Chapter 15. Abnormalities of the Optic Nerve and Retina

Question 1

Question 15-1:
Which of the following statements are true regarding unilateral optic disc edema?
A. Optic nerve function is usually abnormal
B. Appearance overlaps between different causes
C. Can be caused by optic neuritis
D. Most patients wilh acute demyelinating optic neuritis do not have optic disc edema
E. All are true

Answer 1

Answer 15-1: E.
All are true. Optic nerve function· is abnormal
in most patient with unilateral optic disc
edema. Common causes are optic neuritis,
anterior ischemic optic neuropathy, and orbital
compressive lesions. Differentiation between
these causes is often impossible on visual
examination, alone, though the history is
commonly different. About one third of
. patients with acute demyelinating optic
neuritis have optic disc edema

Question 2

Question 15-2:
Which of the following statements is true
regarding the Foster-Kennedy syndrome?
A. Lesion is usually retrobulhar neuritis from multiple sclerosis
B. Patients have unilateral optic nerve edema with the opposite eye unaffected
C. Patients have anterior ischemic optic neuropathy
D. Patients have unilateral optic atrophy with contralateral papilledema

Answer 2

Answer 15-2: D.
Foster-Kennedy syndrome is most commonly
due to a tumor which causes optic atrophy in
one eye and papilledema in the opposite eye.
The optic atrophy is due to nerve damage,
where the contralateral papilledema is due to
increased intracranial pressure. Multiple
sclerosis and AION can produce a syndrome
which can resemble Foster-Kennedy
syndrome

Question 3

Question 15-3: 
A 36-year-old man presents with severe confusion, papilledema, ahd severe hypertension. There are no  focal  findings on examination. What diagnoses should be 
considered? 
A.  Optic neuritis 
B.  Diabetic papillopathy 
C.  Malignant hypertension 
D.  Glaucoma

Answer 3

Answer 15-3: C.
Malignant hypertension can produce
papilledema and confusion, though oot all
patients will have neurologic deficits. Patients
with reoal failure will develop disc edema at a
lower level of blood pressure than patients
with normal renal function.

Question 4

Question 15-4: 
A 30-year-old female presents with encephalopathy and is found on examination to have mUltiple branch retinal artery occlusions and sensorineural hearing loss. CSF shows a mild lymphocytic pleocytosis. MRl shows multiple regions of  increased signal on T2-weighted images. Angiogrnphy is normal. Which is the most likely diagnosis? 
A.  Susac's syndrome 
B.  Multiple sclerosis 
C.  HSVencephalitis 
D.  Vasculitis

Answer 4

Answer 15-4: A.
Susac’s syndrome is a disorder of unknown
cause, with the clinical features of confusion,
sensorineural hearing loss and branch retinal
artery occlusions. CSF shows a mild
lymphocytic pleocytosis. MRI shows multiple
areas of increased intensity TI-weighted
images. While these may radiographically
resemble demyelinating diseases, the retinal
abnormalities argue against this. ANA and
angiography are normal, helping to
differentiate this from vasculitis

Question 5

Question 15-5: 
Which retinal findings would be expected in von Hippel-Lindau disease? 
A.  Astrocytic hamartomas 
B.  Angiomas 
C.  Arteriovenous malformations 
D.  Pigmentary degeneration

Answer 5

Answer 15-5: B.
Retinal angiomas are characteristic of von
Hippel-Lindau disease. Astrocytic
hamartomas are characteristic of tuberous
sclerosis. A VMs are seen in Wybum-Mason
disease. Pigmentary degeneration has a
multitude of causes including but not limited
to Kearns-Sayre syndrome, Refsum’s disease,
Batten’S disease, and Marie’s ataxia

Question 6

Question 15-6: 
Which retinal findings would be expected in tuberous sclerosis? 
A.  Astrocytic hamartomas 
B.  Angiomas 
C.  Arteriovenous malformations 
D.  Pigmentary degeneration

Answer 6

Answer 15-6: A.
Astrocytic hamartomas are seen in the retinae
of patients with tuberous sclerosis. As
described for the previous question, angiomas
are seen in von Hippel-Lindau disease, A VMs
are seen in Wybum-Mason disease, and
pigmentary degeneration is seen in a multitude
of disorders

Question 7

Question 15-7:
Which of the followingis not true regarding retinal vein occlusion ?
A. Most Pastients with retinal vein occlusion are older than 50
B. Disc edema is common with retinal vein occlusion
C. Retinal vein occlusion is usually due to carotid vascular disease
D. Patients with retinaI vein occlusion should be screened for vascular risk factors

Answer 7

Answer 15-7: C.
Carotid vascular disease does not cause retinal
vein occlusion, mther carotid disease causes
retinal arterial ischemia. Therefore, carotid
imaging is seldom needed for patients with
retinal vein occlusion. Disc edema is common
and may be the predominant ophthalmologic
feature. Patients with retinal vein occlusion
typically have vascular risk factors and are
older than 50 years

Question 8

Question 15-8: 
Which of the following disorders does not cause optic atrophy? 
A.  Optic nerve comppression  by tumor 
B.  Optic neuritis
c.  Chiasmal compression by  tumor 
D.  Infarction of  the optic radiations

Answer 8

Answer 15-8: D.
Infarction of the optic radiations from the
geniculate to the occipital cortex would
produce visual loss but would not produce
optic atrophy. All of the other conditions
listed affect the optic nerve axons projecting
to the lateral geniculate. lUly cause of optic
nerve damage can cause optic atrophy,
including tumors, demyelinating disease, and
even inherited conditioDS. However, the
pattern of visual loss and optic nerve
appearance differs with the differing causes

Question 9

Question 15-9:
Which of the following statements are true of Leber’s optic neuropathy?
I. Occurs mainly in males
2. Optic disk may appear swollen
3. Telangiectatic” vessels are seen in the peripapillary nerve fiber layer
4. Fluoroscein angiography shows leakage from the disk
Select: A = 1.2.3. B = 1.3. C =2. 4. D =4 only. E = All

Answer 9

Answer 15-9: A.
Leber’s optic neuropathy produces mild
swelling of the optic disc in the acute phase.
However, fluorescein angiography does not
show leakage from the disk, differentiating
this condition from true disk edema. Leber’s
optic neuropathy is predominantly a disorder
seen in males. The telangiectatic vessels are
characteristic and are a clue to the diagnosis.

Question 10

Question 15-10:
Which of the following conditions are associated with uveoretinal meningoencephalitis?
I. Syphilis
2. Vogt-Koyanagi-Harada syndrome
3. Beheet’s disease
4. Sarcoidosis
Select A = 1.2.3. B = I. 3. C = 2. 4. D = 4 only. E = All

Answer 10

Answer 15-10: E.
All of these conditions can produce inflammatory
changes in the eye and CNS. Vogt-KoyanagiHarada
syndrome is the most common of these, and
is characterized by exudative retinal detachments