Board Certified Ophthalmologist Neuro-Ophthalmology Questions and Answers — Questions and Answers
Question 1: A 28-year-old woman presents with a three-day history of painful, progressively worsening vision in her left eye. On examination, her visual acuity is 20/200 in the affected eye, and a prominent relative afferent pupillary defect is present. Which of the following clinical features is most characteristic of typical optic neuritis associated with multiple sclerosis?
- Severe optic disc edema (papilledema)
- Absence of pain with eye movements
- Pain that is exacerbated by eye movement (Correct answer)
- No improvement in vision after several weeks
Correct answer: Pain that is exacerbated by eye movement
Pain with eye movement is a hallmark symptom of typical optic neuritis, reported in over 90% of cases. This pain occurs because the inflamed optic nerve sheath is tethered to the extraocular muscles. While the optic disc may be swollen, severe papilledema is atypical. An RAPD is expected, and vision typically begins to recover within a few weeks.
Question 2: A 74-year-old female presents with an acute, profound, and painless loss of vision in her right eye. She also reports several weeks of new-onset headaches, scalp tenderness, and pain in her jaw when chewing. Examination reveals a pale, swollen optic disc in the right eye. What is the most critical immediate step in management?
- Order an urgent temporal artery biopsy
- Schedule an MRI of the brain and orbits
- Obtain a stat erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP)
- Administer high-dose systemic corticosteroids immediately (Correct answer)
Correct answer: Administer high-dose systemic corticosteroids immediately
The clinical presentation is highly suggestive of giant cell arteritis (GCA) causing an arteritic anterior ischemic optic neuropathy (A-AION). This is a neuro-ophthalmic emergency. The most critical step is to immediately start high-dose systemic corticosteroids to prevent catastrophic, irreversible vision loss in the fellow eye. While labs and a temporal artery biopsy are necessary for diagnosis, treatment should not be delayed while awaiting these results.
Question 3: A 66-year-old man with a history of type 2 diabetes and hypertension presents with the sudden onset of horizontal double vision. Examination reveals an esotropia in the primary position that worsens on left gaze, with a clear limitation of abduction in the left eye. Pupillary function, eyelid position, and all other extraocular movements are normal. What is the most likely diagnosis?
- Cavernous sinus thrombosis
- Myasthenia Gravis
- Microvascular sixth nerve palsy (Correct answer)
- Third nerve palsy
Correct answer: Microvascular sixth nerve palsy
This is a classic presentation of an isolated sixth (abducens) nerve palsy, characterized by an abduction deficit. In a patient over 50 with vasculopathic risk factors like diabetes and hypertension, a microvascular ischemic etiology is the most common cause. The absence of other cranial nerve involvement, ptosis, or pupillary changes makes other diagnoses like cavernous sinus thrombosis or a third nerve palsy less likely.
Question 4: A patient's visual field testing demonstrates a loss of the outer half of the visual field in both eyes. This defect, known as a bitemporal hemianopia, is most classically caused by a lesion at which location?
- Optic chiasm (Correct answer)
- Occipital lobe
- Optic nerve
- Optic tract
Correct answer: Optic chiasm
A bitemporal hemianopia is caused by a lesion at the optic chiasm. At the chiasm, the nerve fibers from the nasal retinas (which perceive the temporal visual fields) of both eyes cross over. A compressive lesion, such as a pituitary adenoma, will damage these crossing fibers, resulting in loss of the temporal visual fields bilaterally.
Question 5: Which of the following findings is a core component of the classic triad for Horner's syndrome?
- Mydriasis
- Lid retraction
- Exophthalmos
- Miosis (Correct answer)
Correct answer: Miosis
Horner's syndrome results from an interruption of the oculosympathetic pathway. The classic triad of signs consists of miosis (a constricted pupil), ptosis (a droopy eyelid), and anhidrosis (decreased sweating) on the affected side of the face. Mydriasis (pupil dilation), lid retraction, and exophthalmos are signs of sympathetic over-action, not paresis.
Question 6: A 26-year-old obese female presents with a 2-month history of daily headaches, pulsatile tinnitus, and transient visual obscurations. Fundus examination reveals bilateral optic disc edema. An MRI of the brain is normal. A lumbar puncture is performed, which shows an elevated opening pressure of 32 cm H2O with normal cerebrospinal fluid composition. What is the most likely diagnosis?
- Idiopathic Intracranial Hypertension (IIH) (Correct answer)
- Optic neuritis
- Meningioma
- Hypertensive retinopathy
Correct answer: Idiopathic Intracranial Hypertension (IIH)
The patient's demographics (young, obese female), symptoms (headache, pulsatile tinnitus, transient visual obscurations), signs (bilateral papilledema), and diagnostic findings (normal neuroimaging with elevated opening pressure) are all classic for Idiopathic Intracranial Hypertension (IIH), formerly known as pseudotumor cerebri.
