ArticleBrain : a journal of neurology2009
Magnetic resonance diagnostic markers in clinically sporadic prion disease: a combined brain magnetic resonance imaging and spectroscopy study.
Article in Brain : a journal of neurology, 2009. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
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15 citing papers in PubMed, 53 citations in OpenAlex.
- Pons metabolite alterations in narcolepsy type 1.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2025Article
- Subtype Diagnosis of Sporadic Creutzfeldt-Jakob Disease with Diffusion Magnetic Resonance Imaging.Annals of neurology · 2021Article
- Evaluation of a New Criterion for Detecting Prion Disease With Diffusion Magnetic Resonance Imaging.JAMA neurology · 2020Article
- Using NMR spectroscopy to investigate the role played by copper in prion diseases.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2020Review
- Predicting conversion from mild cognitive impairment to Alzheimer's disease using brainNeuroImage. Clinical · 2019Article
- Neuroradiology of human prion diseases, diagnosis and differential diagnosis.La Radiologia medica · 2017Review
- Combined Cerebellar Proton MR Spectroscopy and DWI Study of Patients with Friedreich's Ataxia.Cerebellum (London, England) · 2017Article
- Mathematical models for the diffusion magnetic resonance signal abnormality in patients with prion diseases.NeuroImage. Clinical · 2015Article
- Neuroimaging biomarkers of neurodegenerative diseases and dementia.Seminars in neurology · 2013Review
- MRS in early and presymptomatic carriers of a novel octapeptide repeat insertion in the prion protein gene.Journal of neuroimaging : official journal of the American Society of Neuroimaging · 2013Article
- Temporal evolution of sporadic Creutzfeldt-Jakob disease monitored by 3-Tesla MR spectroscopy.Journal of neurology · 2011Article
- An unusual case of sporadic Creutzfeldt-Jakob disease (CJD).BMJ case reports · 2010Article
- Prion disease: Brain MRI studies in prion disease.Nature reviews. Neurology · 2010Article
- Neuroimaging in human prion disease: Searching in the mist.World journal of radiology · 2009Article
- Quantum dots and prion proteins: is this a new challenge for neurodegenerative diseases imaging?PrionReview
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Authors and funding
15 authors at 3 institutions in 1 country.
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No grant is acknowledged in the PubMed record.
Abstract
The intra vitam diagnosis of prion disease is challenging and a definite diagnosis still requires neuropathological examination in non-familial cases. Magnetic resonance imaging has gained increasing importance in the diagnosis of prion disease. The aim of this study was to compare the usefulness of different magnetic resonance imaging sequences and proton magnetic resonance spectroscopy in the differential diagnosis of patients with rapidly progressive neurological signs compatible with the clinical diagnosis of sporadic prion disease. Twenty-nine consecutive patients with an initial diagnosis of possible or probable sporadic prion disease, on the basis of clinical and electroencephalography features, were recruited. The magnetic resonance protocol included axial fluid-attenuated inversion recovery-T2- and diffusion-weighted images, and proton magnetic resonance spectroscopy of the thalamus, striatum, cerebellum and occipital cortex. Based on the clinical follow-up, genetic studies and neuropathology, the final diagnosis was of prion disease in 14 patients out of 29. The percentage of correctly diagnosed cases was 86% for diffusion-weighted imaging (hyperintensity in the striatum/cerebral cortex), 86% for thalamic N-acetyl-aspartate to creatine ratio (cutoff </=1.21), 90% for thalamic N-acetyl-aspartate to myo-inositol (mI) ratio (cutoff </=1.05) and 86% for cerebral spinal fluid 14-3-3 protein. All the prion disease patients had N-acetyl-aspartate to creatine ratios </=1.21 (100% sensitivity and 100% negative predictive value) and all the non-prion patients had N-acetyl-aspartate to myo-inositol ratios >1.05 (100% specificity and 100% positive predictive value). Univariate logistic regression analysis showed that the combination of thalamic N-acetyl-aspartate to creatine ratio and diffusion-weighted imaging correctly classified 93% of the patients. The combination of thalamic proton magnetic resonance spectroscopy (10 min acquisition duration) and brain diffusion-weighted imaging (2 min acquisition duration) may increase the diagnostic accuracy of the magnetic resonance scan. Both sequences should be routinely included in the clinical work-up of patients with suspected prion disease.
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