Archives for August 2018

Neuro-ophthalmology questions of the week: NOI16-Nystagmus and Other Ocular Oscillations 4 – Other Nystagmoid Eye Movements

Questions:
24. What are the characteristics and the lesion location for convergence-retraction nystagmus?
25.  What are the characteristics, concerns, and treatment for superior oblique myokymia?
26. What are the characteristics, concerns, and the lesion location for ocular bobbing?
27. What are the characteristics of saccadic intrusions?
28. What are the characteristics and concerns for ocular flutter?
29. What are the characteristics and concerns for opsoclonus?
30. What are the characteristics and concerns for square-wave jerks and macrosquare-wave jerks?
31. What are the characteristics, concerns, and lesion location for ocular dysmetria?
32. What are the characteristics of voluntary nystagmus?
33. What are the characteristics, concerns and lesion location for internuclear ophthalmoplegia?

Recommended Reading – Treatment of Nystagmus and Saccadic Oscillations

Recommended Reading – Treatment of Nystagmus and Saccadic Oscillations

Treatment of Nystagmus and Saccadic Oscillations
Matthew J. Thurtell, and John J. Brinkley/ University of Iowa. EyeRounds.org. 2013
http://webeye.ophth.uiowa.edu/eyeforum/tutorials/Nystagmus/

Introduction
Nystagmus is often encountered in ophthalmology practice, having a prevalence of about 24 per 10,000 in the general population.[1] Unlike physiologic nystagmus, where the slow phases of nystagmus minimize retinal image slip, the slow phases of pathologic nystagmus cause retinal image slip. Retinal image slip of greater than 5 degrees per second produces a decline in visual acuity, partly because the image of the object of interest no longer lies on the fovea, and illusory motion of the visual environment known as oscillopsia.[2,3] Saccadic intrusions and oscillations can also cause visual symptoms, such as difficulty reading, since they take the eye off target so that the image of the object of interest no longer lies on the fovea.[2]

Goals of Treatment
The goal of treatment is to reduce visual symptoms (e.g., blurred vision, oscillopsia) by reducing the speed of nystagmus slow phases or by suppressing saccadic oscillations. Treatments that stop the eyes from moving altogether (e.g., botulinum toxin injections into the extraocular muscles) are not ideal, because they cause oscillopsia during head movements (due to loss of the vestibulo-ocular reflex) and diplopia (due to loss of vergence eye movements).[2] Thus, treatments that suppress the abnormal eye movements without affecting normal eye movements are preferred. Note that some types of nystagmus (e.g., gaze-evoked) and saccadic intrusions (e.g., square-wave jerks) do not usually give visual symptoms and, thus, do not require specific treatment.

General Approaches to Treatment
Treatments for nystagmus that have been proposed include medical, optical, surgical, and other miscellaneous treatments (Table 1); few of these have been evaluated in prospective masked clinical trials.[2,4] Likewise, a variety of treatments for saccadic oscillations have been proposed; few have been evaluated in prospective masked clinical trials.[2] Most treatments aim to suppress the abnormal eye movements without affecting normal eye movements, whereas others aim to negate the visual consequences of the abnormal eye movements. Choice of treatment depends on the type of nystagmus or saccadic oscillation and its characteristics. While some patients will derive benefit from one treatment approach, others require a combination of treatments.[2,4]

Full Article http://webeye.ophth.uiowa.edu/eyeforum/tutorials/Nystagmus/

 

Recommended Reading – Nystagmus Videos – Neuro-Ophthalmology Virtual Education Library

Recommended Reading – Nystagmus Videos – Neuro-Ophthalmology Virtual Education Library

List of related Videos from the Novel Moran Eye Center – Neuro-Ophthalmology Virtual Education Library

Novel Moran Eye Center – Neuro-Ophthalmology Virtual Education Library

https://collections.lib.utah.edu/search?facet_setname_s=ehsl_novel_jmec

Neuro-ophthalmology questions of the week: NOI16-Nystagmus and Other Ocular Oscillations 3 – Acquired Nystagmus

