Baló's concentric sclerosis with spontaneous remission and favorable prognosis.

Introduction: Baló's concentric sclerosis (BCS) is a rare type of central nervous system demyelinating disorder. Most patients with BCS are treated with corticosteroids, and spontaneous remission has seldom been described. Case presentation: A 46-year-old man presented with a subacute-onset headache and memory loss. Brain magnetic resonance imaging (MRI) revealed multiple onion-shaped ring lesions with mild enhancement in the outermost ring. A brain biopsy revealed significant myelin loss. The diagnosis of BCS was established based on the MRI results and pathological findings. Interestingly, the patient recovered almost completely without immunotherapy, with repeated brain MRI at the 1-year follow-up showing an obvious reduction in the extent of the lesions. Conclusion: Neurologists should improve the recognition of the typical MRI features of BCS to avoid unnecessary biopsies. Although rare, spontaneous remission can be observed in clinical practice.

Systematic sequential therapy for ex vivo liver resection and autotransplantation: A case report and review of literature.

BACKGROUND: Perihilar cholangiocarcinoma (pCCA) is a highly malignant tumor arising from the biliary tree. Radical surgery is the only treatment offering a chance of long-term survival. However, limited by the tumor's anatomic location and peri-vascular invasion, most patients lose the chance for curative treatment. Therefore, more methods to increase the resectability of tumors as well as to improve outcomes are needed. CASE SUMMARY: A 68-year-old female patient had a hepatic hilar mass without obvious symptoms. Laboratory results showed hepatitis B positivity. Magnetic resonance imaging indicated that the mass (maximum diameter: 41 mm) invaded the left and right branches of the main portal vein, as well as the middle, left and right hepatic veins; enlarged lymph nodes were also detected in the hilum. The patient was diagnosed with pCCA, and the clinical stage was determined to be T4N1M0 (stage IIIC). Considering the tumor's anatomic location and vascular invasion, systematic conversion therapy followed by ex vivo liver resection and autotransplantation (ELRA) was determined as personalized treatment for this patient. Our original systemic sequential therapeutic strategy (lenvatinib and tislelizumab in combination with gemcitabine and cisplatin) was successfully adopted as conversion therapy because she achieved partial response after three cycles of treatment, without severe toxicity. ELRA, anastomotic reconstruction of the middle hepatic vein, right hepatic vein, root of portal vein, inferior vena cava and right hepatic artery, and lymph node dissection were performed at one month after systemic therapy. Pathological and immunohistochemical examination confirmed the diagnosis of pCCA with lymph node metastasis. Although the middle hepatic vein was partially obstructed four months later, hepatic vein stent implantation successfully addressed this problem. The patient has survived for 22 mo after the diagnosis, with no evidence of recurrence or metastasis. CONCLUSION: An effective therapeutic strategy for conversion therapy greatly increases the feasibility and efficiency of ELRA.

Myelin oligodendrocyte glycoprotein antibody-associated disease preceding primary central nervous system lymphoma: causality or coincidence?

INTRODUCTION: Primary central nervous system lymphoma (PCNSL) is a rare extranodal lymphomatous malignancy that affects the brain, spinal cord, leptomeninges, or eyes, in the absence of systemic diffusion. Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is a newly identified benign immune-mediated CNS inflammatory disorder with specific anti-MOG antibody seropositivity. These two seemingly unrelated nosological entities both have abundant clinical and radiological manifestations, and whether there is a potential link between them is unclear. CASE REPORT: We describe a 49-year-old man who presented progressive headache, dizziness, and unsteady gait with multifocal scattered T2 hyperintensities with contrast enhancement. The serum anti-MOG antibody test was positive, and a brain biopsy showed inflammatory infiltration. Initially, he was diagnosed with MOGAD and his condition improved after corticosteroid therapy. The patient relapsed with exacerbation of symptoms and neuroimaging showed new mass-forming lesions four months later. A second brain biopsy confirmed PCNSL. DISCUSSION: This is the first report of histologically confirmed successive MOGAD and PCNSL. Our case broadens the phenotypic spectrum of sentinel lesions in PCNSL. Though rare, PCNSL should be considered in patients diagnosed with benign CNS inflammatory disorder and responding well to steroid treatment when their clinical symptoms worsen and the imaging deteriorates. A timely biopsy is critical for accurate diagnosis and appropriate therapy.

Clinical, Morphological, and Molecular Study on Grade 2 and 3 Pleomorphic Xanthoastrocytoma.

