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history of IIH and new optic neuropathy

  • 1.  history of IIH and new optic neuropathy

    Posted 14 days ago

    Hi all ! 

    Hoping for some input/thoughts. thank you ! 

    22 yo AAF 
    IIH dx  since 2014 ; OP 55 and normal CSF
    11-2025 : seen at our hospital & by one of our ophtho 
    Had LP with OP = 50 (on lasix 80-100mg qd)
    Neurology restarted on diamox
    OCT G146 OD (was 142 LV), G 152 OS (was 175 LV)
    Gmax 192 OD & 195 OS in 7/2021
    HVF  enlarged blind spots OU
    12-2025 I started to see her ; she was on 1000mg diamox and exam no disc edema with normal HVF and good RNFL OU 
    Was also started on Zepbound for weight loss
    Saw her 3-2026 with very stable exam - kept diamox dosing same 
    6-2026 fu visit: she reports a few days after our apt she noted change in her left eye 
     pt woke up with decreased vision OS (describes a large gray line in the center of the vision) and persistent sharp left eye pain (but ?denies pain with eye movements). This occurred twice (first episode was transient and symptoms self-resolved, whereas symptoms were persistent after second episode which occurred two weeks after first episode). The vision decline has been stable since
    She did not call or come in sooner for re-eval 
    6-2026 eval shows APD OS ; VA 20/1250 ; no color plates and atrophy on OCT ; G OS now 74 
    OD stable and no disc edema 
    Initiated workup w repeat MRI and labs 
    MRI with still signs of increased ICP as before and now OS with mild enhancement of the optic nerve but no thickening or T2 signal abnormality 
    Labs: B12, folate, bartonella, NMO, MOG, RF, Lyme, Lysozyme, Syphilis, CRP, Anti dsDNA, ANA, ANCA, ACE all WNL 
    7-2026 : 1 month follow up vision at 20/500 so with some improvement ; rest of exam stable 
    Clearly there is new optic neuropathy/neuritis component OS though I am unclear of etiology 
    Pt is declining new LP w OP and CSF analysis and declined IV steroids. 
    Any thoughts on what else I can do here and on differential for this new finding in the setting of history of IIH which has been well controlled. 
    I also thought about NAION in setting of Zepbound use but unsure if that would be accompanied by intraorbital optic nerve enhancement on the MRI 


  • 2.  RE: history of IIH and new optic neuropathy

    Posted 14 days ago
    Hello,
    If the optic nerve enhancement is real, then NAION associated with GLP1 agonist is less likely. Perhaps you could post a picture of the MRI? So maybe optic neuritis? Maybe helpful to get additional c/t spine MRI and LP for oligoclonal bands and CSF MOG testing if she's agreeable. Also helpful to see if the enhancement resolves over time; it it doesn't, could also consider infiltrative optic neuropathies such as sarcoid. 
    Best,
    Bart

    Bart K. Chwalisz, M.D.

    Neuro-ophthalmology, Headache Unit, and  Skull Base Neurology Clinic. Massachusetts General Hospital and Brigham & Women's Hospital

    Skull Base Clinic: https://link.edgepilot.com/s/d56bfde9/x2C4xlWamUy0ny5onbj6Yg?u=https://www.massgeneral.org/neurology/services/treatmentprograms.aspx?id=2070

    Neurology, Martha's Vineyard Hospital

    Neuro-ophthalmology, Massachusetts Eye & Ear 

     

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  • 3.  RE: history of IIH and new optic neuropathy

    Posted 14 days ago
    image



  • 4.  RE: history of IIH and new optic neuropathy

    Posted 14 days ago
    image



  • 5.  RE: history of IIH and new optic neuropathy

    Posted 14 days ago
    image



  • 6.  RE: history of IIH and new optic neuropathy

    Posted 14 days ago
    The enhancement of the optic nerve sheath and the lateral rectus muscle are most consistent with idiopathic orbital inflammatory syndrome. I think this is coincidental with her IIH and not a sign of ischemic optic neuropathy as has been reported with GLP1RA use. I would push hard on the IV steroids to see if you can get any vision back. If she doesn't improve you could envision a biopsy of the lateral rectus to look for IgG-4 disease. Tough case!

