森 墾

Introduction: Kawasaki disease (KD) is an acute systemic vasculitis in children, but 0.4% of patients with KD exhibit central nervous system involvement. Acute encephalitis and encephalopathy accompanied with KD have been reported to be mostly self-limiting complications. Case Presentation: A 2-year-old girl developed recurrent vomiting, a cluster of generalized seizures, and decreased consciousness on day 12 after the onset of KD. Magnetic resonance imaging (MRI) T2-weighted images on day 13 showed high signal intensities in bilaterally symmetrical and subcortical white matter and thalamus, and linear radial hyperintensities parallel to the cerebral vessels of the periventricular white matter. Diffuse white matter hyperintensity on the apparent diffusion coefficient map suggested vasogenic edema. Subsequently, lethal cerebral edema rapidly progressed in 8 hrs after the MRI examination. Conclusion: To our knowledge, acute fulminant cerebral edema in patients with KD has not been previously reported. We should be aware of the possibility of severe encephalitis related to KD. Furthermore, diffuse white matter vasogenic edema with perivascular abnormalities on MRI may be an alerm, potentially leading to fatal cerebral edema.

BACKGROUND: The coexistence of falcine and occipital sinuses is rare and its natural course has not been reported. CASE REPORTS: Two patients with persistent falcine and occipital sinuses are described. Both patients had straight sinuses. In one, both the transverse and sigmoid sinuses were hypoplastic and the patient had an acquired Chiari I malformation. The other patient had no other venous anomalies and had a normal posterior cranial fossa. CONCLUSION: The coexistence of falcine and occipital sinuses can lead to an acquired Chiari I malformation. These cases suggest the importance of checking other venous and brain anomalies in this situation.

BACKGROUND AND PURPOSE: Recent studies suggest that the autoantibodies against adrenergic/muscarinic receptors might be one of the causes and potential markers of myalgic encephalomyelitis/chronic fatigue syndrome (ME/CFS). The purpose of this study was to investigate the structural network changes related to autoantibody titers against adrenergic/muscarinic receptors in ME/CFS by performing a single-subject gray matter similarity-based structural network analysis. METHODS: We prospectively examined 89 consecutive right-handed ME/CFS patients who underwent both brain MRI including 3D T1-wighted images and a blood analysis of autoantibodies titers against β1 adrenergic receptor (β1 AdR-Ab), β2 AdR-Ab, M3 acetylcholine receptor (M3 AchR-Ab), and M4 AchR-Ab. Single-subject gray matter similarity-based structural networks were extracted from segmented gray matter images for each patient. We calculated local network properties (betweenness centrality, clustering coefficient, and characteristic path length) and global network properties (normalized path length λ, normalized clustering coefficient γ, and small-world network value δ). We investigated the correlations between the autoantibody titers and regional gray matter/white matter volumes, the local network properties, and the global network properties. RESULTS: Betweenness centrality showed a significant positive correlation with β1-AdR-Ab in the right dorsolateral prefrontal cortex. The characteristic path length showed a significant negative correlation with β2-AdR-Ab in the right precentral gyrus. There were no significant correlations between the antibody titers and the regional gray matter/white matter volumes, and the global network properties. CONCLUSIONS: Our findings suggest that β1 AdR-Ab and β2 AdR-Ab are potential markers of ME/CFS.

This study aimed to evaluate the visualization of peripheral bronchioles in normal lungs via quarter-detector computed tomography (QDCT). Visualization of bronchioles within 10 mm from the pleura is considered a sign of bronchiectasis. However, it is not known peripheral bronchioles how close to the pleura in normal lungs can be tracked using QDCT. This study included 228 parts in 76 lungs from 38 consecutive patients who underwent QDCT. Reconstruction was performed with different thicknesses, increments, and matrix sizes: 0.5-mm thickness and increment with 512 and 1024 matrixes (Group5 and Group10, respectively) and 0.25-mm thickness and increment with 1024 matrix (Group10Thin). The distance between the most peripheral bronchiole visible and the pleura was determined in the three groups. The distance between the peripheral bronchial duct ends and the nearest pleural surface were significantly shorter in the order of Group10Thin, Group10, and Group5, and the mean distances from the pleura in Group10Thin and Group10 were shorter than 10 mm. These findings suggest the visualization of peripheral bronchioles in QDCT was better with a 1024 axial matrix than with a 512 matrix, and with a 0.25-mm slice thickness/increment than with a 0.5-mm slice thickness/increment. Our study also indicates bronchioles within 10 mm of the pleura do not necessarily indicate pathology.

