| Identifier |
walsh_2016_s2_c2 |
| Title |
Many Small Lesions, One Big Problem - Abstract |
| Creator |
Harsh V. Gupta; Tuhin Virmani; Rohan Samant; Murat Gokden; Joseph G. Chacko; Sarkis M. Nazarian |
| Affiliation |
(HVG) (TV) (JGC) (SMN) UAMS, Neurology Little Rock, AR; (JGC) (SMN) UAMS, Ophthalmology Little Rock, AR; (RS) UAMS, Radiology Little Rock, AR; (MG) UAMS, Pathology Little Rock, AR |
| Subject |
Autoimmune Diseases; CNS Vasculitidies; Abducens Nerve Palsy; Upbeat Nystagmus |
| Description |
The differential diagnosis, based on the radiological findings of punctate and linear lesions suggesting perivascular space involvement, included Neuro-Behcet disease, primary CNS vasculitis, lymphomatoid granulomatosis, Erdheim-Chester disease, CNS lymphoma, neurosarcoidosis, paraneoplastic encephalitis and chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). A double-dose contrast MRI study revealed bilateral fronto-parietal and right occipital lesions, and a right frontal lesion was biopsied. Histopathology revealed lymphocytic and histiocytic perivascular infiltration, primarily with CD3+ T-lymphocytes and lesser numbers of CD20+ B-lymphocytes. No vasculitis, giant cells, or granulomas were found. Stains for viral, fungal, and acid-fast organisms were negative. No myelin loss was demonstrated. The patient was given a 5-day steroid pulse, and discharged on prednisone 60 mg daily. After one month of therapy, his gait ataxia and dysphagia improved, though his visual complaints were unchanged. His diplopia responded to prism glasses. |
| History |
A 73-year old hypertensive and hyperlipidemic man presented with 18 months of horizontal diplopia, worse at distance and gaze right, oscillopsia on left head turn, and unsteady gait. Six months prior to consult, he developed dysphagia, worse with liquids, and recurrent falls forced him to use a walker. He had memory impairment for three months. On exam, MoCA testing revealed score of 20/30, with both language and visuo-spatial deficits. Visual acuity was 20/40 OD, 20/30- OS. Mydriatic pupil OS, due to old injury, was present; no RAPD was found. Color plates were normal. Visual fields were full to confrontation. Left ptosis from old inury was present.He had nuclear sclerotic cataracts. Optic discs were pink & flat, with 0.2 cup/disc ratio. EOM revealed partial right abduction deficit, comitant left hypertropia, and upbeat nystagmus. Motor and sensory exams were unremarkable. He had dysmetria in his upper extremities, left worse than right. He had an ataxic gait, with tendency to fall to the left, and could not tandem walk. MRI of the brain showed numerous contrast-enhancing lesions, hyperintense on FLAIR, widely distributed in the brainstem, basal ganglia, cerebral and cerebellar hemispheres, in patterns suggesting perivascular localization. CSF analysis revealed elevated protein at 69 mg/dl, with 7 leukocytes per cmm. CSF cultures, cytology, and demyelination studies were all negative. No malignancies were discovered by CT scans of the chest, abdomen, and pelvis, or by whole-body PET scanning. A battery of autoimmune markers was negative. Vitamin E and B12 levels were normal. Lactic dehydrogenase, beta 2 microglobulin, ANCA, and HIV testing were all normal. Serum IgE was elevated, and absolute CD4 and CD8 counts were roughly at a third of low normal, and the CD4:CD8 ratio was high. A diagnostic procedure was performed. |
| Disease/Diagnosis |
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). |
| Date |
2016-02 |
| References |
1. Pittock SJ, Debruyne J, Krecke KN, et al. Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS ). Brain 133:2026-2034, 2010. 2. Dudesek A, Rimmele F, Tesar S, et al. CLIPPERS : Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids: review of an increasingly recognized entity within the spectrum of inflammatory central nervous system disorders. Clin Exp Immunol 175:385-396, 2014. 3. Taieb G, Duflos C, Renard D, et al. Long-term outcomes of CLIPPERS (Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) in a consecutive series of 12 patients. Arch Neurol 69:847-855, 2012. |
| Language |
eng |
| Format |
application/pdf |
| Type |
Text |
| Source |
48th Annual Frank Walsh Society Meeting |
| Relation is Part of |
NANOS Annual Meeting 2016 |
| 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 2016. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK |
ark:/87278/s6rf8rng |
| Setname |
ehsl_novel_fbw |
| ID |
179362 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s6rf8rng |
