Magnetic resonance imaging findings in probable Creutzfeld-Jacob disease: comparison with electroencephalography and cerebrospinal fluid characteristics

Abstract

BackgroundCreutzfeld-Jacob disease (CJD) is a rare, progressive disease that has a vast clinical manifestation range. Cranial magnetic resonance imaging (MRI), electroencephalography (EEG), and measurement of 14-3-3 in cerebrospinal fluid (CSF) may offer a pragmatic approach in the diagnosis of CJD as an alternative to histopathological confirmation.PurposeTo present the symptoms and signs of the CJD patients in regard to radiological and neurophysiological findings.Material and MethodsWe collected all cases with the diagnosis of probable CJD admitted to our neurology department between June 2010 and June 2014. The medical records and laboratory data, clinical features, results of MRI (including diffusion weighted images), EEG and CSF evaluations, and other laboratory data to exclude other possible diagnoses were recorded. None of the patients underwent biopsy or autopsy for histological diagnosis.ResultsOf 20 patients, 11 (55%) were men and nine (45%) were women. The mean age at disease onset was 60.0 ± 9.5 years (age range, 47–80 years). All patients without exception had characteristic abnormalities in DWI and/or FLAIR on admission, about 4 months after the initial symptom. Periodic complexes on EEGs characteristic for CJD were detected only in 10 patients (50%) on admission and in 13 patients (65%) during disease course. Out of 14 patients who underwent CSF examination, 11 (78.5%) were positive for 14-3-3 protein.ConclusionAlthough the definite diagnosis of CJD is made histopathologically, we aimed to discuss the value of magnetic resonance imaging in the diagnosis of CJD in respect to EEG findings and protein 14-3-3 levels in CSF.

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

“…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 ].…”

Autoimmune encephalitis: what the radiologist needs to know

“…AE with a striatal involvement, especially anti-CV2/CRMP5, may resemble other conditions affecting the basal ganglia, and particularly Creutzfeldt-Jacob disease (CJD). In the suspicion of CJD, seeking the typical patterns of DWI restriction in the basal ganglia and/or in the cortex is crucial [ 138 ].…”