Automated diffusion-weighted image analysis along the perivascular space index reveals glymphatic dysfunction in association with brain parenchymal lesions.

Brain glymphatic dysfunction is critical in neurodegenerative processes. While animal studies have provided substantial insights, understandings in humans remains limited. Recent attention has focused on the non-invasive evaluation of brain glymphatic function. However, its association with brain parenchymal lesions in large-scale population remains under-investigated. In this cross-sectional analysis of 1030 participants (57.14 ± 9.34 years, 37.18% males) from the Shunyi cohort, we developed an automated pipeline to calculate diffusion-weighted image analysis along the perivascular space (ALPS), with a lower ALPS value indicating worse glymphatic function. The automated ALPS showed high consistency with the manual calculation of this index (ICC = 0.81, 95% CI: 0.662-0.898). We found that those with older age and male sex had lower automated ALPS values (ß = -0.051, SE = 0.004, p < .001, per 10 years, and ß = -0.036, SE = 0.008, p < .001, respectively). White matter hyperintensity (ß = -2.458, SE = 0.175, p < .001) and presence of lacunes (OR = 0.004, 95% CI < 0.002-0.016, p < .001) were significantly correlated with decreased ALPS. The brain parenchymal and hippocampal fractions were significantly associated with decreased ALPS (ß = 0.067, SE = 0.007, p < .001 and ß = 0.040, SE = 0.014, p = .006, respectively) independent of white matter hyperintensity. Our research implies that the automated ALPS index is potentially a valuable imaging marker for the glymphatic system, deepening our understanding of glymphatic dysfunction.

Utility of Echo-planar Imaging with Compressed Sensitivity Encoding (EPICS) in the Evaluation of Small Breast Cancers Using Diffusion-weighted Imaging with Background Suppression (DWIBS).

PURPOSE: High b-value acquisition and diffusion-weighted imaging with background suppression (DWIBS) are desirable in high-specificity breast cancer diagnosis on non-contrast-enhanced magnetic resonance imaging; however, this inherently results in a lower signal-to-noise ratio (SNR). Compressed sensitivity encoding (C-SENSE), which combines SENSE with compressed sensing, improves the SNR by reducing noise. Recent technological improvements allow us to incorporate this acceleration technique into echo-planar imaging, called echo-planar imaging with C-SENSE (EPICS). This study aimed to compare image quality and reliability of the apparent diffusion coefficient (ADC) between DWIBS obtained using SENSE and EPICS in patients with small breast cancers. METHODS: Thirty-seven patients with pathologically confirmed breast cancer underwent DWIBS, and images were reconstructed using both conventional SENSE (SENSE-DWIBS) and EPICS (EPICS-DWIBS). Two board-certified radiologists independently evaluated lesion conspicuity (LC) and noise using a 5-point grading scale. The same 2 radiologists independently measured SNR, contrast-to-noise ratio (CNR), and the mean cancer ADC. The Pearson coefficient and Bland-Altman plot were applied to assess the accuracy of ADCs. RESULTS: LC scores were higher with EPICS than with SENSE, reaching significance for one reviewer but not the other reviewer. Noise ratings on visual evaluation were significantly lower with EPICS than with SENSE (P < 0.001 for both reviewers). SNR was significantly higher with EPICS than with SENSE (P < 0.005 for both reviewers). CNR was significantly higher with EPICS than with SENSE (P < 0.001 for both reviewers). Bland-Altman plots of cancer ADCs using EPICS-DWIBS and SENSE-DWIBS showed excellent concordance, with a bias of 0.026 × 10-3 mm2/s and limits of agreement ranging 0.054 × 10-3 mm2/s; the Pearson's correlation coefficient was 0.997 (P < 0.0001). CONCLUSION: EPICS enhances breast DWIBS image quality, with improved SNR and CNR and reduced noise levels. The ADCs of breast cancers obtained using EPICS were almost perfectly correlated with those obtained using conventional SENSE.

Non-contrast-enhanced breast MRI for evaluation of tumor volume change after neoadjuvant chemotherapy.

