Wednesday, February 04, 2009

Creutzfeldt-Jacob disease presenting as severe depression: a case report

Case Report Creutzfeldt-Jacob disease presenting as severe depression: a case report Ethan U Cumbler , Kristin Furfari and Jeannette Guerrasio

Cases Journal 2009, 2:122doi:10.1186/1757-1626-2-122

Published: 4 February 2009

Abstract (provisional)

Background An 81 year old female presented with altered mental status after new onset of severe depression and suicidal ideation with recent psychiatric hospitalization.

Case presentation Key clinical features included muscle rigidity, prominent startle reflex, and rapidly progressing cognitive decline. Initial working hypothesis was serotonin syndrome or neuroleptic malignant syndrome but continued deterioration after medication removal prompted evaluation for alternative etiology. Work-up revealed elevated 14-3-3 CSF protein which suggested the prion disorder which was confirmed on post-mortem examination of brain tissue.

Conclusion While the degree of depression was unusually severe, the case highlights the behavioral and psychiatric manifestations which frequently accompany Creutzfeldt-Jacob disease.

The complete article is available as a provisional PDF.

Saturday, January 24, 2009 Bovine Spongiform Encephalopathy h-BSE ATYPICAL USA 2008 Annual Report Research Project: Study of Atypical Bse

Location: Virus and Prion Diseases of Livestock

2008 Annual Report

Thursday, December 04, 2008 2:37 PM

"we have found that H-BSE can infect humans."

personal communication with Professor Kong. ...TSS

Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518

NOR-98 ATYPICAL SCRAPIE 5 cases documented in USA in 5 different states USA 007


Monday, December 1, 2008 When Atypical Scrapie cross species barriers

Saturday, January 24, 2009

Research Project: Detection of TSE Agents in Livestock, Wildlife, Agricultural Products, and the Environment Location: 2008 Annual Report


Wednesday, January 28, 2009 TAFS1 Position Paper on BSE in small ruminants (January 2009)

The Diagnostic Utility of Brain Biopsy Procedures in Patients With Rapidly Deteriorating Neurological Conditions or Dementia

Josephson SA, Papanastassiou AM, Berger MS, et al. J Neurosurg. 2007;106:72-75. Josephson and colleagues1

Josephson and colleagues1 reviewed case records of all brain biopsy procedures at a tertiary care center in an effort to better characterize the diagnostic sensitivity of brain biopsy procedure for patients with rapidly worsening neurologic conditions, including dementia. Patients with HIV immunosuppression, and known nonlymphomatous tumors were excluded from analysis, resulting in a cohort of 171 identified patients. A surprisingly large number of these patients (n = 90, 53% of the initial cohort) were excluded from subsequent analysis because of the absence of "documentation of a complete history or neurological examination." An additional 15 patients were excluded for other reasons, leaving 64 patients in the final analysis. The most common diagnoses made at biopsy are shown in Table 1. In the group of 10 patients found to have CJD (Table 1), the most common symptom categories included motor/pyramidal (90%), cognitive (80%), behavioral/psychiatric (80%), extrapyramidal (60%), and cerebellar (40%). Nine of the 10 patients had symptoms involving 2 of the first 3 groupings. Prominent individual symptoms included memory loss (50%), confusion/disorientation (50%), depression (40%), headache (40%), and sleep disturbances (40%). Cerebrospinal fluid (CSF) examination was performed in 8 of the 10 patients, and none had a CSF pleocytosis. Overlap of symptoms between patients with CJD and primary CNS lymphoma was high, and it was not clearly possible to separate these groups on clinical, serologic, or CSF criteria alone. Unfortunately, information was not provided as to the neuroimaging findings in these 2 groups, which would likely be distinctive. NIH Public Access Author Manuscript Rev Neurol Dis. Author manuscript; available in PMC 2009 January 2. Published in final edited form as: Rev Neurol Dis. 2007 ; 4(3): 168-172. NIH-PA Author Manuscript NIH-PA Author Manuscript NIH-PA Author Manuscript The authors reported that the overall diagnostic sensitivity of brain biopsy was 65%. However, given the numerous exclusions from analysis, this high sensitivity must be viewed with caution. Prior reports have generally suggested that sensitivity of brain biopsy in neurodegenerative disorders is only 20%.

In this context, a word is in order about the US testing program. After the discovery of the first (imported) cow in 2003, the magnitude of testing was much increased, reaching a level of >400,000 tests in 2005 (Figure 4). Neither of the 2 more recently indigenously infected older animals with nonspecific clinical features would have been detected without such testing, and neither would have been identified as atypical without confirmatory Western blots. Despite these facts, surveillance has now been decimated to 40,000 annual tests (USDA news release no. 0255.06, July 20, 2006) and invites the accusation that the United States will never know the true status of its involvement with BSE.

In short, a great deal of further work will need to be done before the phenotypic features and prevalence of atypical BSE are understood. More than a single strain may have been present from the beginning of the epidemic, but this possibility has been overlooked by virtue of the absence of widespread Western blot confirmatory testing of positive screening test results; or these new phenotypes may be found, at least in part, to result from infections at an older age by a typical BSE agent, rather than neonatal infections with new "strains" of BSE. Neither alternative has yet been investigated.