    Bradley J Katz, MD, PhD
    Professor of Ophthalmology and Visual Sciences
    John A Moran Eye Center
    he/him/his





  • 7.  RE: history of IIH and new optic neuropathy

    Posted 14 days ago

    that is very helpful. thank you! I actually hadn't noticed the EOM asymmetry as much but as I run through it again I do see that as well. I will add on IGG4 labs and try to push for steroids again.

    Re the nerve enhancement to me I still think there is proper enhancement within the nerve itself not just the sheath esp in the coronal cuts 




  • 8.  RE: history of IIH and new optic neuropathy

    Posted 14 days ago
    The left lateral rectus is normal, it looks thickened because the muscle is contracted in aBduction (look at visual axis), there is no T2 hyper in the muscle to suggest myositis.

    This looks like optic neuritis, I don't appreciate any nerve sheath enhancement on the coronal post-GAD image.

    If the patient doesn't want IV steroids, there are several series looking at oral equivalent (1250 mg prednisone) three times per day. But since it is not Nmo or MOGAD the benefit or steroids is questionable.

    I agree, this is not consistent with GLP-1 RA associated ischemic optic neuropathy.

    Best, 

    Drew 







  • 9.  RE: history of IIH and new optic neuropathy

    Posted 13 days ago
    The only factor that it is NA ION is the near altitudinal visual field defect , but this can occur with optic neuritis as well , but in case suspecting , do carotid Doppler and just cardiological examination by a cardiologist , I had a case like that , young lady with all the complaints and the disc pointing to neuritis then on Echo heart , there was both mitral and tricuspid valve affection , but note that where I stand rheumatic fever is still have a substantial activity , but for the field defect and ensuring every thing for the other eye it wouldn't hurt having Doppler and cardiological examination 





  • 10.  RE: history of IIH and new optic neuropathy

    Posted 10 days ago

    Thank you ! I agree my primary concern was for optic neuritis (though exact etiology is not yet clear to  me); I saw pt again this week and  is now pinholing to 20/300 ; her HVF was not very reliable and showed general constriction. OCT was stable w loss of GCL and RNFL thinning. She still declining steroids even in oral form (she has concern bc of her bipolar disorder for a severe manic episode). Also still declining LP. 

    Plan is to observe for now but I am just unclear on the cause of the optic neuritis. 




  • 11.  RE: history of IIH and new optic neuropathy

    Posted 10 days ago

    An ischemic etiology though remote is not yet completely excluded if the MRI that was done after overnight worsening blur occurred ~3-21 days after the event (the window within which ischemic lesions could enhance post-GAD). Sounds like the PE post-COVID could indicate a latent coagulopathic process that was unmasked by the acute prothrombotic state during COVID. The other possibility is that the worsening over the years is 2/2 to persistent thrombosis in the setting of "long COVID"?.

    RE: Poiseuille's law, the volume flow rate (Q) depends on the pressure difference (Δ P), pipe radius (r), fluid viscosity (η), and pipe length (L). In her case, there is patchy pipe radius according to the MRV findings you described (i.e., "...The left transverse and sigmoid venous  sinus is small, likely developmental. There may be additional narrowing along the lateral aspect of the left transverse venous sinus...". ) and INCREASE viscosity (pro-thrombotic state from long COVID +/- an undiagnosed PRE-COVID acquired or hereditary coagulopathy). This creates the perfect Virchow scenario for continued thrombosis and persistent elevated ICP that could've led to a sleeping-position dependent unilateral ischemic event; furthermore, unilateral involvement in IIH is not that farfetched.  This is like a DK rule #6-9 scenario (for those not familiar with DK rules, see attached). 

    image

    Does she have sickle disease/trait, a clotting disorder (from a hematologic or solid malignancy),  or some hereditary pro-thrombotic issue?  Re: her bipolar disorder, is she on lithium? That could be indirectly prothrombotic (adding to the mess). From an inflammatory standpoint, is it worth doing a gallium scan? Or PET? Any lesions in the chest?



