuro-muscular transmission deficits(1)

  1. Physiology of the neuromuscular transmission(1)
  2. Myasthenia gravis(1)
  3. Lambert-Eaton myasthenic syndrome(3)
  4. Toxic neuromuscular transmission defect (Botulism, medications)(1)

13. Myopathies (involving the extraocular muscles)

  1. Congenital myopathies/ dystrophies/ Ion Channel Disorders (Myotonia)(3)
  2. Mitochondrial diseases(2) 
    1. Chronic progressive external ophthalmoplegia (CPEO)
  3. Ischemic(1) 
    1. Giant cell arteritis
    2. Orbital ischemic syndrome
  4. Metabolic - Toxic(3) 
    1. Bassen Kornzweig syndrome (abetalipoproteinemia) 
    2. Refsum's 
    3. Drug induced, toxic
  5. Inflammatory(1) 
    1. Thyroid orbitopathy 
    2. Orbital inflammatory disease
  6. Neoplasm/infiltration(3)
  7. Congenital syndromes involving the extraocular muscles 
    1. Anomalous muscle insertions(1) 
    2. Brown's syndrome 
    3. Congenital fibrosis of the extraocular muscles (CFEOM) 
    4. Duane's syndrome 
    5. High myopia 

14. Headache and facial pain

  1. Classification of headaches and facial pain proposed by the International Headache Society (IHS)(3)
  2. Migraine(1) 
    1. Migraine without aura(2) 
    2. Migraine with visual aura(1)
  3. Tension headaches(3) 
    1. Episodic 
    2. Chronic
  4. Cluster headache(1)
  5. Headache associated with increased intracranial pressure(1)
  6. Headache and facial pain of vascular origin(2)
  7. Ocular pain related to ocular of optic nerve disease(1)
  8. Trigeminal neuralgia(2)
  9. Herpes zoster (zoster ophthalmicus) (1)
  10. Projected ocular pain(1) 
    1. Vascular (i.e. dissection) 
    2. Ocular ischemic syndrome
    3. Cavernous sinus syndrome

IV. Ocular diseases commonly associated with- or mimicking neuro-ophthalmologic disorders.


1. Know how to recognize and diagnose various ocular disorders commonly associated with neuro-ophthalmic symptoms and signs.
2. Recognize the neuro-ophthalmic complications of ocular disorders.
3. Recognize the ocular diseases mimicking neuro-ophthalmic disorders.


1. Ocular neoplasms(3)

  1. Benign vs malignant
  2. Primary vs secondary
  3. Pediatric vs adult neoplasms

2. Ocular infections(3)

  1. External
  2. Endophthalmitis
  3. Neuroretinitis

3. Ocular inflammation (uveitis)(3)

  1. Classification of uveitis
  2. Neuro-ophthalmic disorders associated with uveitis(1)
  3. Optic neuritis and uveitis(1)
  4. Meningo-uveitis(2)

4. Retinal disorders

  1. Vascular(2)
  2. Degenerative/hereditary(3)
  3. Inflammatory/infectious(3)
  4. White dot syndromes(3)

5. Glaucoma(3)
6. Ocular causes of acute and chronic visual loss(1)

VI. Neuro-ophthalmic manifestations of iatrogenic diseases


1. Recognize neuro-ophthalmic complications of iatrogenic diseases.


1. Radiation(3)
2. Chemotherapy(3)
3. Various drugs with specific neuro-ophthalmologic complications, including

  1. Cyclosporine(2)
  2. FK-506 (tacrolismus) (2)
  3. Amiodarone(1)
  4. Hydroxychloroquine(1)
  5. Ethambutol(1)
  6. Vasoconstritors(1)
  7. Steroids(1)
  8. Facial and orbital injections(1)

4. Alcohol

  1. Thiamine deficiency(1)
  2. Wernicke encephalopathy(3)

5. Neuro-ophthalmic complications of surgical procedures(1)

  1. Post operative visual loss(1)
  2. Epidural anesthesia(2)
  3. Ocular and orbital surgery(1)
  4. Neurosurgery(1)
  5. Endovascular procedures(1)

VI. Functional disorders


1. Recognize patients with functional visual complaints.


1. Terminology(3)

  1. Factitious (Münchausen's)
  2. Malingering
  3. Conversion reaction
  4. Exaggeration 
    1. Hypochondriasis
    2. Somatization disorder 

2. Clinical presentations(1)

  1. Visual loss(1)
  2. Visual field defects(1)
  3. Spasm of near triad(1)
  4. Nystagmus(1)

3. Specific techniques of evaluation(1)

Proposed by the Curriculum of the North American Neuro-Ophthalmology Society (NANOS)

Next: F. Procedures Commonly Performed/Obtained in Neuro-Ophthalmology