A New Prionopathy OR more of the same old BSe and sporadic CJD

Communicated by: Terry S. Singeltary Sr.

[In submitting these data, Terry S. Singeltary Sr. draws attention to the steady increase in the "type unknown" category, which, according to their definition, comprises cases in which vCJD could be excluded. The total of 26 cases for the current year (2007) is disturbing, possibly symptomatic of the circulation of novel agents. Characterization of these agents should be given a high priority. - Mod.CP],F2400_P1001_PUB_MAIL_ID:1010,39963

There is a growing number of human CJD cases, and they were presented last week in San Francisco by Luigi Gambatti(?) from his CJD surveillance collection.

He estimates that it may be up to 14 or 15 persons which display selectively SPRPSC and practically no detected RPRPSC proteins.

sporadic Fatal Familial Insomnia


MARCH 26, 2003

RE-Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob

disease in the United States

Email Terry S. Singeltary:


I lost my mother to hvCJD (Heidenhain Variant CJD). I would like to comment on the CDC's attempts to monitor the occurrence of emerging forms of CJD. Asante, Collinge et al [1] have reported that BSE transmission to the 129-methionine genotype can lead to an alternate phenotype that is indistinguishable from type 2 PrPSc, the commonest sporadic CJD. However, CJD and all human TSEs are not reportable nationally. CJD and all human TSEs must be made reportable in every state and internationally. I hope that the CDC does not continue to expect us to still believe that the 85%+ of all CJD cases which are sporadic are all spontaneous, without route/source. We have many TSEs in the USA in both animal and man. CWD in deer/elk is spreading rapidly and CWD does transmit to mink, ferret, cattle, and squirrel monkey by intracerebral inoculation. With the known incubation periods in other TSEs, oral transmission studies of CWD may take much longer. Every victim/family of CJD/TSEs should be asked about route and source of this agent. To prolong this will only spread the agent and needlessly expose others. In light of the findings of Asante and Collinge et al, there should be drastic measures to safeguard the medical and surgical arena from sporadic CJDs and all human TSEs. I only ponder how many sporadic CJDs in the USA are type 2 PrPSc?


Hardcover, 304 pages plus photos and illustrations. ISBN 0-387-95508-9

June 2003

BY Philip Yam


Answering critics like Terry Singeltary, who feels that the U.S. under- counts CJD, Schonberger conceded that the current surveillance system has errors but stated that most of the errors will be confined to the older population.

Diagnosis and Reporting of Creutzfeldt-Jakob Disease Singeltary, Sr et al. JAMA.2001; 285: 733-734. Vol. 285 No. 6, February 14, 2001 JAMA

Diagnosis and Reporting of Creutzfeldt-Jakob Disease

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.

Terry S. Singeltary, Sr Bacliff, Tex

1. Gibbons RV, Holman RC, Belay ED, Schonberger LB. Creutzfeldt-Jakob disease in the United States: 1979-1998. JAMA. 2000;284:2322-2323. FREE FULL TEXT

2 January 2000 British Medical Journal U.S. Scientist should be concerned with a CJD epidemic in the U.S., as well

15 November 1999 British Medical Journal vCJD in the USA * BSE in U.S.

Creutzfeldt Jakob Disease


Sunday, April 20, 2008 Progress Report from the National Prion Disease Pathology Surveillance Center April 3, 2008

Atypical forms of BSE have emerged which, although rare, appear to be more virulent than the classical BSE that causes vCJD.

see full text ;

CJD TEXAS (cjd clusters)

Thursday, January 29, 2009 Medical Procedures and Risk for Sporadic Creutzfeldt-Jakob Disease, Japan, 1999-2008 (WARNING TO Neurosurgeons and Ophthalmologists) Volume 15, Number 2-February 2009 Research

Creutzfeldt-Jakob disease (CJD) update report Emerging Infections/CJD Published on: 12 December 2008

Friday, August 29, 2008


Sunday, March 16, 2008

MAD COW DISEASE terminology UK c-BSE (typical), atypical BSE H or L, and or Italian L-BASE

HUMAN and ANIMAL TSE Classifications i.e. mad cow disease and the UKBSEnvCJD only theory JUNE 2008


Tissue infectivity and strain typing of the many variants Manuscript of the human and animal TSEs are paramount in all variants of all TSE. There must be a proper classification that will differentiate between all these human TSE in order to do this. With the CDI and other more sensitive testing coming about, I only hope that my proposal will some day be taken seriously. ...




The statistical incidence of CJD cases in the United States has been revised to reflect that there is one case per 9000 in adults age 55 and older. Eighty-five percent of the cases are sporadic, meaning there is no known cause at present.

Attending Dr.: Date / Time Admitted : 12/14/97 1228

UTMB University of Texas Medical Branch Galveston, Texas 77555-0543 (409) 772-1238 Fax (409) 772-5683 Pathology Report

FINAL AUTOPSY DIAGNOSIS Autopsy' Office (409)772-2858


I. Brain: Creutzfeldt-Jakob disease, Heidenhain variant.


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