Day 12 (4): Neuro-Ophthalmic Evaluation Flashcards

1
Q

What are the pertinent symptoms elicited in the history of neuro-ophthalmology patients?

A
  1. Abnormal vision
  2. Double vision
  3. Eye deviation
  4. Unequal pupils (anisocoria)
  5. Facial asymmetry: ptosis, Bell’s palsy, spasm
  6. Facial or eye pain
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2
Q

What are the common descriptors used to describe abnormal vision?

A
  1. Blurred or dim
  2. With a cloud/screen/curtain
  3. Blind
  4. Distorted
  5. Poor color vision
  6. Difficulty of movement and ambulation due to abnormal vision
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3
Q

What are the pertinent points in the history of pts presenting with abnormal vision?

A
  1. Etiology: differentiate using Pinhole Test
    - Optical: IMPROVES with pinhole
    - Neurosensory: NO improvement
  2. Onset: Sudden or Gradual
    - Sudden: occurred without warning
    - Gradual: subtle and insidious progression
  3. Duration
    - Seconds: Papilledema, Transient Visual Obscurations
    - Minutes: Transient Ischemic Attack, Atherosclerosis, Carotid Artery Disease
    - Hours: Migraine
    - Days: Optic neuritis/neuropathy
  4. Timing or Progression
    - transient, fluctuating or constant
    - improving or worsening

Remember:
Time Course (onset and duration): most important clue to the etiology

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4
Q

How is strabismus classified according to the angle of deviation in the different gaze directions?

A

COMITANT/NON-PARALYTIC
- misalignment or angle of deviation is EQUAL in ALL gaze directions (within 5 PD)
- NO under- or overaction of EOMS
- FULL ocular motility (ductions and versions)
- NORMAL motor evaluation

INCOMITANT/PARALYTIC
- misalignment or angle of deviation VARIES with the gaze direction
- SECONDARY deviation (affected eye is fixating) is GREATER than the PRIMARY deviation (normal eye is fixating)
- INVOLVES under- or overaction of EOMS
- angle INCREASES with gaze TOWARDS the affected muscle
- angle DECREASES with gaze AWAY from the affected muscle

Types based on the EOMs involved:
A. Horizontal Muscle Incomitance
- involve horizontal muscles (MR, LR)
- horizontal angle of deviation in L gaze is different from that in R gaze
- affected eye is the eye with the UNDERacting horizontal EOM
- NO such thing as overacting horizontal muscle

B. Cyclovertical Muscle Incomitance
- involve cyclovertical muscles (SO, IO, SR, IR)
- vertical angle of deviation is unequal in the different vertical and oblique gaze directions

Types based on EOM pathology:
A. PARALYTIC
- UNDERaction or paresis of the involved muscle in that direction of gaze

B. RESTRICTIVE
- OVEraction or tightness of the antagonistic muscle in that direction of gaze, preventing the eye from moving towards that gaze direction

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5
Q

How is double vision characterized?

A

MONOCULAR DIPLOPIA
- RESOLVES when AFFECTED eye occluded
- PERSISTS when UNAFFECTED eye occluded
- due to pathologies that affect transmission and focusing of light into the fovea

BINOCULAR DIPLOPIA
- RESOLVES when EITHER eye is occluded

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6
Q

What is monocular diplopia?

A
  • occurs when TWO images are seen by a SINGLE eye
  • RESOLVES when AFFECTED eye occluded
  • PERSISTS when UNAFFECTED eye occluded
  • may be unilateral or bilateral
  • may even involve triplopia or polyopia

Causes:
1. Eyelid abnormalities
2. Tear film dysfunction
3. Refractive errors and corneal malformations
4. Media opacities: cornea, aqueous, vitreous
5. Iris abnormalities
6. Lens shape and position abnormalities
7. Maculopathies: edema, CSR, membranes
8. Foreign bodies
9. Cerebral polyopia
10. Psychogenic: diagnosis of exclusion

Pathophysiology:
1. Light Diffraction
- images of a single object fall on BOTH foveal and extrafoveal area resulting in multiple images of different clarity:
+ foveal: clear and crisp
+ extrafoveal: hazy and ghost-like
- the brain’s attempt to fuse the two images result in diplopia
- resolves with PINHOLE: focuses light on the fovea and prevents light from reaching extrafoveal areas

  1. Metamorphopsia
    - distortion of retinal images
    - due to maculopathies: edema, CSR, membranes
    - does NOT resolve with pinhole
  2. Cerebral Polyopia
    - BILATERAL monocular diplopia:
    + equal image clarity
    + no overlapping of images
    - diplopia PERSISTS WHICHEVER is closed
    - perception of multiple images due to pathologies in the central visual pathway or visual cortex
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7
Q

What is binocular diplopia?