PURPOSE: Three-dimensional contrast-enhanced magnetic resonance imaging (3D-Ce-MRI) is a most powerful tool for evaluation of neoadjuvant chemotherapy (NAC). However, the use of contrast agent is invasive, expensive, and time consuming, Thus, contrast agent-free imaging is preferable. We aimed to investigate the tumor volume change after NAC using maximum intensity projection diffusion-weighted image (MIP-DWI) and 3D-Ce-MRI. METHOD: We finally enrolled 55 breast cancer patients who underwent NAC in 2018. All MRI analyses were performed using SYNAPSE VINCENT® medical imaging system (Fujifilm Medical, Tokyo, Japan). We evaluated the tumor volumes before, during, and after NAC. Tumor volume before NAC on 3D-Ce-MRI was termed Pre-CE and those during and after NAC were termed Post-CE. The observer raised the lower end of the window width until the tumor was clearly visible and then manually deleted the non-tumor tissues. A month thereafter, the same observer who was blinded to the 3D-Ce-MRI results randomly evaluated the tumor volumes (Pre-DWI and Post-DWI) using MIP-DWI with the same method. Tumor volume change between ΔCE (Pre-CE - Post-CE/Pre-CE) and ΔDWI (Pre-DWI - Post-DWI/Pre-DWI) and the processing time for both methods (Time-DWI and Time-CE) were compared. RESULTS: We enrolled 55 patients. Spearman's rho between ΔDWI and ΔCE for pure mass lesions, and non-mass enhancement (NME) was 0.89 (p < 0.01), 0.63(p < 0.01) respectively. Time-DWI was significantly shorter than Time-CE (41.3 ± 21.2 and 199.5 ± 98.3 respectively, p < 0.01). CONCLUSIONS: Non-contrast-enhanced Breast MRI enables appropriate and faster evaluation of tumor volume change after NAC than 3D-Ce-MRI especially for mass lesions.

Modeling cerebrospinal fluid dynamics across the entire intracranial space through integration of four-dimensional flow and intravoxel incoherent motion magnetic resonance imaging.

BACKGROUND: Bidirectional reciprocal motion of cerebrospinal fluid (CSF) was quantified using four-dimensional (4D) flow magnetic resonance imaging (MRI) and intravoxel incoherent motion (IVIM) MRI. To estimate various CSF motions in the entire intracranial region, we attempted to integrate the flow parameters calculated using the two MRI sequences. To elucidate how CSF dynamics deteriorate in Hakim's disease, an age-dependent chronic hydrocephalus, flow parameters were estimated from the two MRI sequences to assess CSF motion in the entire intracranial region. METHODS: This study included 127 healthy volunteers aged ≥ 20 years and 44 patients with Hakim's disease. On 4D flow MRI for measuring CSF motion, velocity encoding was set at 5 cm/s. For the IVIM MRI analysis, the diffusion-weighted sequence was set at six b-values (i.e., 0, 50, 100, 250, 500, and 1000 s/mm2), and the biexponential IVIM fitting method was adapted. The relationships between the fraction of incoherent perfusion (f) on IVIM MRI and 4D flow MRI parameters including velocity amplitude (VA), absolute maximum velocity, stroke volume, net flow volume, and reverse flow rate were comprehensively evaluated in seven locations in the ventricles and subarachnoid spaces. Furthermore, we developed a new parameter for fluid oscillation, the Fluid Oscillation Index (FOI), by integrating these two measurements. In addition, we investigated the relationship between the measurements and indices specific to Hakim's disease and the FOIs in the entire intracranial space. RESULTS: The VA on 4D flow MRI was significantly associated with the mean f-values on IVIM MRI. Therefore, we estimated VA that could not be directly measured on 4D flow MRI from the mean f-values on IVIM MRI in the intracranial CSF space, using the following formula; e0.2(f-85) + 0.25. To quantify fluid oscillation using one integrated parameter with weighting, FOI was calculated as VA × 10 + f × 0.02. In addition, the FOIs at the left foramen of Luschka had the strongest correlations with the Evans index (Pearson's correlation coefficient: 0.78). The other indices related with Hakim's disease were significantly associated with the FOIs at the cerebral aqueduct and bilateral foramina of Luschka. FOI at the cerebral aqueduct was also elevated in healthy controls aged ≥ 60 years. CONCLUSIONS: We estimated pulsatile CSF movements in the entire intracranial CSF space in healthy individuals and patients with Hakim's disease using FOI integrating VA from 4D flow MRI and f-values from IVIM MRI. FOI is useful for quantifying the CSF oscillation.

A Rare Case of Exercise-Induced Transient Global Amnesia as an Exclusive Presenting Symptom of Hippocampus Infarct.

