EyeSpyOphthalmic Education

Case 03 - Neuro-ophthalmology, orbit, and inflammation

Painless Progressive Visual Loss

42-year-old African-American woman - Painless monocular vision loss progressing over months

Illustrated eye for pathology case review
Case 03Optic Neuropathy / Sarcoidosis

Clinical Presentation

A 42-year-old African-American woman complains of painless loss of vision in one eye which has progressed during the past several months. The optic nerve in the affected eye is illustrated. The opposite eye is normal.

Learner Questions

  1. Describe and explain the pupillary response expected. How would it differ in the two eyes?
  2. Describe the appearance of the optic disc and its significance.
  3. In broad diagnostic categories, what is the differential diagnosis?

Answer Framework

Pupillary response

RAPD (Marcus Gunn pupil) in the affected eye. Swinging flashlight test: both pupils paradoxical the fellow eye. RAPD = asymmetric optic nerve or extensive retinal lesion.

Optic disc

Pale, swollen disc with edema and pallor - infiltrative or compressive optic neuropathy. In thi the leading diagnosis.

DDx

Inflammatory (sarcoidosis #1 in this demographic, MS, syphilis, TB, Lyme) - Compressive (orbital mass, meningioma, pituitary) - Infiltrative (lymphoma, sarcoid) - Ischemic (NAION) - Glaucomatous (NTG) - Hereditary (Leber's LHON). Work-up: ACE, CXR/CT, RPR, PPD, biopsy.

Teaching Pearl

Sarcoidosis predilects young African-American women. Ocular involvement in ~25-50% of cases; optic nerve granulomas are vision-threatening. Work-up: ACE, serum lysozyme, chest CT (hilar adenopathy), RPR (always rule out syphilis). Treatment: systemic corticosteroids.

Original answer transcript 
Pupillary response
RAPD (Marcus Gunn pupil) in the affected eye. Swinging flashlight test: both pupils paradoxically dilate when light swings to the affected eye (weaker afferent signal). Normal direct/consensual in
the fellow eye. RAPD = asymmetric optic nerve or extensive retinal lesion.
Optic disc
Pale, swollen disc with edema and pallor - infiltrative or compressive optic neuropathy. In this patient (African-American woman, painless, gradual): sarcoid granuloma infiltrating the optic nerve is
the leading diagnosis.
DDx
Inflammatory (sarcoidosis #1 in this demographic, MS, syphilis, TB, Lyme) - Compressive (orbital mass, meningioma, pituitary) - Infiltrative (lymphoma, sarcoid) - Ischemic (NAION) - Glaucomatous
(NTG) - Hereditary (Leber's LHON). Work-up: ACE, CXR/CT, RPR, PPD, biopsy.
TEACHING PEARL
Sarcoidosis predilects young African-American women. Ocular involvement in ~25-50% of cases; optic nerve granulomas are vision-threatening. Work-up: ACE, serum
lysozyme, chest CT (hilar adenopathy), RPR (always rule out syphilis). Treatment: systemic corticosteroids.