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Question 1 of 64

A 48-year-old white man presents to his optometrist with a 1-month history of blurred vision
in his left eye. Visual acuity is 6/12 in the affected eye (6/6 in the other eye). Fundal
examination reveals some mottled pigmentary changes at the left macula, associated with
some macular oedema. On closer inspection, both fundi revealed reddish-brown bands deep
to the retina radiating in a spoke-like pattern from the optic disc.
The optician referred the patient to his GP, who noted loose skin folds with yellow striations
and puckering in the neck and flexor aspects of the joints.
What is the most likely diagnosis?
A

Ehlers-Danlos syndrome

B

Paget’s disease

C

Marfan’s syndrome

D

Sickle cell disease

E

Pseudoxanthoma elasticum

Explanation
Angioid streaks Pseudoxanthoma elasticum The fundal examination of this patient reveals the classic signs of angioid streaks, ie orangebrown bands radiating away from the optic disc. The patient has presented to his optometrist
with macular changes suggestive of macular degeneration. This is a known complication of
angioid streaks and occurs secondary to choroidal neovascularisation. Although angioid
streaks are idiopathic in up to 50% of patients the remaining 50% have associations with
systemic disease. The skin changes present on this patient’s neck and flexor surfaces are
typical of those seen with pseudoxanthoma elasticum (the classic plucked chicken
appearance).
Other causes of angioid streaks -

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Angioid streaks can be seen with Ehlers–Danlos syndrome, which is associated with
hyperelasticity of the skin and loose joints. Marfan syndrome is also associated with angioid
streaks but is not associated with the skin changes described. These lesions can also occur in
patients with Paget’s disease, unlikely in a 48-year old man. In patients of Afro-Caribbean
descent, they can also be seen in sickle cell disease.
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Question 2 of 64

Which one of the following is a cause of second-order-neurone Horner syndrome?
A

Cavernous sinus thrombosis

B

Craniopharyngioma

C

Internal carotid artery aneurysm

D

Pancoast’s tumour

E

Syringomyelia

Explanation



The answer is Pancoast’s tumour –
Horner syndrome is also known as oculo-sympathetic paresis and is characterised by
miosis, ptosis and anhydrosis. Lesions along the sympathetic chain along the head and
neck region can cause these clinical appearances. The causes of Horner syndrome can
vary with age, and can be elicited by thinking about lesions that could affect its path.
The first-order (central) neurone descends from the hypothalamus to the cervical spine.
The second-order (preganglionic) neuroneexits the spinal cord and heads towards the
head and neck. It travels along its path with the cervical sympathetic chain, the brachial
plexus, lung apices (Pancoasttumour) to synapse at the superior cervical ganglion. This is
located near the angle of the mandible and is in close proximity to the common carotid
artery.
The third-order (postganglionic) neurone begins at the superior cervical ganglion and
travels along the wall of the internal carotid artery into the cavernous sinus. Lesions
along its path affecting the internal carotid arteryor within the cranium can affect its
function.

Cavernous sinus thrombosis (Option A) is incorrect. The sympathetic chain does not pass
along the cavernous sinus.

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Craniopharyngioma (Option B) is incorrect. The first-order (central) neurone descends from
the hypothalamus to the cervical spine (ciliospinal centre of Budge), and can be affected by
craniopharyngioma.
Internal carotid artery aneurysm (Option C) is incorrect. Lesions of the third-order neurone
affect the internal carotid artery.
Syringomyelia (Option E) is incorrect. The first-order (central) neurone descends from the
hypothalamus to the cervical spine (ciliospinal centre of Budge), and can be affected by
syringomelia.
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Question 3 of 64

A patient with controlled ocular myasthenia gravis develops an acute infection.
Which one of the following antibiotics is contraindicated?
A

Aminoglycosides

B

Macrolides

C

Metronidazole

D

Penicillins

E

Quinolones

Explanation



The answer is Aminoglycosides –
Aminoglycosides are contraindicated in patients with myasthenia gravis as they are
known to interfere with and impair neuromuscular transmission.

