 |
|
Creutzfeldt-Jakob Disease With Florid-Type Plaques After Cadaveric Dura Mater Grafting
Saori Shimizu, MD;
Ken-ichi Hoshi, MD;
Tamaki Muramoto, MD;
Mari Homma, MD;
James W. Ironside, MRCPath;
Shigeki Kuzuhara, MD;
Takeshi Sato, MD;
Teiji Yamamoto, MD;
Tetsuyuki Kitamoto, MD
Arch Neurol. 1999;56:357-362.
Background Many reported cases of iatrogenic Creutzfeldt-Jakob disease (CJD) developed after grafting cadaveric dura mater contaminated with CJD prions (dura-associated CJD). They are known to be clinicopathologically similar to sporadic CJD. We report herein 2 autopsy cases of dura-associated CJD with atypical clinicopathological features.
Patients Two patients presented with progressive ataxia and mental deterioration 10 or 11 years after neurosurgical treatment with cadaveric dural grafting, which led to their deaths at 8 and 17 months, respectively, after onset.
Results The cases were clinically atypical in exhibiting no or late occurrence of myoclonus and periodic synchronous discharges on electroencephalographic studies. They were pathologically unique in several aspects. The most striking feature was the presence of many prion protein (PrP) plaques in multiple areas in the brain. Some of them were the "florid" type surrounded by a zone of spongiform changes known to be a hallmark for the new variant CJD. The distribution of spongiform degeneration was also unique in that it was intense in the thalamus, basal ganglia, and the dentate nuclei of the cerebellum but milder in the cerebrum. There were no mutations in the PrP gene of the patients. There was no major difference in the size and glycoform pattern between the abnormal isoform of PrP extracted from the brain tissue from the dura-associated cases of CJD and that from a sporadic case of CJD.
Conclusions These 2 cases are clinicopathologically distinct from typical dura-associated cases of CJD. They may be a subtype with florid-type plaques in dura-associated CJD.
From the Departments of Neurology, Fukushima Medical College, Fukushima (Drs Shimizu, Homma, and Yamamoto), Kohnodai Hospital, the National Center of Neurology and Psychiatry, Ichikawa (Drs Hoshi and Sato), and Mie University School of Medicine, Tsu (Dr Kuzuhara), the Department of Neurological Science, Tohoku University School of Medicine, Sendai (Drs Muramoto and Kitamoto), Japan; and the Creutzfeldt-Jakob Disease Surveillance Unit, Western General Hospital, Edinburgh, Scotland (Dr Ironside).
RELATED ARTICLE
Archives of Neurology Reader's Choice: Continuing Medical Education
Arch Neurol. 1999;56(3):370-371.
FULL TEXT
THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES
|
Cross-sequence Transmission of Sporadic Creutzfeldt-Jakob Disease Creates a New Prion Strain
Kobayashi et al.
J. Biol. Chem. 2007;282:30022-30028.
ABSTRACT
| FULL TEXT
Clinical features and diagnosis of dura mater graft-associated Creutzfeldt-Jakob disease
Noguchi-Shinohara et al.
Neurology 2007;69:360-367.
ABSTRACT
| FULL TEXT
The MM2-cortical form of sporadic Creutzfeldt-Jakob disease presenting with visual disturbance
Nozaki et al.
Neurology 2006;67:531-533.
ABSTRACT
| FULL TEXT
Iatrogenic Creutzfeldt-Jakob disease: The waning of an era
Brown et al.
Neurology 2006;67:389-393.
ABSTRACT
| FULL TEXT
Involvement of the peripheral nervous system in human prion diseases including dural graft associated Creutzfeldt-Jakob disease
Ishida et al.
J. Neurol. Neurosurg. Psychiatry 2005;76:325-329.
ABSTRACT
| FULL TEXT
Clinical diagnosis of MM2-type sporadic Creutzfeldt-Jakob disease
Hamaguchi et al.
Neurology 2005;64:643-648.
ABSTRACT
| FULL TEXT
Humanized Knock-In Mice Expressing Chimeric Prion Protein Showed Varied Susceptibility to Different Human Prions
Taguchi et al.
Am. J. Pathol. 2003;163:2585-2593.
ABSTRACT
| FULL TEXT
Association of an 11-12 kDa protease-resistant prion protein fragment with subtypes of dura graft-associated Creutzfeldt-Jakob disease and other prion diseases
Satoh et al.
J. Gen. Virol. 2003;84:2885-2893.
ABSTRACT
| FULL TEXT
Sporadic Creutzfeldt-Jakob disease with MM1-type prion protein and plaques
Ishida et al.
Neurology 2003;60:514-517.
ABSTRACT
| FULL TEXT
Mad Cows, Prions, and Wrinkles
Carruthers and Carruthers
Arch Dermatol 2002;138:667-670.
FULL TEXT
Atypical form of dural graft associated Creutzfeldt-Jakob disease: report of a postmortem case with review of the literature
Kimura et al.
J. Neurol. Neurosurg. Psychiatry 2001;70:696-699.
ABSTRACT
| FULL TEXT
Prion proteins and the gut: une liaison dangereuse?
SHMAKOV and GHOSH
Gut 2001;48:443-447.
FULL TEXT
Creutzfeldt-Jakob disease associated with cadaveric dura mater grafts in Japan
Hoshi et al.
Neurology 2000;55:718-721.
ABSTRACT
| FULL TEXT
|
