藤井 裕之

Mucosa-associated lymphoid tissue (MALT) lymphoma commonly arises from chronic inflammation or autoimmune diseases, such as Sjögren syndrome (SjS). Although rare, amyloid deposition in MALT lymphoma has been reported. We present a rare case of parotid gland MALT lymphoma in a 49-year-old woman, in whom preoperative diagnosis was challenging due to atypical imaging findings resulting from amyloid deposits. MRI showed T2-hypointense and T1-iso- to slightly hyperintense masses in the left parotid gland and right sublingual gland, with predominant marginal contrast enhancement and no significant diffusion restriction. Additionally, atrophy and fatty replacement of the parenchyma were noted in bilateral parotid glands, suggesting SjS. Left superficial parotidectomy was performed and pathological findings confirmed MALT lymphoma with extensive amyloid deposition. Histopathological findings of the resected parotid gland parenchyma also suggested SjS. MALT lymphoma should be considered in the differential diagnosis of multiple salivary gland masses in patients with suspected SjS. If MRI reveals atypical imaging findings for malignant lymphoma, particularly T2-hypointensity with no significant diffusion restriction, the possibility of amyloid deposition in MALT lymphoma should be considered.

INTRODUCTION: Moderately differentiated neuroendocrine tumors of the larynx are rare malignant tumors that arise from the submucosa of the larynx, for which surgery is the first-line treatment. PRESENTATION OF CASE: We report a case of moderately differentiated neuroendocrine tumor of the larynx, in which the patient, a 74-year-old man, experienced long-term palliation but an unfortunate outcome of death owing to metastasis. Laryngeal endoscopic examination revealed an elevated submucosal lesion on the laryngeal surface of the epiglottis. Computed tomography and magnetic resonance imaging showed a tumor-like lesion demonstrating a contrasting effect in the submucosa of the epiglottis. A biopsy revealed a moderately differentiated neuroendocrine tumor (formerly called an atypical carcinoid), and a horizontal partial laryngectomy was performed. The patient had a good postoperative course; however, three years and ten months after surgery, he experienced recurrence in the upper gastrointestinal tract and carcinoid syndrome and died four years and three months after the surgery. DISCUSSION: The prognosis of laryngeal neuroendocrine tumors remains poor. In this case, local control was possible without irradiation because the resection margins were negative on pathological examination. This case report has been reported in line with the SCARE Criteria. CONCLUSION: Long-term follow-up of this type of tumor is necessary, as distant metastasis is likely to affect prognosis. In addition to surgery, effective adjuvant therapies, including molecular targeted therapies, should be established.

Introduction Low-grade epilepsy-associated tumors are the second most common histopathological diagnoses in cases of drug-resistant focal epilepsy. However, the connection between neuroimaging features and genetic alterations in these tumors is unclear, prompting an investigation into genotype-relevant neuroimaging characteristics. Methods This study retrospectively analyzed neuroimaging and surgical specimens from 46 epilepsy patients with low-grade epilepsy-associated neuroepithelial tumors that had genetic mutations identified through panel sequencing to investigate their relationship to genotypes. Results Three distinct neuroimaging groups were established: Group 1 had indistinct borders and iso T1-weighted and slightly high or high T2-weighted signal intensities without a diffuse mass effect, associated with 93.8% sensitivity and 100% specificity to BRAF V600E mutations; Group 2 exhibited sharp borders and very or slightly low T1-weighted and very high T2-weighted signal intensities with a diffuse mass effect and 100% sensitivity and specificity for FGFR1 mutations; and Group 3 displayed various characteristics. Histopathological diagnoses including diffuse low-grade glioma and ganglioglioma showed no clear association with genotypes. Notably, postoperative seizure-free rates were higher in Group 1 tumors (BRAF V600E) than in Group 2 tumors (FGFR1). Discussion These findings suggest that tumor genotype may be predicted by neuroimaging before surgery, providing insights for personalized treatment approaches.

PURPOSE: Complete androgen insensitivity syndrome (CAIS) and Mayer-Rokitansky-Küster-Hauser syndrome (MRKHS) share common clinical features such as female phenotype, vaginal hypoplasia, and primary amenorrhea. Magnetic resonance imaging (MRI) is performed to investigate the cause of primary amenorrhea. However, the MRI features are also similar in both disorders. They are ultimately diagnosed by chromosome testing, but there is a possibility of misdiagnosis if chromosome testing is not performed. This study aimed to identify MRI features that are useful for differentiating CAIS from MRKHS. METHOD: This multicenter retrospective study included 12 patients with CAIS and 19 patients with MRKHS. Three radiologists blindly evaluated the following features: (1) detection of vagina, (2) detection of nodular and cystic structures in the lateral pelvis; undescended testicles and paratesticular cysts in CAIS and rudimentary uteri and ovaries in MRKHS, (3) their location, (4) number of cysts in the cystic structures, and (5) signal intensity on diffusion-weighted images (DWI) and apparent diffusion coefficient (ADC) values of the nodular structures. Statistical comparisons were performed using Mann-Whitney U and Fisher's exact tests. RESULTS: Compared with MRKHS, the CAIS group showed significantly detectable vagina, more ventrally located nodular and cystic structures, fewer cysts within the cystic structures, and nodular structures with higher signal intensity on DWI and lower ADC values. CONCLUSIONS: MRI features of detectable vagina, location of nodular and cystic structures, number of cysts within the cystic structures, signal intensity on DWI and ADC values of the nodular structures were useful in differentiating CAIS from MRKHS.