Transient global amnesia (TGA) is a rare condition characterized by a temporary loss of the ability to form new memories. Retrograde episodic memory loss may also occur but to a lesser extent. Although TGA is generally benign, its sudden onset and similarity to more dangerous conditions like transient ischemic attack (TIA) or cerebral vascular accident (CVA) can be concerning. We present the case of a 70-year-old female who experienced confusion and general memory loss after a vigorous workout on her stationary exercise bike. After displaying considerable amnestic symptoms, she was admitted to the hospital for further medical attention and underwent a magnetic resonance imaging (MRI) that concluded a TGA diagnosis. This case report aims to investigate the prognosis associated with risk factors and refine the diagnostic criteria of TGA. We explore whether TGA caused by exercise, leading to unilateral or bilateral hippocampal lesions, is linked to cognitive decline. It is not yet clear if the development of TGA with unilateral infarct or bilateral hippocampal lesions results in different clinical presentations or varying prognoses. Further research is needed to determine the long-term risks of cognitive decline associated with resulting infarcts and clinical presentations.

Enhancing Diagnostic Precision: Evaluation of Preprocessing Filters in Simple Diffusion Kurtosis Imaging for Head and Neck Tumors.

Background: Our initial clinical study using simple diffusion kurtosis imaging (SDI), which simultaneously produces a diffusion kurtosis image (DKI) and an apparent diffusion coefficient map, confirmed the usefulness of SDI for tumor diagnosis. However, the obtained DKI had noticeable variability in the mean kurtosis (MK) values, which is inherent to SDI. We aimed to improve this variability in SDI by preprocessing with three different filters (Gaussian [G], median [M], and nonlocal mean) of the diffusion-weighted images used for SDI. Methods: The usefulness of filter parameters for diagnosis was examined in basic and clinical studies involving 13 patients with head and neck tumors. Results: The filter parameters, which did not change the median MK value, but reduced the variability and significantly homogenized the MK values in tumor and normal tissues in both basic and clinical studies, were identified. In the receiver operating characteristic curve analysis for distinguishing tumors from normal tissues using MK values, the area under curve values significantly improved from 0.627 without filters to 0.641 with G (σ = 0.5) and 0.638 with M (radius = 0.5). Conclusions: Thus, image pretreatment with G and M for SDI was shown to be useful for improving tumor diagnosis in clinical practice.

How Topographic Diffusion-Weighted Imaging Patterns can Predict the Potential Embolic Source.

PURPOSE: To develop an imaging prediction model for patients with embolic stroke of undetermined source (ESUS), we investigated the association of topographic diffusion-weighted imaging (DWI) patterns with potential embolic sources (PES) identified by transesophageal echocardiography. METHODS: From a total of 992 consecutive patients with embolic stroke, 366 patients with the ESUS group were selected. ESUS was defined as no atrial fibrillation (Af) within 24h from admission and no PES after general examination. Clinical variables include age (> 80years, 70-80 years), sex, vascular risk factors and left atrial diameter > 4 cm. Age, sex and vascular risk factors adjusted odds ratio of each DWI for the different PESs were calculated. DWI was determined based on the arterial territories. Middle cerebral arteries were divided into 4 segments, i.e., M1-M4. Moreover, M2 segments were subdivided into superior and inferior branches. RESULTS: The 366 patients consisted of 168 with paroxysmal Af (pAf), 77 with paradoxical embolism, 71 with aortic embolism and 50 with undetermined embolism after transesophageal echocardiography. The variables adjusted odds ratio (OR) of internal carotid artery (OR: 12.1, p = 0.037), M1 (4.2, p = 0.001), inferior M2 (7.5, p = 0.0041) and multiple cortical branches (12.6, p < 0.0001) were significantly higher in patients with pAf. Striatocapsular infarction (12.5, p < 0.0001) and posterior inferior cerebellar artery infarcts (3.6, p = 0.018) were significantly associated with paradoxical embolism. Clinical variables adjusted OR of multiple small scattered infarcts (8.3, p < 0.0001) were significantly higher in patients with aortic embolism. CONCLUSION: The associations of DWI with different PES have their distinctive characteristics and DWI along with clinical variables may help predict PES in patients with ESUS.

Lacrimal Gland Abscess: A Case Report.

