Whipping Up a New Flavor - Video

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Identifier walsh_2018_s1_c1
Title Whipping Up a New Flavor - Video
Creator Meagan Seay, Tory Johnson, Benjamin Larman, Avindra Nath, Myoung-Hwa Lee, Janet Rucker, Jonathan Howard, Ilya Kister, Matija Snuderl, Laura Balcer, Steven Galetta
Subject Progressive Supranuclear Palsy; Infection; Whipple's Disease; Neuro-Degenerative Disease
Presenting Symptom A 43-year old Indian man presented with two years of progressive gait imbalance, dysarthria, and cognitive decline. Neurological exam revealed supranuclear gaze palsy with impaired downgaze and loss of OKN quick phases, impaired horizontal pursuit, ideomotor apraxia, facial and arm dystonia, foot and toe chorea, hyperreflexia, retropulsion, and gait ataxia. Neuropsychological testing suggested subcortical dementia. Brain MRI revealed multiple nonenhancing, confluent, periventricular and juxtacortical T2 hyperintensities and severe atrophy with a hummingbird sign. A paraneoplastic panel, GAD antibodies, and NMDA antibodies were negative. CSF revealed normal white blood cell count, elevated protein (81), markedly elevated IgG index and synthesis, 15 oligoclonal bands, negative paraneoplastic panel, negative Whipple's DNA PCR, and negative CJD testing (negative tau quantity and ambiguous 14-3-3, not supporting the diagnosis of CJD). Body PET-CT and EEG were normal. Treatment with high dose steroids, IVIG, and plasmapheresis were ineffective. Brain biopsy showed a hypercellular cortex and white matter with spongiosis, gliosis, and diffuse inflammatory parenchymal and meningeal infiltrate. The cortex contained numerous inflammatory nodules with neuronophagia. Overall the features were nonspecific and most suggestive of an inflammatory meningoencephalitis, such as a viral infection, autoimmune disease, or paraneoplastic process. The patient continued to deteriorate and died from sepsis six years after disease onset. An autopsy was performed.
Date 2018-03
Language eng
Format video/mp4
Source 2018 North American Neuro-Ophthalmology Society Annual Meeting
Relation is Part of NANOS Annual Meeting 2018: Frank B. Walsh Session 1
Collection Neuro-ophthalmology Virtual Education Library: NOVEL http://NOVEL.utah.edu
Publisher Spencer S. Eccles Health Sciences Library, University of Utah
Holding Institution North American Neuro-Ophthalmology Association. NANOS Executive Office 5841 Cedar Lake Road, Suite 204, Minneapolis, MN 55416
Rights Management Copyright 2018. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright
ARK ark:/87278/s6fb95h6
Contributor Primary Meagan Seay, DO
Setname ehsl_novel_fbw
ID 1320239
Reference URL https://collections.lib.utah.edu/ark:/87278/s6fb95h6
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