| Identifier |
walsh_2020_s2_c5-pdf |
| Title |
Well Here Is Another Uveo-Meningeal Syndrome You Might Muck Up (PDF) |
| Creator |
David DeLeon; Mariel Rojas; Francisco Sanchez; Rosa Tang; Julie Patel; Jade Schiffman |
| Affiliation |
(DD) (MR) (FS) (RT) (JS) Neuro-Eye Clinical Trials Inc., Houston, Texas; (JP) Allergy and Rheumatology Specialists of Houston, Houston, Texas |
| Subject |
Increased Intracranial Pressure, Meningo-Uveitis, Neurologic Disorders, Hearing Loss, Monoclonal Antibody Therapy |
| History |
A 16 y/o Caucasian female with a BMI of 24, went for an eye exam for blurry vision which corrected with refraction, however bilateral disc edema was found. There was a 4-year history of headaches that were intermittent and escalating in nature around 5 days a week, about 1 hour after awakening, and 50% became severe (9/10) lasting about 2 hours. Only during headaches did she note pulsatile tinnitus, and when severe headaches, light sensitivity and nausea. TVO's occurred when standing up quickly. Family believed allergies resulted in her headaches, as she had longstanding intermittent rashes which at times were pruritic and red eyes without photophobia or pruritus. Fatigue, recently developed, such that the patient slept all day/night following school and had to be awakened to eat. Despite this she was an honor student, but had to discontinue PE classes and cheerleading and felt winded with walking, despite a normal chest X-ray. Furthermore, patient noted puffiness and aching in hands, knees and ankles. A school screening test uncovered a bilateral hearing loss, and no obvious change over years. On exam patient had a 20/20 OU vision, normal IOP, pupils were equal, no APD, with slight light near dissociation. Her slit lamp exam showed diffuse conjunctival injection, subtle subepithelial/anterior stromal corneal opacities inferiorly. Anterior chamber had rare cell and 1+ flare OU, anterior vitreous had 1-2+ cell OU. Bilateral chronic appearing disc edema (Frisen 3-4 ) was present, with no obvious retinal, choroid or vascular process after examination with ICG and FA, the later showing disc leakage. VF minor nasal depression. OCT showed bilateral thickening NFL. MRI, MRA/MRV were normal. LP demonstrated an OP of 44cm H20, WBC 60 (mostly PMN) Patient's work-up was negative for infectious/autoimmune serology despite very elevated inflammatory markers. Diagnosis and management with disappearance of symptoms ensued. |
| Disease/Diagnosis |
Uveomeningeal Syndrome with Raised Intracranial Pressure due to Muckle Wells Syndrome, the later also explaining rashes, red eyes, hearing loss, fatigue. |
| Date |
2020-03 |
| References |
i Terreri MTR, Bernardo WM, Len CA, Silva CAAD, Magalhães CMRD, Sacchetti SB, et al. Guidelines for the management and treatment of periodic fever syndromes. Revista Brasileira de Reumatologia (English Edition). 2016;56(1):44-51. ii Kitley JL, Lachmann HJ, Pinto A, Ginsberg L. Neurologic manifestations of the cryopyrin-associated periodic syndrome. Neurology. 2010;74(16):1267-70. iii Oberg TJ, Vitale AT, Hoffman RO, Bohnsack JF, Warner JE. Cryopyrin-associated Periodic Syndromes and the Eye. Ocular Immunology and Inflammation. 2013Oct;21(4):306-9.\ |
| Language |
eng |
| Format |
application/pdf |
| Type |
Text |
| Source |
2020 North American Neuro-Ophthalmology Society Annual Meeting |
| Relation is Part of |
NANOS Annual Meeting Frank B. Walsh Sessions; 2020 |
| 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 2020. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK |
ark:/87278/s6sz1qzx |
| Setname |
ehsl_novel_fbw |
| ID |
1546637 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s6sz1qzx |