Introduction: The aim of the study was to report a rare case of lacrimal gland abscess. Case Presentation: A 47-year-old woman noticed upper eyelid swelling on the right side 1.5 months before referral to our service. Oral antibiotics were administered, based on the diagnosis of acute dacryoadenitis at another clinic. The symptom had once subsided 20 days later but recurred. On the first examination, the right upper eyelid was swollen with tenderness. The right lacrimal gland was palpable. Blood tests revealed positive proteinase 3-anti-neutrophil cytoplasmic antibody. T2-weighted magnetic resonance and diffusion-weighted images showed a high signal intensity lesion in an enlarged right lacrimal gland, while apparent diffusion coefficient map demonstrated the lesion with a low signal intensity. We started administration of intravenous antibiotics. Abscess drainage and lacrimal gland biopsy were performed 4 days after the first examination. Culture test of the abscess showed only 1 colony growth of Cutibacterium acnes. The specimen harvested from the lacrimal gland showed proliferation of fibrous connective tissue and infiltration of inflammatory cells without vasculitis. After the drainage, the swelling gradually subsided. Administration of antibiotics discontinued at 22 days of follow-up. At 4-month follow-up, the patient did not have any symptom related to the lacrimal gland abscess. Conclusion: The diffusion-weighted images and apparent diffusion coefficient map are helpful for the diagnosis of lacrimal gland abscess when the culture tests provide poor results.

Arterial spin labeling image findings in the acute phase in paediatric patients with acute encephalopathy with biphasic seizures and late reduced diffusion.

Introduction: Diagnosing acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) after the first seizure (early seizure/seizures, ES/ESs) is challenging because a reduced apparent diffusion coefficient (ADC) in the cortical or subcortical white matter, often described as having a "bright-tree appearance (BTA)," is usually not observed until secondary seizures (late seizures, LSs) occur. Previous studies have reported hypoperfusion on arterial spin labeling (ASL) within 24 h after ES/ESs in patients with AESD and hyperperfusion within 24 h after LS onset. This study aimed to investigate cerebral blood flow in the hyperacute phase (between ES/ESs and LSs) using ASL in patients with AESD. Methods: Eight ASL images were acquired in six patients with AESD admitted to our hospital from October 2021 to October 2022. ASL findings in the hyperacute phase were investigated and video-electroencephalogram findings obtained around ASL image acquisition in the hyperacute phase were evaluated. Results: Four ASL images were obtained for three patients before LS onset, with three images showing hyperperfusion areas and one image showing hypoperfusion areas. These hyperperfuion regions coincided with BTA on subsequent images of these patients.In one patient, the first ASL image was obtained in the late hyperacute phase and revealed hyperperfusion areas with a slightly abnormal change on diffusion-weighted image (DWI), which were not accompanied by ADC abnormalities. The second ASL image obtained 51 h after the first ASL, and before LS onset revealed more prominent hyperperfusion areas than the first ASL image, which were accompanied by BTA. In another patient, the ASL image obtained 82 h after ES revealed hyperperfusion areas without abnormal change on DWI or ADC. Conclusion: This study revealed that two patients exhibited hyperperfusion regions and another patient exhibited hypoperfusion regions among three patients who underwent ASL imaging during the period from 24 h after ES/ESs to LSs in patients with LSs or cooling initiation in patients without LSs due to early anaesthesia induction (late hyperacute phase). Further prospective studies on cerebral blood flow are required to explore the relationship among the timing of image acquisition, the presence of electrographic seizures, and ASL findings in patients with AESD.

Residual diffusion-weighted imaging hyperintense signal in primary central nervous system lymphoma can predict early recurrence.

BACKGROUND: The treatment response of primary central nervous system lymphomas (PCNSLs) is mainly evaluated using postcontrast T1-weighted imaging (T1WI). Because poorly enhanced lesions may contain residual tumors, the combination of evaluation methods will potentially improve the accuracy of determining treatment effectiveness. In this study, we evaluated the usefulness of diffusion-weighted imaging (DWI) in predicting recurrence among patients with PCNSL who achieved complete response (CR)/unconfirmed CR (CRu). METHODS: Fifty-four patients newly diagnosed with PCNSL who were treated at our institution and achieved CR/CRu at the end of treatment were included in this study. The patients were divided into two groups according to the presence or absence of residual DWI hyperintense signal at the tumor site at the end of treatment. Kaplan-Meier analysis was performed to analyze the median overall survival (OS) and progression-free survival (PFS). RESULTS: The mean age of the 54 patients was 66.4 ± 13.3 years. The induction therapies were HD-MTX in 20 patients, R-MPV in 29 patients, and other chemotherapies in five patients. Radiotherapy was performed in 35 patients, high-dose cytarabine therapy in 14 patients, and autologous hematopoietic stem cell transplantation in one patient, and of the 54 patients, 10 had no consolidation therapy. The residual DWI hyperintense signal sign was observed in 18 patients. The R-MPV regimen was statistically associated with a lower rate of residual DWI hyperintense signal (p = 0.0453). The median PFS was statistically shorter in the residual DWI hyperintense signal group than in the non-residual DWI hyperintense signal group (14.0 months vs. 85.1 months) (p < 0.0001, log-rank test). CONCLUSION: A residual DWI hyperintense signal at the end of treatment was statistically associated with shorter PFS. Among patients who achieved CR/CRu evaluated based on postcontrast T1WI, DWI could be a valuable additional sequence to predict the early recurrence of PCNSL.