Questions:
Acquired Nystagmus
9. What are the characteristics, concerns, lesion location, and treatment for gaze-evoked nystagmus?
10.  What are the characteristics, concerns, lesion location, and treatment for nystagmus with positional vertigo?
11. What are the characteristics, concerns, lesion location, and treatment for downbeat nystagmus?
12. What are the characteristics, concerns, lesion location, and treatment for upbeat nystagmus?
13. What are the characteristics, concerns, lesion location, and treatment for periodic alternating nystagmus?
14. What are the characteristics, concerns, and lesion location for rebound nystagmus?
15.  What are the characteristics and lesion location Brun nystagmus?
16. What are the characteristics, concerns, and treatment for dissociated jerk nystagmus?
17. What are the characteristics, concerns, lesion location, and treatment for acquired pendular nystagmus?
18. What are the characteristics, concerns, lesion location, and treatment for seesaw nystagmus?
19. What are the characteristics, lesion location, and treatment for oculopalatal myoclonus?
20. What are the characteristics, concerns, lesion location, and treatment for oculomasticatory myorhythmia?
21. What are the characteristics, concerns, and treatment for Wernicke encephalopathy?
22. Which patterns of jerk nystagmus have localizing/diagnostic value?
23. Which patterns of pendular nystagmus have localizing/diagnostic value?

Recommended Reading – The clinical evaluation of infantile nystagmus: What to do first and why

Recommended Reading – The clinical evaluation of infantile nystagmus: What to do first and why

The clinical evaluation of infantile nystagmus: What to do first and why.
Morgan Bertsch, Michael Floyd, Taylor Keohea, Wanda Pfeifer, and Arlene V. Dracka Ophthalmic Genet. 2017 ; 38(1): 22–33.
Department of Ophthalmology, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, Iowa, USA

Abstract Introduction—Infantile nystagmus has many causes, some life threating. We determined the most common diagnoses in order to develop a testing algorithm.

Methods—Retrospective chart review. Exclusion criteria were no nystagmus, acquired after 6 months, or lack of examination. Data collected: pediatric eye examination findings, ancillary testing, order of testing, referral, and final diagnoses. Final diagnosis was defined as meeting published clinical criteria and/or confirmed by diagnostic testing. Patients with a diagnosis not meeting the definition were “unknown.” Patients with incomplete testing were “incomplete.” Patients with multiple plausible etiologies were “multifactorial.” Patients with negative complete workup were “motor.”

Results—284 charts were identified; 202 met inclusion criteria. The 3 most common causes were Albinism(19%), Leber Congenital Amaurosis(LCA)(14%) and Non-LCA retinal dystrophy (13%). Anatomic retinal disorders comprised 10%, motor another 10%. The most common first test was MRI (74/202) with a diagnostic yield of 16%. For 28 MRI-first patients, nystagmus alone was the indication; for 46 MRI-first patients other neurologic signs were present. 0/28 nystagmus-only patients had a diagnostic MRI while 14/46 (30%) with neurologic signs did. Yield of ERG as first test was 56%, OCT 55%, and molecular genetic testing 47%. 90% of patients had an etiology identified.

Conclusion—The most common causes of infantile nystagmus were retinal disorders (56%), however, the most common first test was brain MRI. For patients without other neurologic stigmata complete pediatric eye examination, ERG, OCT and molecular genetic testing had a higher yield than MRI scan. If MRI is not diagnostic, a complete ophthalmologic workup should be pursued.

Full Article https://drive.google.com/open?id=1h76ziIrxB6TpINMsPF1mcsWvvAvVkaj3

 

NOI16-Nystagmus and Other Ocular Oscillations 2 – Infantile Nystagmus

Questions:
Infantile (congenital) Nystagmus
5. What are the characteristics, associated conditions, concerns, and treatment for infantile nystagmus?

Other Types of Infantile Nystagmus
6. What are the characteristics and associated conditions for latent nystagmus?
7. What are the characteristics, associated conditions, concerns, lesion location and treatment for spasmus nutans?
8. What are the characteristics, and lesion location for infantile monocular pendular nystagmus?

https://lh4.googleusercontent.com/Gt4dXacAnmGJwfgR9EIJQVdqsG39U_FLEbrM7T1e_YCH8Raza2n7WAW4vHFELSC4gsZ2sCgoBYrlcftn1TA4jji2un1nBpYJzznscv4y6lnJvxBvuKPRD8WBV_2y4u3GDM4hvSmj

Recommended Reading – Mystery Case: A young woman with isolated upbeating nystagmus.