A
  • RESOLVES when EITHER eye is occluded
  • due to misalignment of visual axes of the eyes
  • images fall on exceedingly DISPARATE areas OUTSIDE of the Panum’s Area
    + fixating eye: on the fovea
    + deviated eye: on an extra-foveal area
  • usually due to involvement of the EOMs and the innervating cranial nerves
  • angle of deviation and the separation of the images is GREATEST when the object is viewed in the direction of the field-of-action of the paretic muscle
  • may be transient (MG) or constant (neurologic)

Remember:
1. Cortical fusion of two disparate retinal images can only occur if they BOTH fall in the Panum’s Area (meaning only a small degree of retinal disparity).

  1. Stereopsis
    - results from the FUSION of two slightly DISPARATE retinal images that both still fall WITHIN the Panum’s Area
  2. Diplopia
    - due to NON-fusion of two overly DISPARATE retinal images that already fall OUTSIDE the Panum’s Area
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8
Q

What are the components of the basic neuro-ophthalmologic eye exam?

A
  1. Observation: posture, behavior, appearance
  2. Visual Acuity: BCVA, PHVA
  3. Refraction: BCVA
  4. Pupillary exam
  5. Motility testing
  6. Visual field testing
  7. Ophthalmoscopy
  8. Neurologic exam
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9
Q

How is visual acuity tested?

A
  1. Test with no correction (UCVA)
  2. If abnormal, repeat testing with pinhole (PHVA) or the best available correction (BCVA)
  3. Test for both distance and near VA
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10
Q

Discuss the components of the pupillary exam.

A

Normal Pupil
- balance between the parasympathetic and sympathetic innervation
- round
- 2 - 3 mm symmetric
- equally briskly reactive to light

Components:
1. Direct light reflex:
- response of the examined pupil to light shone directly on that eye

  1. Consensual light reflex:
    - response of the contralateral pupil to light shone on one eye
  2. Swinging light test (RAPD)
    - detect a pupillary AFFERENT pathway defect
  3. Anisocoria tests
    - detect a pupillary EFFERENT pathway defect
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11
Q

What is an RAPD and how is it tested?

A

Relative Afferent Pupillary Defect/Marcus Gunn Pupil
- Defect in the Pupillary pathway on the Afferent side Relative to the other eye
- NOT observed in defects anterior to the retina and posterior to the optic tract
- requires two eyes BUT only one working pupil

Test:
Swinging Light Test
- sensitive and objective method of detecting unilateral or bilateral but asymmetric defects along the pupillary light pathway
- patient is placed in a dark room and a bright light is alternately swung between the two eyes while the patient fixates at a distance

Grading:
+1 or +2: barely detectable; initially no change but gradually dilates

+3 or +4: apparent and immediate dilation

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12
Q

What is Anisocoria and how is it diagnosed?

A

UNEQUAL pupil size

  1. Physiologic/Essential (20%)
    - size difference </= 0.5 mm
    - no changes in the size difference in both light and dark conditions
  2. Pathologic/Acquired (80%)
    - size difference > 0.5 mm
    - anisocoria decreases or increases depending on light conditions

Tests:
1. Pupillary light reflex of each pupil
2. Measure pupil size in both bright and dim conditions

Results:
1. Sympathetic lesions
- SMALLER pupil is pathologic
- EQUALLY reactive to light
- Dark: anisocoria increases
- Light: anisocoria decreases (reversal)

  1. Parasympathetic lesions
    - LARGER pupil is pathologic
    - SLUGGISH constriction in affected eye
    - Dark: anisocoria decreases (reversal)
    - Light: anisocoria increases
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13
Q

What are the levels of EOM motor control?

A

SUPRANUCLEAR
1. Frontal Eye Fields: saccades, vergence
2. Parieto-Occipito-Temporal Junction: pursuits

NUCLEAR
- location of the cranial nerve nuclei responsible for eye movements
- pre-motor coordination of conjugate eye movements
- coordination of the vestibulo-ocular system
1. PPRF: Horizontal gaze center
2. riMLF/INC: Vertical gaze center
3. Vestibular nucleus: VO reflex
4. Motor nuclei of III, IV and VI

INFRANUCLEAR
1. Oculomotor, Trochlear and Abducens nerves
2. Neuromuscular junctions
3. EOMs

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14
Q

What are the components of the motility test?

A
  1. Examine in primary gaze
  2. Examine binocular (version) movement in the horizontal, vertical and oblique gazes
    - note for deviations, under- or overshooting
    - if with noted abnormalities, may test
    monocular (duction) movements individually
  3. Check for vergence
  4. Check for both saccades (fast movements) and pursuits (slow tracking movements)
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15
Q

What are the ancillary tests used to examine strabismus patients?

