Clinicopathologic characteristics of five autopsied cases of dura mater-associated Creutzfeldt-Jakob disease.

Clinicopathologic characteristics of five autopsied cases of dura mater-associated Creutzfeldt-Jakob disease. Research Abstract Details 

Research Abstract Table of Contents

Jump to the:

Clinicopathologic characteristics of five autopsied cases of dura mater-associated Creutzfeldt-Jakob disease. Abstract Text:

Yasushi Iwasaki,Maya Mimuro,Mari Yoshida,Yoshio Hashizume,Tetsuyuki Kitamoto,Gen Sobue,Yasushi Iwasaki,Maya Mimuro,Mari Yoshida,Yoshio Hashizume,Tetsuyuki Kitamoto,Gen Sobue,

We present five cases of dura mater-associated Creutzfeldt-Jakob disease (dura-CJD) that were analyzed clinicopathologically and review previous reports. The average age at dura mater transplantation was 54.4 +/- 7.3 years, and the average age at CJD onset was 66.0 +/- 8.2 years, with an average latency period of 11.6 +/- 1.1 years. The average age at death was 67.6 +/- 8.7 years, with an average CJD disease duration of 16.8 +/- 10.4 months. Symptoms of CJD onset in four patients who received dura mater transplantation below the cerebellar tent reflected cerebellar or brainstem dysfunction, whereas symptoms of one patient who received transplantation above the cerebellar tent reflected cerebral cortical involvement. All patients showed rapidly progressive cognitive impairment, and both periodic sharp-wave complexes on electroencephalogram and myoclonus were observed in the early disease stage. Neuropathologic evaluation showed one case of subacute spongiform encephalopathy and four cases of panencephalopathic-type CJD. Widespread cerebral neocortical, subcortical gray matter and cerebellar cortical involvement were observed to varying degrees, and severity tended to be associated with CJD disease duration. There were no instances of kuru plaques or florid plaques. Prion protein (PrP) immunostaining showed widespread synaptic-type PrP deposition. No differences between our dura-CJD cases and typical cases of sporadic CJD were found with respect to clinicopathologic findings, except history of dura mater transplantation. Although a specific association between the dura mater graft site and neuropathologic observations was not evaluated in the present study, the initial symptoms appear to be closely related to the graft site, indicating a direct transmission of CJD from the graft site to the adjacent brain.

Clinicopathologic characteristics of five autopsied cases of dura mater-associated Creutzfeldt-Jakob disease. Publishing Authors By Initials

Y Iwasaki,M Mimuro,M Yoshida,Y Hashizume,T Kitamoto,G Sobue,Y Iwasaki,M Mimuro,M Yoshida,Y Hashizume,T Kitamoto,G Sobue,

For similar abstracts research abstracts see: abstracts research

PUBMED ID PMID:

MEDLINE DATE:

Clinicopathologic characteristics of five autopsied cases of dura mater-associated Creutzfeldt-Jakob disease. Journal Published:

PUBLICATION TYPE: Journal Article

Journal: Neuropathology : official journal of the Japanese

VOLUME: 28

Page Numbers: 51-61

Journal Abbreviation:

ISSN: 0919-6544

DAY: 9

MONTH: Feb

YEAR: 2008

Clinicopathologic characteristics of five autopsied cases of dura mater-associated Creutzfeldt-Jakob disease. Information

Number of References:

LANGUAGE: eng

NlmUniqueID: 9606526

Clinicopathologic characteristics of five autopsied cases of dura mater-associated Creutzfeldt-Jakob disease. Keywords Mesh Terms:

KEYWORDS:

MESH TERMS:

Chemical & Substance for Abstract: Clinicopathologic characteristics of five autopsied cases of dura mater-associated Creutzfeldt-Jakob disease. Information

Substance Name:

Registry Number:

Grant and Affiliation Information for Clinicopathologic characteristics of five autopsied cases of dura mater-associated Creutzfeldt-Jakob disease.

AFFILIATION: Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan.

Country: Australia

AGENCY:

GRANT:

ACRONYM:

MEDLINETA: Neuropathology

REFSOURCE:

DATABASENAME:

ACCESSION NUMBER:

Number Hits: 0

Clinicopathologic characteristics of five autopsied cases of dura mater-associated Creutzfeldt-Jakob disease Related Publications

• A message from the New Editor-in-Chief.
• A novel centrosomal ring-finger protein, dorfin, mediates ubiquitin ligase activity.
• CAG repeat size correlates to electrophysiological motor and sensory phenotypes in SBMA.
• Cancer incidence and incidence rates in Japan in 2001 based on the data from 10 population-based cancer registries.
• Cisplatin neurotoxicity presenting as reversible posterior leukoencephalopathy syndrome.
• Clinicopathologic characteristics of five autopsied cases of dura mater-associated Creutzfeldt-Jakob disease.
• Contrast-enhanced 2D cine phase MR angiography for measurement of basilar artery blood flow in posterior circulation ischemia.
• Correlation between multiple phosphorylation of gizzard myosin light chains and actin-activated myosin ATPase activity.
• Cyclosporin A inhibits late steps of T lymphocyte activation after transmembrane signaling.
• Dobutamine stress test unmasks cardiac sympathetic denervation in Parkinson's disease.
• Dorfin localizes to Lewy bodies and ubiquitylates synphilin-1.
• Dorfin localizes to the ubiquitylated inclusions in Parkinson's disease, dementia with Lewy bodies, multiple system atrophy, and amyotrophic lateral sclerosis.
• Dorfin prevents cell death by reducing mitochondrial localizing mutant superoxide dismutase 1 in a neuronal cell model of familial amyotrophic lateral sclerosis.
• Dorfin ubiquitylates mutant SOD1 and prevents mutant SOD1-mediated neurotoxicity.
• Dual roles of myocardin-related transcription factors in epithelial mesenchymal transition via slug induction and actin remodeling.
• Isolation and characterization of a calmodulin binding fragment of chicken gizzard caldesmon.
• Multiple cerebellar infarcts: clinical and pathophysiologic features.
• Nonmyelinating Schwann cell involvement with well-preserved unmyelinated axons in Charcot-Marie-Tooth disease type 1A.
• Physical and functional interaction between Dorfin and Valosin-containing protein that are colocalized in ubiquitylated inclusions in neurodegenerative disorders.
• Pupillary supersensitivity and visual disturbance in Parkinson's disease.
• Pyramidal tract degeneration in sporadic Creutzfeldt-Jakob disease.
• Quantitative determinations of calmodulin in the supernatant and particulate fractions of mammalian tissues.
• Small neurons may be preferentially affected in ganglionopathy.
• The Japan cancer surveillance report: incidence of childhood, bone, penis and testis cancers.
• The video images of sleep attacks in Parkinson's disease.
• Usefulness of combined fractional anisotropy and apparent diffusion coefficient values for detection of involvement in multiple system atrophy.
• [Animal models of neurodegenerative diseases on the road to disease-modifying therapy: spinal and bulbar muscular atrophy]
• [Envisioning quality measurement systems of cancer care]
• [Gene expression profile of spinal ventral horn in ALS]
• [Neurodegenerative diseases and Dorfin]