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Acute bilateral visual constriction, mild disc edema and abnormal mfERG

  • 1.  Acute bilateral visual constriction, mild disc edema and abnormal mfERG

    Posted 05-11-2025 20:36

    Hi Dear All, 

    Any suggestions would be greatly appreciated!

    A 44-year-old male with no previous medical history. He had sudden peripheral visual field defects in both eyes accompanied by blurred vision for half a month, along with photophobia, especially with LED white light. This is more obvious in the subway station and improves slightly outdoors. There is no pain with eye movement and no headache. There is no transient vision loss or tinnitus. He denies any change in color vision.

    He has been treated with methylprednisolone 500mg for 3 days and 250mg for 3 days in the local hospital. 

    The visual field is slightly better after getting up in the morning but returns to the previous state in the afternoon.

    The corrected visual acuity of both eyes is 20/20, and the color vision is 8/8. The anterior chamber is deep and quiet, RAPD is negative, the optic discs are slightly edematous, the cup-disc ratio is 0.3, and no lesions are seen in the retina.

    The brain and orbital MRI w/wo is normal. 

    The visual fields of both eyes show symmetrical concentric constriction. Fluorescein angiography (FFA) shows leakage of the optic discs in both eyes. The autofluorescence is normal.

    AQP4, MOG and GFAP are negative, ANA is 1:320, and SSA is positive. The mfERG shows a severe decrease wave in the macula, while the ffERG is normal.

    Any thoughts?

    Thanks!

    Best,



    ------------------------------
    Yan
    ------------------------------


  • 2.  RE: Acute bilateral visual constriction, mild disc edema and abnormal mfERG

    Posted 05-11-2025 21:34

    Can you share a cross sectional scan of OCT the macula?

    The disc edema is fairly mild and not proportional to the vision loss.

    The MRI appears to show mild enhancement of the pre-chiasmatic optic nerve on the left.

    This is a bit of a strange presentation for an acute optic neuropathy. The mf-ERG seems to be congruous to the VF loss than optic disc edema of optic nerve enhancement. You may want to send a more expanded encephalitis / paraneoplastic panel including IgLON5.

    Did the patient have any photopsias? These are common in acute photoreceptor diseases.

    I would recommend checking for syphilis and other infectious causes.

    ICG angiography may show choroiditis not apparent on fluorescein angiography.

    Best,

    Drew






  • 3.  RE: Acute bilateral visual constriction, mild disc edema and abnormal mfERG

    Posted 05-12-2025 05:20
    Good morning 
    I have SLE patients with atypical optic neuritis , which for me considered as a rather known association 
    So would I do is to get the immunologist onboard the decision making as regards steroids or other immune modulating drugs for SLE , what I see in those patients is those who knows about their illness and neglected their long term treatments or those who do not know about their illness and knew through optic neuritis , many patients I believe wheee tiggers by vague viral infection in a few cases of mine COVID was the trigger 



    Original Message


  • 4.  RE: Acute bilateral visual constriction, mild disc edema and abnormal mfERG

    Posted 05-12-2025 07:29

    This pattern may be more consistent with perineuritis rather than optic neuritis. Is there sheath enhancement on the coronal sequences? Perineuritis may be a subset or non-specific orbital inflammation which is more commonly associated with systemic rheumatic conditions compared to optic neuritis.



    ------------------------------
    Andrew Carey
    Associate Professor
    Wilmer Eye Institute, Johns Hopkins Medicine
    Baltimore MD
    ------------------------------

    Original Message


  • 5.  RE: Acute bilateral visual constriction, mild disc edema and abnormal mfERG

    Posted 05-12-2025 08:33
    Definitely agree , I just include both under the term optic neuritis , though here the enhancement is unilateral and bit atypical 







  • 6.  RE: Acute bilateral visual constriction, mild disc edema and abnormal mfERG

    Posted 05-12-2025 11:22

    Thank you both for your replies!

    The main symptom of this patient is obvious photophobia when looking at LED white lights or fluorescent lights, especially in the subway station, but there is no photophobia under sunlight. 

    The macular line scan is normal, and more coronal MRI images are as shown. Syphilis and TB (T-SPOT) were negative. 

    This patient consulted in the online clinic. When I heard his chief complaint, I suspected a retinal disease. Moreover, his visual field is inconsistent with the MRI findings. I will see him in person this Thursday. 

    What confuses me most is his mfERG. However, I just called him and he told me that they performed ffERG on him and directly carried out the mfERG without letting him rest. Even at the beginning, he couldn't see the red cross of the fixation point, and the technician had to repeat the exam.