A
  • done when a misalignment or deviation is observed in the motility exam
  1. Hirschberg’s/Corneal Light Reflex Test
    - qualitative assessment of eye deviations
  2. Cover and Uncover Test
    - diagnose tropias and uncover phorias
  3. Prisms Tests
    - quantify angle of deviations in a tropic or phoric eye
  4. Red Glass Test
    - diagnose diplopia
  5. Bielschowsky Three Step Test
    - isolate and localize the paretic muscle causing a cyclovertical eye deviation
  6. Forced Duction Test
    - toothed forceps or cotton-tipped applicator is used to grasp the conjunctiva and passively move the anesthetized eye towards a particular gaze direction
    - if no movement or if resistance is noted: RESTRICTIVE strabismus
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16
Q

How does the cranial nerve palsies present in terms of the EOM they innervate?

A

Symptom: Diplopia

Signs:
1. Oculomotor nerve palsy
- down-and-out eye: IO and MR
- ptosis: LPS
- mydriasis: iris sphincter

  1. Trochlear nerve palsy: SO
    - hypertropic and externally rotated with compensatory head tilt
  2. Abducens nerve palsy: LR
    - esotropic
17
Q

What is the normal monocular and binocular visual field?

A

MONOCULAR Visual Field

  • Horizontal gaze: 160 degrees total
    + temporal: 100 degrees
    + nasal: 60 degrees
  • Vertical gaze: 130 degrees total
    + superior: 60 degrees
    + inferior: 70 degrees

BINOCULAR Visual Field:

  • Horizontal gaze: 140 degrees total
    + temporal: 90 degrees
    + nasal: 50 degrees
  • Vertical gaze: 110 degrees total
    + superior: 50 degrees
    + inferior: 60 degrees
  • Peripheral vision: 10 degrees per direction
18
Q

What are the different tests used to assess the visual field?

A

A. Confrontation Test
- subjective but reliable if done properly

Targets:
- finger counting
- hand movement
- light source or bright objects

Steps:
1. Position patient 2 - 3 feet away.
2. One eye is covered while the other maintains fixation on the examiner.
3. Each quadrant of the visual field is tested by asking the patient to count the number of fingers held up or to confirm if a test target appears within the field of vision.
4. Repeat above steps for the other eye.

B. Amsler Grid
- screening test to assess the central visual field

Steps:
1. A chart consisting of a grid with a dot at the center is placed 1 foot away from the patient.
2. The patient is asked to fixate on the dot at the center and describe what is seen.

Results:
1. Metamorphopsia: distortion of the grid
- macular diseases (membranes, CSR, edema)

  1. Central scotoma: central grid and dot not visualized
    - optic nerve disease

C. Perimetry
- objective test used to document, confirm and quantify visual field defects
- also measures sensitivity to stimuli at multiple locations in the visual field

Standard Automated Perimetry/ White-on-White Perimetry
- most common form of visual field testing
- a white stimulus is projected randomly on a white background to determine threshold values
- removes bias by perimetrist
- able to detect early visual field defects
- results are reproducible and can be reviewed

Types based on presentation of stimulus:
1. Kinetic: Goldmann
- stimulus is MOVING from a non-seeing area to a seeing area
- location where object is first seen is recorded

  1. Static: Humphrey, Octopus
    - STATIONARY stimuli are presented at defined points in the visual field
    - stimuli presented for longer durations of time may be seen better as a result of temporal summation of information

Note:
Humphrey 24-2
- extended partially to beyond 30 degrees nasally to detect NASAL STEP scotoma

19
Q

Review of the components of the visual pathway.

A
  1. Retina
  2. Optic Nerve
  3. Optic Chiasm
  4. Optic Tract
  5. Lateral Geniculate Nucleus
  6. Optic Radiations (Temporal and Parietal)
  7. Primary Visual (Striate) Cortex
20
Q

What is a scotoma?

A
  • area of depressed or absent vision surrounded on all sides by relatively better vision
  • depression or absence of vision anywhere EXCEPT the anatomic blindspot is abnormal
21
Q

What are the different patterns of visual field defects?