PURPOSE: This study aimed to clarify associations between subacute hematoma on placental magnetic resonance imaging (MRI), antenatal bleeding, and preterm deliveries in patients with placenta previa (PP) without placenta accreta spectrum (PAS). MATERIALS AND METHODS: This retrospective study investigated 78 consecutive patients with PP (median age, 34.5 years; interquartile range [IQR], 31-37 years) who underwent placental MRI in the third trimester. Patients with PAS detected intraoperatively or pathologically were excluded. Two radiologists evaluated the presence of subacute hematomas and their locations on placental MRI. We examined associations between presence of subacute hematoma and antenatal bleeding, emergency cesarean section (CS), hysterectomy, gestational age (GA) at delivery, birth weight, and amount of blood loss at CS. We also examined the association between perinatal outcome and subacute hematoma location: marginal, retro-placental, or intra-placental. Inter-observer agreement for the detection of subacute hematoma was calculated using kappa analysis. RESULTS: Subacute hematomas were identified on MRI in 39 of the 78 patients (50.0%). Antenatal bleeding and emergency CS were more prevalent in patients with subacute hematoma on MRI (20 patients [51.3%] and 18 patients [46.2%], respectively) than in patients without (7 patients [17.9%], Fisher's exact test, p = 0.004 and 7 patients [17.9%], p = 0.014, respectively). GA at delivery was significantly lower in patients with subacute hematoma (median 36w3d, IQR 35w4d-37w1d) than in patients without (median 37w1d, IQR 36w4d-37w2d; Mann-Whitney test: p = 0.048). Marginal hematoma was significantly associated with antenatal bleeding and emergency CS. Inter-observer agreement for the presence of subacute hematoma was moderate (κ = 0.573). CONCLUSION: Subacute hematoma on placental MRI was associated with antenatal bleeding, emergency CS and shorter GA at delivery in patients with PP. Marginal hematoma was also associated with antenatal bleeding and emergency CS. Placental MRI appears useful for predicting antenatal bleeding and preterm delivery in patients with PP.

A 45-year-old woman was hospitalized with severe coronavirus disease 2019 pneumonia. Following cytokine storm-induced multiorgan failure and lethal arrhythmia, the patient developed a sustained coma with flaccid quadriplegia. A cerebrospinal fluid examination excluded infectious and immunogenic encephalopathies, and diffusion-weighted magnetic resonance imaging demonstrated high-intensity areas in the white matter with a cortex-sparing distribution, suggesting delayed post-hypoxic leukoencephalopathy. As a result of intensive cardiopulmonary support for a month, the neurological function gradually recovered. Based on the reversible clinical course noted in this patient, accurate diagnosis and persistent medical approaches are important for the management of coronavirus disease 2019-related delayed post-hypoxic leukoencephalopathy.

Maxillary angiosarcoma, an aggressive tumor derived from vascular endothelial cells, is very rare. Recently, antivascular endothelial growth factor (VEGF) therapies have attracted considerable attention. We describe the clinical course of a patient with maxillary angiosarcoma and discuss the expression of VEGF signaling molecules assessed via immunohistological analysis. An 81-year-old man presented with an aggressive tumor in the left maxillary sinus. Biopsy revealed atypical nuclear cell proliferation, and the tumor was suspected to be a sarcoma. The maxillary malignancy was treated using a multidisciplinary approach with a combination of surgery, radiotherapy, and regional chemotherapy. Examination of the specimen obtained in the first surgery revealed maxillary angiosarcoma, found to be positive for CD31, while negative for CD34, D2-40, and factor Ⅷ. Although no pathological residual tumor was observed after the planned wide surgery, cervical lymph node and distant metastases occurred. The patient died 24 months after the first surgery. Staining revealed VEGF receptor (VEGFR) 1, VEGFR2, phosphorylated Ak strain transforming, mitogen-activated protein kinase, and signal transducer and activator of transcription 3 positivity. Although our findings do not indicate that anti-VEGF therapy is beneficial for treating maxillary angiosarcomas, we found that VEGFR signaling pathways were activated in maxillary angiosarcomas similar to angiosarcomas originating at other sites. Herein, we report a case of maxillary angiosarcoma, focused on VEGFR and signaling pathway activation. To our knowledge, this is the first report to describe VEGFR system immunostaining findings in maxillary angiosarcoma.