BACKGROUND AND PURPOSE: Hypophysitis is one of the well-known adverse effects of immune checkpoint inhibitors. Immune checkpoint inhibitor-induced hypophysitis frequently causes irreversible hypopituitarism, which requires long-term hormone replacement. Despite the high frequency and clinical significance, characteristic MR imaging findings of immune checkpoint inhibitor-induced hypophysitis have not been established. In the present study, we aimed to review and extract the MR imaging features of immune checkpoint inhibitor-induced hypophysitis. MATERIALS AND METHODS: This retrospective international multicenter study comprised 20 patients with melanoma who were being treated with immune checkpoint inhibitors and clinically diagnosed with immune checkpoint inhibitor-induced hypophysitis. Three radiologists evaluated the following MR imaging findings: enlargement of the pituitary gland and stalk; homogeneity of enhancement of the pituitary gland; presence/absence of a well-defined poorly enhanced area and, if present, its location, shape, and signal intensity in T2WI; and enhancement pattern in contrast-enhanced dynamic MR imaging. Clinical symptoms and hormone levels were also recorded. RESULTS: Enlargement of the pituitary gland and stalk was observed in 12 and 20 patients, respectively. Nineteen patients showed poorly enhanced lesions (geographic hypoenhancing lesions) in the anterior lobe, and 11 of these lesions showed hypointensity on T2WI. Thyrotropin deficiency and corticotropin deficiency were observed in 19/20 and 12/17 patients, respectively, which persisted in 12/19 and 10/12 patients, respectively, throughout the study period. CONCLUSIONS: Pituitary geographic hypoenhancing lesions in the anterior lobe of the pituitary gland are characteristic and frequent MR imaging findings of immune checkpoint inhibitor-induced hypophysitis. They reflect fibrosis and are useful in distinguishing immune checkpoint inhibitor-induced hypophysitis from other types of hypophysitis/tumors.

OBJECTIVES: To compare CT findings of early (within 3 weeks post-onset)- and later (within 1 month before or after diagnostic criteria were satisfied, and later than 3 weeks post-onset) stage thrombocytopenia, anasarca, fever, reticulin fibrosis, renal dysfunction, and organomegaly (TAFRO) syndrome. METHODS: Between 2014 and 2019, 13 patients with TAFRO syndrome (8 men and 5 women; mean age, 54.9 years) from nine hospitals were enrolled. The number of the following CT findings (CT factors) was recorded: the presence of anasarca, organomegaly, adrenal ischaemia, anterior mediastinal lesion, bony lesion, and lymphadenopathy. Records of adrenal disorders (adrenomegaly, ischaemia, and haemorrhage) throughout the disease course were also collected. Differences in CT factors at each stage were statistically compared between remission and deceased groups. RESULTS: Para-aortic oedema and mild lymphadenopathy were observed in all patients, whereas pleural effusion, ascites, and subcutaneous oedema were found in 5/13, 7/13, and 7/13 cases, respectively, at the early stage. CT factors at the early stage were significantly higher in the deceased than in the remission group (mean, 11 vs 6.5; p = 0.04), while they were nonsignificant at the later stage. Adrenal disorders were present in 7/13 cases throughout the course including 6 of adrenomegaly and 4 of ischaemia at the early stage. CONCLUSIONS: Para-aortic oedema and mild lymphadenopathy are most common at the early stage. Anasarca, organomegaly, lymphadenopathy, and adrenal disorders on early-stage CT are useful for unfavourable prognosis prediction. Moreover, adrenal disorders are frequent even at the early stage and are useful for early diagnosis of TAFRO syndrome. KEY POINTS: • CT findings facilitate early diagnosis and prognosis prediction in TAFRO syndrome. • Adrenal disorders are frequently observed in TAFRO syndrome. • Adrenal disorders are useful for differential diagnosis of TAFRO syndrome.

This pilot study tests the feasibility of rapid carotid MR angiography using the liver acquisition with volume acceleration-flex technique (LAVA MRA). Seven healthy volunteers and 21 consecutive patients suspected of carotid stenosis underwent LAVA and conventional time-of-flight (cTOF) MRAs. Artery-to-fat and artery-to-muscle signal intensity ratios were manually measured. LAVA MRA exhibited a significantly larger artery-to-fat signal intensity ratio compared with cTOF MRA in all slices (P < 0.001) and exhibited a larger (P < 0.001) or equivalent (P = 1.0) artery-to-muscle signal intensity ratio in the extracranial carotid arteries. The image quality of the cervical carotid bifurcation and the signal change on each MRA were visually assessed and compared among the MRAs. There was no significant difference between the two MRAs in visual assessment. LAVA MRA can provide visualization similar to cTOF MRA in the evaluation of the cervical carotid bifurcation while reducing scan time by one-fifth.

