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Abstract Background Tubulointerstitial nephritis and uveitis (TINU) syndrome is an uveits characterized by complications of idiopathic acute tubulointerstitial nephritis, and most cases present only anterior uveitis. We report a case of TINU syndrome in which the presence of choroiditis was revealed by multimodal imaging. Case presentation A 12-year-old male visited our hospital with a 6-day history of ocular pain and hyperemia. Conjunctival and ciliary injections, 1 + flare and 3 + cells of anterior chamber inflammation with mutton fat keratic precipitates were observed in both eyes (OU), together with redness and swelling of the optic disc OU. Laboratory tests showed slightly high levels of soluble IL-2R and serum β2 microglobulin and markedly high levels of urinary β2 microglobulin. The diagnosis of probable TINU syndrome was established on the basis of bilateral uveitis and urinalysis results in accordance with a clinical criteria of tubulointerstitial nephritis. With treatment with oral prednisolone (PSL) at 20 mg/day, ocular findings improved, and the dose of PSL was gradually reduced and withdrawn 6 months later. However, 1 month later from the withdrawal, ocular inflammation recurred with the presence of retinal exudates and snowball vitreous opacities in the peripheral retina OU. Fluorescein angiography showed leakages from peripheral retinal vessels and staining corresponding to retinal exudates. Indocyanine green angiography showed hypofluorescent dots scattered over the ocular fundus. Optical coherence tomography revealed the presence of choroidal thickening. Laser speckle flowgraphy color map showed a relatively cooler color. Findings from these multimodal images indicated the presence of subclinical choroiditis; therefore, oral PSL was administered again, and ocular inflammatory findings were improved. Conclusions TINU syndrome can exhibit subclinical choroiditis detected with multimodal imaging. Further studies are necessary to determine the frequency of subclinical choroiditis in TINU syndrome.

Objective Using segmental dynamic and static factors, we aimed to elucidate the pathogenesis and relationship between ossification of the posterior longitudinal ligament (OPLL) and the severity of cervical myelopathy. Methods Retrospective analysis of 163 OPLL patients' 815 segments. Imaging was used to evaluate each segmental space available for the spinal cord (SAC), OPLL diameter, type, bone space, K-line, the C2–7 Cobb angle, each segmental range of motion (ROM), and total ROM. Magnetic resonance imaging was used to evaluate spinal cord signal intensity. Patients were divided into the myelopathy group (M group) and the without myelopathy group (WM group). Results Minimal SAC (p = 0.043), (C2–7) Cobb angle (p = 0.004), total ROM (p = 0.013), and local ROM (p = 0.022) were evaluated as an independent predictor of myelopathy in OPLL. Different from the previous report, the M group had a straighter whole cervical spine (p < 0.001) and poorer cervical mobility (p < 0.001) compared to the WM group. Total ROM was not always a risk factor for myelopathy, as its impact depended on SAC, when SAC > 5 mm, the incidence rate of myelopathy decreased with the increase of total ROM. Lower cervical spine (C5–6, C6–7) showing increased “Bridge-Formation,” along with spinal canal stenosis and segmental instability (C2–3, C3–4) in the upper cervical spine, could cause myelopathy in M group (p < 0.05). Conclusion Cervical myelopathy is linked to the OPLL’s narrowest segment and its segmental motion. The hypermobility of the C2–3 and C3–4, contributes significantly to the development of myelopathy in OPLL.

