Archives for February 2019

Neuro-ophthalmology Illustrated Chapter 6 – Transient Visual Loss 3

29. What should be done immediately when a patient presents with a recent vascular TMVL?
30. What work-up should be done for transient monocular visual loss thought to be of vascular origin?
31.  What should be done emergently in the presence of an acute central retinal artery or branch retinal artery occlusion?
32.  What is the chance and timeline of stroke after TMVL in the presence of ipsilateral atheromatous internal carotid stenosis ≥50%?
33. What is the yearly risk of vascular death (myocardial infarction) in patients with TMVL and atheromatous disease? 34. What tests should be done in all patients with transient monocular visual loss over age 50?

Recommended Reading – Unusual presentation of an Adie-like pupil Cat’s eye pupil

Unusual presentation of an Adie-like pupil Cat’s eye pupil
Robert A. Egan, Carla Avruskin
Neurology. 2018; 91 (15)

A 55-year-old woman noticed that her right pupil was oddly shaped, associated with a mild ache. Her afferent visual examination, ocular and eyelid motility, and fundi were normal. Her left pupil was round and reactive (figure 1).

Figure 1 Close-up shows normal left pupil and tonic right cat’s eye pupil
The right pupil was elongated (points at 11:00 and 5:00) and tonic (figure 2).

Figure 2 There was sectoral paralysis of the iris between 12:00 and 4:00 and also from 6:00 to 10:00 with mild thinning of the pupillary ruff on the temporal iris characteristic of an Adie-like pupil

There was no ocular hypertension or corneal edema. A year later, the pupil shape became triangular; evaluation revealed ocular hypertension without corneal edema, confirming the iridocorneal endothelial (ICE) syndrome. This case is an unusual presentation of a cat’s eye Adie-like pupil as the harbinger for ICE syndrome.

Neuro-ophthalmology Illustrated Chapter 6 – Transient Visual Loss 2

10. What provides the blood supply to the anterior cerebral hemispheres and to the eyes?
11. What is the blood supply to the occipital lobes?
12. What finding does occlusion of a posterior cerebral artery produce?
13. A patient complains of loss of visual field in one eye, what
 should be ruled-out on examination?
14. What 5 mechanisms should be considered in the differential diagnosis of transient monocular visual loss?
15. What is the most likely cause of transient visual obscurations (brief blackouts or grey-outs) on change of posture?
16. What is the likely cause of gaze-evoked episodes of transient monocular vision loss?
17. Which ocular diagnoses involving the anterior segment of the eye should be considered when a patient complains of transient monocular blurring of vision?
18. How long does the transient loss of vision last when the cause is retinal emboli?
19. What is the typical length of time of transient monocular vision loss in optic nerve head anomalies?
20. What should be considered in a patient who has eye or brow pain with transient monocular vision loss?
21. What should be considered in a patient who has transient monocular vision loss and neck pain or ipsilateral Horner syndrome?
22. What should be considered in a patient who has transient monocular vision loss and simultaneous contralateral hemisensory or motor findings?
23. What should be considered in a patient who has transient monocular vision loss and presyncope?
24. Will the transient monocular vision loss due to carotid occlusive disease be partial vision loss or complete vision loss?
25. What is the major source of collateral circulation to the eye?
26. What are 5 vascular mechanisms of transient monocular visual loss?
27. Should the presence of emboli in the eye found on a routine exam prompt a workup for their source?
28. How long does the monocular visual loss from spasm of the central retinal artery last?

Recommended Reading – Teaching NeuroImages: Kearns-Sayre syndrome

Teaching NeuroImages: Kearns-Sayre syndrome 
Michael T.B. Nguyen, Jonathan Micieli, Edward Margolin
RESIDENT & FELLOW SECTION. Neurology. 2019; 92 (5)


A 19-year-old man presented 6 months postimplantation of permanent pacemaker for complete heart block with bilateral nonfatigable symmetric ptosis, diminished levator superioris function, and symmetric ophthalmoplegia (figure 1). Funduscopy revealed bilateral pigmentary retinopathy (figure 2). Skeletal muscle biopsy revealed presence of ragged-red fibers, consistent with Kearns-Sayre syndrome. This mitochondrial disorder is characterized by the triad of onset before age 20, chronic progressive external ophthalmoplegia, and pigmentary retinopathy. Other findings can include complete heart block, cerebellar ataxia, deafness, and endocrinopathies. CSF folate levels should be measured and supplemented if low. There is no definitive treatment but annual surveillance for comorbidities is required.1,2

Figure 1 Ptosis and ophthalmoplegia
Photographs of eye movement demonstrate severe bilateral ptosis and mild diffuse ophthalmoplegia in all directions of attempted gaze.

