NANOS: Curriculum Outline

The 2020 Update to the North American Neuro-ophthalmology Society Neuro-Ophthalmology Curriculum

The 2020 update to the NANOS neuro-ophthalmology curriculum includes revisions to all sections of the curriculum previously written in 2006. We have streamlined the sections to make it relevant to neuro-ophthalmologists from different training backgrounds and consistent with United States and international training requirements. We have included recent developments in our specialty and eliminated redundant items within the curriculum. 

The goal of the curriculum is to provide a framework that defines the scope of work expected of a practicing neuro-ophthalmologist. The curriculum should not be considered an exhaustive list of conditions that a neuro-ophthalmologist may expect to see. Rather, we expect it to serve as the recommended educational plan for fellowship training and self-directed learning for practicing neuro-ophthalmologists throughout their career. It is our expectation that this curriculum will provide a framework for development and/or revision of existing curricula for medical students and residents (specifically, neurology, ophthalmology, otolaryngology, and neurosurgery). The specific learning objectives is expected to be different for students, residents, and fellows. The curriculum is weighted with the following levels:  

  • Basic or Level 1: We suggest this level for students (medical, optometry or neuroscience graduates) and practitioners who want to gain a basic understanding of the visual and nervous system. 
  • Intermediate or Level 2: We suggest this level for trainees who have a basic knowledge of the visual and nervous system. This level would be appropriate for residents or fellows in ophthalmology, neurology, and optometry. Some of the educational content will also be appropriate for trainees in otolaryngology, neurosurgery, neuro-oncology and physiatry.
  • Advanced or Level 3: This is the recommended curricula for fellowship training in neuro-ophthalmology. In addition, we suggest this level for practitioners who wish to develop and maintain expertise in neuro-ophthalmology and neuro-otology.
  • *: this superscript indicates that recommended level may be modified for a resident in either neurology or ophthalmology.

 

  1. ANATOMY AND PHYSIOLOGY FOR THE NEURO-OPHTHALMOLOGIST
  2. Goals:

    • Describe the anatomy of the visual pathways.
    • Describe the surgical anatomy of the eye and orbits.
    • Discuss intracranial and orbital vascular anatomy of relevance to the neuro-ophthalmologist

    Topics:

    1. Developmental anatomy (1)
      1. Embryological origin of the eye and orbit (1)
      2. Visual maturation (1)
    2. Bony anatomy (1)
      1. Skull and skull base (1)
        1. Sella turcica (1)
        2. Cavernous sinus (1) 
        3. Anterior cranial fossa (1)
        4. Middle cranial fossa (1)
        5. Posterior cranial fossa (1)
      2. Bony communications (1)
        1. Optic canal (1)
        2. Superior orbital fissure (1)
        3. Inferior orbital fissure (2)
        4. Ethmoidal foramina (2)
      3. Orbit (1)
    3. Ophthalmic and orbital anatomy and physiology (1)
      1. Orbit (1)
      2. Eyelids (1)
      3. Lacrimal pathways (2)
      4. Ocular surface, cornea, lens (1)
      5. Aqueous and vitreous humor (1)
      6. Uvea (1)
      7. Retina (1)
      8. Physiology of intraocular pressure (2)
    4. Afferent visual pathways (1) 
      1. Optic nerve (1)
      2. Optic chiasm (1)
      3. Optic tract (1)
      4. Lateral geniculate nucleus (1)
      5. Optic radiations (1)
      6. Calcarine cortex (1)
      7. Visual association areas (1)
    5. Efferent visual pathways (1)
      1. Cortical input (2)
      2. Subcortical structures (2)
      3. Cerebellar pathway (2)
      4. Vestibular pathways (2)
      5. Nuclear centers (1)
      6. Ocular motor nerves (1)
        1. Abducens (VI) (1)
        2. Trochlear (IV) (1)
        3. Oculomotor (III) (1)
      7. Extraocular muscles (1)
    6. Neuroanatomy relevant to neuro-ophthalmology (1)
      1. Other cranial nerves (1)
        1. Sensory anatomy CN 5 (trigeminal system) (2)
        2. Facial motor anatomy CN 7 (2)
        3. Auditory and vestibular anatomy CN 8 (2)
      2. Brainstem (1)
      3. Cerebellum (1)
      4. Basal Ganglia (1)
      5. Cerebral hemisphere (1)
      6. Pupillary pathway (1)
        1. Sympathetic (1)
        2. Parasympathetic (1)
      7. Autonomic pathway (1)
        1. Sympathetic (1)
        2. Parasympathetic (1)
    7. Vascular anatomy (1)
      1. Arterial anatomy (1)
        1. Aortic arch (1)
        2. Internal carotid arteries and their branches (1)
        3. External carotid arteries and their branches (1)
        4. Vertebrobasilar system (1)
        5. Circle of Willis (1)
        6. Blood supply of the orbit, eye, and optic nerve (1)
          1. Ophthalmic artery (1)
          2. Central retinal artery (1)
          3. Cilioretinal artery (2)
          4. Orbital arteries (2)
      2. Venous anatomy (1)
        1. Cerebral venous sinuses and deep venous system (1)
          1. Cavernous sinus (1)
          2. Other dural venous sinus (1)
          3. Deep venous system (2)
          4. Cortical veins (2)
        2. Venous drainage in the neck (1)
        3. Venous drainage of the eyes and orbits (1)
          1. Superior and inferior ophthalmic veins (1)
          2. Central retinal vein (1)
    8. Cerebrospinal fluid (1)
      1. CSF production, flow, drainage and dynamics (1)
      2. CSF composition (1)

     

  3. OCULAR AND NEUROLOGIC EVALUATION
  4. Goals: 

    • Discuss the components of neuro-ophthalmologic examination in a clinical setting.
    • Describe modifications of examination techniques used in the evaluation of comatose, pediatric, and non-organic visual loss subjects.
    • Apply relevant clinical and ancillary diagnostic techniques in a subject with visual disorder.

