Serial MR imaging in Creutzfeldt-Jakob disease

Uchino, Akira; Yoshinaga, Masatomi; Shiokawa, Osamu; Hata, H.; Ohno, Masato

doi:10.1007/bf00587828

Cited by 33 publications

(9 citation statements)

References 12 publications

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“…All these disorders demonstrate prolonged T2 relaxation, except for Hallervorden Spatz disease, which often shows pronounced hypointensity on the long TR images in the basal ganglia from iron deposit. Symmetry of the findings makes neoplastic lesions very unlikely [6,7]. Although there is a wide range of differential diagnoses, we believe that, considering clinical course and EEC examination, characteristic signal alterations on early MRI are definitely helpful for evaluating the clinical diagnosis of early-stage CJD in patients with rapidly progressive dementia.…”

Section: Discussionmentioning

confidence: 95%

MRI follow-up in a case of clinically diagnosed Creutzfeld-Jakob disease

Hutzelmann

Biederer

1998

European Radiology

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We report the case of a 75-year-old woman suffering from Creutzfeld-Jakob disease (CJD). As brain biopsy was refused, diagnosis had to be based on clinical examination, EEC and findings on cranial MRI. Over a 4-month period MRI examinations demonstrated progressive cortical atrophy and bilateral enhanced signal intensity on T2-weighted images of caudate nuclei and putamina indicating development of spongioform degeneration. As clinical course and the characteristic pattern of brain lesions corresponded to cases of neuropathologically confirmed CJD, we suggest that MRI should be considered a valuable diagnostic tool in clinical diagnosis of the disease.

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Section: Discussionmentioning

confidence: 95%

MRI follow-up in a case of clinically diagnosed Creutzfeld-Jakob disease

Hutzelmann

Biederer

1998

European Radiology

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“…The nature of the white matter abnormalities remains obscure, but can possibly be attributed to Wallerian degeneration. 6 Most CT or MRI abnormalities in human prion diseases in previous reports mention either no abnormalities, cortical atrophy, or high signal intensities in the basal ganglia. White matter abnormalities, as we report, appear to be rare in spongiform encephalopathy, which is referred to as the panencephalopathic type of Creutzfeldt-Jakob disease (CJD).…”

Section: Discussionmentioning

confidence: 98%

“…White matter abnormalities, as we report, appear to be rare in spongiform encephalopathy, which is referred to as the panencephalopathic type of Creutzfeldt-Jakob disease (CJD). 6 There are only a few case reports of sporadic CJD with white matter degeneration and cortical atrophy. 6,7 Including our patient, at least four patients with a hereditary prion disease have had white matter changes.…”

Section: Discussionmentioning

confidence: 98%

A new mutation in the prion protein gene: A patient with dementia and white matter changes

Harten¹,

Gool²,

Langen³

et al. 2000

Neurology

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The authors describe the clinical characteristics, MRI abnormalities, and molecular findings in a patient with a novel variant of a two-octarepeat insertion mutation in the prion protein gene. This patient presented with moderately progressive dementia of presenile onset and gait ataxia. MRI showed extensive cortical atrophy and white matter abnormalities. The mutation consists of a two-octarepeat insertion mutation and irregularities in the nucleotide sequence of the octarepeat region.

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“…3) (8). With the evolving course of the disease, atrophy is seen, a finding usually revealed after the typical DWI changes (8,41). A large multinational study demonstrated that abnormal signal in the basal ganglia was found in 71% on DWI, and 63% on FLAIR, whereas widespread abnormal signal within the cortex was found in 66% on DWI and 38% on FLAIR (6).…”

Section: Sporadic Creutzfeldt-jakob Diseasementioning

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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Serial MR imaging in Creutzfeldt-Jakob disease

Cited by 33 publications

References 12 publications

MRI follow-up in a case of clinically diagnosed Creutzfeld-Jakob disease

MRI follow-up in a case of clinically diagnosed Creutzfeld-Jakob disease

A new mutation in the prion protein gene: A patient with dementia and white matter changes

Imaging of prion diseases

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