Neurosarcoidosis with enlargement of the dorsal root ganglia: A case report.

We report the case of a woman in her 40s who presented with sensory disturbances in all 4 limbs and left facial palsy. MRI revealed asymmetric enlargement of the dorsal root ganglia, which was enhanced by gadolinium-a chest CT scan identified enlarged supraclavicular, mediastinal, and hilar lymph nodes. A biopsy of a hilar lymph node showed noncaseating epithelioid granulomas, confirming a sarcoidosis diagnosis. Prednisolone treatment led to symptomatic improvements. In sarcoidosis of the peripheral nervous system, there might be observable enlargement of the dorsal root ganglion alongside enhanced gadolinium contrast. Obtaining a biopsy from the dorsal root ganglion poses challenges, and radiologists should be mindful of this specific imaging characteristic.

Ultrasound-based radiomic analysis of the peripheral nerves for differentiation between CIDP and POEMS syndrome.

BACKGROUND: Demyelinating peripheral neuropathy is characteristic of both polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome and chronic inflammatory demyelinating polyneuropathy (CIDP). We hypothesized that the different pathogeneses underlying these entities would affect the sonographic imaging features. PURPOSE: To investigate whether ultrasound (US)-based radiomic analysis could extract features to describe the differences between CIDP and POEMS syndrome. MATERIAL AND METHODS: In this retrospective study, we evaluated nerve US images from 26 with typical CIDP and 34 patients with POEMS syndrome. Cross-sectional area (CSA) and echogenicity of the median and ulnar nerves were evaluated in each US image of the wrist, forearm, elbow, and mid-arm. Radiomic analysis was performed on these US images. All radiomic features were examined using receiver operating characteristic analysis. Optimal features were selected using a three-step feature selection method and were inputted into XGBoost to build predictive machine-learning models. RESULTS: The CSAs were more enlarged in patients with CIDP than in those with POEMS syndrome without significant differences, except for that of the ulnar nerve at the wrist. Nerve echogenicity was significantly more heterogeneous in patients with CIDP than in those with POEMS syndrome. The radiomic analysis yielded four features with the highest area under the curve (AUC) value of 0.83. The machine-learning model showed an AUC of 0.90. CONCLUSION: US-based radiomic analysis has high AUC values in differentiating POEM syndrome from CIDP. Machine-learning algorithms further improved the discriminative ability.

Cerebral cortex swelling in V180I genetic Creutzfeldt-Jakob disease: comparative imaging study between sporadic and V180I genetic Creutzfeldt-Jakob disease in the early stage.

The most common genetic Creutzfeldt-Jakob disease (gCJD) in Japan is caused by a point mutation in which isoleucine replaces valine at codon 180 of the prion protein (PrP) gene (V180I gCJD). Evidence suggests that cerebral cortex swelling, which appears as abnormal hyperintensities on diffusion-weighted imaging (DWI), is a characteristic magnetic resonance imaging (MRI) finding of V180I gCJD. However, no study has directly compared the MRI findings between V180I gCJD and sporadic CJD (sCJD). The current study, therefore, aims to clarify the imaging features of V180I gCJD, which would lead to prompt genetic counselling and analysis of the PrP gene, particularly focusing on cerebral cortex swelling. We included 35 patients with sCJD (n = 23) or V180I gCJD (n = 12). Cerebral cortex swelling on T2-weighted imaging (T2WI) or fluid-attenuated inversion recovery (FLAIR) wherein abnormal cortical hyperintensities were observed on DWI, and the distribution of grey matter hyperintensities on DWI were visually evaluated. V180I gCJD patients had significantly more cerebral cortex swelling (100% vs. 13.0%, p < 0.001), an overall correct classification of 91.4%, and parahippocampal gyrus hyperintensities on DWI (100% vs. 39.1%, q = 0.019) than sCJD patients. Cerebral cortical hyperintensities on DWI with swelling on T2WI or FLAIR are characteristic imaging findings of V180I gCJD and are useful for differentiating it from sCJD.

Late Nonanastomotic Pseudoaneurysm From the Prosthetic Graft: A Successful Transcatheter Arterial Embolization Using Coils.

A 71-year-old man who received a total arch replacement with a knitted Dacron® graft presented aneurysmal sac re-expansion due to leakage at the distal anastomotic site of the graft. He did not tolerate the stress of general anesthesia due to severe pulmonary function impairment. Therefore, thoracic endovascular aortic repair (TEVAR) in zone 3 was performed under epidural anesthesia. Contrast-enhanced computed tomography (CT) revealed another leakage into the aneurysmal sac in zone 1 after performing TEVAR. Because open surgical repair and debranching TEVAR were contraindicated, transcatheter arterial embolization was performed with careful consideration of his comorbidities. Follow-up contrast-enhanced CT performed 2 weeks after embolization indicated no opacification of the aneurysmal sac, and noncontrast-enhanced CT a year after embolization showed no dilatation of the aneurysmal sac.

Transnasal endoscopic resection of Epstein-Barr virus-associated cavernous sinus tumour.

Epstein-Barr virus-associated smooth muscle tumour (EBV-SMT) is a rare disease occurring in immunosuppressed patients, such as those with AIDS, post-transplantation immunodeficiency and congenital immunodeficiency. Intracranial EBV-SMT after solid organ transplantation has been reported. However, intracranial lesions after bone marrow transplantation are extremely rare. We report the case of a 47-year-old man with a history of acute myeloid leukaemia and bone marrow transplantation. He had symptoms of trigeminal neuralgia, and MRI revealed a left cavernous sinus tumour. He started taking oral gabapentin, but his symptoms did not improve. We performed transnasal endoscopic surgery. Postoperative MRI showed complete removal of the cavernous sinus lesion. Pathological examination showed spindle-shaped cells positive for smooth muscle markers and EBV-encoded small RNA in situ hybridisation. EBV-SMT was pathologically diagnosed. His symptoms improved after surgery. No tumour recurrence was noted on follow-up MRI after 15 months without adjuvant radiation or chemotherapy.

Diagnostic performance and inter-operator variability of apparent diffusion coefficient analysis for differentiating pleomorphic adenoma and carcinoma ex pleomorphic adenoma: comparing one-point measurement and whole-tumor measurement including radiomics approach.

BACKGROUND AND PURPOSE: The purpose of this study was to compare the diagnostic performance between apparent diffusion coefficient (ADC) analysis of one-point measurement and whole-tumor measurement, including radiomics for differentiating pleomorphic adenoma (PA) from carcinoma ex pleomorphic adenoma (CXPA), and to evaluate the impact of inter-operator segmentation variability. MATERIALS AND METHODS: One hundred and fifteen patients with PA and 22 with CXPA were included. Four radiologists with different experience independently placed one-point and whole-tumor ROIs and a radiomics-predictive model was constructed from the extracted imaging features. We calculated the area under the receiver-operator characteristic curve (AUC) for the diagnostic performance of imaging features and the radiomics-predictive model. RESULTS: AUCs of the imaging features from whole-tumor varied between readers (0.50-0.89). The most experienced radiologist (Reader 1) produced significantly high AUCs than less experienced radiologists (Reader 3 and 4; P = 0.01 and 0.009). AUCs were higher for the radiomics-predictive model (0.82-0.87) than for one-point (0.66-0.79) in all readers. CONCLUSION: Some imaging features of whole-tumor and radiomics-predictive model had higher diagnostic performance than one-point. The diagnostic performance of imaging features from whole-tumor alone varied depending on operator experience. Operator experience appears less likely to affect diagnostic performance in the radiomics-predictive model.