    Topics

    1. Evaluation of visual function (1)
      1. Visual acuity including basic principles of refraction (1)
      2. Color vision (1)  
      3. Contrast sensitivity (2)
      4. Stereopsis (1)
      5. Visual fields (1)
    2. Pupillary examination (1)
      1. Pupillary size and reflex (1)
      2. Relative afferent pupillary defect (1)
      3. Pharmacological examination of anisocoria (2)
    3. Other relevant ocular examination (2)
      1. Slit lamp examination (2)
      2. Intraocular pressure testing (1)  
    4. Funduscopic examination (1)
      1. Ophthalmoscopy (1) 
      2. Retinal fundus photography (1)  
    5. Examination of the orbit and extraocular structures (2)
      1. External examination, globe position and exophthalmometry (2)
      2. Examination of the eyelids and lacrimal system (2)
    6. Examination of eye movements and alignment 
      1. Normal eye movements 
        1. Range of eye movements (1)
        2. Optokinetic nystagmus (2)
        3. Saccades, Smooth pursuit (2)
        4. Vestibulo-ocular reflex (VOR) (2)
          1. Slow (2)
          2. Fast (Head impulse test) (2)
          3. Suppression (2)
        5. Convergence and divergence (2)
      2. Ocular alignment (2)
    7. Examination of the Audio-vestibular system  
      1. Hearing assessment and otoscopy (3)
      2. Dynamic visual acuity (3) 
      3. Maneuvers to provoke vertigo and nystagmus (3)
        1. Valsalva, Sound (3)
        2. Dix-Hallpike test (2)*
        3. Modified Epley maneuver (3)*
        4. Caloric testing (2)*
    8. Neurological examination 
      1. Examination of mental status (1)
        1. Clinical examination of mental status (1)
        2. Clinical Examination of higher order visual dysfunction (3)  
      2. Cranial nerve examination (1)
      3. Motor examination and reflexes (1)  
      4. Sensory examination (1)
      5. Coordination (1)
      6. Gait (1)
    9. Diagnostic tests- ophthalmic 
      1. Visual field testing (2) 
        1. Automated static perimetry (2) 
        2. Kinetic perimetry (2)
          1. Goldmann perimetry (2)
        3. Tangent screen (2) 
      2. Electrophysiology (2)  
        1. Full-field and multifocal electroretinogram (ERG) (2)
        2. Electro-oculogram (EOG) (2)  
        3. Visual evoked potential (2) 
        4. Nerve conduction study and electromyography (NCS/EMG) (3) * 
      3. Pupillometry (3)  
      4. Ophthalmic imaging
        1. Fundus fluorescein and indocyanine green angiography (2)  
        2. Fundus autofluorescence (2) 
        3. Optical coherence tomography (OCT) (2)  
          1. Imaging the optic nerve (2)  
            1. Retinal nerve fiber layer (RNFL) (2)  
            2. Enhanced depth imaging (EDI) (2)  
          2. Imaging the macula (2)
            1. Ganglion cell layer (GCL) (2)    
          3. OCT-angiography (3) 
        4. Ocular and orbital ultrasonography (2)  
    10. Diagnostic testing- neurological 
        Computed tomography (CT) (1)  
      1. Magnetic resonance imaging (MRI) (1)
      2. Vascular imaging (2)* 
        1. Color Doppler ultrasonography (2)* 
        2. CT and MR based angiography of head and neck (2)*
        3. CT and MR based venography of head and neck (2)*
        4.  Catheter angiogram of head and neck (2)* 
      3. Lumbar puncture with opening pressure, including fluoroscopic guidance (1)
    11. Diagnostic testing: Audio-vestibular  (3)
      1. Vestibular evoked myogenic potentials (3)  
      2. Audiogram (3)
      3. Video nystagmographyElectronystagmography and Video oculography (3) 
    12. Examination of the pediatric patient (2)
    13. Examination of the comatose patient (2)*
    14. Nonorganic disorders (2)  
      1. Visual acuity loss (2)
      2. Visual field loss (2)* 
      3. Spasm of near triad (2)* 
      4. Voluntary nystagmus (2)*

     

  5. NEURO-OPHTHALMIC SYMPTOMS AND SIGNS 
  6.  

    Goals:

    • Elicit and interpret symptoms and signs commonly encountered in neuro-ophthalmology.

    Topics:

    1. Visual loss (1)
      1. Transient(1)
      2. Permanent(1)
    2. Visual field loss (1)
    3. Positive visual phenomena (2)
      1. Visual illusions, distortion, and hallucinations (2)
    4. Disorders of visual perception (1)
    5. Ocular motility and alignment
      1. Oscillopsia, nystagmus, ocular oscillations(1)
      2. Monocular diplopia (1)
      3. Binocular diplopia (1)
    6. Normal and abnormal optic nerve(1)
      1. Normal optic nerve appearance (1)
      2. Abnormal optic nerve appearance (1)
        1. Optic disc edema (1)
        2. Optic atrophy (1)
        3. Optic disc cupping (1)
        4. Pseudo-disc edema (pseudopapilledema) (2)
        5. Hypoplastic and dysplastic optic nerve  (2)
        6. Optic disc drusen (2)
        7. Myelinated nerve fibers (2)
        8. Other anomalous optic nerve appearance (2)
        9. Optociliary shunt vessels (retinochoroidal collaterals) (2)
    7. Eye lid signs and symptoms (1)
      1. Ptosis (1)
      2. Lagophthalmos (1)
      3. Lid retraction (2)
      4. Lid lag (2)
    8. Orbital signs (2)
      1. Proptosis (1)
      2. Enophthalmos (2)
      3. Exophthalmos (2)
    9. Pupillary changes (1)
      1. Anisocoria (1)
      2. Irregular pupils (1)
      3. Non-reactive pupils (1)
      4. Relative afferent pupillary defect (1)
      5. Light near dissociation (1)
    10. Abnormal facial movements (2)
      1. Facial weakness including aberrant regeneration (2)
      2. Hemifacial spasm (2)*
      3. Facial myokomia (2)
    11. Ocular pain, facial pain, headache (1)
      1. Photophobia (1)
      2. Eye pain (1)
      3. Face pain (2)
    12. Dizziness
      1. Vertigo (1)

