Neuro-ophthalmology Question of the Week: Paraneoplastic Syndromes in Neuro-Ophthalmology

August 30, 2017 By

Question: When should an ophthalmologist consider the diagnosis of a paraneoplastic syndrome?  

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Question with answers: When should an ophthalmologist consider the diagnosis of a paraneoplastic syndrome?
“In terms of afferent symptoms, an unexplained, painless, progressive vision loss is typical. With retinal involvement, there may be photopsias, night blindness, or ring scotomas. In the optic neuropathies, there is most commonly bilateral disc swelling often accompanied by vitritis. Efferent symptoms include myasthenic-like presentation or the presence of opsoclonus/myoclonus syndrome (OMS).”1

Neuro-ophthalmology Questions of the Week-Neuromyelitis Optica (Devic Disease)

August 23, 2017 By

Questions:
1. Does the Transverse myelopathy of Neuromyelitis Optica (NMO) precede or follow the onset of optic neuritis?
2. Is there an antibody test for NMO?
3. What is the prognosis for NMO?
4. What are the diagnostic criteria for NMO?
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Trial of Tocilizumab in Giant-Cell Arteritis

August 11, 2017 By 
John H. Stone, M.D., M.P.H., Katie Tuckwell, Ph.D., Sophie Dimonaco, M.Sc., Micki Klearman, M.D., Martin Aringer, M.D., Daniel Blockmans, M.D., Ph.D., Elisabeth Brouwer, M.D., Ph.D., Maria C. Cid, M.D., Bhaskar Dasgupta, M.B., B.S., M.D., Juergen Rech, M.D., Carlo Salvarani, M.D., Georg Schett, M.D., Hendrik Schulze-Koops, M.D., Ph.D., Robert Spiera, M.D., Sebastian H. Unizony, M.D., and Neil Collinson, Ph.D.
N Engl J Med 2017; 377:317-328July 27, 2017DOI: 10.1056/NEJMoa1613849

BACKGROUND
Giant-cell arteritis commonly relapses when glucocorticoids are tapered, and the prolonged use of glucocorticoids is associated with side effects. The effect of the interleukin-6 receptor alpha inhibitor tocilizumab on the rates of relapse during glucocorticoid tapering was studied in patients with giant-cell arteritis.

METHODS
In this 1-year trial, we randomly assigned 251 patients, in a 2:1:1:1 ratio, to receive subcutaneous tocilizumab (at a dose of 162 mg) weekly or every other week, combined with a 26-week prednisone taper, or placebo combined with a prednisone taper over a period of either 26 weeks or 52 weeks. The primary outcome was the rate of sustained glucocorticoid-free remission at week 52 in each tocilizumab group as compared with the rate in the placebo group that underwent the 26-week prednisone taper. The key secondary outcome was the rate of remission in each tocilizumab group as compared with the placebo group that underwent the 52-week prednisone taper. Dosing of prednisone and safety were also assessed.

RESULTS
Sustained remission at week 52 occurred in 56% of the patients treated with tocilizumab weekly and in 53% of those treated with tocilizumab every other week, as compared with 14% of those in the placebo group that underwent the 26-week prednisone taper and 18% of those in the placebo group that underwent the 52-week prednisone taper (P<0.001 for the comparisons of either active treatment with placebo). The cumulative median prednisone dose over the 52-week period was 1862 mg in each tocilizumab group, as compared with 3296 mg in the placebo group that underwent the 26-week taper (P<0.001 for both comparisons) and 3818 mg in the placebo group that underwent the 52-week taper (P<0.001 for both comparisons). Serious adverse events occurred in 15% of the patients in the group that received tocilizumab weekly, 14% of those in the group that received tocilizumab every other week, 22% of those in the placebo group that underwent the 26-week taper, and 25% of those in the placebo group that underwent the 52-week taper. Anterior ischemic optic neuropathy developed in one patient in the group that received tocilizumab every other week.

CONCLUSIONS
Tocilizumab, received weekly or every other week, combined with a 26-week prednisone taper was superior to either 26-week or 52-week prednisone tapering plus placebo with regard to sustained glucocorticoid-free remission in patients with giant-cell arteritis. Longer follow-up is necessary to determine the durability of remission and safety of tocilizumab. (Funded by F. Hoffmann–La Roche; ClinicalTrials.gov number, NCT01791153.)

