Neuroimaging findings in human prion disease

Macfarlane, Rebecca; Wroe, S; Collinge, John; Yousry, Tarek; Jäger, Hans Rolf

doi:10.1136/jnnp.2006.094821

Cited by 89 publications

(67 citation statements)

References 88 publications

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“…In animal models of cerebral ischemia, cell lysis and membrane disruption preferentially increase the extracellular volume and allow less restricted movement of water molecules, increasing the apparent diffusion coefficient and leading to pseudonormalization in DWI [41]. In prion diseases, Macfarlane et al [42] suggested that inherited forms, but not other, display cerebellar atrophy. Our finding of atrophy around the fourth ventricle in mostly genetic E200K patients is similar to the two cases they show, with the 6-OPRI and P102L mutations.…”

Section: Discussionmentioning

confidence: 98%

MRI Detection of the Cerebellar Syndrome in Creutzfeldt–Jakob Disease

et al. 2009

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Creutzfeldt-Jakob Disease (CJD) is characterized by bilateral basal ganglia hyperintensities on T2W and diffusion-weighted imaging (DWI) magnetic resonance imaging (MRI) scans, consistent with its extrapyramidal neurological manifestations. MRI is diagnostically uninformative about the cerebellar symptoms, equally prominent in CJD. This study was undertaken to explain this apparent paradox. Eleven CJD patients with definite cerebellar or brain stem symptoms were selected from a large prospective study, as well as 11 healthy controls matched for age and gender. All subjects participated in a standardized MRI protocol, including SPGR, fluid-attenuated inversion recovery (FLAIR), DWI and diffusion tensor imaging (DTI). All subjects underwent detailed examination by a neurologist blinded to the radiological findings, who predicted the expected site of cerebral abnormalities. MRI showed good sensitivity for the abnormalities predicted in the cortex (80-90%) and basal ganglia (100%). None of the standard MRI sequences, including DWI, DTI, and FLAIR, revealed any tissue abnormalities in cerebellum or brain stem. Apparent diffusion coefficient (ADC) values, however, were substantially and significantly elevated in several cerebellar structures, where also the volumetric (VBM) analysis revealed elevated cerebrospinal fluid volume, suggesting focal cerebellar atrophy in these CJD patients. In patients with CJD, DWI appears sensitive to the reduced diffusivity in cortex and basal ganglia but insensitive to cerebellar involvement. We propose that the radiological hallmark of cerebellar pathology in CJD is atrophy, revealed quantitatively by both VBM and elevated diffusivity, which is identifiable on ADC maps but poorly visualized in nonquantitative DWI images.

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Section: Discussionmentioning

confidence: 98%

MRI Detection of the Cerebellar Syndrome in Creutzfeldt–Jakob Disease

et al. 2009

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“…[5][6][7] With the routine clinical use of DWI, MR imaging has gained increasing importance in the diagnosis of human prion diseases. 8 MR imaging is now included in the diagnostic criteria for vCJD, and DWI has enabled earlier and more accurate diagnosis of sCJD. The combined use of DWI and FLAIR sequences increases the diagnostic sensitivity and specificity for sCJD to Ͼ91%.…”

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confidence: 99%

High-b-Value Diffusion MR Imaging and Basal Nuclei Apparent Diffusion Coefficient Measurements in Variant and Sporadic Creutzfeldt-Jakob Disease

Hyare¹,

Thornton²,

Stevens³

et al. 2009

AJNR Am J Neuroradiol

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“…The estimated incidence of prion disease is relatively stable across the world with an incidence of approximately 1 case per 1 million inhabitants per year (2). In humans, the disease is most often secondary to the sporadic form of Creutzfeldt Jacob Disease (sCJD), accounting for approximately 85% of cases.…”

Section: Epidemiology and Pathologymentioning

confidence: 99%

“…Contrary to the various molecular subtypes of sCJD, vCJD has consistent neuroimaging features, a characteristic that has been attributed to the fact that only one strain is responsible for the disease (2). The pulvinar sign is relatively specific for the new variant of CJD and is included in the diagnostic criteria established for probable vCJD (see Table 2; Fig.…”

Section: Variant Cjdmentioning

confidence: 99%

Imaging of prion diseases

Létourneau-Guillon

Wada²,

Kucharczyk

2012

Magnetic Resonance Imaging

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Prion diseases are caused by self‐replicating proteins that induce lethal neurodegenerative disorders. In the last decade, the understanding of the different clinical, pathological, and neuroimaging phenotypes of this group of disorders has evolved paralleling the advances in prion molecular biology. From an imaging standpoint, the implementation of diffusion‐weighted imaging in routine practice has markedly facilitated the detection of prion diseases, especially Creutzfeldt‐Jakob. Less frequent prion‐related disorders, including genetic diseases, may also benefit from progresses in the field of quantitative diffusion‐weighted imaging, MR spectroscopy or molecular imaging. Herein, we present a review of the neuroimaging features of the prion disorders known to affect humans emphasizing the important contribution of MRI in the diagnosis of this group of disorders. J. Magn. Reson. Imaging 2012;35:998‐1012. © 2012 Wiley Periodicals, Inc.

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Neuroimaging findings in human prion disease

Cited by 89 publications

References 88 publications

MRI Detection of the Cerebellar Syndrome in Creutzfeldt–Jakob Disease

MRI Detection of the Cerebellar Syndrome in Creutzfeldt–Jakob Disease

High-b-Value Diffusion MR Imaging and Basal Nuclei Apparent Diffusion Coefficient Measurements in Variant and Sporadic Creutzfeldt-Jakob Disease

Imaging of prion diseases

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