| Identifier |
walsh_2018_s1_c1-2 |
| Title |
Whipping Up a New Flavor - Slides |
| 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 |
| History |
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. |
| Disease/Diagnosis |
Chronic Dengue Encephalitis |
| 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 |
2018-05 |
| References |
Kutiyal A, Malik C, Hyanki G. Dengue Haemorrhagic Encephalitis: Rare Case Report with Review of Literature. Journal of Clinical and Diagnostic Research. 11(7):10-12, 2017 Varatharaj A. Encephalitis in the clinical spectrum of Dengue infection. Neurology India. 58(4):585-591, 2010 Solomon R, Dung N, Vaughn D, et al. Neurological manifestations of Dengue infection. Lancet. 355(9209):1053-1059, 2000 Penn R, Guarner J, Sejvar J, et al. Persistent neuroinvasive West Nile virus infection in an immunocompromised patient. Clinical Infectious Diseases. 42(5):680-683, 2006 |
| Language |
eng |
| Format |
application/pdf |
| Type |
Text |
| 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 - Walsh Session Annual Meeting Archives https://novel.utah.edu/Walsh/index3.html |
| Publisher |
Spencer S. Eccles Health Sciences Library, University of Utah |
| Holding Institution |
Spencer S. Eccles Health Sciences Library, University of Utah, 10 N 1900 E SLC, UT 84112-6053 |
| Rights Management |
Copyright 2018. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright |
| ARK |
ark:/87278/s65j1kj5 |
| Setname |
ehsl_novel_fbw |
| ID |
1326570 |
| Reference URL |
https://collections.lib.utah.edu/ark:/87278/s65j1kj5 |
