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Sporadic Jakob-Creutzfeldt Disease Presenting as Primary Progressive Aphasia

Posted Feb 25 2013 12:28pm
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
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