A lecture given to residents of the Kuwait Neurolgy Board

1. The Approach to A
Patient With Decreased
Vision
Raed Behbehani , MD

2. Initial Evaluation-History
• Unilateral vs Bilateral
• Onset (acute , sub-acute, chronic)
• Course (constant, progressive,improvement)
• Associated Symptoms

3. Evaluation-Color Vision
• Optic nerve vs Macular Disease
• Psuedoisochromatic color plates (congenital color
deficiency and acquired optic nerve diseases)
• Other (HRR plates-blue and Yellow , Fansworth-
Munsell D15 Panel)

4. Evaluation-Pupil
• RAPD is the hall mark of optic neuropathy.
• Swinging flash-light test.
• Reverse RAPD (wrong name) if one pupil is dilated
or does not constrict for mechanical causes.

5. RAPD

6. Fundus Evaluation

7. Visual Field Testing

8. Ancillary Tests
• Photo-stress Recovery Tests
• IVFA
• OCT
• ERG (Panfiled , Multifocal)

9. Classification
• Macular Media
• Retinopathy
• Optic Neuropathy
• Chiasmopathy
• Visual Pathway
• Occipital Cortex

10. MEWDS- AIBSE
• Photopsias
• Temporal Scotoma
• Visual Field-enlarged blind spot
• Mild disc edema, small deep white retinal spots
• Early wrath-like hyperflourescence
• ERG : MFERG (depressed peripapilary regsion) , Full-Field
ERG (depressed a-wave).
• OCT : attenuation of the outer retinal layers

11. MEWDS/AIBSE

12. Vitamin A Deficiency
• Bariatric Surgery / Mal-absorption syndromes
• Look for other deficiencies (zinc , copper , Vitamin
B1,B6,B12).

13. Other Retinopathies
• Cone Dystrophy
• Para-neoplastic Retinopathy

14. Optic Neuropathy

15. Non-Arteritic Anterior Ischemic
Optic Neuropathy (NAION)

16. Non-Arteritic Anterior Ischemic
Optic Nuropathy (NAION)
• Age> 40.
• Unilateral visual acuity/field loss.
• Disc edema ( initially pallid ) , can be sectoral or diffuse.
• Small cup/disc ratio (anamolous disc).
• Vascular risk factors (diabetes,hypertension, smoking,
hypercholesterolemia).
• Usually remains static but can improve in 42.7 % or
progress over several weeks in 25 %.

17. NAION
Hemorrhage
Anamolous
disc

18. NAION
Narrowed arterioles
Segmental pallor

19. Arteritic anterior ischemic
optic neuropathy
• Age >70
• GCA symptoms
• Vision loss is often more severe
• Chalky-white swelling of the disc
• Retinal infarcts/cotton wools spots
• Delayed filling IVFA
• Other disc has normal C/D ratio

23. Posterior Ischemic Optic
Neuropathy
• After severe blood loss, intra-operative hypotension, renal
dialysis, severe anemia, spinal procedures, and coronary
bypass surgery.
• Disc are usually normal.
• Pupillary light reflexes assessment.

24. PION

25. Retinal Artery Occlusion

27. Optic neuritis
• Young, female
• Pain ( dull-aching , periocular headache , worse with
EOM)
• Visual acuity can be normal.
• RAPD
• Visual field defect

28. OCT in ON
Acute RNFL thickening
Late RNFL loss

29. GCL loss in ON

30. MRI brain

31. • A 12 year old with history of sudden, sequential, bilateral
vision loss x 4 days.
• First right eye and 1 day later the left eye.
• No photophobia, or redness.
• No fever, headache or neck pain.
• Otherwise healthy.
• No allergies.
Case

32. Case
• Was admitted to Amiri Hospital .
• CT and MRI brain was normal.
• Received IV steroids x 2 days with mild improvement in
vision.
• CBC , ESR Normal.
• Ophthalmology consult.