    ------------------------------
    Regards, 
    Kemar E. Green, DO, FANA
    Rare & Complex Disease Neurologist | Neuro-Ophthalmologist | Oto-Neurologist
    Founder & CEO  | NeuroAgent AI, Inc. |HazyEyes, Inc.
    Affiliate Faculty, JHU Data Science & AI Institute

    Former Assistant Professor of Neurology, Johns Hopkins University
    ------------------------------



  • 12.  RE: history of IIH and new optic neuropathy

    Posted 9 days ago
    History of COVID could simply be correlated to both optic neuritis and NA ION but dis she recently had any flu like illness that could be correlated to recent complaint 






  • 13.  RE: history of IIH and new optic neuropathy

    Posted 13 days ago
    You can consider ordering IL6, il2 for sarcoidosis and neuro filament light chain in serum - sensitive marker for ms. 
    Given her demographics, inflammatory and demyelinating are in the differentials. 






  • 14.  RE: history of IIH and new optic neuropathy

    Posted 13 days ago

    Interesting. What is the temporal relationship between the worsening and MRI? Could the gad enhancement be a manifestation of a subacute ischemic event? The worsening upon awakening is particularly interesting. What is the likelihood that the persistent elevated ICP caused a secondary ischemic event overnight due to isolated intracanicular (?watershed zone) ischemia from elevated pressure decreasing arterial flow? Any hx of OSA? Do we know if she was prone, supine, or in the L or R lateral decubitus position the night before the symptoms were discovered?  Any h/o BRCA mutation (and/or non-BRCA breast and/or ovarian Ca)? Any thrombocytosis and/or other cell line elevation? Any hx or signs of acquired or hereditary coagulopathies? I am assuming MRV or CTV head is normal, is the CTV/MRV neck also normal?...Was there any evidence of CSF pleocytosis (if yes, flow? cytopathy?) and/or CSF hyperproteinorachia? 



    ------------------------------
    Regards, 
    Kemar E. Green, DO, FANA
    Rare & Complex Disease Neurologist | Neuro-Ophthalmologist | Oto-Neurologist
    Founder & CEO  | NeuroAgent AI, Inc. |HazyEyes, Inc.
    Affiliate Faculty, JHU Data Science & AI Institute

    Former Assistant Professor of Neurology, Johns Hopkins University
    ------------------------------



  • 15.  RE: history of IIH and new optic neuropathy

    Posted 10 days ago

    she is declining LP.  

    The only other thing I have found in further review of her history is she had PE after COVID infection back in 2022; seems was seen by heme-onc but I cannot find coag workup.  But I am not sure how that would correlate to her current clinical picture? 

    her IIH diagnosis was prior to this event and no venous thrombosis on previous MRV dating back to 2021. 




  • 16.  RE: history of IIH and new optic neuropathy

    Posted 13 days ago
    Hello
    This is Irene Vanek doing IIH at st Michael's in Toronto
    Interesting case
    What exactly is her MRV and CT V
    What is her VEP
    Neuroopth , neurosurgery staff
    Thanks Irene





  • 17.  RE: history of IIH and new optic neuropathy

    Posted 10 days ago

    Her last MRV was May 2025; I did not repeat MRV as I was not suspecting an increased ICP component as the culprit for the sudden vision loss given her other eye has no edema at all and she has no current increased ICP symptoms on diamox and the MRI showing post contrast enhancement which is more consistent with optic neuritis. Thank you! 

    this is the radiology read:

    Head MRV: 
    Dominant flow is seen from the superior sagittal sinus into the right 
    transverse and sigmoid venous sinus. The left transverse and sigmoid venous 
    sinus is small, likely developmental. There may be additional narrowing along 
    the lateral aspect of the left transverse venous sinus. On the right side 
    where there is no significant narrowing, the overall caliber of the lateral 
    transverse venous sinus and sigmoid venous sinus is somewhat small relative to 
    the jugular bulb and proximal transverse venous sinus. This decrease in 
    caliber is also more pronounced when compared to the preceding study from 
    7/24/2021. The superior sagittal sinus, internal cerebral veins, vein of 
    Galen, straight sinus and adjoining cortical veins are relatively unremarkable 
    for technique. 




  • 18.  RE: history of IIH and new optic neuropathy

    Posted 8 days ago

    The enhancement pattern is too (atypical) extensive for AION, which typically shows confined optic disc enhancement. What about DWI? How well is her weight being managed?

    Given the possibility of false-negative results, it is reasonable to repeat serum AQP4 and MOG (live CBA). 

    Best,



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