Observation|Feb 2013
Sporadic Jakob-Creutzfeldt Disease Presenting as Primary Progressive
Aphasia
David Y. Johnson, MD; Diana L. Dunkelberger, MA; Maya Henry, PhD; Aissatou
Haman, MD; Michael D. Greicius, MD, PhD; Katherine Wong, BA; Stephen J.
DeArmond, MD, PhD; Bruce L. Miller, MD; Maria Luisa Gorno-Tempini, MD, PhD;
Michael D. Geschwind, MD, PhD
JAMA Neurol. 2013;70(2):254-257. doi:10.1001/2013.jamaneurol.139.
ABSTRACT
Objective
To report the clinical, neuropsychological, linguistic,
imaging, and neuropathological features of a unique case of sporadic
Jakob-Creutzfeldt disease in which the patient presented with a logopenic
variant of primary progressive aphasia.
Design Case report.
Setting
Large referral center for atypical memory and aging disorders,
particularly Jakob-Creutzfeldt disease.
Patient
Patient presenting with logopenic variant primary progressive
aphasia initially thought to be due to Alzheimer disease.
Results
Despite the long, slow 3.5-year course, the patient was shown to
have pathology-proven sporadic Jakob-Creutzfeldt disease.
Conclusions
These findings expand the differential of primary progressive
aphasia to include prion disease.
Saturday, January 22, 2011
Alzheimer's, Prion, and Neurological disease, and the misdiagnosis there
of, a review 2011
Monday, August 20, 2012
CASE REPORTS CREUTZFELDT-JAKOB DISEASE: AN UNDER-RECOGNIZED CAUSE OF
DEMENTIA
see the Duke, Pa, Yale, and Mexican study here, showing the misdiagnosis
of CJD TSE prion disease as Alzheimers ;
Saturday, October 13, 2012
On the issue of transmissibility of Alzheimer disease: A critical review
Friday, February 15, 2013
Scottish TSE Network November Symposium Announcement Event: 12 November
2012 Title: Is Alzheimer’s Disease a transmissible disease? SUMMARY
Thursday, February 21, 2013
National Prion Disease Pathology Surveillance Center Cases Examined January
16, 2013
Letters|February 14, 2001
Diagnosis and Reporting of Creutzfeldt-Jakob Disease
Terry S. Singeltary, Sr
JAMA. 2001;285(6):733-734.
doi:10-1001/pubs.JAMA-ISSN-0098-7484-285-6-jlt0214.
To the Editor: In their Research Letter, Dr Gibbons and colleagues1
reported that the annual US death rate due to Creutzfeldt-Jakob disease (CJD)
has been stable since 1985. These estimates, however, are based only on reported
cases, and do not include misdiagnosed or preclinical cases. It seems to me that
misdiagnosis alone would drastically change these figures. An unknown number of
persons with a diagnosis of Alzheimer disease in fact may have CJD, although
only a small number of these patients receive the postmortem examination
necessary to make this diagnosis. Furthermore, only a few states have made CJD
reportable. Human and animal transmissible spongiform encephalopathies should be
reportable nationwide and internationally.
TSS