Lymphoepithelial carcinoma (LEC) is an uncommon neoplasm strongly associated with Epstein-Barr virus (EBV). LEC of the salivary glands (LECSG) is very rare: the most commonly affected site is the parotid gland, followed by the submandibular gland. This report describes the case of a 58-year-old man who presented with a left submandibular mass that had gradually increased in size over five years. On magnetic resonance imaging (MRI), the mass showed low apparent diffusion coefficient (ADC) values, rapid initial enhancement before reaching a plateau on dynamic contrast-enhanced MRI (DCE-MRI), and internal septal-like enhancement. Histopathologically, the tumor comprised polygonal or round tumor cells with atypical or pleomorphic nuclei and numerous lymphocytes, separated by heavy fibrosis. Immunohistological findings were positive for AE/AE3, CD20, and EBV-encoded small RNA in situ hybridization (EBER-ISH), consistent with LEC. A low ADC value with rapid initial enhancement before reaching a plateau on DCE-MRI was thought to reflect abundant cellular components with tumor neoangiogenesis, whereas internal septal-like enhancement reflects separating heavy fibrosis. To the best of our knowledge, this is the first case report describing ADC value and DCE-MRI findings of LECSG, and these findings can be considered characteristic MRI findings of LECSG.

Pulmonary vein stenosis (PVS) is a serious complication of catheter ablation (CA) for atrial fibrillation (AF). PVS generally occurs several months after CA and presents with non-specific symptoms and imaging findings. There have been reports of delayed diagnoses due to a misdiagnosis as infection, interstitial pneumonia, or organizing pneumonia. We introduced six cases of PVS after CA, all of which showed narrowing of the unilateral pulmonary vessels with or without lobar volume loss in the left lung on unenhanced computed tomography. We report these findings as important results indicating the possibility of PVS after CA for AF and contributing to the early diagnosis and management of PVS.

Posterior reversible encephalopathy syndrome (PRES) is a clinico-radiological condition characterized by reversible vasogenic edema of the white matter and acute neurological symptoms. PRES typically affects the parieto-occipital regions but rarely affects the optic pathway. Herein, we describe a case of central-variant PRES in a 57-year-old man with abnormal signal intensities in the optic pathway on magnetic resonance imaging (MRI). The patient underwent hemodialysis, initiated diuretics and antihypertensive medications, and the abnormal signal intensities of the brainstem and optic pathway on MRI improved. Although rare, PRES can affect the optic pathway, thus suggesting the possibility of fragility of the optic pathway itself to hyperperfusion.

Chordomas are rare, locally aggressive, primary bone tumors derived from primitive notochord remnants. They almost always arise within the axial skeleton, particularly in the skull base and the sacrococcygeal region. They usually present as extradural tumors, but rarely, they present as entirely intradural tumors. This report describes a case of intradural chordoma that mimicked an epidermoid cyst. A 72-year-old woman was incidentally found to have a prepontine extra-axial mass on magnetic resonance imaging. The mass gradually increased in size, and she felt discomfort in the right cheek area. The mass showed similar signal intensity to cerebrospinal fluid on T1-weighted images and T2-weighted images, but high signal intensity on fluid-attenuated inversion recovery images and diffusion-weighted images. Because the presence of very faint contrast enhancement was not noticed, the mass was preoperatively diagnosed as an epidermoid cyst. Tumor resection was performed, and the histopathological diagnosis was chondroid chordoma. Since intradural chordoma may resemble an epidermoid cyst on imaging, radiologists should check carefully for the presence of contrast enhancement and suggest the possibility of intradural chordoma.

Decidualization can originate in ovarian endometrioma by elevated serum progesterone levels during pregnancy, which mimics malignancy on ultrasonography. Moreover, decidualized ovarian endometrioma may rupture and cause acute abdominal pain during pregnancy. Magnetic resonance imaging (MRI) is reportedly useful in differentiating decidualized ovarian endometriomas from malignancies. However, to our knowledge, serial MRI of decidualized ovarian endometrioma before and after rupture has not been reported. Herein, we report the case of a 39-year-old woman with a ruptured decidualized ovarian endometrioma in which serial MRI was useful for adequate management. She had a history of right ovarian endometrioma. Transvaginal ultrasonography at 20 weeks of gestation showed the known right ovarian endometrioma with mural nodules that were not evident before pregnancy. MRI for further evaluation showed ovarian endometrioma with mural nodules with signals similar to those of the placenta. Based on the MRI findings, we diagnosed a decidualized ovarian endometrioma. At 27 weeks of gestation, she complained of sudden abdominal pain, for which MRI was performed. MRI showed disappearance of the ovarian endometrioma and bloody ascites, based on which we diagnosed a ruptured ovarian endometrioma. The abdominal pain subsided immediately, and a conservative observational treatment approach was taken. At 37 weeks of gestation, right ovarian cystectomy was performed simultaneously with an elective cesarean section, which revealed a ruptured decidualized ovarian endometrioma. Our findings demonstrate that the accurate diagnosis of a ruptured decidualized ovarian endometrioma on serial MRI can contribute to its management.