Synthetic MRI plus FSE-PROPELLER DWI for differentiating malignant from benign head and neck tumors: a preliminary study.

Background: Preoperative classification of head and neck (HN) tumors remains challenging, especially distinguishing early cancerogenic masses from benign lesions. Synthetic MRI offers a new way for quantitative analysis of tumors. The present study investigated the application of synthetic MRI and stimulus and fast spin echo diffusion-weighted imaging with periodically rotated overlapping parallel lines with enhanced reconstruction (FSE-PROPELLER DWI) to differentiate malignant from benign HN tumors. Materials and methods: Forty-eight patients with pathologically confirmed HN tumors were retrospectively recruited between August 2022 and October 2022. The patients were divided into malignant (n = 28) and benign (n = 20) groups. All patients were scanned using synthetic MRI and FSE-PROPELLER DWI. T1, T2, and proton density (PD) values were acquired on the synthetic MRI and ADC values on the FSE-PROPELLER DWI. Results: Benign tumors (ADC: 2.03 ± 0.31 × 10-3 mm2/s, T1: 1741.13 ± 662.64 ms, T2: 157.43 ± 72.23 ms) showed higher ADC, T1, and T2 values compared to malignant tumors (ADC: 1.46 ± 0.37 × 10-3 mm2/s, T1: 1390.06 ± 241.09 ms, T2: 97.64 ± 14.91 ms) (all P<0.05), while no differences were seen for PD values. ROC analysis showed that T2+ADC (cut-off value, > 0.55; AUC, 0.950) had optimal diagnostic performance vs. T1 (cut-off value, ≤ 1675.84 ms; AUC, 0.698), T2 (cut-off value, ≤ 113.24 ms; AUC, 0.855) and PD (cut off value, > 80.67 pu; AUC, 0.568) alone in differentiating malignant from benign lesions (all P<0.05); yet, the difference in AUC between ADC and T2+ADC or T2 did not reach statistical significance. Conclusion: Synthetic MRI and FSE-PROPELLER DWI can quantitatively differentiate malignant from benign HN tumors. T2 value is comparable to ADC value, and T2+ADC values could improve diagnostic efficacy., apparent diffusion coeffificient, head and neck tumors.

Ultra high b-value diffusion weighted imaging enables better molecular grading stratification over histological grading in adult-type diffuse glioma.

PURPOSE: Accurate preoperative radiological staging of adult-type diffuse glioma is crucial for effective prognostic stratification and selection of appropriate therapeutic interventions. The purpose of this study was to compare the effectiveness of apparent diffusion coefficient (ADC) maps generated from ultrahigh b-value diffusion-weighted imaging (DWI) for molecular grading with that for histological grading of adult-type diffuse glioma, and to evaluate the correlation between these ADC maps and molecular and histological biomarkers. METHODS: This study retrospectively enrolled forty adult-type diffuse glioma patients, diagnosed using the 2021 WHO classification criteria. Preoperative imaging data, including multiple b-value DWI and conventional magnetic resonance imaging, were collected. Tumors were graded using both histological and molecular criteria. Histogram analysis was conducted to generate 14 parameters for each tumor. Receiver operating characteristic curves and the area under the curve (AUC) were used to evaluate tumor grading and molecular status differentiation. Analysis of histological biomarkers was performed by calculating the Pearson and Spearman correlation coefficients of continuous and hierarchical variables, respectively. RESULTS: The intensity-related parameters for molecular grading were found to be superior to those for histological grading for the identification of WHO grade 4 (WHO4) adult-type diffuse glioma. The AUC of both grading systems increased with increasing b-values, with ADC8000-based histogram parameters showing the best results (molecular grading, square root: AUC = 0.897; histological grading, median: AUC = 0.737). The intensity-related parameters could also differentiate molecular WHO4 gliomas from histologically lower-grade gliomas (ADC8000-based square root: AUC = 0.919), and different ADC8000-based kurtosis was observed between molecular and histological WHO4 gliomas (AUC = 0.833). Significant correlations between the Ki-67 index and molecular status prediction for IDH, CDKN2A, and EGFR were also demonstrated. CONCLUSION: The histogram parameters derived from high b-value ADC maps were found to be more effective for differentiating molecular grades of WHO4 adult-type diffuse glioma than for differentiating histological grades.

