M & M Conference (Not on Morbidity and Mortality)

Update Item Information
Identifier walsh_2004_s1_c4
Title M & M Conference (Not on Morbidity and Mortality)
Creator William D. Freeman, MD; Paul W. Brazis, MD; Frank A. Rubino, MD; Dennis Dickson, MD; Steven Weindling, MD; David Kostick, MD; Elizabeth Shuster, MD; David R. Chabolla, MD
Affiliation Jacksonville, FL
Subject Diplopia; Creutzfeldt-Jakob Syndrome; PrPSc Proteins; Prions; Paresis; Aphasia; Postural Balance; Ocular Motility Disorders; Primary Progressive Nonfluent Aphasia
History A 76-year old female with intermittent horizontal diplopia and balance difficulties.
Pathology Protease-resistant scrapie protein consistent with prion disease
Disease/Diagnosis Creutzfeldt-Jakob Disease (sporadic)-MM1 type
Clinical VA: 20/30 OU; Color plates: 8.5/10 OD, 9/10 OS; 3+ nuclear sclerotic cataracts OU
Presenting Symptom Diplopia; Difficulties with balance
Neuroimaging MRI
Treatment Anticonvulsants; Steroids
Date 2004
References 1. National Prion Disease Pathology Surveillance Center Report. 2. Parchi P, Giese A, Capellari S, Brown B, Schulz-Schaeffer W, Windl O, et al. Classification of Sporadic Creutzfeldt-Jakob Disease Based on Molecular and Phenotypic Analysis of 300 Subjects. Annals of Neurology. 46:224-233. 3. Yokota T, Tsuchiya K, Yamane M, Hayashi M, Tanabe H, Tsukagoshi H. Geotropic ocular deviation with skew and absence of saccade in Creutzfelt-Jakob disease. Journal of the Neurological Sciences. 106(2):175-8, 1991 Dec. 4. Helmchen C, Buttner U. Centripetal nystagmus in a case of Creutzfeldt-Jakob disease. Neuro-ophthalmology 1995; 15:187-192. 5. Brazis, PW, Biller, J, Masdeu, JC. Localization in Clinical Neurology. Fouth Edition. LWW. 2001. 6. Grant MP, Cohen M, Petersen RB, Halmagyi GM, McDougall A, Tusa RJ, Leigh RJ. Abnormal ye movements in Creutzfeldt-Jakob Disease. Annals of Neurology. 34(2):192-7, 1993 Aug. 7. Bertoni JM, Brown P, Goldfarb LG, Rubenstein R, Gajdusek DC. Familial Creutzfeldt-Jakob Disease (codon 200 mutation) with supranuclear palsy.JAMA. 268(17):2413-5. 8. Johnson, RT, Gibbs, CJ. Creutzfeldt-Jakob Disease and Related Transmissible Spongiform Encephalopathies. New England Journal of Medicine. Vol 339(27): 1994-2004. 9. Beaudry P, Cohen P, Brandel JP, et al. 14-3-3 Protein, neuron-specific enolase, and the S-100 protein in cerebrospinal fluid of patients with Creutzfeldt-Jakob Disease. Dement Geriatr Cogn Disord. Vol.10:40-46. 10. Hsich G, Kenney K, Gibbs CH, Lee KH, Harrington MG. The 14-3-3 brain protein in cerebrospinal fluid as a marker for transmissible spongiform encephalopathies. New England Journal of Medicine. Vol 335: 924-930. 11. Poser, S, Mollenhauer B, Kraubeta A, et al. How to improve the clinical diagnosis of Creutzfeldt-Jakob Disease. Brain. Vol.122:2345-2351. 12. Geschwind MD, Martindale J, Miller D, DeArmond SJ, Uyehara-Lock J, Gaskin D, et al. Challenging the Clinical Utility of the 14-3-3 Protein for the Diagnosis of Sporadic Creuztfeldt-Jakob Disease. Archives of Neurology. Vol. 60: 813-816. 13. Aksamit AJ. Cerebrospinal Fluid 14-3-3 Protein: Variability of Sporadic Creutzfeldt-Jakob Disease, Laboratory Standards, and Quantitation. Archives of Neurology. Vol. 60: 803-804. 14. Schroter A, Zerr I, Henkl K, et al. Magnetic Resonance Imaging in the Clinical Diagnosis of Creutzfeldt-Jakob Disease. Archives of Neurology. Vol. 57: 1751-1757. 15. Vrancken AFJE, Frijns CJM, Ramos LMP. FLAIR MRI in sporadic Creutzfeldt-Jakob Disease. Neurology. Vol 55: 147. 16. Demaerel P, Heiner L, Robberecht W, Sciot R, Wilms G. Diffusion-weighted MRI in sporadic Creutzfeldt-Jakob Disease. Neurology. Vol 52: 205-207. 17. Urbach H, Klisch J, Wolf HK, et al. MRI in sporadic Creutzfeldt-Jakob Disease: Correlation with clinical and neuropathological data. Neuroradiology. Vol 40:65-70. 18. Levy, SR, Chiappa, KH, Burke, CJ, Young, RR. Early Evolution and Incidence of Electroencephalographic Abnormalities in Creutzfeldt-Jakob Disease. Journal of Clinical Neurophysiology. 3 (1):1-21.
Language eng
Format application/pdf
Type Text
Source 30th Annual North American Neuro-Ophthalmology Society Meeting, Frank B. Walsh Session CD-ROM
Relation is Part of NANOS Annual Meeting Frank B. Walsh Sessions; 2004
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 2008. For further information regarding the rights to this collection, please visit: https://NOVEL.utah.edu/about/copyright
ARK ark:/87278/s6qc0660
Setname ehsl_novel_fbw
ID 177581
Reference URL https://collections.lib.utah.edu/ark:/87278/s6qc0660