Abstract Background Vaccination against the worldwide pandemic coronavirus disease 2019 (COVID-19) is underway; however, some cases of new onset uveitis after vaccination have been reported. We report a case of bilateral acute posterior multifocal placoid pigment epitheliopathy-like (AMPPE-like) panuveitis after COVID-19 vaccination in which the patient’s pathological condition was evaluated using multimodal imaging. Case presentation A 31-year-old woman experienced bilateral hyperemia and blurred vision starting 6 days after her second inoculation of the COVID-19 vaccination. At her first visit, her visual acuity was decreased bilaterally, and severe bilateral anterior chamber inflammation and bilateral scattering of cream-white placoid lesions on the fundus were detected. Optical coherence tomography (OCT) showed serous retinal detachment (SRD) and choroidal thickening in both eyes (OU). Fluorescein angiography (FA) revealed hypofluorescence in the early phase and hyperfluorescence in the late phase corresponding to the placoid legions. Indocyanine green angiography (ICGA) showed sharply marginated hypofluorescent dots of various sizes throughout the mid-venous and late phases OU. The patient was diagnosed with APMPPE and was observed without any medications. Three days later, her SRD disappeared spontaneously. However, her anterior chamber inflammation continued, and oral prednisolone (PSL) was given to her. Seven days after the patient’s first visit, the hyperfluorescent lesions on FA and hypofluorescent dots on ICGA partially improved; however, the patient’s best corrected visual acuity (BCVA) recovered only to 0.7 OD and 0.6 OS, and the impairment of the outer retinal layer was broadly detected as hyperautofluorescent lesions on fundus autofluorescence (FAF) examination and as irregularity in or disappearance of the ellipsoid and interdigitation zones on OCT, which were quite atypical for the findings of APMPPE. Steroid pulse therapy was performed. Five days later, the hyperfluorescence on FAF had disappeared, and the outer retinal layer improved on OCT. Moreover, the patient’s BCVA recovered to 1.0 OU. Twelve months after the end of treatment, the patient did not show any recurrences. Conclusions We observed a case of APMPPE-like panuveitis after COVID-19 vaccination featuring some atypical findings for APMPPE. COVID-19 vaccination may induce not only known uveitis but also atypical uveitis, and appropriate treatment is required for each case.

Abstract Background Intraocular lymphoma (IOL) is a masquerade syndrome that mimics uveitis, making diagnosis difficult. The serum soluble interleukin-2 receptor (sIL-2R), which is cleaved by matrix metalloproteinase (MMP) -2 and MMP-9, has been recognized as a tumor-related biomarker of malignant lymphomas. The aim of this study was to review the reliability of serum and vitreous sIL-2R for distinguishing IOL from uveitis. Methods Patients who underwent diagnostic vitrectomy for marked vitreous haze at Hokkaido University Hospital between April 2014 and June 2019 were enrolled. The patients were divided into an IOL group and a uveitis group, according to the pathology of their vitreous samples. The IOL group was further divided at the time of vitrectomy into patients who already had extraocular involvement (IOL with extraocular involvement group) and patients with no evidence of having extraocular involvement (IOL without extraocular involvement group). Serum sIL-2R, and intravitreal sIL-2R, MMP-2, and MMP-9 levels were assessed. Results Twenty-five eyes of 25 patients, and 15 eyes of 15 patients were included in the IOL group and uveitis group, respectively. The serum sIL-2R levels were significantly lower in the IOL group than in the uveitis group (P

PurposeAngiopoietin (Ang) 2 is released from vascular endothelial cells by the stimulation of vascular endothelial growth factor (VEGF)A. Ang2 increases the expression of leukocyte adhesion molecules on endothelial cells via nuclear factor κB. The aim of this study was to evaluate the effects of Ang2 and VEGFA on ocular autoimmune inflammation.MethodsWe measured the concentrations of Ang2 and VEGFA in vitreous samples among patients with uveitis. Vitreous samples were collected from 16 patients with idiopathic uveitis (uveitis group) and 16 patients with non-inflammatory eye disease (control group). Experimental autoimmune uveoretinitis (EAU) was induced in B10.BR mice with a human interphotoreceptor retinoid-binding protein-derived peptide. The retinochoroidal tissues of the EAU mice were removed, and the mRNA levels of Ang2 and VEGFA were examined. EAU mice treated with anti-Ang2, anti-VEGFA, a combination of anti-Ang2 and anti-VEGFA, anti-Ang2/VEGFA bispecific, or IgG control antibodies were clinically and histopathologically evaluated.ResultsThe protein levels of Ang2 and VEGFA were significantly higher in the vitreous samples of patients with uveitis than in controls (PConclusionsBased on these results, VEGFA and Ang2 were shown to be upregulated locally in the eye of both uveitis patients and models of uveitis. Dual inhibition of Ang2 and VEGFA is suggested to be a new therapeutic strategy for uveitis.