MRI features of neuronal intranuclear inclusion disease, combining visual and quantitative imaging investigations.

OBJECTIVE: To observe the radiological characteristics of Neuronal Intranuclear Inclusion Disease (NIID) on lesion locations and diffusion property using quantitative imaging analysis. METHODS: Visual inspection and quantitative analyses were performed on MRI data from 31 retrospectively included patients with NIID. Frequency heatmaps of lesion locations on T2WI and DWI were generated using voxel-wise analysis. Gray matter volume (GMV), white matter volume (WMV) and diffusion property of apparent diffusion coefficient (ADC) values of patients were voxel-wisely compared with healthy controls. Moreover, the ADC values within the DWI-detected lesion were compared with those within the adjacent cortical gray matter and white matter. Voxel-based lesion symptom mapping (VLSM) techniques, were used to determine the relationship between DWI lesion location and disease durations. RESULTS: By visual inspection on the imaging findings, we proposed an "cockscomb flower sign" for describing the radiological feature of DWI hyperintensity within the corticomedullary junction. A "T2WI-DWI mismatch of spatial distribution" pattern was also revealed with visual inspection and frequency heatmaps, for describing the feature of a wider lesion distribution covering white matter shown on T2WI than that on DWI. Voxel-based morphometry comparison revealed that wildly reduced GMV and WMV, both the lesion areas detected by DWI and T2WI demonstrated ADC increase in patients. Furthermore, the ADC values within the DWI-detected lesion were intermediate between the adjacent cortex and the deep white matter with highest ADC. VLSM analysis revealed that frontal lobe, parietal lobe and internal capsule damage were associated with higher NIID durations. CONCLUSION: NIID features with "cockscomb flower-like" DWI hyperintensity in area of corticomedullary junction, based on a "T2WI-DWI mismatch of spatial distribution" of lesion locations. The pathological substrate of corticomedullary junction hyperintensity on DWI, can not be explained as diffusion restriction. These typical radiological features of brain MRI would be helpful for diagnosis of NIID.

The advances in the early and accurate diagnosis of Creutzfeldt-Jakob disease and other prion diseases: where are we today?

INTRODUCTION: Before the introduction of MRI diffusion-weighted images (DWI), the diagnosis of Creutzfeldt-Jakob disease (CJD) relied upon nonspecific findings including clinical symptoms, EEG abnormalities, and elevated levels of cerebrospinal fluid 14-3-3 protein. Subsequently, the use of DWI has improved diagnostic accuracy, but it sometimes remains difficult to differentiate CJD from encephalitis, epilepsy, and other dementing disorders. The revised diagnostic criteria include real-time quaking-induced conversion (RT-QuIC), detecting small amounts of CJD-specific prion protein, and clinically sensitive DWI. Combining these techniques has further improved diagnostic accuracy, enabling earlier diagnosis. AREAS COVERED: Herein, the authors review the recent advances in diagnostic methods and revised diagnostic criteria for sporadic CJD. They also discuss other prion diseases, such as variant CJD and chronic wasting disease, where the emergence of new types is a concern. EXPERT OPINION: Despite improvements in diagnostic methods and criteria, some subtypes of prion disease are still difficult to diagnose, and even the diagnosis using the most innovative RT-QuIC test remains a challenge in terms of accuracy and standardization. However, these revised criteria can be adapted to the emergence of new types of prion diseases. It is essential to continue careful surveillance and update information on the latest prion disease phenotypes.

Evaluating Biliary Malignancy with Measured and Calculated Ultra-high b-value Diffusion-weighted MR Imaging at 3T.

PURPOSE: Although diffusion-weighted imaging (DWI) with ultra-high b-values is reported to be advantageous in the detection of some tumors, its applicability is not yet known in biliary malignancy. Therefore, this study aimed to evaluate the impact of measured b = 1400 s/mm2 (M1400) and calculated b = 1400 s/mm2 (C1400) DWI on image quality and quality of lesion discernibility using a modern 3T MR system compared to conventional b = 800 s/mm2 DWI (M800). METHODS: We evaluated 56 patients who had pathologically proven biliary malignancy. All the patients underwent preoperative or baseline 3T MRI using DWI (b = 50, 400, 800, and 1400 s/mm2). The calculated DWI was obtained using a conventional DWI set (b = 50, 400, and 800). The tumor-to-bile contrast ratio (CR) and tumor SNR were compared between the different DWI images. Likert scores were given on a 5-point scale to assess the overall image quality, overall artifacts, ghost artifacts, misregistration artifacts, margin sharpness, and lesion discernibility. Repeated-measures analysis of variance with post hoc analyses was used for statistical evaluations. RESULTS: The CR of the tumor-to-bile was significantly higher in both M1400 and C1400 than in M800 (Pa < 0.01). SNRs were significantly higher in M800, followed by C1400 and M1400 (Pa < 0.01). Lesion discernibility was significantly improved for M1400, followed by C1400 and M800 for both readers (Pa < 0.01). CONCLUSION: Using a 3T MRI, both measured and calculated DWI with an ultra-high b-value offer superior lesion discernibility for biliary malignancy compared to the conventional DWI.

