Diagnostic value of diffusion-weighted brain magnetic resonance imaging in patients with sporadic Creutzfeldt-Jakob disease: a systematic review and meta-analysis

Park, Ho Young; Kim, Minjae; Suh, Chong Hyun; Kim, Sang Yeong; Shim, Woo Hyun; Kim, Sang Joon

doi:10.1007/s00330-021-08031-4

Cited by 14 publications

(14 citation statements)

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“…DWI abnormalities in MRI showed a high diagnostic value for CJD with 93.8% specificity and 92.3% sensitivity [ 7 ]. In a recent meta-analysis, MRI changes have 91% sensitivity and 97% specificity for CJD diagnosis [ 8 ]. Therefore, MRI is an important non-invasive diagnostic modality that should be performed on any CJD suspected patient to help in CJD diagnosis and exclude other differential diagnoses such as vascular, infectious, metabolic, malignant, and other causes of rapidly progressive cognitive decline.…”

Section: Discussionmentioning

confidence: 99%

Rapidly Progressive Probable Sporadic Creutzfeldt-Jakob Disease

Elziny¹,

Elsaid²

2022

Cureus

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Creutzfeldt-Jakob disease (CJD) is a rare, fatal brain infection caused by a human prion. Because CJD is associated with rapidly progressive neurological degeneration, it requires high suspicion for diagnosis. We report the case of a 79-year-old patient who presented with a rapidly progressive neurological clinical picture. The patient had positive 14-3-3 proteins in cerebrospinal fluid, electroencephalography was significant for periodic discharges, and magnetic resonance imaging of the brain showed both diffusion restriction and increased fluid-attenuated inversion recovery signal in different cortical regions, consistent with probable sporadic CJD infection. The patient was enrolled under hospice and palliative care. The patient passed away two months after the onset of her symptoms. We discuss the probable sporadic CJD diagnostic criteria and possible risk factors that might have led to a faster progressive course.

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Section: Discussionmentioning

confidence: 99%

Rapidly Progressive Probable Sporadic Creutzfeldt-Jakob Disease

Elziny¹,

Elsaid²

2022

Cureus

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“…T2/FLAIR sequences were normal. The pooled diagnostic yield of diffusion-weighted imaging in sporadic Creutzfeldt-Jakob disease has been reported as high as 91% [13]. The diagnostic performance of diffusion-weighted Fig.…”

Section: Discussionmentioning

confidence: 99%

Establishing a committee for antemortem reviews of suspect Creutzfeldt-Jakob disease cases in Ireland

et al. 2022

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Background Creutzfeldt-Jakob disease (CJD) is a rapidly progressive, neurodegenerative disease. In Ireland, clinical diagnostics and laboratory testing remain the responsibility of the managing clinician and the Neuropathology Department at the Beaumont Hospital, respectively. Centralized review of individual cases is not undertaken. Aims To determine how diagnostic processes for CJD could be improved in Ireland and to outline the structure and referral process for a new CJD review panel at the Beaumont Hospital. Methods We surveyed Irish neurologists’ experiences on the management of CJD in Ireland. We measured turnaround times (TAT) for CSF samples referred for diagnostic CJD testing. Finally, we retrospectively reviewed imaging of autopsy-proven CJD cases to compare with initial reports. Results Ninety-three percent of neurologists supported a national central review of suspect CJD cases. A second clinical opinion was considered to be of likely benefit by 79%. Additionally, 93% reported that a centralized review of neuroradiology would be useful. All respondents felt that expediting turnaround of CSF analysis would be of benefit. The average TAT for CSF testing was 35.4 days. In retrospective review of imaging, all patients demonstrated MRI findings consistent with CJD. However, in only one of these cases were the initial pre-autopsy radiological findings reported as being consistent with CJD. Conclusions These findings support the need for improvements to the Irish National CJD Surveillance Unit to maximize antemortem diagnostic accuracy. On foot of this, a clinical CJD Multidisciplinary Team (CJD MDT) has been established to provide a second opinion on (i) the patient’s clinical history, (ii) neuroradiology and (iii) and neurophysiology reports (where available).

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“…While the diagnostic yield of DWI for CJD was low (0.33% [11/3,298]), CJD is an important cause of subacute cognitive impairment for which an early diagnosis is a requirement for future treatment [ 26 – 28 ]. The ACR Appropriateness Criteria recommends DWI to be included in patients with suspected prion disease [ 29 ], and previous studies showed the detection rate of DWI for sporadic CJD to range from 73% to 100% with a pooled detection rate of 91% in a recent meta-analysis [ 30 – 35 ]. A newly proposed criterion incorporating DWI findings in at least 1 positive brain region showed the superior diagnostic performance to that of standard diagnostic criteria for sporadic CJD and similar diagnostic performance to that of the cerebrospinal fluid test [ 33 ].…”

Section: Discussionmentioning

confidence: 99%

Diagnostic yield of diffusion-weighted brain MR imaging in patients with cognitive impairment: Large cohort study with 3,298 patients

Kim

Suh

et al. 2022

PLoS ONE

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Objective There is a paucity of large cohort-based evidence regarding the need and added value of diffusion-weighted imaging (DWI) in patients attending outpatient clinic for cognitive impairment. We aimed to evaluate the diagnostic yield of DWI in patients attending outpatient clinic for cognitive impairment. Materials and methods This retrospective, observational, single-institution study included 3,298 consecutive patients (mean age ± SD, 71 years ± 10; 1,976 women) attending outpatient clinic for cognitive impairment with clinical dementia rating ≥ 0.5 who underwent brain MRI with DWI from January 2010 to February 2020. Diagnostic yield was defined as the proportion of patients in whom DWI supported the diagnosis that underlies cognitive impairment among all patients. Subgroup analyses were performed by age group and sex, and the Chi-square test was performed to compare the diagnostic yields between groups. Results The overall diagnostic yield of DWI in patients with cognitive impairment was 3.2% (106/3,298; 95% CI, 2.6–3.9%). The diagnostic yield was 2.5% (83/3,298) for acute or subacute infarct, which included recent small subcortical infarct for which the diagnostic yield was 1.6% (54/3,298). The diagnostic yield was 0.33% (11/3,298) for Creutzfeldt-Jakob disease (CJD), 0.15% (5/3,298) for transient global amnesia (TGA), 0.12% (4/3,298) for encephalitis and 0.09% (3/3,298) for lymphoma. There was a trend towards a higher diagnostic yield in the older age group with age ≥ 70 years old (3.6% vs 2.6%, P = .12). There was an incremental increase in the diagnostic yield from the age group 60–69 years (2.6%; 20/773) to 90–99 years (8.0%; 2/25). Conclusion Despite its low overall diagnostic yield, DWI supported the diagnosis of acute or subacute infarct, CJD, TGA, encephalitis and lymphoma that underlie cognitive impairment, and there was a trend towards a higher diagnostic yield in the older age group.

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Diagnostic value of diffusion-weighted brain magnetic resonance imaging in patients with sporadic Creutzfeldt-Jakob disease: a systematic review and meta-analysis

Cited by 14 publications

References 57 publications

Rapidly Progressive Probable Sporadic Creutzfeldt-Jakob Disease

Rapidly Progressive Probable Sporadic Creutzfeldt-Jakob Disease

Establishing a committee for antemortem reviews of suspect Creutzfeldt-Jakob disease cases in Ireland

Diagnostic yield of diffusion-weighted brain MR imaging in patients with cognitive impairment: Large cohort study with 3,298 patients

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