     

  7. DISORDERS OF THE AFFERENT AND EFFERENT VISUAL PATHWAYS
  8. Goals:

    • To localize, diagnose and manage disease processes involving the afferent, association, pupillary and efferent visual pathway.

    Topics

    1. Diseases of the optic nerve
      1. Optic neuropathy, Ischemic (1)
        1. Anterior (1)
        2. Posterior (2)
        3. Diabetic papillopathy (2)
        4. Radiation optic neuropathy (2)
      2. Inflammation (1)
        1. Non-infectious
          1. Typical and atypical optic neuritis (1)
          2. Neuroretinitis (2)
          3. Optic perineuritis (2)
          4. Orbital inflammation (2)
        2. Infectious (2)
        3. Paraneoplastic (2)
      3. Compression/infiltration (1)
        1. Orbit (1)
        2. Sella (1)
        3. Skull base (2)
      4. Traumatic (1)
        1. Direct (2)
        2. Indirect (2)
      5. Toxic including adverse effects of medications (2)
      6. Nutritional (2)
      7. Metabolic (2)
      8. Hereditary (2)
      9. Congenital optic nerve anomaly (2)
        1. Bergmeister papilla (2)
        2. Optic nerve dysplasia and aplasia/hypoplasia (2)
        3. Optic nerve coloboma (2)
        4. Optic nerve pit (2)
        5. Morning glory syndrome (2)
        6. Optic nerve drusen (2)
        7. Tilted disc (2)
        8. Myelinated nerve fiber (2)
        9. Staphyloma (2)
        10. Other syndromes (2)
      10. Papilledema secondary to raised intracranial pressure (1)
    2. Diseases of the chiasm
      1. Chiasmal visual field defects (1)
      2. Compression/infiltration(2)
        1. Lesions of sella, suprasella and parasellar regions (1)
        2. Skull base lesions (1)
      3. Inflammation(2)
        1. Chiasmal optic neuritis (2)
        2. Hyphophysitis (2)
        3. Abscess (2)
      4. Trauma (2)
      5. Ischemia/hemorrhage (2)
    3. Diseases of the retrochiasmal visual pathways (1)
      1. Optic tract (1)
        1. Optic tract syndrome (2)
      2. Lateral geniculate (1)
      3. Optic radiations (1)
      4. Calcarine cortex (1)
    4. Association areas (2)
      1. Physiology of the dorsal and ventral streams (1)
      2. Specialized syndromes (2)*
        1. Cerebral achromatopsia (2)
        2. Agnosia (2)
          1. Prosopagnosia (2)
          2. Topographagnosia (2)
        3. Anton syndrome (2)
        4. Balint syndrome (2)
          1. Simultanagnosia (2)
          2. Ocular motor apraxia (2)
          3. Optic ataxia (2)
        5. Gerstmann syndrome (2)
        6. Alexia (2)
          1. With agraphia (2)
          2. Without agraphia (2)
        7. Visual neglect (2)
        8. Constructional apraxia (2)*
        9. Blind-sight and residual vision (3)
        10. Riddoch syndrome: Stato-kinetic dissociation (3)
        11. Akinetopsia (3)
        12. Visual illusions, distortion, and hallucinations (2)
          1. Visual snow (2)
          2. Cerebral diplopia/polyopia (2)
          3. Palinopsia (2)
          4. Illusory visual spread (2)
          5. Alice in Wonderland syndrome (2)
          6. Formed and unformed visual hallucinations (2)
            1. Charles-Bonnet syndrome (2)
            2. Peduncular hallucinosis (3)
            3. Neurodegenerative disorders (3)
            4. Toxic (3)
          7. Other visual distortions (3)
    5. Pupillary pathology
      1. Normal pupillary responses (1)
      2. Effects of drugs on the pupils (1)
      3. Congenital pupillary abnormalities (2)
      4. Pupillary changes secondary to ocular diseases (2)
        1. Trauma (2)
        2. Inflammation (2)
        3. Pupillary block (2)*
        4. Neovascularization (2)*
        5. Ocular surgery, laser (2)*
        6. Other (siderosis, foreign body, melanoma) (2)*
      5. Evaluation and management of anisocoria (2)
        1. Evaluation and management of a large or a small pupil (2)
      6. Evaluation and management of specific pupillary disorders (1)
        1. Physiologic anisocoria (1)
        2. Pharmacologically dilated or constricted pupil (2)
        3. Afferent pupillary defect (1)
        4. Light near dissociation (2)
        5. Horner syndrome (1)
        6. Third nerve palsy (1)
        7. Adie’s tonic pupil (2)
        8. Episodic pupillary mydriasis (2)
        9. Tadpole pupil (3)
        10. Argyll-Robertson pupil (2)
        11. Corectopia (2)
    6. Eye movement systems pathology
      1. Supranuclear and internuclear disorders
        1. Lesions of Medulla (2)
          1. Skew deviation (2)
          2. Ocular tilt reaction (2)
          3. Wallenberg syndrome (2)
          4. Syndrome of the anterior inferior cerebellar artery (2)
        2. Lesions of Cerebellum (2)
          1. Impaired VOR cancelation (3)
          2. Other cerebellar signs (3)
        3. Lesions of Pons (2)
          1. Internuclear ophthalmoplegia (1)
          2. Horizontal gaze palsy (2)
          3. One and a half syndrome (2)
          4. Foville syndrome (2) 