Purpose: Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a disease characterized by multiple yellowish-white placoid lesions. Although most lesions resolve spontaneously, some turn into scars and lead to permanent visual dysfunction. In this report, we found suggestive findings in fundus autofluorescence (FAF) that may be useful for distinguishing severe lesions requiring treatment in APMPPE. Observation: Case 1: A 29-year-old woman was referred to our hospital with multiple yellowish-white placoid lesions on the fundi of both eyes (OU). FAF showed hyperautofluorescence in some of these placoid lesions. Based on the findings of fluorescein angiography, a diagnosis of APMPPE was established, and oral prednisolone (PSL) was initiated, given that some lesions were located in the macula. One week later, exacerbation occurred with the newly developed hyperautofluorescent lesions. Some lesions in the right eye (OD) that were hyperautofluorescent at the first visit became hypoautofluorescent. Afterward, although all hypoautofluorescent lesions persisted, most of the hyperautofluorescent lesions disappeared, so oral PSL could be stopped. Two months later, however, the recurrence occurred along with multiple new placoid lesions. Some lesions located at the macula were hyperautofluorescent on FAF OU, indicating the possibility of becoming scar lesions with hypoautofluorescence. Accordingly, oral PSL was given again. Case 2: A 47-year-old woman noticed decreased vision OD, and she was referred to us. Multiple yellowish-white placoid lesions were seen in the fundi OU. FAF showed hyperautofluorescence both with and without corresponding hypoautofluorescence in the placoid lesions OU. A diagnosis of APMPPE was established, and oral PSL was initiated. Four months later, some lesions that were hyperautofluorescent at the first visit had turned isoautofluorescent, and some lesions OU became hypoautofluorescent. However, all hypoautofluorescent lesions remained hypoautofluorescent OU. Only some hyperautofluorescent lesions recovered to isoautofluorescence without scars. Conclusions and Importance: In APMPPE, lesions showing hyperautofluorescence on FAF may change into hypoautofluorescence indicating scar formation. Therefore, the presence of hyperautofluorescent lesions in the macula may be a good indicator of the need for intensive corticosteroid treatments to avoid leaving hypoautofluorescent scars that are related to irreversible visual dysfunction.

This study aimed to assess the accuracy of predicting pelvic lymph node status using sentinel lymph node (SLN) biopsy with indocyanine green (ICG) and to examine the outcomes of SLN biopsy-guided abdominal radical trachelectomy (ART). Patients with stage IA2–IB2 cervical cancer from January 2009 to January 2021 were included. ICG was injected before ART and SLNs were identified, excised, and assessed intraoperatively using fast-frozen sections. Systemic pelvic lymphadenectomy was subsequently performed. The SLN detection rate, sensitivity, and false-negative rate were determined. Thirty patients desiring fertility preservation were enrolled, of whom 26 successfully completed ART and four underwent radical hysterectomies because of metastatic primary SLNs. Bilateral SLNs were identified in all patients. The sensitivity, false-negative rate, and negative predictive value were 100%, 7.7%, and 92.3%, respectively. Three (12%) patients were lost to follow-up: two relapsed and one died of tumor progression. Of the nine patients who tried to conceive after surgery, four achieved pregnancy and three delivered healthy live infants. In women with early-stage cervical cancer who desired to conserve fertility, SLN mapping with ICG had a very high detection rate, sensitivity, and low false-negative rate. SLN biopsy-guided ART is a feasible and accurate method for assessing pelvic node status.

Purpose: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a necrotizing vasculitis syndrome characterized by the destruction of small vessels, leading to various organ disorders. Here, we report a case of posterior scleritis with AAV successfully treated with prednisolone and rituximab (RTX) combination therapy. Observations: A 69-year-old female suffered from ocular pain and redness in her left eye for 2.5 years. She had been diagnosed with idiopathic otitis media before a year. At her initial visit, scleral injection with nodular elevated scleral lesions, vitreous haze, and serous retinal detachment (SRD) in the inferior periphery were observed in the left eye. Enhanced computed tomography revealed the enhancement and thickening of the left sclera. The results of laboratory analysis were positive for myeloperoxidase ANCA. Accordingly, she was diagnosed with AAV. Owing to the exacerbation of vitreous haze and SRD, topical treatment and steroid pulse therapy were initiated. Following therapy, anterior and posterior scleritis improved, and additional RTX was administered to maintain the remission. Following treatment, the patient has maintained remission with 10 mg/day prednisolone to date. Conclusions and importance: We encountered a case of posterior scleritis with AAV in which inflammatory manifestations subsided with RTX and glucocorticoid combination therapy. RTX administration likely contributed to the maintenance of remission.