Genital arteriovenous malformations are rare and present unique surgical challenges in preserving urogenital function, abdominal wall integrity, and lower limb perfusion. A 32-year-old man with a giant abdominoscrotal arteriovenous malformation presented with recurrent heavy bleeding. Due to the high risk of rebleeding and fatal hemorrhage, surgery with curative intent was proposed and the patient was counseled on the risks of ischemia to the lower limb, testes, and penis. Preoperative embolization of the feeding vessels was performed. Three days later, surgical excision of the mass with the affected scrotum, left rectus muscle, sheath, and overlying abdominal skin followed. The testes were dissected from the malformation and preserved along with the right internal pudendal artery. The left thigh skin was advanced to the scrotal remnants and a neoscrotum created. The resulting large abdominal wall defect was reconstructed in layers with a pedicled anterolateral thigh flap, including innervated vastus lateralis muscle, to prevent herniation. Recovery was uneventful, and a 4-year follow-up revealed no significant clinical or radiological recurrence with recovery of flap sensation, retained erectile function, and no herniation. We report this case due to rarity of giant abdominoscrotal arteriovenous malformations and present preoperative embolization, surgical resection, and functional anterolateral thigh flap reconstruction as a valuable treatment option of this life-threatening illness.

<title>Abstract</title> Accumulated experience supports the efficacy of allogenic haematopoietic stem cell transplantation in arresting the progression of childhood-onset cerebral form of adrenoleukodystrophy in early stages. For adulthood-onset cerebral form of adrenoleukodystrophy, however, there have been only a few reports on haematopoietic stem cell transplantation and the clinical efficacy and safety of that for adulthood-onset cerebral form of adrenoleukodystrophy remain to be established. To evaluate the clinical efficacy and safety of haematopoietic stem cell transplantation, we conducted haematopoietic stem cell transplantation on 12 patients with adolescent-/adult-onset cerebral form/cerebello-brainstem form of adrenoleukodystrophy in a single-institution-based prospective study. Through careful prospective follow-up of 45 male adrenoleukodystrophy patients, we aimed to enrol patients with adolescent-/adult-onset cerebral form/cerebello-brainstem form of adrenoleukodystrophy at early stages. Indications for haematopoietic stem cell transplantation included cerebral form of adrenoleukodystrophy or cerebello-brainstem form of adrenoleukodystrophy with Loes scores up to 13, the presence of progressively enlarging white matter lesions and/or lesions with gadolinium enhancement on brain MRI. Clinical outcomes of haematopoietic stem cell transplantation were evaluated by the survival rate as well as by serial evaluation of clinical rating scale scores and neurological and MRI findings. Clinical courses of eight patients who did not undergo haematopoietic stem cell transplantation were also evaluated for comparison of the survival rate. All the patients who underwent haematopoietic stem cell transplantation survived to date with a median follow-up period of 28.6 months (4.2–125.3 months) without fatality. Neurological findings attributable to cerebral/cerebellar/brainstem lesions became stable or partially improved in all the patients. Gadolinium-enhanced brain lesions disappeared or became obscure within 3.5 months and the white matter lesions of MRI became stable or small. The median Loes scores before haematopoietic stem cell transplantation and at the last follow-up visit were 6.0 and 5.25, respectively. Of the eight patients who did not undergo haematopoietic stem cell transplantation, six patients died 69.1 months (median period; range 16.0–104.1 months) after the onset of the cerebral/cerebellar/brainstem lesions, confirming that the survival probability was significantly higher in patients with haematopoietic stem cell transplantation compared with that in patients without haematopoietic stem cell transplantation (P = 0.0089). The present study showed that haematopoietic stem cell transplantation was conducted safely and arrested the inflammatory demyelination in all the patients with adolescent-/adult-onset cerebral form/cerebello-brainstem form of adrenoleukodystrophy when haematopoietic stem cell transplantation was conducted in the early stages. Further studies are warranted to optimize the procedures of haematopoietic stem cell transplantation for adolescent-/adult-onset cerebral form/cerebello-brainstem form of adrenoleukodystrophy.