Full article available at 
http://sfx.stanford.edu/local?sid=Entrez:PubMed&id=pmid:28745999
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Neuro-ophthalmology Questions of the Week: Idiopathic Demyelinating Optic Neuritis

August 7, 2017 By

Questions:
1. What is the most common acute optic neuropathy in persons under the age of 45?   
2. What is the most common cause of optic neuritis?
3. What condition is a common presenting sign of multiple sclerosis?

Neuro-ophthalmology Questions of the Week: Inflammatory Optic Neuropathy

August 2, 2017 By

Questions:

1. What historical factors are important to assess in a patient with suspected optic neuropathy?

2. What tests should be considered based on the assessment of appropriate historical factors?

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Neuro-ophthalmology Questions of the Week-Optic Neuropathy Patient Evaluation

July 26, 2017 By

Questions:

1. Are some maculopathies associated with mild optic nerve pallor?
2. Can maculopathies have dyschromatopsia?
3. In evaluating optic neuritis, when should a lumbar puncture for CSF analysis be considered?


Fig.8.5
(a) Isolated right inflammatory optic neuropathy. There is a large central scotoma seen as diffuse depression on a 24–2 Humphrey visual field test.
(b)Coronal and axial T1-weighted magnetic resonance imaging with fat suppression and contrast showing enhancement of the orbital portion of the right optic nerve(arrow).

Neuro-ophthalmology Question of the Week: Optic Neuropathies Introduction

July 19, 2017 By

Question:
1. In acute optic neuropathy, how long after onset does the optic nerve head become pale?
2. When is electrophysiologic testing useful in acute optic neuropathies?
3. What does a painful orbital apex syndrome in a diabetic patient suggest?

Fig.8.1

Neuro-ophthalmology Questions of the Week-Ocular Vascular Disease

July 13, 2017 By

Questions:

  1. What may develop in patients with severe stenosis or occlusion of the ipsilateral common carotid artery or internal carotid artery and poor collateral circulation?
  2. What condition should venous stasis retinopathy, or hypotensive retinopathy, suggest?
  3. What are the symptoms of the ocular ischemic syndrome?
  4. What are signs of the ocular ischemic syndrome?
  5. What is the prognosis of the ocular ischemic syndrome?
  6. What should be ruled-out in a patient who has a rapidly worsening ocular ischemic syndrome?
  7. What should be checked in all patients with headache and bilateral optic nerve swelling?
  8. What 10 classic systemic disorders are associated with retinal vasculitis?
  9. Is radiation retinopathy a chronic, painless, progressive retinal vasculopathy?
  10. Radiation retinopathy may be unilateral or bilateral and occur months or years after radiotherapy. True or False?
  11. Radiation retinopathy is more common in patients with underlying retinal vascular disease (e.g., hypertension or diabetes). True or False?
  12. What are 5 key findings of radiation retinopathy?
  13. What are 6 Complications of radiation retinopathy?
  14. What is the chief feature of Purtscher retinopathy?
  15. What is the significance of bilateral retinal vascular tortuosity?
  16. What are 2 retinal vascular malformations?

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Neuro-ophthalmology Questions of the Week-Retinal Ischemia

July 5, 2017 By

Questions:
1. What should be done for a patient with monocular vision loss, not due to nonarteritic anterior ischemic optic neuropathy, arteritic anterior ischemic optic neuropathy, or other ophthalmologic disease, but with branch or central retinal artery occlusions or amaurosis fugax?
2. What should be considered if a central artery is associated with pain?
3. What is the most common cause of ophthalmic artery occlusion?
4. What are the findings from ophthalmic artery occlusion
5. What finding seen in acute central artery occlusion is not seen with acute ophthalmic artery occlusion?
6. What work-up should be done when retinal emboli are found in an asymptomatic patient?
7. What is the appearance of cholesterol emboli?
8. What are common sources for cholesterol emboli?
9. What is the appearance of talc emboli?
10. What condition is associated with talc emboli?
11. In a patient with acute retinal ischemia what tests should be ordered for thrombophilia?

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Neuro-ophthalmology Recommended Reading – Seesaw Nystagmus – with video

July 4, 2017 By
www.nejm.org
Recommended by Yaping Joyce Liao, M.D., Ph.D.

A 52-year-old man presented to the emergency department with a 1-year history of headache and reduced visual acuity. Physical examination showed seesaw nystagmus; a …

http://www.nejm.org/doi/full/10.1056/NEJMicm1613244#t=article