Purpose: Epstein-Barr virus (EBV) is a herpes virus known to cause infectious mononucleosis and several other human disorders. Ocular EBV infections that have been reported include uveitis, retinal vasculitis, and acute retinal necrosis (ARN). ARN is usually caused by herpes simplex virus (HSV) or varicella-zoster virus (VZV). ARN that is caused by EBV (EBV-ARN) is rarely seen, and only a few cases have been reported. The visual prognosis for EBV-ARN is poor, and no treatment strategy has been established. We report on a patient who was treated successfully for EBV-ARN. Observation: An 80-year-old female who had been treated with prednisolone at 5 mg/day and methotrexate at 2 mg/week for rheumatoid arthritis visited our hospital because of blurred vision in her left eye. Her left visual acuity was 20/50, and extensive white-yellowish retinal lesions at the temporal periphery with retinal hemorrhages were seen through vitreous haze. The DNA sequence of EBV, but not of HSV, VZV, or cytomegalovirus, was detected by a polymerase chain reaction (PCR) assay in the aqueous humor (4.2 × 106 copies/ml), with EBV also being positive in serum (3.5 × 102 copies/ml). The patient received 2 mg of intravitreal ganciclovir injections twice with a 3-day interval and intravenous infusion of acyclovir at 750 mg/day for 7 days; however, the retinal white lesions expanded rapidly, then dose of prednisolone was increased (40 mg/day) and vitrectomy was performed 10 days after the initial visit. After the surgery, the retinal lesion continued to enlarge. Vitreous samples showed high copies of EBV (1.2 × 108 copies/ml). Following treatment with intravenous foscarnet (4800 mg/day), which replaced the acyclovir application, the retinal white lesions gradually diminished, leaving retinal scars. To date, the patient has developed no retinal detachment and shows visual acuity over 6/60 in the left eye along with silicone oil. Conclusions: We experienced a case of EBV-ARN that was refractory to systemic acyclovir and topical ganciclovir but responded effectively to systemic foscarnet after vitrectomy. Although the clinical management remains challenging in this disease, foscarnet is considered to be one of the candidate drugs for EBV infections.

Background:. Ossification of the posterior longitudinal ligament (OPLL), characterized by ectopic new bone formation in the spinal ligament, causes neurological impairment due to narrowing of the spinal canal. However, the etiology has not been fully elucidated yet. Several biomarkers may be related to the pathogenesis of OPLL. The present study focused on the serum level of periostin, which is recognized as an important bone formation regulator. Methods:. This study included 92 patients with OPLL and 54 control patients without OPLL. For the case-control analysis, 54 age and sex-matched patients were randomly included in the OPLL group. The serum fibroblast growth factor-23 (FGF-23), creatinine, inorganic phosphate, calcium, alkaline phosphatase, and periostin levels were assessed. Furthermore, the calcium, creatinine, and inorganic phosphate levels in urine and the percentage of tubular reabsorption of phosphate were also analyzed. Moreover, the relationship between the biomarkers and the extent of OPLL was analyzed. The data were compared between patients with OPLL progression (the progression group) and without OPLL progression (the non-progression group). Results:. The mean serum FGF-23 and periostin levels in the OPLL group were higher than that in the control group. The serum inorganic phosphate level in the OPLL group was lower than that in the control group. No correlation was found between any of the biomarkers and the extent of ossification. The serum periostin level in the progression group was higher than that in the non-progression group. No significant difference in the serum FGF-23 level was noted between the progression and non-progression groups. Moreover, no correlation was found between serum periostin and FGF-23 levels. Conclusions:. The serum periostin level is related to OPLL progression. Level of Evidence:. Prognostic Level III. See Instructions for Authors for a complete description of levels of evidence.

Abstract Background Hematologic malignancies occasionally cause serous retinal detachment (SRD); however, its pathogenesis remains unclear. Here we present the imaging characteristics of metastatic choroidal lymphoma masquerading as Vogt-Koyanagi-Harada (VKH) disease. Case presentation A 45-year-old Japanese woman was referred to our clinic because of bilateral SRD with blurred vision. Fluorescein angiography revealed multiple pinpoint leakage followed by pooling OU. Enhanced depth imaging optical coherence tomography showed marked choroidal thickening OU. Laser speckle flowgraphy detected choroidal circulation impairment OU. Although these results totally agreed with the inflammatory manifestations of acute VKH disease, indocyanine green angiography demonstrated various sizes of sharply marginated hypofluorescent lesions that seemed atypical for the finding of VKH disease, i.e., vaguely marginated hypofluorescent small dots. Cerebrospinal fluid pleocytosis was not detected. Blood tests revealed leukocytosis together with elevation of lactate dehydrogenase and soluble interleukin-2 receptor levels. Corticosteroid pulse therapy did not improve any ocular findings. Bone marrow biopsy was then performed, leading to a definite diagnosis of diffuse large B-cell lymphoma. After starting systemic chemotherapy, both SRD and choroidal thickening resolved rapidly with visual recovery. However, choroidal hypoperfusion persisted, which contrasted distinctly with the inflammatory pattern of VKH disease, i.e., the restoration of choroidal blood flow in parallel with normalization of choroidal thickness. Conclusions Our detailed multimodal observations highlighted the differential imaging features of choroidal lymphoma despite close resemblance to VKH disease especially at the initial stage. Impaired circulation in the thickened choroid marked the pseudo-inflammatory pathogenesis of SRD due to choroidal involvement with neoplastic, but not inflammatory cells.

