| Identifier |
walsh_2019_s3_c2-slides |
| Title |
Not the Rolling Stones |
| Creator |
Ari Shemesh; Anthony Brune; Lilja Solnes; Avindra Nath; John Probasco; Daniel Gold |
| Affiliation |
(AS) (AB) (JP) (DG) Department of Neurology, Johns Hopkins School of Medicine, Baltimore, Maryland; (JS) Department of Radiology, Johns Hopkins School of Medicine, Baltimore, Maryland; (AN) National Institute of Health, Bethesda, Maryland |
| Subject |
Vertigo; Eye Movements; Cerebellum; Creutzfeld-Jacob Disease |
| History |
A 68-year-old woman presented to the clinic complaining of "bouncing" vision when waking up at night for one month. Two years ago, she experienced discrete episodes of spinning for seconds provoked by head movements, which resolved spontaneously. Her ocular motor and vestibular exams were normal aside from apogeotropic nystagmus (brought on by roll more than Dix-Hallpike testing), most intense in right roll test (Figure 1). The general neurological examination was unremarkable except for mild difficulty in tandem gait. She was diagnosed with apogeotropic horizontal canal (HC) benign paroxysmal positional vertigo (BPPV) and underwent treatment with repositioning maneuvers and head-shaking in the clinic and with vestibular PT, although there was no change in symptoms or nystagmus. Given refractory positional nystagmus, contrast-enhanced MRI of the brain was ordered and was unremarkable. Over the next several weeks, balance worsened, positional symptoms persisted, and dizziness was experienced constantly without provocation. Examination was now notable for gaze-evoked with rebound nystagmus, saccadic smooth pursuit and VOR suppression, horizontal head shaking induced downbeat nystagmus and ataxic gait. Positional apogeotropic nystagmus was still present. Paraneoplastic panel was unrevealing (ANNA-1/2/3, PCA-1/2/Tr, Amphiphysin, CRMP-5, VGKC, VGCC, GAD-65, Ma/Ta and ZIC4), as was CT chest, abdomen and pelvis and other labs to investigate a progressive cerebellopathy (negative/normal CRP, ESR, Vitamins B1/B12/ E, Cu, celiac panel, anti-TPO, HIV and +ANA titer of 1:320, which was a chronic finding). Given continued deterioration, she was admitted for expeditious LP and PET scan. Repeat contrast-enhanced brain MRI was normal. Lumbar puncture demonstrated normal WBC, protein and glucose levels. Extensive CSF studies were negative including: gram stain, Cryptococcus, VDRL, HSV/CMV/VZV PCR, WNV-IgG, Mycobacteria/Whipple's PCR, Oligoclonal IgG, Autoimmune panel, Cytology and Flow cytometry. She underwent whole-body FDG-PET that was notable for symmetric cerebellar hypometabolism (Figure 2). Further diagnostic testing was performed. |
| Disease/Diagnosis |
Sporadic Creutzfeldt-Jakob disease (sCJD) |
| Date |
2019-03 |
| References |
1. Seung-Han Lee, Sang-Jun Park, Jae-Hwan Choi, Tai-Seung Nam, Central Positional Nystagmus Associated with Cerebellar Tumors, Neurology, Volume 86, 16 Supplement, 2016. 2. Jeong-Yoon Choi, Ji Hyun Kim, Hyo Jung Kim, Stefan Glasauer, Ji-Soo Kim, Central Paroxysmal Positional Nystagmus - Characteristics and Possible Mechanisms, Neurology, Volume 84, Page 2238-46, 2015 3. Aaron Foutz, Brian S. Appleby, Clive Hamlin, Xiaoqin Liu, Sheng Yang et al, Diagnostic and Prognostic Value of Human Prion Detection in Cerebrospinal Fluid. Annals of Neurology, Volume 81, Page 79-92, 2017. 4. Brian S. Appleby, Kristin K. Appleby, Peter V. Rabins, Does The Presentation of Creutzfeldt-Jakob Disease Vary By Age of Presumed Etiology? A meta-analysis of the past 10 years, The Journal of Neuropsychiatry and Clinical Neurosciences, Volume 19, Page 428-435, 2007. |
| Language |
eng |
| Format |
application/pdf |
| Format Creation |
Microsoft PowerPoint |
| Type |
Text |
| Source |
2019 North American Neuro-Ophthalmology Society Annual Meeting |
| Relation is Part of |
NANOS Annual Meeting Frank B. Walsh Sessions; 2019 |
| 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 2019. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK |
ark:/87278/s65q9d9q |
| Setname |
ehsl_novel_fbw |
| ID |
1431988 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s65q9d9q |