Ectopic adrenocortical tissue can arise along the path of embryonic migration, such as the celiac axis, broad ligament, adnexa of the testis, and spermatic cord. Occasionally, ectopic adrenocortical tissues undergo marked hyperplasia and develop into ectopic adrenocortical adenomas. This report describes the case of a 60-year-old man who was incidentally found to have a lipid-containing mass with early enhancement and delayed washout in the right renal hilum. A renal cell carcinoma was suspected, and robot-assisted partial nephrectomy was performed, but the final diagnosis was an ectopic adrenocortical adenoma. We should include ectopic adrenocortical adenoma in the differential diagnosis when we find a lipid-containing tumor adjacent to the kidney.

OBJECTIVE: Previous studies have demonstrated structural brain network abnormalities in patients with temporal lobe epilepsy (TLE) using cortical thickness or gray matter (GM) volume. However, no studies have applied single-subject GM network analysis. Here, we first applied an analysis of similarity-based single-subject GM networks to individual patients with TLE. MATERIALS AND METHODS: We recruited 51 patients with TLE and unilateral hippocampal sclerosis (22 left, 29 right TLE) and 51 age- and gender- matched healthy controls. Single-subject structural networks were extracted from three-dimensional T1-weighted magnetic resonance images for each subject. In this method, nodes were defined as small cortical regions and edges representing connecting regions that have high statistical similarity. The constructed graphs were analyzed using the graph theoretical approach. The following global and local network properties were calculated: betweenness centrality, clustering coefficient, and characteristic path length. In addition, small world properties (normalized path length λ, normalized clustering coefficient γ, and small-world network value σ) were obtained and compared with those for the controls. RESULTS: Although the small-world configurations were retained, impaired global clustering coefficient was observed in left and right TLE. At a regional level, patients with left TLE showed a widespread decrease of the clustering coefficient beyond the ipsilateral temporal lobe and a decreased characteristic path length in the ipsilateral temporal pole. On the other hand, patients with right TLE showed a localized decrease of the clustering coefficient in the ipsilateral temporal lobe. CONCLUSIONS: Our findings suggest that global and local network properties disrupted and moved toward randomized networks in TLE patients in comparison to controls. This network alteration was more extensive in left TLE than in right TLE patients. Single-subject GM networks may contribute to a better understanding of the pathophysiology of TLE.

Objective: The aim of this study was to evaluate tau-related structural network metrics derived from gray matter magnetic resonance imaging (MRI) scans in cognitively normal (CN) older adults. Methods: We recruited 47 amyloid-negative CN older adults (mean age ± standard deviation, 65.0 ± 7.9 years; 26 women). All participants underwent 3D T1-weighted MRI and 11C-Pittsburgh compound-B and 18F-THK5351 positron emission tomography scans. Four local network metrics (betweenness centrality, clustering coefficient, characteristic path length, and degree) were computed and rendered on individual brain images. We then evaluated the correlations between 18F-THK5351 positron emission tomography images and local network metric images at the voxel level. Results: Significant positive correlations of the four local network metrics with 18F-THK5351 were detected in the bilateral caudate. Conclusion: Our findings suggest that tau and neuroinflammation in CN older adults may influence the gray matter structural network in the caudate.

Objective: This study aimed to examine the alterations in gray matter networks related to tau retention in Alzheimer's disease (AD) patients and cognitively normal (CN) older individuals. Methods: Eighteen amyloid-positive AD patients and 30 age- and sex-matched amyloid-negative CN controls were enrolled. All underwent 3D T1-weighted MRI, amyloid positron-emission tomography imaging (PET) with 11C-Pittsburgh Compound B (PiB), and tau PET with 18F-THK5351. The structural networks extracted from the T1-weighted MRI data based on cortical similarities within single subjects were analyzed. Based on graph theoretical approach, global and local network properties across the whole brain were computed. Group comparisons of global and local network properties were evaluated between the groups. Then, we correlated the global and local network measures with total cerebral 18F-THK5351 retention. Results: AD patients moved toward more randomized global network compared to controls and regional differences were observed in the default mode network (DMN) area. No significant correlations existed between global network properties and tau retention. On a local level, AD and controls showed opposite relationships between network properties and tau retention mainly in the DMN areas; CN controls showed positive correlations, whereas AD showed negative correlations. Conclusion: We found opposite relationships between local network properties and tau retention between amyloid-positive AD patients and amyloid-negative controls. Our findings suggest that the presence of amyloid and induced exacerbated tau retention alter the relationship of local network properties and tau retention.

Dentatorubral-pallidoluysian atrophy (DRPLA) is an autosomal dominant neurodegenerative disorder caused by a CAG nucleotide repeat expansion in atrophin 1. A previous report described cerebellar white matter lesions on magnetic resonance imaging (MRI) in elderly-onset DRPLA patients, but this finding has not been fully investigated in a total population of DRPLA patients, including juvenile or early-adult onset patients. Herein, we attempted to determine the frequency, distribution pattern, and features of the cerebellar white matter lesions in 30 consecutive DRPLA patients. We also assessed the relationships between the cerebellar white matter lesions and clinical parameters and other MRI findings. The cerebellar white matter lesions were found in 43% of the 30 DRPLA patients, and in 70% of the late adult-onset DRPLA patients. In approx. Two-thirds of the patients with cerebellar white matter lesions, the lesions were localized in the paravermal area (paravermal lesions). Multiple logistic regression analyses revealed that the Fazekas grade of 'cerebral' white matter lesions was independently associated with 'cerebellar' white matter lesions. In conclusion, cerebellar white matter lesions are one of the distinctive MRI features in DRPLA patients, especially in patients with older age at onset. Cerebellar white matter lesions, as well as cerebral white matter lesions, might originate from the disease process of DRPLA itself, and they often have a characteristic distribution of paravermal lesions.