Abstract Background Diffuse idiopathic skeletal hyperostosis (DISH) is characterized by the ossification of vertebral bodies and peripheral entheses. However, variations in sacroiliac (SI) joint change in patients with DISH have not been fully clarified. The purpose of this study was to evaluate SI joint variation in patients with DISH in comparison with a non-DISH population. Methods A total of 342 SI joints in 171 patients (DISH+, n = 86; DISH-, n = 85) who had undergone lumbar spine surgery were analyzed by computed tomography examination. SI joint variations were classified into four types: Type 1, normal or tiny peripheral bone irregularity; Type 2, subchondral bone sclerosis and osteophytes formation; Type 3, vacuum phenomenon; and Type 4, bridging osteophyte and bony fusion. The type of bridging osteophyte in SI joints and the prevalence of ossification in each spinal segment from C1 to SI joint were also examined. Results The most common SI joint variation in the DISH+ group was bony fusion (Type 4), with 71.6% exhibiting anterior paraarticular bridging. On the other hand, SI joint vacuum phenomenon (Type 3) was the most frequent change (57.1%) in the DISH- group. The middle to lower thoracic spine and SI joints were highly affected in DISH and caused bony ankylosis. Conclusions Anterior paraarticular bridging was the most common type of SI joint change in patients with DISH who underwent lumbar spine surgery. The present results regarding variations of SI joint changes in DISH should help understand the etiology of DISH.

Abstract Background Lumbar epidural lipomatosis (LEL) is characterized by abnormal accumulation of unencapsulated adipose tissue in the spinal epidural space. Such accumulation compresses the dural sac and nerve roots, and results in various neurological findings. However, the pathophysiology of LEL remains unclear. This study examined the associations between imaging and clinical findings in detail, and investigated the mechanisms underlying symptom onset by measuring intraoperative epidural pressures in LEL. Methods Sixteen patients (all men; mean age, 68.8 years) were enrolled between 2011 and 2015. Mean body mass index was 26.5 kg/m2. Four cases were steroid-induced, and the remaining 12 cases were idiopathic. All patients presented with neurological deficits in the lower extremities. Cauda equina syndrome (CES) alone was seen in 8 patients, radiculopathy alone in 4, and both radiculopathy and CES (mixed CES) in 4. All patients subsequently underwent laminectomy with epidural lipomatosis resection and were followed-up for more than 1 year. We investigated the clinical course and imaging and measured epidural pressures during surgery. Results Subjective symptoms improved within 1 week after surgery. Mean Japanese Orthopaedic Association (JOA) score was 15.2 ± 2.8 before surgery, improving to 25.4 ± 2.5 at 1 year after surgery. On magnetic resonance imaging, all lipomatosis lesions included the L4–5 level. On preoperative computed tomography, saucerization of the laminae was not observed in radiculopathy cases, whereas saucerization of the posterior vertebral body was observed in all radiculopathy or mixed CES cases. Intraoperative epidural pressures were significantly higher than preoperative subarachnoid pressures. The results suggest that high epidural pressure resulting from the proliferation of adipose tissue leads to saucerization of the lumbar spine and subsequent symptoms. Conclusions Clinical courses were satisfactory after laminectomy. In LEL, epidural pressure increases and symptoms develop through the abnormal proliferation of adipose tissue. Higher epidural pressures induce saucerization of the laminae and/or posterior vertebral body. Furthermore, the direction of proliferative adipose tissue (i.e., site of saucerization) might be related to the types of neurological symptoms.