          5. Millard-Gubler syndrome (2)
          6. Raymond-Cestan syndrome (2)
          7. Locked-in syndrome (2)
        4. Lesion of the midbrain (2)
          1. Vertical gaze palsy (2)
          2. Disturbance of convergence and divergence (3)
          3. Weber syndrome (2)
          4. Benedickt syndrome (2)
          5. Nothnagel syndrome (2)
          6. Claude syndrome (2)
          7. Dorsal midbrain syndrome (2)
          8. Top of basilar syndrome (3)
        5. Lesions of the cerebral hemispheres (2)
        6. Lesions of the thalamus and basal ganglia (3)
          1. Thalamic esotropia (3)
      2. Lesions of the ocular motor nerves and nucleus (1)
        1. Oculomotor nerve palsy (1)
        2. Trochlear nerve palsy (1)
        3. Abducens nerve palsy (1)
        4. Multiple cranial neuropathy (1)
          1. Cavernous sinus and superior orbital fissure syndrome (2)
      3. Other disorders of ocular motility (2)
        1. Spasm of the near triad (2)
        2. Convergence insufficiency (2)
        3. Divergence insufficiency (2)
      4. Strabismus (2)*
        1. Classification (2)
        2. Phoria and tropia (2)
        3. Esotropia (1)
        4. Exotropia (1)
        5. Hypertropia and cyclovertical strabismus (2)
        6. Decompensation of phoria (2)
        7. Paretic and restrictive strabismus (2)
        8. Congenital Cranial Dysinnervation Disorders (CCDD) (3)
          1. Congenital fibrosis of extraocular muscles (CFEOM) (3)
          2. Duane syndrome (2)
          3. Mobius syndrome (2*)
          4. Monocular elevation deficit (3)
          5. Horizontal gaze palsy with progressive scoliosis (3)
          6. Congenital ptosis (2)
        9. Mechanical forms of strabismus (2)
          1. Brown syndrome (2)*
          2. Age related distance esotropia (sagging eye syndrome) (3)
          3. Heavy eye syndrome (3)
          4. Post ocular surgery strabismus (3)
          5. Silent sinus syndrome (3)
        10. Principles of management of strabismus (2)
          1. Optical methods (2)*
          2. Surgical correction (2)*
      5. Other abnormal eye movements (3)
        1. Superior oblique myokymia (2)
        2. Ocular neuromyotonia (3)
        3. Cyclic oculomotor paresis (3)
        4. Aberrant regeneration of oculomotor nerve palsy (2)
        5. Oculomasticatory myorhythmia (3)
    7. Nystagmus and disorders of ocular stability
      1. Anatomic and physiologic basis for gaze stability (3)
      2. Classification of nystagmus (2)
        1. Physiologic vs. pathologic nystagmus (2)
        2. Congenital nystagmus vs. acquired nystagmus (2)
        3. Central nystagmus vs. peripheral nystagmus (2)
        4. Pendular vs. jerk nystagmus (2)
      3. Specific types of nystagmus and their localizing value
        1. Horizontal jerk nystagmus (1)
        2. Pendular nystagmus (2)
        3. Down beat nystagmus (2)
        4. Upbeat nystagmus (2)
        5. Rebound nystagmus (2)
        6. Bruns nystagmus (2)
        7. Periodic alternating nystagmus (2)
        8. Convergence retraction nystagmus (2)
        9. See saw nystagmus (2)
        10. Divergence nystagmus (2)
        11. Sensory nystagmus (2)
        12. Infantile nystagmus syndrome (Congenital nystagmus) (2) 
        13. Spasmus nutans/dissociated nystagmus/monocular nystagmus (2)
        14. Rotary (torsional) nystagmus (2)
        15. Eyelid nystagmus (2)
        16. Oculopalatal tremor (3)
      4. Saccadic intrusions and oscillations (3)
        1. Square wave jerks, macro-square wave jerks (2)
        2. Ocular flutter (2)
        3. Opsoclonus (2)
        4. Ocular bobbing (2)
        5. Voluntary nystagmus (2)
      5. Treatment of nystagmus and ocular oscillation (3)
    8. Eyelid position abnormalities
      1. Ptosis (1)
        1. Congenital ptosis (2)
          1. With elevator palsy (2)
          2. Marcus Gunn Jaw Wink (2)
          3. Blepharophimosis (2)*
        2. Acquired ptosis
          1. Disorders of levator palpebrae aponeurosis (disinsertion, dehiscence) (2)
          2. Restrictive or infiltrative ptosis (2)
        3. Apraxia of eyelid opening  (3)
        4. Pseudoptosis (3)
      2. Eyelid twitch (1)
      3. Excessive eyelid closure (2)
        1. Blepharospasm (2)
        2. Eyelid nystagmus(3)
      4. Eyelid retraction (2)
      5. Lagophthalmos (2)
    9. Facial nerve dysfunction
      1. Central and peripheral facial palsy (1)
        1. Aberrant regeneration (2)
      2. Facial myokymia(2)
      3. Hemifacial dystonia (Meige syndrome) (3)
      4. Oculomasticatory myorhythmia (Whipple disease) (3)
      5. Facial tics (3)
      6. Hemifacial contraction syndrome (3)

     

  9. SYSTEMIC, NEUROLOGIC, AND OPHTHALMIC DISORDERS COMMONLY ASSOCIATED WITH NEURO-OPHTHALMIC MANIFESTATIONS
  10. Goals:

    • To diagnose and manage neuro-ophthalmic manifestations from neurologic, ophthalmic, and systemic disease processes.