Macrolides (Option B) is incorrect. Macrolides have been associated with anecdotal reports
of increased myasthenic weakness, although the mechanism is less clear, with rare published
incidence.
Metronidazole (Option C) is incorrect. Metronidazole is very rarely associated with the
disease.
Penicillins (Option D) is incorrect. Penicillins have been associated with anecdotal reports of
increased myasthenic weakness, although the mechanism is less clear, with rare published
incidence.
Quinolones (Option E) is incorrect. Quinolones have been associated with anecdotal reports
of increased myasthenic weakness, although the mechanism is less clear, with rare published
incidence.
45524

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Question 4 of 64

A patient presents with a first episode of a painful reduction in visual acuity in one eye. The
acuity is 6/60, ocular movements produce pain and the optic disc is normal.
Which one of the following investigations supports the diagnosis of optic neuritis?
A

Abnormal visual evoked potentials (VEP)

B

Abnormal electroretinogram (ERG)

C

Abnormal electro-oculogram (EOG)

D

Abnormal electroencephalogram (EEG)

E

Abnormal electronystagmogram (ENG)

Explanation



The answer is Abnormal visual evoked potentials In optic neuritis, demyelination causes a reduction in the velocity of the propagation of
action potentials and so the VEP response is delayed. The ERG is normal as the retina is
unaffected.

Abnormal electroretinogram (Option B) is incorrect. The ERG measures retinal electrical
activity and is often used to validate the significance of an abnormal VEP response (ie if the
VEP is abnormal but so is the ERG, then the VEP abnormality might be secondary to an eye
condition and be a false-positive for optic nerve disease).
Abnormal electro-oculogram (Option C) is incorrect. The EOG measures the overall electrical
activity of the eye and is reduced in degenerative eye conditions affecting the pigment
epithelial layer, such as Best’s disease.
Abnormal electroencephalogram (Option D) is incorrect. An EEG is not an optimal
investigation for assessing vision impairment.
Abnormal electronystagmogram (Option E) is incorrect. The ENG is simply a method of
documenting nystagmus using the electrical charge of the eye.
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Question 5 of 64

A diabetic 46-year-old man is found in the Diabetic Clinic to have reduced visual acuity.
During a telephone referral to the Eye Clinic, the ophthalmologist asks if the patient has any
risk factors for macular oedema.
Which one of the following should the referring physician bring to his attention?
A

Background diabetic retinopathy

B

Low glycosylated haemoglobin

C

Hypercholesterolaemia

D

Proteinuria

E

Peripheral vascular disease

Explanation
Diabetic macular oedema
The Wisconsin Epidemiological Study showed that the incidence of macular oedema was 2–
6% in background diabetic retinopathy (DR), 20–63% in preproliferative DR and 70–74% in
proliferative DR. The prevalence increased with greater duration of diabetes, higher
glycosylated haemoglobin and greater proteinuria levels.
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Question 6 of 64

A 71-year-old man presents with deteriorating vision. He complains of blurred central vision
when looking through both eyes, although the left eye is slightly worse than the right.
Fluorescein angiography shows choroidal neovascularisation with leakage at the macula in
both eyes.
When assessing possible risk factors for his condition, which one of the following is the most
important?
A

Smoking

B

Alcohol

C

Hypertension

D

Diabetes

E

Cataract surgery

Explanation
Macular degeneration
Pathogenesis
Smoking, hypertension and previous cataract surgery have all been implicated in the
pathogenesis of age-related macular degeneration (ARMD), but smoking is regarded as the
most important modifiable risk factor. Important non-modifiable risk factors include white
European ethnicity and a positive family history. Recent studies have pointed to genetic
differences in the complement pathway having an important role to play in the pathogenesis
of the condition.
Treatment
Anti-vascular endothelial growth factor (anti-VEGF) therapies have recently been introduced
for the treatment of exudative (wet) ARMD, although the National Institute for Health and
Care Excellence (NICE) appraisal process has allowed only limited use of such agents for the
time being, on the basis of cost-effectiveness.
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Question 7 of 64

During a routine insurance medical examination, a GP notices that a 30-year-old female
patient has absent ankle jerks and unequal pupils.
Which one of the following is the most likely diagnosis?
A