Introduction: In more than 20 years' follow-up after cervical laminoplasty, some patients died at an early stage, within 5 years postoperatively. The details remain unclear. This study was conducted to elucidate the clinical features in patients who died at an early stage after cervical laminoplasty to determine possible preventive measures against early death after surgery. Methods: A total of 74 patients who died with the follow-up period were included. The patients were divided into two groups: patients with a short survival period (S group, died 5 years after surgery) and patients with a long survival period (L group, died >5 years after surgery). Diseases, gender, age, causes of the death, general complications before surgery, and the pre- and postoperative JOA scores were compared between the two groups. Results: Eleven patients (15%) died within 5 years after laminoplasty. The average age at death in the S group was 68.7 years which was considerably younger than that in the L group (80.2 years). The ratio of pneumonia was higher in the S group, compared to that in the L group. Postoperative JOA score in the S group was lower than that in the L group. Conclusions: 11 patients out of 74 patients (15%) died within 5 years after laminoplasty. The average age at the death in the S group was much younger than that in the L group. The postoperative JOA score in the S group was lower than that in the L group. As pneumonia was more prevalent in the S group, it might be reasonable to give the information for the protection of pneumonia after cervical laminoplasty.

Abstract Background Osteosarcoma, the most common primary bone malignancy, has an extremely poor prognosis and a high rate of local recurrence and distal metastases. Because osteosarcomas of the head and neck region are rare, accounting for less than 10% of all osteosarcoma cases, limited information is available about their treatment and prognosis. Because of the high rate of distal metastases associated with extragnathic osteosarcoma, surgery combined with chemotherapy is currently considered essential in its treatment. However, the role of chemotherapy has not been well elucidated in the treatment of head and neck osteosarcoma because of the rarity of this condition. Case presentation In this report, we present the case of a 58-year-old Japanese woman with osteosarcoma of the mandible that was treated with radical surgery combined with neoadjuvant and adjuvant chemotherapy. Because the tumor showed rapid growth during neoadjuvant chemotherapy, neoadjuvant chemotherapy was suspended and surgical resection was performed, followed by adjuvant chemotherapy. No evidence of local recurrence and distal metastasis was found 14 months after initial treatment. Local control is considered a principal prognostic factor for head and neck osteosarcoma. Conclusions Wide surgical excision should be considered a primary goal even during neoadjuvant chemotherapy, especially in cases that respond poorly to neoadjuvant chemotherapy.

Abstract Background Fibroma of tendon sheath (FTS) is a benign tumor arising from the synovium of the tendon sheath that occurs mostly around small joints such as the fingers, hands, and wrist. However, FTS rarely arises around a large joint (knee, shoulder, elbow, and ankle) with intra-articular or extra-articular involvement. The clinical characteristics of FTS arising around a large joint are unclear. An additional 3 cases of FTS arising around a large joint are presented. Furthermore, the published cases and the present cases are reviewed with respect to their clinical characteristics and imaging and histopathology findings. Methods The 43 reported cases including the present 3 patients were summarized, and the patients’ profiles, symptoms, sites and locations in the joint involved by FTS, magnetic resonance imaging (MRI) findings, surgical procedures, clinical courses, and cytogenetic analyses were reviewed. Results The average age of 26 cases was 40.9 years (range 13–69 years), and about 60% of the patients were male. About 10% of the patients had a past history of trauma to the knee joint. Of the present 3 cases, one case was extra-articular around the elbow joint, one case was extra-articular around the knee joint, and one case was intra-articular involving the knee joint. The common symptoms were pain (62.5%), swelling or palpable mass (54.2%), and limited range of motion of the involved joint (50%). The most commonly involved joint was the knee, with 32 cases (74.4%), followed by the elbow in 5 cases (11.6%), ankle in 4 (9.3%), and shoulder in 2 (4.7%). The tumor typically exhibited iso to low signal intensity on T1-weighted MRI. T2-weighted images showed various patterns, but mostly low signal intensity relative to muscle. The surgical margin was marginal resection in all cases. There were no recurrences after surgery. On chromosomal analysis, only the present Case 3 showed an abnormality. Conclusions A total of 43 FTS cases that occurred around large joints were summarized. The most common site was around the knee joint. In FTS cases around large joints, it is necessary to distinguish between various fibroblastic and/or fibrohistiocytic tumors.

