| Identifier |
walsh_2013_s4_c5-1 |
| 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 |
| 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 |
| Format Creation |
Microsoft PowerPoint |
| Type |
Text |
| Source |
45th Annual Frank Walsh Society Meeting |
| Relation is Part of |
NANOS Annual Meeting Frank B. Walsh Sessions; 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/s6pk3cr4 |
| Setname |
ehsl_novel_fbw |
| ID |
179169 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s6pk3cr4 |