    Topics

    1. Developmental and congenital anomalies with neuro-ophthalmologic consequences
      1. Normal visual maturation (1)
      2. Amblyopia(1)
      3. Perinatal complications (prematurity, low birth weight, birth injuries) (1)
        1. Cerebral palsy (1)
        2. Retinopathy of prematurity (1)
      4. Congenital hydrocephalus (2)
      5. Cranial dysostoses (craniosynostosis) (2*)
      6. Common malformations of the eye and orbit (2)
      7. Skull base malformations. 
        1. Chiari malformation (2)
      8. Fibrous dysplasia (2)
      9. Ocular manifestations of child abuse (1)
    2. Genetic disorders commonly associated with neuro-ophthalmologic manifestations
      1. Principles of genetics and genetic disorders (1)
      2. Testing for genetic disorders (2)
      3. Neurocutaneous syndromes (1)
        1. Neurofibromatosis (1)
          1. NF-1 (1)
          2. NF-2 (1)
        2. Tuberous sclerosis (1)
        3. von Hippel-Lindau (1)
        4. Sturge-Weber (1)
        5. Ataxia telangiectasia (2)
        6. Wyburn-Mason (2)
        7. Other genetic disorders
      4. Mitochondrial diseases (1)
        1. Classification and pathogenesis of mitochondrial genetics and disorders (2)
        2. Disorders of interest to neuro-ophthalmology
          1. Leber Hereditary Optic Neuropathy (LHON) (1)
          2. Chronic progressive external ophthalmoplegia (CPEO) (2)
          3. Kearns-Sayre syndrome (2)
          4. Mitochondrial encephalopathy, lactic acidosis, and stroke like episodes (MELAS) (3)
          5. Other mitochondrial disorders (3)
      5. Dominant Optic Atrophy (2)
        1. Isolated optic neuropathy (2)
        2. Diabetes insipidus, diabetes mellitus, optic atrophy, and deafness (DIDMOAD) syndrome (2)
      6. Papillorenal syndrome (2)
    3. Vascular disorders commonly associated with neuro-ophthalmologic manifestations (2)
      1. Cardiovascular risk factors (1)
      2. Prevention of vascular disease (1)
      3. Ocular and Cerebrovascular diseases 
        1. Acute ischemic stroke (1)
          1. Classification and mechanisms (2)
          2. Diagnosis and evaluation of stroke(1)
          3. Principles of acute stroke care and management (2)
          4. Secondary prevention of stroke (2)
          5. Neuro-ophthalmologic manifestations of stroke (2)
        2. Intracranial hemorrhage (1)
          1. Classification and mechanisms (2)
          2. Principles of management (2)
          3. Neuro-ophthalmic manifestations 
            1. Terson syndrome (3)*
        3. Other cerebrovascular diseases of interest
          1. Reversible cerebral vasoconstriction syndrome (RCVS) (2)*
          2. Posterior reversible encephalopathy syndrome (PRES) (2)*
        4. Cerebral venous sinus thrombosis (2)
          1. Cavernous sinus thrombosis (2)
        5. Intracranial vascular malformations, diagnosis and neuro-ophthalmologic manifestations
          1. Aneurysm (2)
          2. Arteriovenous malformation (2)
          3. Dural fistula (3)
          4. Carotid cavernous fistula (direct, indirect) (2)
            1. Orbital and ophthalmic manifestations (2) 
          5. Cavernous hemangiomas (2)
          6. Orbital vascular malformations (2)
        6. Ocular ischemic syndrome (2)
        7. Retinal vascular diseases (2)
          1. Retinal artery occlusive diseases (2)
            1. Susac syndrome (3)
            2. Retinal vasculitis (2)
          2. Retinal venous occlusive diseases (2)
          3. Hypertensive retinopathy (1)
          4. Diabetic retinopathy (1)
    4. Inflammatory disorders commonly associated with neuro-ophthalmologic manifestations
      1. Classification of systemic inflammatory diseases (2)
      2. Specific inflammatory diseases with ocular and neurologic manifestations
        1. Giant cell arteritis (1) (Temporal arteritis)
        2. Sarcoidosis (1)
        3. Systemic lupus erythematosus (1)
        4. Behçet disease (1)
        5. Granulomatosis with polyangiitis (1)
        6. IgG4- related disease (2)
      3. Neuro-inflammatory diseases
        1. Classification and pathophysiology (2)
        2. Multiple sclerosis (1)
          1. Relationships between optic neuritis and multiple sclerosis (2)
          2. Neuro-ophthalmology of multiple sclerosis (2)
          3. Classification and diagnostic criteria (2)
          4. Treatment strategies (2)
        3. Neuromyelitis optica spectrum disorders (2)
        4. Myelin Oligodendrocyte Glycoprotein (MOG) antibody disease (2)
        5. Acute disseminated encephalomyelitis (2)*
        6. Anti-glutamic acid decarboxylase antibody disease
        7. Other antibody mediated neuro-inflammatory diseases of interest
          1. Neuronal antibody mediated disorders (3)
          2. Other forms of autoimmune encephalitides (3)
        8. Inflammatory polyradiculopathy (2)
          1. Guillain- Barre syndrome (2)
          2. Miller Fisher variant (2)
      4. Orbital inflammatory diseases
        1. Thyroid orbitopathy (2)
        2. Idiopathic orbital inflammatory disease (2)
        3. Secondary orbital inflammation (2)
        4. Tolosa Hunt syndrome (2)
      5. Ocular inflammatory diseases 
        1. Classification of uveitis and retinal vasculitis (2)
        2. Evaluation and treatment (2)
        3. Neuro-ophthalmic manifestations and complications from uveitis (3)
        4. Specific conditions:
          1. White dot syndromes (2)*
              Acute Idiopathic Blind-Spot Enlargement (2)*
            1. Acute Zonal Occult Outer Retinopathy (2)*
            2. Multiple Evanescent White Dot Syndrome (2)*
          2. Uveo-meningitis (3)
          3. Vogt-Koyanagi-Harada syndrome (2)*
          4. Autoimmune and paraneoplastic retinopathy (3)
          5. Other inflammatory diseases 
    5. Infectious disorders commonly associated with neuro-ophthalmologic manifestations
      1. Classification and principles of management of infections of the head and neck(2)
        1. Intracranial infections (2)
          1. Meningitis, encephalitis, and meningoencephalitis (1)
          2. Abscess (1)
        2. Orbital infections (2)
          1. Cellulitis (1)
        3. Invasive sinusitis (2)
        4. Ophthalmic infections of interest
          1. Herpes zoster ophthalmicus and its complications (2)
          2. Acute retinal necrosis (2)
          3. Other ophthalmic infections
      2. Specific infectious diseases with ocular and neurologic manifestations
        1. HIV and AIDS related diseases (1)
        2. Human herpes infections (1)
        3. Syphilis (1)
        4. Tuberculosis (1)
        5. Toxoplasmosis (2)
        6. Whipple disease (2)
        7. Prion disease (2)*
        8. John Cunningham virus (JC) (2)
        9. Borrelia burgdorferi (Lyme) disease (2)
    6. Neuro-ophthalmic manifestations of trauma
      1. Traumatic brain injury (2)
        1. Classification, mechanisms, and principles of management (2)*
        2. Concussion and sports related head trauma (2)*
        3. Neurological complications of head injury (2)*
          1. Post-concussion headache and syndrome (2)*
      2. Orbital and facial trauma (2)
        1. Classification, mechanisms, and principles of management (2)*
        2. Direct and indirect traumatic optic neuropathy (2)
        3. Orbital fracture (2)
        4. Orbital hemorrhage (2)
        5. Orbital entrapment syndrome (2)
      3. Ophthalmic trauma (2)
        1. Purtscher traumatic retinopathy (2)*
    7. Neoplastic disorders commonly associated with neuro-ophthalmic manifestations  
      1. Intracranial neoplasm (2)
      2. Orbital neoplasm (2)
      3. Ocular neoplasm (2)
      4. Paraneoplastic disorders (3)
      5. Neuro-ophthalmic complications of chemotherapy (3)
      6. Neuro-ophthalmic complications of radiation therapy (3)
    8. Metabolic disorders commonly associated with neuro-ophthalmologic manifestations
      1. Nutritional deficiencies (1)
        1. Vitamin A (1)
        2. Vitamin B1 (1)
          1. Wernicke encephalopathy (1)
          2. Korsakoff syndrome (1)
        3. Vitamin B12 (1)
        4. Folate (1)
      2. Toxicity (2)
        1. Methanol (2)
      3. Metabolic storage diseases (2)
      4. Amyloidosis (2)
    9. Neuro-ophthalmic manifestations during pregnancy (2)
    10.  