Ossification of the posterior longitudinal ligament (OPLL) is characterized by replacement of ligamentous tissue by ectopic new bone formation. OPLL causes narrowing of the spinal canal, resulting in neurological impairment. However, the pathogenesis of OPLL has not been fully elucidated. We investigated whether inflammation occurs in OPLL or not using high-sensitivity CRP (hs-CRP) in a case-control study.This study included 103 patients with OPLL in the patient group and 95 age- and sex-matched volunteers with degenerative spinal disease in the control group. Of the 103 OPLL patients, 88 patients who were available for more than 2 years follow-up were checked for OPLL progression. A blood sample was obtained and Hs-CRP, and other routine data, including total protein (TP), albumin (ALB), lactate dehydrogenase (LDH), alkaline phosphatase (ALP), glucose (Glu), calcium (Ca), inorganic phosphate (Pi), white blood cell count (WBC), hemoglobin (Hb) and platelet (PLT), were analyzed. The data were compared between the patients with OPLL and the controls. The severity of the ossified lesions in the whole spine were evaluated by the ossification index (OS index) in patients with OPLL. The data were also compared between the patients with OPLL progression (the progression group) and the patients without OPLL progression (the non-progression group). In the results, the mean hs-CRP in the OPLL group was higher than that in the controls. The Pi in the OPLL group was lower than that in the control group. A negative correlation was found between the Pi and the OS index. The mean hs-CRP in the progression group was higher than that in the non-progression group. There was a positive correlation between the average length of the OPLL progression per year and the hs-CRP.The results may suggest the occurrence of local inflammation in OPLL and the inflammation might cause OPLL progression. These facts are important for understanding the pathology of OPLL.

We sought to examine social network diversity as a potential determinant of oral health, considering size and contact frequency of the social network and oral health behaviors.Our cross-sectional study was based on data from the 2010 Japan Gerontological Evaluation Study. Data from 19,756 community-dwelling individuals aged 65 years or older were analyzed. We inquired about diversity of friendships based on seven types of friends. Ordered logistic regression models were developed to determine the association between the diversity of social networks and number of teeth (categorized as ≥20, 10-19, 1-9, and 0).Of the participants, 54.1% were women (mean age, 73.9 years; standard deviation, 6.2). The proportion of respondents with ≥20 teeth was 34.1%. After adjusting for age, sex, socioeconomic status (income, education, and occupation), marital status, health status (diabetes and mental health), and size and contact frequency of the social network, an increase in the diversity of social networks was significantly associated with having more teeth (odds ratio = 1.08; 95% confidence interval, 1.04-1.11). Even adjusted for oral health behaviors (smoking, curative/preventive dental care access, use of dental floss/fluoride toothpaste), significant association was still observed (odds ratio = 1.05 (95% confidence interval, 1.02-1.08)).Social connectedness among people from diverse backgrounds may increase information channels and promote the diffusion of oral health behaviors and prevent tooth loss.

We examined the relationship between incident functional disability and social participation from the perspective of number of types of organizations participated in and type of social participation in a prospective cohort study.The study was based on the Aichi Gerontological Evaluation Study (AGES) Cohort Study data. We followed 13,310 individuals aged 65 years or older for 4 years. Analysis was carried out on 12,951 subjects, excluding 359 people whose information on age or sex was missing. Social participation was categorized into 8 types.Compared to those that did not participate in any organizations, the hazard ratio (HR) was 0.83 (95% CI: 0.73-0.95) for participation in one, 0.72 (0.61-0.85) for participation in two, and 0.57 (0.46-0.70) for participation in three or more different types of organizations. In multivariable adjusted models, participation in the following types of organization was protective for incident disability: local community organizations (HR = 0.85, 95% CI: 0.76-0.96), hobby organizations (HR = 0.75, 95% CI: 0.64-0.87), and sports organizations (HR = 0.64, 95% CI: 0.54-0.81).Social participation may decrease the risk of incident functional disability in older people in Japan. This effect may be strengthened by participation in a variety of different types of organizations. Participating in a local community, hobby, or sports group or organization may be especially effective for decreasing the risk of disability.

To evaluate the association between relative deprivation (lacking daily necessities) and subjective health in older Japanese adults, we performed a cross-sectional analysis using data from the Japan Gerontological Evaluation Study (JAGES). The data were obtained from functionally independent residents aged ≥65 years from 24 municipalities in Japan (n = 24,742). Thirteen items in three dimensions were used to evaluate relative deprivation of material conditions. Approximately 28% of older Japanese people indicated that they lacked some daily necessities (non-monetary poverty). A two-level Poisson regression analysis revealed that relative deprivation was associated with poor self-rated health (PR = 1.3-1.5) and depressive symptoms (PR = 1.5-1.8) in both men and women, and these relationships were stronger than those observed in people living in relative poverty (monetary poverty). The interaction effect between relative deprivation and relative poverty was not associated with poor health. As a dimension of the social determinants of health, poverty should be evaluated from a multidimensional approach, capturing not only monetary conditions but also material-based, non-monetary conditions.