33. Case
• VA : HM , CF 2 meters.
• Color vision : 0/13 OU.
• Pupils : sluggish OU , Right RAPD
• Motility : Full, orthophoric.
• Orbits : Normal RTR.
• TA : 12 mm/Hg OU.
• SLE : Normal anterior segment , no cells or falre.

35. Neuro-retinitis
• Cat scratch (Bartonella henselae).
• TB
• Syphilis
• Sarcoidosis
• EBV / CMV / HSV / HZV / Mumps
• Lyme
• Toxoplasmosis

36. Neuro-retinitis
http://medstat.med.utah.edu/NOVEL

37. Infiltrative Optic Neuropathy

38. Radiation Optic Neuropathy
 Follows XRT to the head and neck.
 Interval 12-36 months.
 Endothelial cell injury ( ischemia ).
 Associated radiation retinopathy.
 MRI will show optic nerve enhancement with
gadolinium.
 Rule out recurrent tumor !
 ? Hyperbaric oxygen, antiplatelets, and
steroids.

39. RON
NOVEL online library

40. RON

41. Leber’s Hereditary Optic Neuropathy
 Unilateral painless vision loss.
 Usually acute, but some are chronic.
 Sequential bilateral involvement in weeks or
months.
 6-80 year old.
 Central or cecocentral visual field defect.
 disk swelling, thickening of the peripapillary retinal
nerve fiber layer and peripapillary retinal
telangectatic vessels.

42. LHON
Acute LHON
End stage LHON

43. LHON
 4 primary mitochondrial genome mutations;
11778, 3460, 14485, 14459.
 males > females ratio = 2.5:1.
 Spontaneous recovery of vision can occur.
 Cardiac conduction defects.

44. Compressive Optic Neuropathy
 Thyroid eye disease, meningioma, pituitary
tumors, glioma, aneurysms, Hydrocephalus,
and carniopharyngiomas.
 Disc can be pale or normal.
 Visual fields defects help localization.
 Neuro-imaging is essential.

45. TED

46. Compressive Optic Neuropathy
• 5%-7% of TED
• Direct compression of the optic nerve at the orbital apex
• Dyschromatopsia , RAPD ( absent if bilateral)
• Disc edema in 40%
• Visual fields
• Often in the active phase of the disease
• Proptosis may be minimal (tight lids)

47. Thyroid CON

48. TED
 TED compressive ON: steroids, radiation and
orbital decompression.

49. Optic Nerve Sheath Meningioma

50. ONSM
NOVEL online library

51. Aneurysm

52. Aneurysm

53. Orbital Apex Lymphoma

54. Hereditary Optic Neuropathy
 Dominanat (Kjer’s).
 Recessive.
 X-linked.
 Mitochondrial (Leber’s)

55. Dominant OA
 Visual acuity 20/20 – CF.
 Blue yellow color vision blindness.
 Central visual field defects.
 Optic disc shows excavation.
 Examine family members.
 OPA1 gene mutation.

56. Dominant Optic Atrophy
NOVEL online library

57. Dominant OA
NOVEL online library

58. Optic atrophy with other
neurological manifestations
 Optic atrophy with spinocerebellar ataxias.
 Wolfram syndrome (DIDMOAD).
 Fredreich's ataxia.

59. Toxic-nutritional Optic Neuropathy
 Tobacco-alcohol amblyopia in alcohol
abusers, smokers, and in nutritional
deficiency.
 Vitamin deficiency : thiamine (B1), riboflavin
(B2), Folate , B 12 and B6.
 Stop inciting agents and give thiamine.
 Recovery is usually slow.