A

A. Papillomacular Bundle
1. Central: fixation point
2. Cecocentral: fixation point + blindspot
3. Paracentral: adjacent to fixation point

B. Nasal Bundle
1. Wedge: temporal wedge scotoma

C. Arcuate Bundle
1. Seidel: comma-shaped extension of blindspot
2. Arcuate/Bjerrum/Scimitar: extension of Seidel ending at the nasal field
3. Nasal Step of Roenne: localized defect in the nasal field
4. Altitudinal: involves entire horizontal half

D. Hemianopia: loss of a half of the visual field
1. Homonymous: same-sided (one temporal, one nasal)
2. Bitemporal: both temporal fields

E. Quadrantanopia
- loss of a quarter of the visual field

22
Q

What are common visual field defects seen in lesions involving the visual pathway?

A

Optic Nerve: monocular
1. Papillomacular scotomas
2. Nasal scotomas
3. Arcuate scotomas

Optic Chiasm:
- Bitemporal hemianopsia

Optic Tract:
- CONTRALATERAL homonymous hemianopsia

Optic Radiation:
1. Parietal: Pie-on-the-Floor
- CONTRALATERAL Inferior homonymous quadrantanopia

  1. Temporal: Pie-in-the-Sky
    - CONTRALATERAL Superior homonymous quadrantanopia

Occipital Lobe:
- CONTRALATERAL Macular-sparing defect

  1. Anterior occipital lobe
    - monocular contralateral temporal crescent defect
  2. Posterior occipital lobe
    - homonymous hemianopsia WITHOUT the central homonymous hemifields
  3. Occipital tip
    - central homonymous hemianopsia
23
Q

What are the normal ophthalmoscopy findings?

A

Media: clear with good ROR
Optic disc: pink, oval with distinct borders
Cup-Disc ratio: 0.3 - 0.5
A-V ratio: 2:3 - 3:4
No hemorrhages or exudates
Note of spontaneous venous pulsations

Note:
1. ROR: due to rich choroidal vasculature
2. Optic Cup: depression at the center of the disc devoid of axons
3. Disc borders: becomes indistinct due to swelling of the axons and edema from damage to the optic nerve

24
Q

What is Papilledema?

A
  • bilateral optic disc swelling secondary to increased ICP
  • optic nerve is an extension of the brain and is surrounded by meninges and CSF
  • any increase in ICP is transmitted to the meningeal subarachnoid space surrounding the optic nerve
  • elevated pressure causes hypoxia and axonal injury manifesting as disc edema
  • severity is proportional to the ICP level

Causes: LIFE-THREATENING
1. Brain tumors
2. Subarachnoid hemorrhage
3. Meningitis
4. Hydrocephalus
5. Head trauma

Findings:
1. Bilateral
2. Swollen, hyperemic disc w/ indistinct borders
3. Absence of optic cup
4. Venous distention with NO pulsations
5. Hemorrhages, exudates, cotton-wool spots
6. NFL infarcts

Note: Optic disc EDEMA
- optic disc swelling from other causes

25
Q

What are the components of the general neurologic exam?

A
  1. Mental status
  2. Cranial nerves (others)
  3. Motor system
  4. Sensory system
  5. Reflexes
  6. Cerebellum (posture, gait, coordination)
26
Q

What are the other ancillary tests used to diagnose neuro-ophthalmologic conditions?

A

Color Tests

  1. Ishihara
    - screening test for red-green color deficiencies
  2. Farnsworth-Munsell 100 Hue Test
    - more detailed and accurate quantification of color vision accuracy

Comparison Tests
- best used for monocular disorders because comparison with a normal eye is necessary

  1. Red Color Comparison Test
    - detects early ON damage which presents initially as impaired red-color discrimination
    - the patient is asked to assess differences between two identical red objects
    - affected eye would see a faded red
  2. Light Brightness Comparison Test
    - more specific for optic nerve diseases by assessing the afferent pathway
    - light is shone on both eyes and any difference in perceived brightness is noted
  3. Photo Stress Comparison Test
    - bright light is shone on the eye for 10 seconds to saturate the photoreceptors
    - light is removed and the patient is asked to read one line above the BCVA

Results:
1. Normal (< 60 seconds): ON lesions
2. Equivocal (60 - 90 seconds): either
3. Prolonged (> 90 seconds): RETINAL lesions

27
Q

Differentiate true optic disc edema/papilledema from pseudopapilledema based on fluorescein angiography

A

True Edema
- (+) dye leakage from damaged vessels
- (+) tissue staining: persists for hours

Pseudopapilledema
- (-) dye leakage
- (-) tissue staining

28
Q

What is visual electrophysiology?

A
  • objective and non-invasive technique for evaluation of visual function by measuring the electrical signals coursing through the visual pathway
  1. Electro-Oculogram
    - assesses function of the RPE
  2. Electro-Retinogram
    - assesses function of the neurosensory retina (PRL –> GCL)
  3. Visual Evoked Potentials
    - assess function of the proximal visual pathway
    (NFL –> visual cortex)