| Identifier |
walsh_2022_s3_c6 |
| Title |
The Grey Area of White Matter |
| Creator |
Parker Bohm; Leanne Stunkel; Gregory Van Stavern |
| Affiliation |
(PB) (LS) (GV) Washington University in St. Louis, St. Louis, Missouri |
| Subject |
sarcoidosis, JC virus, magnetic resonance imaging (MRI), autoimmune diseases |
| History |
A 76 year-old man presented with two months of progressive confusion, vision loss, and left-sided weakness. His medical; history was notable for hypothyroidism, type 2 diabetes mellitus, coronary artery disease, basal cell carcinoma of the; forehead status-post excision, deep vein thrombosis, and several years of recurrent dyspnea. Two months prior to neuroophthalmology; evaluation, he was hospitalized for dyspnea, and lung biopsy revealed non-necrotizing granulomas.; Corticosteroids were initiated for pulmonary sarcoidosis. He was readmitted 1 month later for dyspnea after corticosteroid; taper and complained of difficulty reading text messages. Neurologic examination revealed a right homonymous; hemianopia. MRI brain revealed multifocal T2 hyperintensities without enhancement, and CSF evaluation demonstrated; isolated elevated protein of 77 with negative cytology and oligoclonal bands. MRI cervical spine showed two chronic nonenhancing; cord lesions. Five doses of intravenous methylprednisolone 1g daily were administered; he was discharged on; prednisone 20mg without improvement in neurologic symptoms. At his neuro-ophthalmology consultation two weeks after; discharge, he had developed worsening left hemiparesis, inability to read, and needed assistance with daily activities.; Examination was most pertinent for a generalized encephalopathy, visual acuity of only 20/90 OD and 20/100 OS with; single optotypes, right homonymous hemianopia, inability to draw a clock, and perseveration. Within 1 week of evaluation,; he was hospitalized due to falls, incontinence, and left hemiparesis. MRI brain in November demonstrated increased size of; the multifocal T2 hyperintensities without enhancement. Broad serum work-up was notable for positive ANA 1:160; (homogenous pattern) and P-ANCA (negative on repeat testing), mildy elevated ACE, and low CD3/CD8 count. CSF; evaluation showed protein of 66, but was otherwise normal, including flow cytometry, JC virus PCR, and autoimmune; encephalopathy panel. PET CT skull to thigh showed reduced FDG uptake corresponding with MRI brain lesions and no; areas of increased FDG uptake. A diagnostic procedure was performed. |
| Disease/Diagnosis |
Progressive multifocal leukoencephalopathy |
| Date |
2022-02 |
| References |
1 Cortese, Reich, Nath, Progressive Multifocal Leukoencephalopathy and the Spectrum of JC Virus-related Disease, Nat; Rev Neurol, 17(1):37-51, 2021.; 2 Davis, Khan, Royal 3rd, Progressive Multifocal Leukoencephalopathy as the First Manifestation of Occult Sarcoidosis:; Case Report and Review of the Literature, Neurologist, 19(1):26-9, 2013.; 3 Henegar et al., Progressive Multifocal Leukoencephalopathy in Patients with Systemic Lupus Erythematosus: a; Systematic Literature Review, Lupus, 25(6):617-26, 2016.; 4 Askamit, Review of Progressive Multifocal Leukoencephalopathy and Natalizumab, Neurologist, 12(6):293-8, 2006.; 5 Berger et al., PML Diagnostic Criteria: Consensus Statement from the AAN Neuroinfectious Disease Section, Neurology,; 9;80(15):1430-8, 2013.; 6 Cortese, Muranski, Enose-Akahata, Ha, Smith et al, Pembrolizumab Treatment for Progressive Multifocal; Leukoencephalopathy, New England Journal of Medicine, 380(17):1597-1605, 2019. |
| Language |
eng |
| Format |
application/pdf |
| Type |
Text |
| Source |
54th Annual Frank Walsh Society Meeting |
| Relation is Part of |
NANOS Annual Meeting Frank B. Walsh Sessions; 2022 |
| 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 2022. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK |
ark:/87278/s6trrr61 |
| Setname |
ehsl_novel_fbw |
| ID |
2100244 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s6trrr61 |