PURPOSE: The purpose of this study was to evaluate the delineation of nerve fiber bundles in the brainstem and optic radiation in infants associated with aging on T1WI, T2WI, and phase difference-enhanced (PADRE) images. MATERIALS AND METHODS: We retrospectively reviewed 21 consecutive subjects < 2 years old who underwent brain MRI without abnormal imaging findings. Two neuroradiologists evaluated the eight nerve fiber bundles in the brainstem and optic radiation using a 3-point scale focused on the contrast to surrounding brain parenchyma. We also evaluated the signal ratio of the optic radiation to surrounding white matter on PADRE for each month age. RESULTS: T2WI was able to delineate nerve fiber bundles better than T1WI at 1 month old, and the images gradually became unclear with aging. On PADRE, almost all nerve fiber bundles were unclear or invisible at 1 month old but gradually became clearer with aging. There was a significant negative correlation between age and the signal ratio of the optic radiation to surrounding white matter. CONCLUSIONS: The PADRE imaging was able to delineate the nerve fiber bundles in infants, and the delineation gradually became clearer with aging. The combination of PADRE, T1WI, and T2WI would be useful for evaluation of nerve fiber bundles in infants.

PURPOSE: To investigate the ability of three-dimensional double-echo steady-state with water excitation sequence (3D-DESS-WE) in detecting the greater occipital nerve (GON) and the lesser occipital nerve (LON). MATERIALS AND METHODS: We retrospectively reviewed 66 consecutive patients who underwent magnetic resonance imaging, including 3D-DESS-WE. Two readers evaluated the detectability [0 (poor) to 3 (excellent)] and thickness of the GON and LON. We calculated the average evaluation scores and interobserver variability for the detectability of the GON and LON. We also calculated the average nerve thickness for each of the two readers and the interobserver reproducibility for the thickness of the GON and LON. The interobserver variability was calculated using a weighted kappa analysis and the intraclass correlation coefficient (ICC) was used to evaluate the interobserver reproducibility of the nerve thickness. RESULTS: The respective average score, interobserver variability, thickness and interobserver reproducibility were 3.00, excellent (κ = 1.00), 1.07 ± 0.12 mm, and almost perfect (ICC = 0.82) for the GON and 1.81, good (κ = 0.73), 0.84 ± 0.11 mm, and substantial (ICC = 0.78) for the LON. The GON was significantly thicker than the LON (p < 0.001). CONCLUSION: The 3D-DESS-WE demonstrated excellent visualization of the GON and relatively good visualization of the LON.

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.

BACKGROUND AND PURPOSE: Beta-propeller protein-associated neurodegeneration (BPAN) is one subtype of neurodegeneration with brain iron accumulation. It is difficult to diagnose BPAN due to the non-specificity of their clinical findings and neuroimaging in early childhood. We experienced four pediatric patients with serial brain MRI and evaluated the alteration of the findings through their course. METHODS: We retrospectively reviewed the clinical findings and 21 MRI findings of the four patients with genetically confirmed pediatric BPAN. We also performed a quantitative MR assessment using the quantitative susceptibility mapping (QSM) values of the globus pallidus (GP), substantia nigra (SN), and deep cerebellar nuclei (DCN) compared to 10 age-matched disease controls. RESULTS: Only one patient was suspected of BPAN based on imaging findings before the genetic diagnosis was made. The other three patients could not be suspected until their Whole-exome sequencings (WES) done. In all four cases, no abnormal signals were noted in the GP and SN at the initial brain MRI, but hypointensities were observed after the ages of 4-7 years on T2-weighted images and after the ages of 2-7 years on susceptibility-weighted images. In three patients, T2 hyperintensity in the bilateral DCN was persistently observed throughout the observational period. Three patients showed transient T2 hyperintensity and swelling in the GP, SN and/or DCN during the episodes of pyrexia and seizures. The other findings included cerebral and cerebellar atrophy, thinning of the corpus callosum, and delayed myelination. The QSM values of the GP and SN were significantly higher in the patients compared to the controls (P=0.005, respectively), but that of the DCN did not differ significantly (P=0.16). CONCLUSION: Brain MRI is a useful method to establish the early diagnosis of BPAN.