Recommended Reading – Mystery Case: A young woman with isolated upbeating nystagmus.
Charlene Ong, Kevin Patel, Erik Musiek, Gregory Van Stavern.
Neurology 2015; 84 (4) RESIDENT AND FELLOW SECTION
http://n.neurology.org/content/84/4/e17.full

ARTICLE
A 15-week pregnant 21-year-old woman initially presented with nausea, vomiting, and abdominal pain. The patient admitted to decreased oral intake over the past 4 weeks, including her prescribed prenatal vitamins. She was hypokalemic with elevated transaminases and gallstone pancreatitis was confirmed by imaging. Prior to cholecystectomy, fetal heart tones were lost and intrauterine fetal demise occurred. The patient underwent dilation and evacuation as well as cholecystectomy. She was discharged home but returned within 1 week with persistent nausea and vomiting. She had no neurologic complaints at the time. Basic metabolic panel on admission was unremarkable. On hospital day 2, she developed oscillopsia. Her examination was remarkable for large amplitude upbeating nystagmus (UBN) in primary position. She had gaze-evoked UBN in all other directions. The amplitude of the UBN increased on upgaze and dampened on downgaze. Smooth pursuit was impaired in all directions and saccades were dysmetric (video https://www.youtube.com/watch?v=b8j3LcwY2ZM).

Extraocular movements were intact with no evidence of ophthalmoplegia. Pupils were equal and reactive, and fundus examination was normal. Reflexes were present and symmetric, and gait was normal. The patient had no deficits on mental status examination. She was oriented to name, date, place, and situation and had no difficulty with complex commands, calculations, or short-term or long-term memory. Language was similarly intact. She demonstrated no ataxia or other focal abnormalities on examination.

Questions for consideration:
1. What is the differential diagnosis with this history and examination?
2. What is the next step in management for this patient? What tests would you order?

Neuro-ophthalmology questions of the week: NOI16-Nystagmus and Other Ocular Oscillations 1 – Basics

Questions:
Nystagmus Basics
1. What 14 features should be assessed in the evaluation of a patient with nystagmus?
2. What are the characteristics of physiologic nystagmus?
3. What are the characteristics, lesion locations, associated conditions, concerns, and treatment for peripheral vestibular nystagmus?
4. What are the characteristics, lesion locations, associated conditions, and concerns for central nystagmus?

Recommended Reading – IMAGES IN CLINICAL MEDICINE Orbital Varix

Recommended Reading – IMAGES IN CLINICAL MEDICINE Orbital Varix
Kiang L, Kahana A. N Engl J Med 2015; 372:e9v

A 63-year-old man was referred to our clinic with a 13-year history of intermittent vision loss, binocular diplopia, and blepharoptosis of the left eye during bending or straining that had worsened over the previous year. Other than uneventful cataract surgery, the patient had no clinically significant ocular history, and prior computed tomographic (CT) scans of the head and orbit did not identify any abnormality. An orbital vascular anomaly was suspected. A CT scan of the head was obtained while the patient performed the Valsalva maneuver, revealing the expansile orbital mass. On presentation to us, the patient’s visual acuity was 20/20 in the right eye and 20/50 in the left eye, with a relative afferent pupillary defect in the left eye. There was no proptosis on examination (Panel A).

Visual-field testing revealed severe constriction.

A Valsalva maneuver induced 6 mm of proptosis in the left eye, with anterior superior globe displacement and blepharoptosis (Panel B, and video).

   Orbital Varix. (00:19)

Proptosis quickly reversed on relaxation. CT of the orbit while the patient waw at at rest was unremarkable (Panels C and D show the axial and coronal views, respectively),

but a Valsalva maneuver revealed an orbital mass causing anterior superior globe displacement (Panels E and F).

An orbital angiogram confirmed the presence of an expansile orbital mass (Fig. 2 in the Supplementary Appendix).

Although the differential diagnosis of an orbital mass is broad and includes lymphoma, metastatic tumors, and inflammatory masses, the clinical findings and imaging studies in this patient were pathognomonic of an orbital vascular anomaly and were most consistent with a distensible venous anomaly (i.e., varix). In the confines of the orbit, an expansile mass can cause intermittent orbital compartment syndrome and compressive optic neuropathy. The patient underwent successful endovascular and transorbital sclerosing treatment. The postoperative visual acuity was 20/30 in the left eye, with a stable visual field.

 

Neuro-ophthalmology questions of the week: NOI15-Cavernous Sinus and Orbital Vascular Disorders 4

Questions:
18. What is the cause of “orbital varices”?
19. What should be considered when a crying infant eye bulges?