BackgroundWe sought to examine prospectively the difference in the association between incident functional disability and exercise with or without sports organization participation.MethodsThe study was based on the Aichi Gerontological Evaluation Study (AGES) Cohort Study data. In October 2003, self-reported questionnaires were mailed to 29,374 non-disabled Japanese individuals aged 65 years or older. Of these, 13,310 individuals were introduced to the Study, and they were followed for 4 years. Analysis was carried out on 11,581 subjects who provided all necessary information for the analysis.ResultsAnalysis was carried out on incident functional disability by 4 groups of different combinations of performance of exercise and participation in a sports organization Active Participant (AP), Exercise Alone (EA), Passive Participant (PP) and Sedentary (S). Compared to the AP group, the EA group had a hazard ratio (HR) of 1.29 (1.02-1.64) for incident functional disability. No significant difference was seen with the PP group, with an HR of 1.16 (0.76-1.77). When a measure of social networks was added to the covariates, the HR of the EA group dropped to 1.27 (1.00-1.61), and significant differences disappeared. In contrast, it showed hardly any change when social support was added.ConclusionThe results suggested that, even with a regular exercise habit, incident functional disability may be better prevented when a person participates in a sports organization than when he/she does not. In addition, participation in a sports organization correlates positively with social networks, which may lead to a small decrease in incident functional disability.

BACKGROUND: Liposarcomas are the most common class of soft tissue sarcomas, and myxoid liposarcoma is the second most common liposarcoma. EWSR1-DDIT3 is a chimeric fusion protein generated by the myxoid liposarcoma-specific chromosomal translocation t(12;22)(q13;q12). Current studies indicate that multipotent mesenchymal cells are the origin of sarcomas. The mechanism whereby EWSR1-DDIT3 contributes to the phenotypic selection of target cells during oncogenic transformation remains to be elucidated. METHODOLOGY/PRINCIPAL FINDINGS: Reporter assays showed that the EWSR1-DDIT3 myxoid liposarcoma fusion protein, but not its wild-type counterparts EWSR1 and DDIT3, selectively repressed the transcriptional activity of cell lineage-specific marker genes in multipotent mesenchymal C3H10T1/2 cells. Specifically, the osteoblastic marker Opn promoter and chondrocytic marker Col11a2 promoter were repressed, while the adipocytic marker Ppar-γ2 promoter was not affected. Mutation analyses, transient ChIP assays, and treatment of cells with trichostatin A (a potent inhibitor of histone deacetylases) or 5-Aza-2'-deoxycytidine (a methylation-resistant cytosine homolog) revealed the possible molecular mechanisms underlying the above-mentioned selective transcriptional repression. The first is a genetic action of the EWSR1-DDIT3 fusion protein, which results in binding to the functional C/EBP site within Opn and Col11a2 promoters through interaction of its DNA-binding domain and subsequent interference with endogenous C/EBPβ function. Another possible mechanism is an epigenetic action of EWSR1-DDIT3, which enhances histone deacetylation, DNA methylation, and histone H3K9 trimethylation at the transcriptional repression site. We hypothesize that EWSR1-DDIT3-mediated transcriptional regulation may modulate the target cell lineage through target gene-specific genetic and epigenetic conversions. CONCLUSIONS/SIGNIFICANCE: This study elucidates the molecular mechanisms underlying EWSR1-DDIT3 fusion protein-mediated phenotypic selection of putative target multipotent mesenchymal cells during myxoid liposarcoma development. A better understanding of this process is fundamental to the elucidation of possible direct lineage reprogramming in oncogenic sarcoma transformation mediated by fusion proteins.

Purpose: We investigated efficacy and safety of adalimumab (ADA) treatment for exacerbation or recurrence of Vogt-Koyanagi-Harada (VKH) patients. Methods: Medical records of 70 VKH patients who received ADA treatment for more than 6 months were retrospectively investigated. Results: The mean age of VKH patients was 54.8 ± 15.1 years, and male/female ratio was 34/36, and sunset glow fundus was observed in 71.4%. Subfoveal choroidal thickness, indocyanine green angiography scores, and corticosteroid and cyclosporine doses were significantly reduced by ADA treatment for 6 months compared to baseline, while LogMAR and flare counts were also improved without being statistically significant. Adverse events were observed in 17.1%, in which tuberculosis was at 7.14% and psoriasis was at 2.86%; however, ADA treatment was continued in 91.4%. Conclusions: ADA was shown to be effective to achieve remission of VKH disease refractory to conventional treatments and was generally well tolerated with few serious adverse events. [ABSTRACT FROM AUTHOR]