    So I suggested that he make an appointment to repeat the mfERG tomorrow. At the same time, recheck FFA and do an ICGA.

    I suggested he go to the rheumatologist to further investigate the positive result of Sjögren's syndrome antibody (anti-Ro) and ANA.

    I agree this could be optic perineuritis but Is there any possibility that this is an autoimmune retinopathy (normal ffERG, OCT, and AF)? 



    ------------------------------
    Yan
    ------------------------------

    Original Message


  • 7.  RE: Acute bilateral visual constriction, mild disc edema and abnormal mfERG

    Posted 05-12-2025 12:34
    Very interesting case
    A few of my thoughts , not too sure though

    There are 2 structures involved in the visual pathway .
    The outer retina and optic nerves
    The pathology may be single
    Visual fields and mfERG changes due to the retinal pathology 
    Optic disc swelling and the MRI changes in the nerve not seen clinically as yet, at least not responsible for the HVF changes
    Should do a PET CT or the CAR antibody panel also , May just be a CTD related disease but haven't seen this presentation in SLE, SLE retinopathy and neuropathy can present simultaneously . But the retinopathy has some colour shower on retina- haem/ exudates etc
    Best
    Shikha



    Dr Shikha Bassi
    Sankara Nethralaya 
    Chennai
    India



    Original Message


  • 8.  RE: Acute bilateral visual constriction, mild disc edema and abnormal mfERG

    Posted 05-12-2025 13:46

    If the full field ERG is normal (including b:a wave ratio >1.3) then it is not a variant of autoimmune retinopathy ( CAR, MAR, or non-paraneoplastic). If the repeat mf-ERG is normal then retina is ruled out. The antibody tests for CAR are of low sensitivity with frequent false positives. I would not order these as a screening / rule out test.

    PET-CT may help to rule out an auto-immune / paraneoplastic optic neuropathy given the atypical nature.

    Sarcoid can cause optic neuropathy with normal or near normal MRI although rare. PET-CT should be helpful for sarcoid as well.

    Best,

    Drew





    Original Message


  • 9.  RE: Acute bilateral visual constriction, mild disc edema and abnormal mfERG

    Posted 05-12-2025 13:59
    Unless I imagined Autofluorescence OU at the posterior pole retina seems to be involved :)



    Dr Shikha Bassi
    Sankara Nethralaya
    Chennai
    India


    Original Message


  • 10.  RE: Acute bilateral visual constriction, mild disc edema and abnormal mfERG

    Posted 05-12-2025 14:40
    May I please not receive information from NANOSNET anymore?

    I am overwhelmed with emails.

    Thanks,

    Fiona

     

    Fiona Costello, MD FRCPC

    Professor

    Departments of Clinical Neurosciences and Surgery

    Associate Dean, Chairs and Professorships

    Cumming School of Medicine, University of Calgary

    Hotchkiss Brain Institute

     

    Foothills Medical Centre, 12th Floor

    1403 - 29th Street NW, Calgary AB, Canada T2N 2T9

    T: 403-944-8389 

     

     

     




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  • 11.  RE: Acute bilateral visual constriction, mild disc edema and abnormal mfERG

    Posted 05-12-2025 16:25
    Hi Fiona and all,
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  • 12.  RE: Acute bilateral visual constriction, mild disc edema and abnormal mfERG

    Posted 05-15-2025 16:42
      |   view attached

    Repeated mfERG is still abnormal.



    ------------------------------
    Yan
    ------------------------------

    Original Message


  • 13.  RE: Acute bilateral visual constriction, mild disc edema and abnormal mfERG

    Posted 05-15-2025 23:53
    Yan, are you on the old NANOSNET group? Dr Martha Schatz is discussing something so similar.
    Though the mfERG is not too abnormal , has blunting of the peak , but with a history of photophobia with this mfERG, with no past history of cancer, you should get the pt evaluated by an oncologist despite some suspicion of CTD on previous blood tests. 
    Retinal antibodies per se May not give the clarity as can be positive in patients with autoimmune diseases

    Shikha




    Dr Shikha Bassi
    Sankara Nethralaya 
    Chennai
    India



    Original Message


  • 14.  RE: Acute bilateral visual constriction, mild disc edema and abnormal mfERG

    Posted 05-18-2025 10:43

    Hi Shikha,

    Thank you for drawing my attention to that case. Yes, it's indeed similar. 

    Best，



    ------------------------------
    Yan
    ------------------------------

    Original Message


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