Holmes–Adie syndrome

B

Horner syndrome

C

IIIrd nerve palsy

D

Mescaline ingestion

E

Argyll Robertson pupils

Explanation



The answer is Holmes-Adie syndrome –
• Adie’s pupil can be attributed to a postviral degeneration in the ciliary ganglion
• The pupil is initially dilated, with a tonic reaction to light and sectorial vermiform
movements • The pupil is hypersensitive to 0.125% pilocarpine. A normal pupil will be
unaffected by this weak dilution of pilocarpine, whereas an Adie pupil will constrict due
to denervation hypersensitivity
• The near vision is impaired by accommodative spasm, but this recovers in a few months
• The pupil sphincter later becomes fibrosed and the pupil miosed (‘little old Adies’)
• Holmes–Adie syndrome is the combination of Adie’s pupil with hyporeflexia and
typically occurs in women in this age group.

Horner syndrome (Option B) is incorrect. Horner syndrome is another cause of anisocoria
(unequal pupils), but the affected pupil is smaller. Horner syndrome is not typically
associated with abnormal deep tendon reflexes.
IIIrd Nerve palsy (Option C) is incorrect. Third nerve palsies do cause anisocoria and an
abnormally large pupil, but are mainly characterised by reduced ocular motility and an
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associated ptosis.

Mescaline ingestion (Option D) is incorrect. Recreational drug use (eg mescaline) can cause
abnormalities in pupil size, but these are bilateral and unlikely to cause anisocoria.
Argyll Robertson pupil (Option E) is incorrect. Argyll Robertson pupils are bilateral small
pupils which constrict to accommodation, ie there is light-near dissociation (the pupils
accommodate but do not react).
41818

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Question 8 of 64

A 40-old woman with AIDS presents to the Genitourinary Clinic with a shadow in her vision in
one eye.
Which one of the following supports a diagnosis of cytomegalovirus retinitis?
A

Conjunctival injection

B

Mydriasis on the affected side

C

Disc swelling

D

Macular oedema

E

Retinal haemorrhages

Explanation
Cytomegalovirus retinitis
Cytomegalovirus (CMV) retinitis causes haemorrhages at the edge of areas of retinal
necrosis. The lesions are usually along the vascular arcades and involve the macula late in the
disease. The prognosis for life expectancy was very poor before the advent of HAART (highly
active antiretroviral therapy) and CMV retinitis was often a harbinger of death in these
patients. Nowadays patients can survive for many years with CMV retinitis, which can
periodically flare up and require treatment, usually with ganciclovir or foscarnet.
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Question 9 of 64

A 49-year-old woman with a history of systemic lupus erythematosus (SLE) presents with a
sudden decrease in vision in her left eye. On examination her blood pressure is 155/85 mmHg.
Ophthalmological examination reveals flame-shaped haemorrhages and retinal oedema
affecting the temporal region of the retina.
Which one of the following is the most likely cause of her symptoms?
A

Vitreous haemorrhage

B

Central retinal vein thrombosis

C

Retinal artery thrombosis

D

Branch retinal artery thrombosis

E

Branch retinal vein thrombosis

Explanation
Retinal vein thrombosis
Causes
Inflammatory conditions such as sarcoidosis and Lyme disease increase the risk of retinal vein
thrombosis, as do conditions which increase the risk of thrombosis such as protein C and
protein S deficiencies, antithrombin III deficiency and systemic lupus erythematosus (SLE).
Management
Anticoagulants and fibrinolytic agents have no effect on the prognosis of branch retinal vein
thrombosis. Small studies incorporating anti-vascular endothelial growth factor (anti-VEGF)
therapies or intraocular triamcinolone injection have shown promising results but these tend
to be small, single-arm studies. Retinal neovascularisation can increase the risk of vitreous
haemorrhage and retinal detachment, so ophthalmology follow-up is advised.
Branch vs retinal vein occlusion:
The fact that only the temporal region has been affected fits with branch rather than central
retinal vein occlusion. For branch retinal vein occlusion the blockage occurs before the lamina
cribrosa and not all venous drainage is affected, hence the flame haemorrhages affect only
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part of the retina.
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Question 10 of 64

A patient is examined in the Diabetic Clinic and found to have a vitreous haemorrhage, which
is precluding a view of his fundus. He is admitted for bed rest. After 3 days the fundus can be
visualised.
What is the most likely diagnosis?
A