60. Toxic ON

61. Swollen ON in TON
A 35 year old woman underwent a gastric by-pass for weight loss.
Nausea and vomiting, had significant weight loss.
She had no alcohol consumption.
She presented with extra-ocular muscle abnormalities, ptosis, confusion,
ataxia, and responded to IV Thiamine

62. Methanol Optic Neuropathy

63. Drug-Induced Optic Neuropathy
 Ethambutol.
 Amiodarone.
 Chloramphenicol.
 Cyclosporine.
 Methotrexate.

64. Chiasmal Disorders
• Visual fields respecting vertical meridien
• Most common visual field loss - Bitemporal
Hemianopsia
• Junctional scotoma - lesion at junction of the optic
nerve and chiasm

71. Chiasmal Syndrome

72. Junctional Scotoma

73. Band Atrophy

74. Extrinsic Causes of
Chiasmal Syndrome
• Pituitary adenoma - Apoplexy
• Craniopharyngiona
• Parasellar meningiom
• ICA aneurysm
• Dilated 3d ventricle due obstruction

75. Delayed Vision Loss Post-
Treatment
•Tumor recurrence
•Delayed radionecrosis of the chiasm or optic
nerves
•Chiasmal distortion due to adhesions or
secondary empty sella syndrome, with descent
and traction on the chiasm
•Chiasmal compression from expansion of
intraoperative overpacking of the sella with fat

76. Intrinsic Causes of Chiasmal
Disorders
• Infection (TB)
• Inflammation (Sarcoid , NMO, IgG4RD)
• Neoplasm (Glioma)
• Metastatic
• Traumatic

77. Neuro-sarcoidosis

79. Retrochiasmal Lesions
• Homonymous Hemianopsia
• Congruity
• Contr-alteral RAPD

80. Optic Tract Lesions
•Mass lesions (aneurysms)
•Inflammation, demyelination
•Ischemic lesions (rare)

81. Bow-tie Atrophy

82. Lateral Geniculate Lesions
•Congruous horizontal sectoranopia
•Posterolateral choroidal artery, a branch of the
PCA

83. Lateral Geniculate Lesions

84. Temporal Lobe Lesions

85. Pie in the Sky

86. Parietal Lobe Lesions
• “Pie on the floor” homonynous defect.
• Associated neurologic signs and symptoms (e.g.,
hemiplegia, hemisensory loss, visual, or neglect) may be
present.
• Gertsman’s syndrome-dominant lobe (acalculia, agraphia,
finger agnosia, and left right confusion)
• Lesions in the nondominant parietal lobe can produce
contralateral neglect.

87. Occipital Lobe Lesions

88. Occipital Lobe Lesions
• Congruous homonymous hemianopia, possibly sparing the
fixational region .
• A monocular defect of the temporal crescent involving only the
most anterior portion of the occipital
• lobe
• Homonymous lesion sparing the temporal crescent in the eye
contralateral to the lesion
• Homonymous hemianopia that respects both the vertical and
horizontal meridians

89. Occipital Lobe Lesions
• Isolated (Stroke)
• Cortical blindness (Bilateral occipital lobe destruction)
• Stroke, severe blood loss, Eclampsia, hypertension, angiography, CO
poisoning, cyclosporine.

90. Higher Cortical Visual
Dysfunction

91. “What" Pathway
• Object recognition , color, shape, and pattern.
• Continuation of the parvocellular pathway.
• V1 V2->V4-> inferotemporal cortex-> angular gyrus->
limbic structures.
• Alexeia, anomia, agnosia, amenesia.

92. “Where” Pathway
• Dorsal stream (occipitoparietal): Spatial orientation ,visual
guidance of movement.
• V1 V3-> V5->Parietal and superotemporal cortex.
• Continuation of magnocellular pathway.
• Simultagnosia, optic ataxia, acquired oculomotor apraxia,
and hemispatial neglect.

93. Cortical Blindness
• Due to bilateral occipital lobe lesions.
• Often misdiagnosed as functional vision loss.
• Stroke, severe blood loss, Eclampsia,
hypertension, angiography, CO poisoning,
cyclosporine.

94. Alexia without Agraphia
• Loss of ability to
read but can write.
• Left occipital lobe
and splenium of
corpus callosum.