PURPOSE: The purpose of this retrospective study was to evaluate the usefulness of serum tumour markers and morphological characteristics in CT/MRI to differentiate between ovarian metastases from colorectal carcinomas (OMCRC) and primary ovarian carcinomas (POC). METHOD: Preoperative radiological images of 41 OMCRCs from 27 patients (mean age ± SD: 52.2 ± 10.7 years) and 46 POCs from 36 patients (52.1 ± 12.7 years) were included. Three blinded gynecological radiologists classified tumour morphology into 'mille-feuille sign', 'solid and cystic', 'multicystic without nodules', and 'multicystic with nodules' groups and analysed using Fisher's exact test. Serum carcinoembryonic antigen (CEA), cancer antigen 125 (CA125), and carbohydrate antigen 19-9 levels were compared by Wilcoxon rank-sum test. RESULTS: 'Mille-feuille sign' indicated OMCRC (OMCRC: 8/41, POC: 1/46, specificity = 0.98, p = 0.011) and had excellent interobserver agreement (Fleiss's kappa value = 0.96). 'Solid and cystic' indicated POC (18/41 vs 41/45, p < 0.001) and 'multicystic without nodules' indicated OMCRC (8/41 vs 2/46, p = 0.041). There was no significant difference in 'multicystic with nodules'. CA125 levels were higher in POCs (292.5 U/mL vs. 41.0 U/mL, p = 0.003). CEA levels were higher in OMCRCs (24.5 ng/mL vs 2 ng/mL, p < 0.001). CEA (< 6.3 ng/mL) AND (CA125 (≥87.0 U/mL) OR 'solid and cystic') indicated POC with high accuracy (3/41 vs 44/46, accuracy = 0.94, p < 0.001). CONCLUSIONS: Our new method with morphological classification and tumour markers were useful for differentiating the two tumours. In particular, the 'mille-feuille sign' frequently indicated OMCRC with high specificity and excellent interobserver agreement.

OBJECTIVES: To understand the sources of variability in diagnostic performance among experienced radiologists. DESIGN: All prostate MRI examinations performed between 2016 and 2018 were retrospectively reviewed. SETTING: University hospital in Japan. PARTICIPANTS: Data derived from 334 pathology-proven cases (male, mean age: 70 years; range: 35-90 years) that were interpreted by 10 experienced radiologists were subjected to the analysis. PRIMARY AND SECONDARY OUTCOME MEASURES: Diagnostic performance measures of the radiologists were compared with candidate factors, including interpretive volume of prostate MRIs, volume of self-directed and assigned total annual interpretive work, and years of experience. The potential influence of fatigue was also evaluated by examining the effect of the report's issue time. RESULTS: There were 186 prostate cancer cases. Performance was based on accuracy, sensitivity and specificity (86%, 85% and 84%, respectively). While performance was not correlated with the volume of prostate MRIs, per se (ρ=-0.15, p=0.69; ρ=-0.01, p=0.99; ρ=-0.33, p=0.36) or the total MRIs assigned for each radiologist (p>0.6) or years of experience (p>0.4), all measures were strongly correlated with voluntary work represented by the interpretive volume of abdominal CTs (r=0.79, p<0.01; r=0.80, p<0.01; r=0.64, p=0.048). The performance did not differ based on the issue time of the report (morning, afternoon and evening) (χ2(2)=3.65, p=0.16). CONCLUSIONS: Greater autonomy, represented as enhanced self-directed interpretive work, was most significantly correlated with the performance of prostate MRI interpretation. The lack of a correlation between the performance and assigned volume confirms the complexity of human learning. Together, these findings support the hypothesis that successful promotion of internal drivers could have a pervasive positive impact on improving diagnostic performance.

Introduction: Multinodular and vacuolating neuronal tumor (MVNT) had been initially described as an epilepsy-related brain tumor, but recent studies demonstrated it could be found incidentally in non-epilepsy patients. Case report: A 33-year-old woman with intractable post-encephalitis epilepsy presented a cluster of multinodular T2 hyperintensity in the left temporal lobe, which was very similar to the characteristics of MVNT. Long-term video electroencephalogram demonstrated that the habitual seizures were originated from bilateral temporal area and the interictal epileptic discharges were seen multifocally, although the lesions with MVNT appearance were localized in the left temporal lobe. It was presumed that the epilepsy in this patient was due to encephalitis in the past, and the link between the lesions and the epilepsy in this patient seemed weak. Conclusion: Although MVNT had been considered as an epilepsy-related brain tumor, we suggest it is not necessarily preferable to perform surgical resection of MVNT even on patients with epilepsy, unless epileptic foci are highly related to MVNT.