    11. Neurologic disorders commonly associated with neuro-ophthalmologic manifestations*
      1. Abnormal  intracranial pressure conditions (2)
        1. Intracranial hypertension (2)
          1. Pathophysiology of intracranial hypertension (2)
          2. Neuro-ophthalmic manifestations and management (2)
          3. Differential diagnosis (2)
            1. Idiopathic intracranial hypertension (1)
            2. Secondary causes of intracranial hypertension (1)
          4. Management of intracranial hypertension (2)
        2. Intracranial hypotension (2)
          1. Neuro-ophthalmic manifestations and management (2)
          2. Differential diagnosis (3)
          3. Management of intracranial hypotension (3)
      2. Epilepsy (2)*
        1. Classification and principles of management (2)
        2. Neuro-ophthalmic manifestations
          1. Occipital seizures (2)
          2. Other manifestations (3)
      3. Neuro-degenerative diseases (2)
        1. Parkinson related syndromes
          1. Parkinson disease (1)
          2. Progressive supranuclear palsy (2)
          3. Multiple systems atrophy (3)
          4. Dementia with Lewy bodies (3)
        2. Dementia (2)*
          1. Classification of dementia (2)
          2. Conditions of interest
            1. Alzheimer disease (1)
            2. Posterior cortical atrophy (3)
            3. Vascular dementias (3)
            4. Frontotemporal dementia (3)
        3. Amyotrophic lateral sclerosis (2)
      4. Neuro-muscular transmission deficits (2)
        1. Physiology of the neuromuscular transmission (2)
        2. Myasthenia gravis (1)
        3. Lambert-Eaton myasthenic syndrome (3)
        4. Toxic neuromuscular transmission defect (Botulism, medications) (2)
        5. Other neuro-muscular transmission deficits (3)
      5. Myopathies
        1. Muscular dystrophy (2)
          1. Classification (3)
          2. Disorders of relevance to the neuro-ophthalmologist
            1. Myotonic dystrophy (3)
            2. Facioscapulohumeral muscular dystrophy (FSHD) (3)
            3. Oculopharyngeal muscular dystrophy (OPMD) (3)
        2. Other forms of myopathy (3) 
      6. Headache and facial pain of relevance to the neuro-ophthalmologist
        1. Classification of headaches and facial pain (International Headache Society (IHS)) (2)
        2. Migraine (1)
          1. Migraine with visual aura (1)
          2. Migraine without aura (1)
          3. Chronic migraine (1)
          4. Aura without headache (2)
          5. Complications of migraine (3)
            1. Persistent aura without infarction (3)
            2. Migraine aura with infarction (3)
          6. Vestibular migraine (3)
          7. Other variants of migraine (3)
        3. Tension type headaches (1)
          1. Episodic (1)
          2. Chronic (2)
        4. Trigeminal autonomic cephalagias (TAC) (2)*
          1. Cluster headache (1)
          2. Hemicrania continua (3)
          3. Other TAC disorders (3)
        5. Important secondary headache disorders  
          1. Headaches attributed to injury of head or neck (Post-traumatic headache) (3)
          2. Headaches attributed to cranial or cervical vascular disorder (2)
            1. Intracranial hemorrhage (1)
            2. Arterial dissection (ICA or vertebral) (2)
            3. Arterial inflammation (Giant cell arteritis) (1)
            4. Cavernous sinus thrombosis (2)
            5. Pituitary apoplexy (2)
            6. Other vascular disorders (3)  
          3. Headache associated with low or high intracranial pressure(2)
          4. Headache attributed to disorders of the eye (2)
            1. Angle closure glaucoma (1)
            2. Ocular inflammation (2)
            3. Refractive error (2)
            4. Trochlear headache (3)
            5. Ocular surface disorder (2)*
            6. Herpes zoster ophthalmicus (2)
          5. Painful lesions of the cranial nerves and other facial pain
            1. Trigeminal neuralgia and neuropathy (2)
            2. Painful optic neuritis (2)
            3. Recurrent painful ophthalmoplegic neuropathy (3)
            4. Tolosa-Hunt syndrome (2)
    12. Ophthalmic disorders commonly associated with neuro-ophthalmologic manifestations* 
      1. Hereditary and acquired degenerative retinal diseases (2)
        1. Retinitis pigmentosa (1)
        2. Albinism (2)
        3. Pathologic myopia (2)
        4. Age related macular degeneration (1)
        5. Vitreomacular traction syndrome (2)
        6. Macular edema (2)
        7. Foveal displacement diplopia syndrome (3)
      2. Glaucoma(1)
        1. Classification of glaucoma (2)
        2. Evaluation and principles of management (2)
    13. Neuro-ophthalmic manifestations of iatrogenic diseases 
      1. Radiation (2)
        1. Radiation retinopathy (2)
        2. Radiation optic neuropathy (2)
      2. Chemotherapeutic agents (3)
        1. Classifications of chemotherapeutic agents (3)
        2. Classification of immunomodulating (biological) agents (3)
        3. Specific (commonly used) drugs with neuro-ophthalmologic complications 
          1. Ethambutol (2)
          2. Hydroxychloroquine (2)
          3. Amiodarone (2)
          4. Cyclosporine (3)
          5. Tacrolimus (3)
          6. Other agents of interest (3)
      3. Neuro-ophthalmic complications of surgical procedures
        1. Peri-operative visual loss (2)
        2. Ocular and orbital surgery (2)
        3. Neuro-endovascular procedures (2)
        4. Skull base surgical and other neurosurgical procedures (3)
        5. Epidural anesthesia (3)
        6. Other procedures
    14. Non organic visual disorders
      1. Classification (2)
        1. Somatic Symptom and related disorders (2)
          1. Somatic symptom disorder (3)
          2. Illness anxiety disorder (3)
          3. Conversion disorder (Functional neurological symptom disorder) (2)
          4. Factitious disorder (2)
        2. Malingering (2)
      2. Clinical presentations and examination (3)
      3. Principles of management (3)
        1. Cognitive behavioral therapy (3)

