Creutzfeldt-Jakob Disease Research Today is a free monthly online journal that collates and summarizes the latest research about Creutzfeldt-Jakob Disease, including details on mad cow disease, symptoms, causes, variants.

Creutzfeldt-Jakob disease: Which diffusion-weighted imaging abnormality is associated with periodic EEG complexes?

Kandiah N, Nagaendran K, Tan K, Pan AB, Au WL, Venketasubramanian N, Tchoyoson Lim CC, Tan NC

Level 3, Clinical Staff Office, National Neuroscience Institute, 11 Jalan Tan Tock Seng, Singapore, 308433, nagaendran_kandiah@nni.com.sg.

BACKGROUND : Clinical diagnosis of CJD remains important due to lack of access to a genetic or histopathological diagnosis. Using current WHO criteria, diagnostic certainty can be increased from "possible" to "probable" CJD if periodic complexes are recorded on EEG. OBJECTIVE : To study the correlation between patterns of MRI-DWI hyperintensity and typical EEG findings among patients with CJD. METHODS : Demographics, clinical findings, MRI-DWI and EEG findings of CJD patients were retrospectively reviewed. RESULTS : A total of 14 patients ranging in age from 35 to 81 years were identified. All had dementia and cerebellar ataxia. Psychiatric manifestations were seen in 5 patients. Seven patients had both cortical and striatal DWI changes, five had isolated cortical DWI changes and two had isolated striatal DWI changes. All twelve patients with cortical DWI changes also had periodic EEG changes. In ten, periodic EEG was recorded within seven days of the DWI. The two patients with isolated striatal DWI changes did not develop periodic EEG complexes despite serial EEG recordings, 40 and 88 days from their respective DWI scans. CONCLUSIONS : Serial EEGs are not useful for patients with isolated striatal DWI hyperintensity but will increase diagnostic certainty from "possible" to probable" CJD for patients with cortical DWI hyperintensity.

Published 25 June 2008 in J Neurol.
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Articles on Creutzfeldt-Jakob Disease published 24 June 2008:

The variant Creutzfeldt-Jakob Disease. Risk, uncertainty or safety in the use of the blood and blood derivatives?   Int Arch Med, 1(1): 9.

ABSTRACT: Long period of time has elapsed since the French physician Jean-Baptiste Denys carried out the first successful blood transfusion to a human being. Using bird feathers as canules, sheep blood was transfused to a young man which died soon after Denys' treatment and Denys was accused of murder. Now, in the our present century, the biotechnology century, we face new challenges in Medicine. New emerging and reemerging diseases like the Creutzfeldt-Jakob disease (CJD) or "mad cow ... [Abstract] [Full-text]

Articles on Creutzfeldt-Jakob Disease published 13 June 2008:

Cerebral amyloid angiopathy with co-localization of prion protein and beta-amyloid in an 85-year-old patient with sporadic Creutzfeldt-Jakob disease.   Acta Neuropathol.

We report on an 85-year-old woman with hypertensive cerebral arteriolosclerosis who presented with rapidly progressive encephalopathy leading to death within 4 months. Magnetic resonance imaging showed mild cortical atrophy consistent with her age and diffuse leukoaraiosis. Her CSF 14-3-3 protein was positive. Neuropathology showed severe spongiform change and gliosis in the grey matter and immunohistochemistry revealed diffuse prion protein deposition in a predominant synaptic pattern. She had ... [Abstract] [Full-text]

PRNP 1368 polymorphism is not associated with sporadic Creutzfeldt-Jakob disease in the Korean population.   Eur J Neurol.

Background: Human prion protein gene (PRNP) is considered a critical and fundamental gene in determining the incidence of human prion diseases. Codons 129 and 219 play an important role in the susceptibility to sporadic Creutzfeldt-Jakob disease (CJD). An association between sporadic CJD and the polymorphism (PRNP 1368) in an upstream of PRNP exon 1 has been reported in the British and German populations, but study in the Dutch population has failed to confirm an association. Purpose: To ... [Abstract] [Full-text]

Articles on Creutzfeldt-Jakob Disease published 10 June 2008:

Different expression patterns of CK2 subunits in the brains of experimental animals and patients with transmissible spongiform encephalopathies.   Arch Virol, 153(6): 1013-1020.

To address the possible alteration of casein kinase 2 (CK2) in transmissible spongiform encephalopathies (TSEs), the levels and patterns of CK2 in the brain tissues of hamsters or C57BL mice inoculated intracerebrally with scrapie agents 263K or 139A were evaluated by Western blots, followed by quantitative analysis. Specific semi-quantitative RT-PCR for evaluating the mRNA transcripts of CK2 subunits was performed in parallel. Compared with normal animals, the levels of CK2alpha and CK2beta in ... [Abstract] [Full-text]

Experimental transmission of bovine spongiform encephalopathy to European red deer (Cervus elaphus elaphus).   BMC Vet Res, 4: 17.

BACKGROUND: Bovine spongiform encephalopathy (BSE), a member of the transmissible spongiform encephalopathies (TSE), primarily affects cattle. Transmission is via concentrate feed rations contaminated with infected meat and bone meal (MBM). In addition to cattle, other food animal species are susceptible to BSE and also pose a potential threat to human health as consumption of infected meat products is the cause of variant Creutzfeldt-Jakob disease in humans, which is invariably fatal. In the ... [Abstract] [Full-text]

Articles on Creutzfeldt-Jakob Disease published 2 June 2008:

Ultrastructural study of florid plaques in variant Creutzfeldt-Jakob disease: a comparison with amyloid plaques in kuru, sporadic Creutzfeldt-Jakob disease and Gerstmann-Sträussler-Scheinker disease.   Neuropathol Appl Neurobiol.

Background: Although the histological features of the amyloid plaques in variant Creutzfeldt-Jakob disease (vCJD) are distinct from those in other forms of prion disease [kuru, sporadic Creutzfeldt-Jakob disease (sCJD) and Gerstmann-Sträussler-Scheinker disease (GSS)], their ultrastructural features have only been described in a single case report. Aims: To study vCJD plaques systematically and compare them with plaques in kuru, sCJD, GSS and Alzheimer disease (AD). Methods: Amyloid plaques ... [Abstract] [Full-text]

Articles on Creutzfeldt-Jakob Disease published 29 May 2008:

Close association of water channel AQP1 with amyloid-beta deposition in Alzheimer disease brains.   Acta Neuropathol.

Aquaporin-1 (AQP1), a membrane water channel protein, is expressed exclusively in the choroid plexus epithelium in the central nervous system under physiological conditions. However, AQP1 expression is enhanced in reactive astrocytes, accumulating in brain lesions of Creutzfeldt-Jakob disease and multiple sclerosis, suggesting a role of AQP1-expressing astrocytes in brain water homeostasis under pathological conditions. To clarify a pathological implication of AQP1 in Alzheimer disease (AD), we ... [Abstract] [Full-text]

An Antibody to the Aggregated Synthetic Prion Protein Peptide (PrP106-126) Selectively Recognizes Disease-Associated Prion Protein (PrP(Sc)) from Human Brain Specimens.   Brain Pathol.

Human prion diseases are characterized by the conversion of the normal host cellular prion protein (PrP(C)) into an abnormal misfolded form [disease-associated prion protein (PrP(Sc))]. Antibodies that are capable of distinguishing between PrP(C) and PrP(Sc) may prove to be useful, not only for the diagnosis of these diseases, but also for a better understanding of the molecular mechanisms involved in disease pathogenesis. In an attempt to produce such antibodies, we immunized mice with an ... [Abstract] [Full-text]

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