Autosomal recessive PRUNE1 mutations on chromosome 1q21.3 are reported to cause a neurodevelopmental disorder with microcephaly, hypotonia, and variable brain malformations. Here, we report a Japanese case with a reported PRUNE1 mutation whose brain magnetic resonance imaging (MRI) showed specific imaging findings that have not been reported before. The patient was a 12-month-old girl, the first child of healthy and nonconsanguineous Japanese parents. She showed global developmental delay, intellectual disability, hypotonia, spastic quadriparesis, and hyperreflexia. Brain MRI showed cerebral and cerebellar atrophy, thin corpus callosum, white matter changes, and abnormal signal intensity of the brainstem, all of which were reported in the literature. In addition, we emphasize the three following imaging findings: a transient cerebral subcortical white matter lesion, atrophy of the midbrain and pontine tegmentum with a preserved pontine base, and abnormal signal intensity of the bilateral swelling putamina and medial portions of the thalami, which emerged after 4 years of age. The whole-exome sequencing (WES) analysis performed at the age of 4 years identified biallelic PRUNE1 variants, namely compound heterozygous mutations (c.[316G > A];[540 T > A],p.[Asp106Asn];[Cys180*]). Although the diagnosis of PRUNE1-related disorder requires WES, we think that these new characteristic MRI findings may help in the diagnosis of PRUNE1-related disorder.

We describe the case of an adult female with a huge pelvic arteriovenous malformation (AVM) measuring approx. 8 × 10 × 13 cm, treated via the transvenous approach alone. Management of huge pelvic AVMs is challenging; there is no consensus on a standardized treatment strategy. In our patient, multiple arterioles shunted to prominently dilated outflow veins. Selective occlusion of prominent outflow veins as close to the nidus as possible achieved significant flow reduction through the nidus. Our treatment may be a safe and acceptable option to control hematuria for huge pelvic AVMs associated with prominent outflow veins, especially when both transarterial embolization and direct puncture are difficult.

We used quantitative susceptibility mapping (QSM) to assess the brain iron deposition in 28 patients with the cerebellar subtype of multiple system atrophy (MSA-C), nine patients with spinocerebellar ataxia type 6 (SCA6), and 23 healthy controls. Two reviewers independently measured the mean QSM values in brain structures including the putamen, globus pallidus, caudate nucleus, red nucleus, substantia nigra, and cerebellar dentate nucleus. A receiver operating characteristics (ROC) analysis was performed to assess the diagnostic usefulness of the QSM measurements. The QSM values in the substantia nigra were significantly higher in the MSA-C group compared to the HC group (p = .007). The QSM values in the cerebellar dentate nucleus were significantly higher in MSA-C than those in the SCA6 and HC groups (p < .001), and significantly lower in the SCA6 patients compared to the HCs (p = .027). The QSM values in the cerebellar dentate nucleus were correlated with disease duration in MSA-C, but inversely correlated with disease duration in SCA6. In the ROC analysis, the QSM values in the cerebellar dentate nucleus showed excellent accuracy for differentiating MSA-C from SCA6 (area under curve [AUC], 0.925), and good accuracy for differentiating MSA-C from healthy controls (AUC 0.834). QSM can identify increased susceptibility of the substantia nigra and cerebellar dentate nucleus in MSA-C patients. These results suggest that an increase in iron accumulation in the cerebellar dentate nucleus may be secondary to the neurodegeneration associated with MSA-C.

BACKGROUND AND PURPOSE: Chorea-acanthocytosis, a rare neurodegenerative disease, affects both the striatum and the medial temporal lobe which may cause involuntary movements and epilepsy, respectively. We examined the imaging changes of the hippocampus/amygdala and the striatum as well as clinical symptoms. MATERIALS AND METHODS: We retrospectively reviewed 29 MRI and 13 SPECT studies and the clinical findings of seven genetically confirmed chorea-acanthocytosis patients. We evaluated the time-dependent imaging changes of the hippocampus/amygdala and striatum and examined the relationships among these images and symptoms. RESULTS: The initial symptom was epilepsy in four patients and involuntary movements in three patients. These symptoms were eventually noted in five and all seven patients, respectively. On MRI, most patients showed striatum atrophy before a hippocampus/amygdala abnormality emerged, but one patient showed a hippocampus/amygdala abnormality before striatum atrophy. Abnormal MRI findings of hippocampus/amygdala were noted in five patients and atrophy of striatum in all seven patients. SPECT demonstrated hypoperfusion of hippocampus/amygdala in three patients and that of striatum in all five available patients. Four patients demonstrated hypoperfusion of striatum earlier than that of hippocampus/amygdala and one patient showed hypoperfusion of both simultaneously. Many imaging abnormal lesions were accompanied by their corresponding symptoms, but not always so. CONCLUSION: Striatum abnormalities were the initial imaging findings in many chorea-acanthocytosis patients, but epilepsy or hippocampus/amygdala imaging abnormalities may be the only findings at the early stage. It is important to understand the detailed clinical and imaging time courses for the diagnosis of chorea-acanthocytosis.