     

  11. PROCEDURES COMMONLY PERFORMED/OBTAINED IN NEURO-OPHTHALMOLOGY
  12. Goal:

    • Describe the principles and complications of common ophthalmic and neurological surgical and non-surgical (neurostimulation and chemodenervation) procedures 

    Topic 

    1. Surgical and endovascular procedures and their complications (2)*
      1. Extraocular procedures relevant to neuro-ophthalmology (2)
        1. Temporal artery biopsy (2)
        2. Principles and complications of strabismus surgery (2)
        3. Canthotomy, cantholysis (2)
        4. Approaches for orbital biopsies and orbital tumors (3)
        5. Optic nerve sheath fenestration (3)
        6. Orbital decompression (3)
        7. Other relevant procedures
      2. Neurosurgical procedures of relevance to neuro-ophthalmology
        1. CSF shunting procedures (3)
        2. Monitoring of intracranial pressure (3)
        3. Pituitary surgery (transphenoidal adenomectomy) (3)
        4. Other neurosurgical procedures of interest (3)
      3. Interventional neuroradiology techniques (3)
        1. Cerebral angiography and venography (3)
        2. Embolization and coiling (3)
        3. Vessel angioplasty and stenting (3)
        4. Intravenous and intra-arterial thrombolysis (2)
        5. Thrombectomy in acute ischemic stroke (2)
      4. Neuro-stimulation and neuro-ablative procedures (3)
      5. Common intraocular surgical procedures of relevance to neuro-ophthalmology
          Principles and neuro-ophthalmic complications from cataract surgery (2)
        1. Principles and neuro-ophthalmic complications from glaucoma procedures (2) 
        2. Principles and neuro-ophthalmic complications from vitreo-retinal procedures (2)
    2. Botulinum toxin therapy (2)*
      1. Treatment of blepharospasm, hemifacial spasm, Meige syndrome(3)
      2. Treatment of strabismus (3) 
      3. Treatment of chronic migraine (2)

     

  13. PATIENT COUNSELING
  14. Goal:

    • Appropriately counsel patients and family resources available to overcome challenges of obesity, low vision, and genetic diseases. 

