OMG, I Can't C

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Identifier walsh_2013_s4_c5
Title OMG, I Can't C
Creator Boisvert, Chantal J; Sand, Daniel; D'Auria, Andrea; Quiros, Peter A
Subject Vision loss; Headaches; Lumbar puncture
Presenting Symptom A 46 year old Hispanic male presented to the ER with progressive visual loss over 1 week in both eyes to the point where he could only see light.
History Headaches for the past 8 months that became more severe in the last few weeks. They were pressure like, came and went, started occipital and radiated to the front. He was able to get initial relief with ibuprofen, but for the past week it didn't help. His past medical history was significant for DM, HTN and hyperlipidemia. He reported being treated for several months for rheumatoid arthritis 8 years ago.
Clinical On exam he was hand motion OD and NLP OS. His pupils were sluggish (os more than od) but round and reactive with a trace APD OS. IOP was normal as was extraocular motility. Slit lamp exam showed nuclear sclerotic cataracts OU and DFE showed non proliferative diabetic retinopathy. He was admitted for work up. The following labs were negative/normal: SS-A, SS-B, thyroid function, homocysteine, fasting lipid panel, liver panel, coags, toxicology screen, HIV, RPR, rheumatoid factor, anti-DS DNA; Positive/Eleveated labs included: ESR 115, CRP 66.8, ANA (positive nuclear pattern 1:80). MRI brain/orbits showed diffuse enhancement of the dura with apparent thickening greater on the left side. In addition bilateral enhancement of the posterior aspect of the intraconal optic nerve sheath was seen.
Neuroimaging CT Scan; X-Ray; MRI
Pathology On exam he still had a trace APD OS and his color plates were 1/8 OU. More CSF studies returned showing an elevated IgG synthesis rate (31.1mg/24hr), a high IgG index (1.09) and normal albumin (20.9). A dural biopsy showed pachymeningitis with increased IgG4 plasma cells. The patient was discharged on PO prednisone 60 mg with follow up. IgG4 pachymeningitis appears to be one of the manifestations in the newly recognized IgG4-related disease spectrum.
Disease/Diagnosis IgG4 pachymeningitis
Treatment Steroid; Glucocorticoid Sparing Agents; Rituximab
References Attached
Relation is Part of Case presented at the NANOS 2013 Walsh Session, February 10
Contributor Primary Thurtell, Matthew J
Contributor Secondary Van Stavern, Gregory P
Publisher Spencer S. Eccles Health Sciences Library, University of Utah
Date 2013-02-10
Type Text
Format application/pdf
Source 45th Annual Frank Walsh Society Meeting
Holding Institution North American Neuro-Ophthalmology Association (NANOS), Copyright 2013. For further information regarding the rights to this collection, please visit
Collection Neuro-ophthalmology Virtual Education Library: NOVEL
Language eng
ARK ark:/87278/s65q7sn0
Setname ehsl_novel_fbw
Date Created 2013-12-12
Date Modified 2021-05-06
ID 179142
Reference URL