NOTCH2NLC GGC repeat expansion causes retinal pathology with intranuclear inclusions throughout the retina and causes visual impairment.

The retinal pathology of genetically confirmed neuronal intranuclear inclusion disease (NIID) is yet unknown. We report the ocular findings in four NIID patients with NOTCH2NLC GGC repeat expansion to investigate the pathology of retinopathy. All four NIID patients were diagnosed by skin biopsy and NOTCH2NLC GGC repeat analysis. Ocular findings in patients with NIID were studied using fundus photographs, optical coherence tomographic images (OCT), and full-field electroretinograms (ERGs). The histopathology of the retina was studied on autopsy samples from two cases with immunohistochemistry. All patients had an expansion of the GGC repeat (87-134 repeats) in the NOTCH2NLC. Two patients were legally blind and had been diagnosed with retinitis pigmentosa prior to the diagnosis of NIID and assessed with whole exome sequencing to rule out comorbidity with other retinal diseases. Fundus photographs around the posterior pole showed chorioretinal atrophy in the peripapillary regions. OCT showed thinning of the retina. ERGs showed various abnormalities in cases. The histopathology of autopsy samples showed diffusely scattered intranuclear inclusions throughout the retina from the retinal pigment epithelium to the ganglion cell layer, and optic nerve glial cells. And severe gliosis was observed in retina and optic nerve. The NOTCH2NLC GGC repeat expansion causes numerous intranuclear inclusions in the retina and optic nerve cells and gliosis. Visual dysfunction could be the first sign of NIID. We should consider NIID as one of the causes of retinal dystrophy and investigate the GGC repeat expansion in NOTCH2NLC.

Usefulness of intravoxel incoherent motion MRI for visualizing slow cerebrospinal fluid motion.

BACKGROUND: In the cerebrospinal fluid (CSF) dynamics, the pulsations of cerebral arteries and brain is considered the main driving force for the reciprocating bidirectional CSF movements. However, measuring these complex CSF movements on conventional flow-related MRI methods is difficult. We tried to visualize and quantify the CSF motion by using intravoxel incoherent motion (IVIM) MRI with low multi-b diffusion-weighted imaging. METHODS: Diffusion-weighted sequence with six b values (0, 50, 100, 250, 500, and 1000 s/mm2) was performed on 132 healthy volunteers aged ≥ 20 years and 36 patients with idiopathic normal pressure hydrocephalus (iNPH). The healthy volunteers were divided into three age groups (< 40, 40 to < 60, and ≥ 60 years). In the IVIM analysis, the bi-exponential IVIM fitting method using the Levenberg-Marquardt algorithm was adapted. The average, maximum, and minimum values of ADC, D, D*, and fraction of incoherent perfusion (f) calculated by IVIM were quantitatively measured in 45 regions of interests in the whole ventricles and subarachnoid spaces. RESULTS: Compared with healthy controls aged ≥ 60 years, the iNPH group had significantly lower mean f values in all the parts of the lateral and 3rd ventricles, whereas significantly higher mean f value in the bilateral foramina of Luschka. In the bilateral Sylvian fossa, which contain the middle cerebral bifurcation, the mean f values increased gradually with increasing age, whereas those were significantly lower in the iNPH group. In the 45 regions of interests, the f values in the bilateral foramina of Luschka were the most positively correlated with the ventricular size and indices specific to iNPH, whereas that in the anterior part of the 3rd ventricle was the most negatively correlated with the ventricular size and indices specific to iNPH. Other parameters of ADC, D, and D* were not significantly different between the two groups in any locations. CONCLUSIONS: The f value on IVIM MRI is useful for evaluating small pulsatile complex motion of CSF throughout the intracranial CSF spaces. Patients with iNPH had significantly lower mean f values in the whole lateral ventricles and 3rd ventricles and significantly higher mean f value in the bilateral foramina of Luschka, compared with healthy controls aged ≥ 60 years.