Normal fundi

B

Background retinopathy

C

Maculopathy

D

Preproliferative retinopathy

E

Proliferative retinopathy

Explanation
Proliferative diabetic retinopathy
There must be a source for the bleeding, and the most likely source of bleeding in a diabetic
patient is fragile neovascular tissue.
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Question 11 of 64

An 82-year-old woman presents with sudden loss of vision in her left eye. On further
questioning, she complains of a left-sided headache over the past few weeks, associated with
tenderness of her head when she brushes her hair. On examination, her vision is reduced to
counting fingers in the left eye. A left relative afferent pupillary defect is present. Fundoscopy
reveals a pale, swollen left optic disc with some flame-shaped haemorrhages. The right eye is
entirely normal. An urgent erythrocyte sedimentation rate (ESR) is elevated at 72 mm/hour.
Which one of the following management options should be carried out first?
A

Temporal artery biopsy

B

Ophthalmic outpatient review

C

Administration of high-dose systemic steroids

D

Automated visual field assessment

E

Computed tomography (CT) scan of brain and orbits

Explanation
Temporal arteritis
Arteritic ischaemic optic neuropathy is a devastating ocular complication of giant-cell
arteritis (GCA), presenting with sudden, painless loss of vision. GCA should always be
suspected in a patient over the age of 50 who presents with a history of headache and scalp
tenderness. Other systemic symptoms include jaw claudication, proximal joint and muscular
aches, anorexia, weight loss and fever. Examination reveals an afferent pupillary defect and a
swollen optic disc. The ESR is usually raised.
Differential diagnosis
Compressive optic nerve tumours can also present with painless loss of vision associated with
disc swelling. However, visual loss is generally only slowly progressive and such patients
demonstrate few if any systemic signs of GCA. Computed tomography of the visual pathway
should identify such lesions.
Management

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Once suspected, high-dose systemic steroids should be started immediately as visual loss can
rapidly become bilateral. This will not affect the result of any subsequent temporal artery
biopsy performed within the next week. An ophthalmic review should be sought – any
subsequent visual field test may reveal an altitudinal or central field defect.
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Question 12 of 64

A 45-year-old woman is found by her optometrist to have band keratopathy and is referred
to the Eye Clinic.
Which one of the following investigations is likely to be helpful in determining an underlying
cause?
A

Cholesterol

B

Ferritin

C

U&E

D

Gamma GT

E

Serum calcium

Explanation
Band keratopathy
Band keratopathy is caused by calcium deposition in Bowman’s layer of the cornea. It can
result from the degenerative phase of chronic eye diseases or from hypercalcaemia. Patients
who present with band keratopathy should have a serum calcium and phosphate level drawn
unless the deposition has been documented previously and a known underlying cause exists.
Parathyroid hormone levels should be checked in otherwise idiopathic cases.
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Question 13 of 64

A 32-year-old woman with rheumatoid arthritis presents to the Rheumatology Clinic with a
complaint of severe pain and reduced vision in one of her eyes. The medical specialist trainee
notes that one eye is dusky red in colour and that the vision in this eye is reduced to 6/36.
What is the appropriate management?
A

The patient should be booked for an urgent CT scan

B

The patient should be investigated urgently for coexisting inflammatory bowel

disease

C

The patient should be started immediately on immunosuppressant therapy

D

The patient should be started on artificial tears

E

The patient should be referred urgently to the ophthalmology clinic

Explanation



The answer is The patient should be referred urgently to the ophthalmology clinic –
A red painful eye in an individual with a history of connective tissue disease should be
referred urgently to the Ophthalmology Clinic for urgent diagnosis and treatment.
Scleritis and uveitis are diagnoses that must be ruled out and treated promptly for visual
preservation. Often they are started on artificial tears or antibiotics as empiric treatment,
which offer no benefit. Further along in their management they may be investigated for
other coexisting inflammatory diseases.

The patient should be booked for an urgent CT scan (Option A) is incorrect. CT scan is
unnecessary at the first instance, unless advised by the ophthalmologists following a full
ocular examination.
The patient should be investigated urgently for coexisting inflammatory bowel disease

(Option B) is incorrect. This is unnecessary unless the patient complains specifically of
associated gastrointestinal symptoms. Even so, it is not usually an ‘urgent’ investigation.

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