BACKGROUNDS: Although neurofibrillary tangles (NFTs) mainly accumulate in the medial temporal lobe with human aging, only a few imaging studies have investigated correlations between NFT accumulation and gray matter (GM) volume in cognitively normal older adults. Here, we investigated the correlations between 18F-THK5351 accumulation and GM volume at the voxel level. MATERIAL AND METHODS: We recruited 47 amyloid-negative, cognitively normal, older adults (65.0 ± 7.9 years, 26 women), who underwent structural magnetic resonance imaging, 11C-Pittsburgh compound-B and 18F-THK5351 PET scans, and neuropsychological assessment. The magnetic resonance and 18F-THK5351 PET images were spatially normalized using Statistical Parametric Mapping 12. Voxel-wise correlations between 18F-THK5351 accumulation and GM volume were evaluated using the Biological Parametric Mapping toolbox. RESULTS: A significant negative correlation (p < 0.001) between 18F-THK5351 accumulation and GM volume was detected in the bilateral medial temporal lobes. CONCLUSIONS: Voxel-wise correlation analysis revealed a significant negative correlation between 18F-THK5351 accumulation and GM volume in the medial temporal lobe in individuals without amyloid-β deposits. These results may contribute to a better understanding of the pathophysiology of primary age-related tauopathy in human aging.

BACKGROUND AND PURPOSE: Reliable preoperative facial nerve mapping may help avoid or minimize facial nerve injury during parotid tumor resection. The purpose of this study was to investigate the diagnostic performance of the 3D double-echo steady-state with water excitation sequence in localizing parotid gland tumors through direct visualization of the intraparotid facial nerve in comparison with indirect methods of estimating the facial nerve location. MATERIALS AND METHODS: We retrospectively reviewed 91 parotid gland tumors in 90 patients who underwent surgical resection and preoperative MR imaging, including the 3D double-echo steady-state with water excitation sequence. The tumor locations were categorized as deep or superficial on the basis of direct and 3 indirect methods: the facial nerve line, retromandibular vein, and Utrecht line. Surgical localization was considered the criterion standard. The diagnostic performance for localizing deep lobe lesions using direct and indirect methods was calculated and compared using the McNemar test. RESULTS: Surgical localization confirmed 75 superficial lesions and 16 deep lesions. The interobserver variability of the 3D double-echo steady-state with water excitation sequence was excellent (κ = 0.870). The diagnostic accuracy, sensitivity, specificity, positive predictive value, and negative predictive value for localizing deep lobe lesions using the 3D double-echo steady-state with water excitation method were 97.8%, 87.5%, 100%, 100%, and 97.4%, respectively. These findings were significantly higher than the facial nerve line in sensitivity, the retromandibular vein in sensitivity, and the Utrecht line in accuracy and specificity (P < .05). Overall, the direct method was the most accurate, sensitive, and specific in localizing parotid gland tumors. CONCLUSIONS: We can achieve higher diagnostic performance in localizing parotid gland tumors by directly visualizing the intraparotid facial nerve using the 3D double-echo steady-state with water excitation sequence compared with indirect methods.

Rasmussen's aneurysm is a peripheral pulmonary artery pseudoaneurysm (PAP) within a tuberculosis cavity. Because it can be perfused from the bronchial and pulmonary arterial circulations, combined embolization via the bronchial and pulmonary arteries is sometimes required. Herein, we present case of a 51-year-old man with Rasmussen's aneurysm that was successfully treated by proximal coil blocking with n-butyl-2-cyanoacrylate (NBCA) injection via the pulmonary artery alone. With proximal coil blocking, a sufficient amount of NBCA could be injected without unintended reflux of the NBCA cast to the proximal pulmonary artery. To our knowledge, there has been no report that attempted NBCA injection under proximal coil blocking for Rasmussen's aneurysm. Our treatment approach may be safe and effective for infectious lung disease-related PAP, which has to be treated from the pulmonary artery side.

Thyroglossal duct (TGD) carcinoma is a rare malignant tumor arising from remnants of thyroid tissue or the wall of the duct and generally occurs along the anatomic course of the TGD. TGD carcinoma originating in the hyoid bone is extremely rare but can occur since the TGD penetrates the hyoid bone on rare occasions. This report describes the case of a 30-year-old man with TGD carcinoma originating in the hyoid bone. Computed tomography demonstrated a mass in the hyoid bone that expanded the cortical bone of the hyoid. The mass had a central solid component with calcification and a marginal cystic component. When we encounter a calcified mass in the hyoid bone, we should consider TGD carcinoma among the differential diagnoses.

Spontaneous intracranial hypotension (SIH) is a well-known cause of orthostatic headache. Although subdural fluid collection is a usual complication of SIH, SIH as a risk factor for cerebral venous thrombosis (CVT) is not well-known. There are several mechanisms that could contribute to the development of CVT in SIH. Herein, we report a case of a 33-year-old woman with SIH complicated by CVT. She was treated with anticoagulation but did not receive a blood patch for the SIH, because there was resolution of orthostatic headache with bed rest and sufficient hydration. Follow-up magnetic resonance imaging showed resolution of the findings of SIH and CVT. Patients with SIH should be closely observed for any change in the headache pattern, which might suggest the development of CVT.

Synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome is a disorder characterized by pustular skin lesions and osteoarticular lesions. Mandibular involvement of SAPHO syndrome is clinically rare, and it is difficult to reach a diagnosis of SAPHO syndrome from only mandibular manifestations. This report describes the case of a 26-year-old woman who presented with mandibular osteitis. Orthopantomogram and computed tomography showed sclerotic change of the right body of the mandible with periosteal reaction without odontogenic infection, which suggested the possibility of SAPHO syndrome. Detailed medical interview found that she had a history of palmoplantar pustulosis treated at a local dermatology clinic and additional bone scintigraphy showed diffuse increased uptake in the right mandible, as well as in the sternum and the sternocostoclavicular joints. She was eventually diagnosed as having SAPHO syndrome. We should consider SAPHO syndrome when we encounter a patient with mandibular osteitis of unknown etiology.

Pleura is a very thin structure. Normal pleura is not visible, except for interlobar fissures. The pleura becomes visible when there is an abnormality present. Pleural abnormalities can be subtle and it is important to check carefully around the edge of each lung, which makes a significant contribution to the workload of radiologists. The aims of this article are to present the chest radiograph and CT imaging appearances of various pleural conditions. Radiologists should be aware of both the common and the atypical manifestations and should become familiar with the various pleural lesions.

Massive hemoptysis is a life threatening respiratory emergency with high mortality and the bronchial artery (BA) is its most frequent source. Herein, we report a case of a 76-year-old man with recurrent hemoptysis due to an aberrant right BA arising from the right internal mammary artery (IMA), an extremely rare origin, that was clearly depicted on pretreatment computed tomography angiography (CTA). The patient was treated successfully by transcatheter bronchial artery embolization (BAE) of the aberrant BA and the hemoptysis has since been controlled for 9 months. Knowledge of the detailed BA anatomy is essential for performing BAE, especially in cases of aberrant BA. CTA is a promising tool for pretreatment planning of emergency BAE in patients with hemoptysis.

BACKGROUND AND PURPOSE: Although visualization of the extracranial branches of the cranial nerves has improved with advances in MR imaging, only limited studies have assessed the detection of extracranial branches of the mandibular nerve (V3). We investigated the detectability of the branches of V3 on a 3D double-echo steady-state with water excitation sequence. MATERIALS AND METHODS: We retrospectively evaluated the detectability of the 6 branches of the V3, the masseteric, buccal, auriculotemporal, lingual, inferior alveolar, and mylohyoid nerves, by using a 5-point scale (4, excellent; 3, good; 2, fair; 1, poor; and 0, none) in 86 consecutive patients who underwent MR imaging with the 3D double-echo steady-state with water excitation sequence. Weighted κ analysis was used to calculate interobserver variability among the 3 readers. RESULTS: The detection of the lingual and inferior alveolar nerves was the most successful, with excellent average scores of 3.80 and 3.99, respectively. The detection of the masseteric, the buccal, and the auriculotemporal nerves was good, with average scores of 3.31, 2.67, and 3.11, respectively. The mylohyoid nerve was difficult to detect with poor average scores of 0.62. All nerves had excellent interobserver variability across the 3 readers (average weighted κ value, 0.95-1.00). CONCLUSIONS: The 3D double-echo steady-state with water excitation sequence demonstrated excellent visualization of the extracranial branches of V3 in most patients. The 3D double-echo steady-state with water excitation sequence has the potential for diagnosing V3 pathologies and preoperatively identifying peripheral cranial nerves to prevent surgical complications.

We report a case of iatrogenic brachial artery injury treated using N-butyl cyanoacrylate (NBCA) with both endovascular approach and direct puncture approach. For the treatment of brachial artery injury, appropriate approaches should be selected depending on patient's condition and vascular anatomy. Embolization using NBCA may be a safe and effective alternative in some cases.

OBJECTIVE: The objective of this study was to compare the diagnostic accuracy of dual-time-point 18F-fluorodeoxyglucose ((18)F-FDG) positron emission tomography (PET) to single-time-point (18)F-FDG PET for staging of preoperative lung cancer. METHODS: Between November 2008 and December 2009, 107 patients who were diagnosed as having lung cancer or strongly suspected of having lung cancer were enrolled. They underwent dual-time-point (18)F-FDG PET following conventional imaging. Dual-time-point (18)F-FDG PET imaging (whole body) was performed at 1-h (early) post-FDG injection and repeated (2 h delayed) after injection. The diagnostic accuracy of pre-PET staging and post-PET staging was retrospectively evaluated, and the diagnostic accuracy of dual-time-point (18)F-FDG PET was compared to that of single-time-point (18)F-FDG PET. RESULTS: In 100 patients, the early (18)F-FDG PET scan resulted in upstaging of the tumor in ten (10 %) and down-staging of the tumor in five (5 %) compared to the conventional scan. The delayed phase of (18)F-FDG PET provided no additional information on staging for lung cancer patients. The remaining seven patients were diagnosed as not having lung cancer. CONCLUSION: This study confirmed that dual-time-point (18)F-FDG PET is useful for differential diagnosis between benign and malignant lesions, but has no major impact on staging and therapeutic management of patients with pathologically proven lung cancer.