OMG, I Can't C

Update Item Information
Identifier walsh_2013_s4_c5
Title OMG, I Can't C
Creator Chantal J. Boisvert; Daniel Sand; Andrea D'Auria; Peter A. Quiros
Affiliation (CJB) (DS) (PAQ) USC-LAC / Doheny Eye Institute, Los Angeles, CA; (AD) USC-LAC/Department of Pathology, Los Angeles, CA
Subject Vision loss; Headaches; Lumbar puncture
Description A repeat ophthalmic exam two days after presentation showed vision to be hand motion OU. CSF analysis showed 1 RBC, 21 WBC, lymph 80%, glucose 90 (serum 164), protein 44 and negative cytology as well as no organisms or host cells on gram stain. The decision was made to start IV solumedrol to salvage vision. Other CSF studies that were negative included: CMV PCR, CMV IgG/IgM, crypto Ag, HSV, RPR and FTA-ABS. Chest-Xray was normal, PPD was negative. The patient also reported scrotal pain and a rash on his legs. Biopsy of the rash and a scrotal ultrasound were non-revealing. MRI T- and C-Spine were normal as well. Four days after initiation of IV methylprednisolone the patient's vision improved to 20/20 OU. 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. This disease was first recognized in 2001 in sclerosing pancreatitis and in 2003 as a systemic condition. At this point in time there are only a few papers describing reports on the rare presentation of involvement of the CNS, with little information being available about clinical presentation, diagnostic criteria and optimal treatment. The treatment modalities available are based on Class IV evidence. Most of the information is currently being extrapolated from the more common presentation of autoimmune pancreatitis and include initial treatment with IV steroids, followed by glucocorticoid sparing agents and/or rituximab in the case of refractory disease.
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.
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
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.
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.
Neuroimaging CT Scan; X-Ray; MRI
Treatment Steroid; Glucocorticoid Sparing Agents; Rituximab
Date 2013-02
References 1. Shapiro KA, Bové RM, Volpicelli ER, Mallery RM, Stone JH. Relapsing course of immunoglobulin G4- related pachymeningitis. Neurology. 2012 Aug 7;79(6):604-6. doi: 10.1212/WNL.0b013e31826356fc. Epub 2012 Jul 25. 2. Della Torre E, Bozzolo EP, Passerini G, Doglioni C, Sabbadini MG. IgG4-related pachymeningitis: evidence of intrathecal IgG4 on cerebrospinal fluid analysis. Ann Intern Med. 2012 Mar 6;156(5):401-3. 3. Riku S, Hashizume Y, Yoshida M, Riku Y. [Is hypertrophic pachymeningitis a dural lesion of IgG4-related systemic disease?]. [Article in Japanese] Rinsho Shinkeigaku. 2009 Sep;49(9):594-6. 4. Chan SK, Cheuk W, Chan KT, Chan JK. IgG4-related sclerosing pachymeningitis: a previously unrecognized form of central nervous system involvement in IgG4-related sclerosing disease. Am J Surg Pathol. 2009 Aug;33(8):1249-52.
Language eng
Format application/pdf
Type Text
Source 45th Annual Frank Walsh Society Meeting
Relation is Part of NANOS Annual Meeting 2013
Collection Neuro-Ophthalmology Virtual Education Library: Walsh Session Annual Meeting Archives: https://novel.utah.edu/Walsh/
Publisher North American Neuro-Ophthalmology Society
Holding Institution Spencer S. Eccles Health Sciences Library, University of Utah
Rights Management Copyright 2013. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright
ARK ark:/87278/s65q7sn0
Setname ehsl_novel_fbw
ID 179142
Reference URL https://collections.lib.utah.edu/ark:/87278/s65q7sn0