    Topic

    1. Visual impairment (2)
      1. Classification of visual impairment based on USA and WHO standards (2)
      2. Definition of legal blindness(1)
      3. Legal requirements for driving (visual) in the state in which you practice (2)
    2. Counseling of the visually impaired patient(2)
      1. Liaison with rehabilitation services (neurologic and for the visually impaired) (2)
      2. Liaison with a low vision service (2)
      3. Principles of low vision rehabilitation (2)
      4. Injury prevention in the visually impaired (2)
    3. Genetic counseling(2)
      1. Financial, legal, and social implications of genetic testing (2)
      2. Counsel patient and family with genetic diseases (2)
    4. Other
      1. Counseling for weight loss (2)
      2. Counseling for vascular risk factor management (2)

     

  15. RESEARCH, ADMINISTRATION, EDUCATIONAL RESOURCES
  16. Goal:

    • Model a career in neuro-ophthalmology through utilization of resources for practice management, research, and organizational resources 

    Topics:

    1. History of neuro-ophthalmology (3)
    2. The practice of neuro-ophthalmology (3)
      1. Practice management
        1. Credentialing, licensure, board certification (2)
        2. Principles of practice management  (3)
          1. Billing and coding requirements (3)
          2. Carrier relations (3)
        3. Accreditation (JCAHO) requirements (staffing, equipment and supplies, facilities, quality insurance) (3)
      2. Systems-based practice (2)
        1. Principles of patient safety and health care quality in neuro-ophthalmology (3)
          1. Medical errors, reporting and prevention (2)
          2. Health care costs, resource allocation, quality of care, access to care issues (2)
        2. Health care disparity and access to care in neuro-ophthalmology (3)
        3. Medico-legal aspects of neuro-ophthalmology practice (3)
          1. Overview of medical malpractice acts, torts, and tort reform (3)
          2. Role of expert witness in medico-legal procedures(3)
          3. Laws relating to drug dispensing, regulation, and abuse (2)
      3. Professionalism: (2)
        1. Adherence to ethical principles in personal and professional career (2)
        2. Participation in professional organizations (2)
      4. Interpersonal communication
        1. Health care team and other professionals (2)
        2. Patients and families (2)
        3. Public (3)
      5. Practice based learning and improvement (2)
        1. Certification and maintenance of certification (3)
        2. Use of evidence-based guidelines (2)
        3. Critical review of literature (2)
    3. Research 
      1. Read and analyze scientific articles (1)
      2. Research funding (3)
      3. Development of a research project (3)
      4. Ethical issues in research, including consent and researchers’ interactions with corporate funding sources (conflict of interest) (3)
      5. Write and publish a manuscript (3)
      6. Major studies of interest to the neuro-ophthalmologist (the list is not exhaustive):
        1. Optic Neuritis Treatment Trial (ONTT) (1)
        2. Longitudinal Optic Neuritis Study (LONS) (2)
        3. Ischemic Optic Neuropathy Decompression Trial (IONDT) (2)
        4. Idiopathic intracranial hypertension treatment trial (IIHTT) (2)
        5. Trial of Tocilizumab in Giant-Cell Arteritis (2)
        6. Teprotumumab for Thyroid-Associated Ophthalmopathy (2)
        7. Other major studies of relevance (3)
    4. Neuro-ophthalmologic resources (3)
      1. Organizations(3)
        1. North American Neuro-Ophthalmology Society (NANOS)
        2. European Neuro-Ophthalmology Society (EUNOS)
        3. American Academy of Neurology (AAN)
        4. American Neurological Association (ANA)
        5. American Academy of Ophthalmology (AAO)
        6. Association for Research in Vision and Ophthalmology (ARVO)
        7. Regional organizations
        8. National Eye Institute (NEI)
        9. National Institute of Neurological Disorders and Stroke (NINDS)
        10. Consortium for Pediatric Neuro-Ophthalmologists (CPNO)
      2. Journals(2)
      3. NOVEL database(3)
        1. NOVEL website:   https://novel.utah.edu/

     

  17. References
    1. American Academy of Neurology. Neuro-Ophthalmology/Neuro-otology residency core curriculum. ( https://www.aan.com/siteassets/home-page/tools-and-resources/academic-neurologist--researchers/teaching-materials/aan-core-curricula-for-program-directorstor/neuroopthalmology-residency-core-curriculum_tr.pdf
    2. American Academy of Ophthalmology. 2019-2020 Basic and Clinical Science Course. Section 5. Neuro-Ophthalmology (last major revision 2017-2018). 
    3. American Academy of Neurology. Continuum (Minneap Minn). Dinkin M (guest editor). Neuro-Ophthalmology. 2019 
    4. American Board for Psychiatry and Neurology. Certification examination in neurology. 2019 Content Outline ( https://www.abpn.com/wp-content/uploads/2019/11/2020_Neurology_CERT_Content_Specifications.pdf
    5. International council of ophthalmology residency and neuro-ophthalmology subspecialty curriculum ( http://www.icoph.org/refocusing_education/curricula.html#ICO%20Residency%20Curriculum
    6. Miller NR, Newman NJ, Biousse V, Kerrison JB. Walsh & Hoyt's Clinical Neuro-Ophthalmology. Lippincott Williams & Wilkins, 6th edition. 2005.
    7. Liu GT, Volpe NJ, Galetta SL. Neuro-Ophthalmology. Diagnosis and Management (3rd ed). Elsevier (2018).
    8. Headache Classification Committee of the International Headache Society (IHS) The International Classification of Headache Disorders, 3rd edition. Cephalalgia. 2018;38(1):1–211. doi:10.1177/0333102417738202.
    9. Society for Academic Emergency Medicine (SAEM) webpage. How to design a curriculum? https://www.saem.org/education/saem-online-academic-resources/curriculum
  1. Prepared by the Curriculum Subcommittee
      1. S. Kedar (Chair)
      2. K. Digre (Vice Chair)
      3. V. Biousse