Figure 2 Pigmentary retinopathy
Fundus examination of right (A) and left eye (B) shows bilateral pigmentary retinopathy. There is diffuse depigmentation of the retinal pigment epithelium in a salt-and-pepper pattern of pigment clumping and involvement of the peripapillary zone.

1. Kearns TP, Sayre GP. Retinitis pigmentosa, external ophthalmoplegia, and complete heart block. Arch Ophthalmol 1958;60:280–289.
2. Shemesh A, Margolin E. Kearns Sayre syndrome. In: StatPearls [Internet]. Treasure Island, FL: StatPearls Publishing; 2018.

Neuro-ophthalmology Illustrated Chapter 6 -Transient Visual Loss 1

1. What is the preferred term for abrupt and temporary vision loss in one eye?
2. What is the most common cause of transient monocular vision loss?
3. What is amaurosis fugax?
4. What is the first step in evaluating transient vision loss?
5. Why is it important to determine whether transient vision loss is unilateral or bilateral?
6. What 3 items are included in the differential diagnosis of transient binocular visual loss?
7. What are the characteristics of an occipital seizure?
8. Are occipital seizures more common in children or adults?
9. What are the characteristics of an occipital transient ischemic attack?

Neuro-ophthalmology Illustrated Chapter 5 – Vision Loss: An Overview 2

10. Other than vascular disease, what 10 conditions can cause transient monocular visual loss?
11. What is Uhthoff’s phenomenon?
12. What conditions should be considered in the absence of an RAPD in cases where the cause of unilateral or asymmetric visual loss is not apparent?
13. What simple clinical test may help in determining if a maculopathy is present where the cause of unilateral or asymmetric visual loss is not apparent?
14. What are five retinal conditions that may mimic optic neuropathies?
15. What is the acquired idiopathic blind spot enlargement syndrome (AIBSE)?
16. What are 4 bilateral retinal conditions that may be difficult to distinguish from bilateral symmetric primary optic neuropathies?
17. What is the fundus appearance early in the course of cone dystrophies?
18. What specific historical items should be sought in cases of unexplained monocular visual acuity loss but with a normal visual field?
19. What exam findings may be found that help confirm the diagnosis of amblyopia?
20. Why should the blood pressure be checked in patients with a history of transient vision loss?
21. What are the findings of the posterior reversible encephalopathy syndrome?

Recommended Reading – Volitional opsoclonus

Recommended Reading – Volitional opsoclonus

Volitional opsoclonus

Patricio S. Espinosa, Joseph R. Berger. Neurology. 2005; 65 (5)


Opsoclonus (saccadomania) is defined as random, uncalled-for, large-amplitude, back-to-back saccades without intersaccadic intervals. An abnormality of the omnipause neurons in the brainstem nucleus raphe interpositus has been suggested, but not proven, as the etiology of opsoclonus.
1 Opsoclonus occurs with paraneoplastic, infectious, postinfectious, toxic, or metabolic disorders; however, it is often idiopathic. Intentional fixation instability may result in ocular movements closely resembling opsoclonus.2 We describe a patient with the volitional ability to replicate “opsoclonus.”

This 55-year-old man presented with transient left-sided numbness which, after thorough evaluation, was attributed to a transient ischemic episode. His neurologic examination, including ocular motility, was perfectly normal. However, he was voluntarily able to display wildly chaotic eye movements (video clip). He commented that he has been aware of this ability since childhood. No other family members, to the best of his knowledge, are able to replicate these eye movements. Analysis of the eye movements permitted by looping and pausing the video segment reveals that the actual eye movements are either diagonal (horizontal and vertical in phase) or, in some cases, elliptical (horizontal and vertical 90º out of phase), indicating that it is “voluntary opsoclonus,” also referred to as “voluntary multiplanar flutter” (Louis Dell’Osso, Cleveland, OH, personal communication, January 4, 2005).

The identification of volitional eye movements in clinical practice is important because it permits the distinction between neurologic and factitious disorders.



1. Ridley A, Kennard C, Scholtz CL, Buttner-Ennever JA, Summers B, Turnbull A. Omnipause neurons in two cases of opsoclonus associated with oat cell carcinoma of the lung. Brain 1987;110(Pt 6):1699–1709.

2. Yee RD, Spiegel PH, Yamada T, Abel LA, Suzuki DA, Zee DS. Voluntary saccadic oscillations, resembling ocular flutter and opsoclonus. J Neuroophthalmol 1994;14:95–101.