The utility of diffusion-weighted imaging for differentiation of phyllodes tumor from fibroadenoma and breast cancer.

Objective: To evaluate the utility of apparent diffusion coefficient (ADC) values for differentiating breast tumors. Methods: The medical records of 17 patients with phyllodes tumor [PT; circular regions of interest (ROI-cs) n = 171], 74 patients with fibroadenomas (FAs; ROI-cs, n = 94), and 57 patients with breast cancers (BCs; ROI-cs, n = 104) confirmed by surgical pathology were retrospectively reviewed. Results: There were significant differences between PTs, FAs, and BCs in ADCmean, ADCmax, and ADCmin values. The cutoff ADCmean for differentiating PTs from FAs was 1.435 × 10-3 mm2/s, PTs from BCs was 1.100 × 10-3 mm2/s, and FAs from BCs was 0.925 × 10-3 mm2/s. There were significant differences between benign PTs, borderline PTs, and malignant PTs in ADCmean, ADCmax, and ADCmin values. The cutoff ADCmean for differentiating benign PTs from borderline PTs was 1.215 × 10-3 mm2/s, and borderline PTs from malignant PTs was 1.665 × 10-3 mm2/s. Conclusion: DWI provides quantitative information that can help distinguish breast tumors.

Computed tomography, magnetic resonance imaging, and positron emission tomography/computed tomography findings for the diagnosis of malignant struma ovarii: A case report.

Malignant struma ovarii (MSO) is an extremely rare disease arising from struma ovarii. Preoperative diagnosis is still challenging due to the lack of criteria for imaging findings. Herein, we report a case of MSO with suggestive imaging findings for a 50-year-old woman who presented with a pelvic tumor. The tumor did not typically show characteristic imaging findings of struma ovarii; however, the findings implied colloids of thyroid tissue within solid components on the magnetic resonance imaging (MRI) and computed tomography. Additionally, the solid components showed hyperintensity on diffusion-weighted image and hypointensity on apparent diffusion coefficient maps. Total abdominal hysterectomy, bilateral salpingo-oophorectomy, and omentectomy were performed. Histopathological examination revealed MSO of the right ovary, pT1aNXM0. The distribution of papillary thyroid carcinoma tissue corresponded to restricted diffusion area on MRI. In conclusion, the coexistence of imaging findings suggesting thyroid tissue and restricted diffusion in the solid component on MRI could indicate MSO.

A feasibility study of reduced full-of-view synthetic high-b-value diffusion-weighted imaging in uterine tumors.

OBJECTIVES: This study aimed to evaluate the feasibility of reduced full-of-view synthetic high-b value diffusion-weighted images (rFOV-syDWIs) in the clinical application of cervical cancer based on image quality and diagnostic efficacy. METHODS: We retrospectively evaluated the data of 35 patients with cervical cancer and 35 healthy volunteers from May to November 2021. All patients and volunteers underwent rFOV-DWI scans, including a 13b-protocol: b = 0, 25, 50, 75, 100, 150, 200, 400, 600, 800, 1000, 1200, and 1500 s/mm2 and a 5b-protocol: b = 0, 100, 400, 800,1500 s/mm2. rFOV-syDWIs with b values of 1200 (rFOV-syDWIb=1200) and 1500 (rFOV-syDWIb=1500) were generated from two different multiple-b-value image datasets using a mono-exponential fitting algorithm. According to homoscedasticity and normality assessed by the Levene's test and Shapiro-Wilk test, the inter-modality differences of quantitative measurements were, respectively, examined by Wilcoxon signed-rank test or paired t test and the inter-group differences of ADC values were examined by independent t test or Mann-Whitney U test. RESULTS: A higher inter-reader agreement between SNRs and CNRs was found in 13b-protocol and 5b-protocol rFOV-syDWIb=1200/1500 compared to 13b-protocol rFOV-sDWIb=1200/1500 (p < 0.05). AUC of 5b-protocol syADCmean,b=1200/1500 and syADCminimum,b=1200/1500 was equal or higher than that of 13b-protocol sADCmean,b=1200/1500 and sADCminimum,b=1200/1500. CONCLUSIONS: rFOV-syDWIs provide better lesion clarity and higher image quality than rFOV-sDWIs. 5b-protocol rFOV-syDWIs shorten scan time, and synthetic ADCs offer reliable diagnosis value as scanned 13b-protocol DWIs.