PURPOSE: Pleomorphic xanthoastrocytoma (PXA) is an uncommon astrocytoma that tends to occur in children and young adults and has a relatively favorable prognosis. The 2021 WHO classification of tumors of the central nervous system (CNS WHO), 5th edition, rates PXAs as grade 2 and grade 3. The histological grading was based on mitotic activity (≥2.5 mitoses/mm2). This study specifically evaluates the clinical, morphological, and, especially, the molecular characteristics of grade 2 and 3 PXAs. METHODS: Between 2003 and 2021, we characterized 53 tumors with histologically defined grade 2 PXA (n = 36, 68%) and grade 3 PXA (n = 17, 32%). RESULTS: Compared with grade 2 PXA, grade 3 PXA has a deeper location and no superiority in the temporal lobe and is more likely to be accompanied by peritumoral edema. In histomorphology, epithelioid cells and necrosis were more likely to occur in grade 3 PXA. Molecular analysis found that the TERT promoter mutation was more prevalent in grade 3 PXA than in grade 2 PXA (35% vs. 3%; p = 0.0005) and all mutation sites were C228T. The cases without BRAF V600E mutation or with necrosis in grade 3 PXA had a poor prognosis (p = 0.01). CONCLUSION: These data define PXA as a heterogeneous astrocytoma. Grade 2 and grade 3 PXAs have different clinical and histological characteristics as well as distinct molecular profiles. TERT promoter mutations may be a significant genetic event associated with anaplastic progression. Necrosis and BRAF V600E mutation play an important role in the prognosis of grade 3 PXA.

Lymphomatosis cerebri: a rare diffuse leukoencephalopathy you should never miss.

INTRODUCTION: Lymphomatosis cerebri (LC) is a rare variant of primary central nervous system lymphoma that diffusely involves throughout the brain. In recent years, increasingly reported cases have notably broadened the spectrum of clinical and radiological features; however, it remains a great diagnostic challenge. CASE REPORT: We reported an atypical case of LC presented with subacute onset of focal neurological deficits and diffuse T2 hyperintensities without contrast enhancement on magnetic resonance imaging. He was initially considered as inflammatory leukoencephalopathy and received empirical corticosteroids, showing a dramatically clinical response. Three months later, the patient relapsed with deteriorating symptoms and enlarged brain lesions with mass-like enhancement. A diagnosis of LC was finally established according to the radiological and pathological findings. DISCUSSION: Though rare, LC should always be kept as a differential diagnosis of diffuse leukoencephalopathy. Neurologists should be aware of every detailed information about LC to avoid a delay of diagnostic biopsy in clinical practice.

MicroRNA-21-5p as a novel therapeutic target for osteoarthritis.

OBJECTIVE: Growing evidence indicates that microRNAs (miRNA) play a critical role in the pathogenesis of OA, and overexpressing or silencing miRNA expression in OA models can contribute to the development of miRNA-based therapeutics. The objective of this study was to determine whether intra-articular injection of miRNA can inhibit OA progression. METHODS: The miRNA expression profile was determined in OA cartilage tissues and controls. Functional analysis of the miRNAs on extracellular matrix degradation was performed after miRNA mimic or inhibitor transfection. Luciferase reporter assays and western blotting were employed to determine miRNA targets. To investigate the functional mechanism of miR-21-5p in OA development, miR-21-5pfl/flCol2a1-CreER and wild-type mice were subject to surgical destabilization of the medial meniscus. Therapeutically, wild-type mice undergoing surgical destabilization of the medial meniscus were treated with intra-articular injection of agomir- and antagomir-21-5p. RESULTS: We found that expression of miR-21-5p was significantly up-regulated in OA cartilage tissues. The articular cartilage degradation of miR-21-5p conditional knockout mice was significantly alleviated compared with that of wild-type mice in spontaneous and destabilization of the medial meniscus models. Through gain-of-function and loss-of-function studies, miR-21-5p was shown to significantly affect matrix synthesis genes expression, and chondrocyte proliferation and apoptosis. Further, fibroblast growth factor 18 (FGF18) was identified as a target of miR-21-5p. Intra-articular injection of antagomir-21-5p significantly attenuated the severity of experimental OA. Clinically, FGF18 expression level was correlated with miR-21-5p expression and a modified Mankin scale. CONCLUSION: Our findings reveal a miRNA functional pathway important for OA development, highlighting miRNA-21-5p silencing as an attractive therapeutic regimen in future clinical trials involving patients with OA.

Primary alveolar rhabdomyosarcoma of retrorectal-presacral space in an adult patient: A case report of an uncommon tumor with rare presentation.

RATIONALE: Rhabdomyosarcoma (RMS) has known as a highly malignant soft tissue sarcoma, representing 5% to 10% of all solid tumors in childhood. Alveolar rhabdomyosarcoma (ARMS) of the retrorectal-presacral space is an extremely rare lesion for adult, no study has been reported in the English literature. PATIENT CONCERNS: A 51-year-old male presented with abdominal pain for 1 month, significantly worse when having a bowel movement. DIAGNOSIS: Computed tomography (CT) and magnetic resonance imaging (MRI) of the pelvis showed a solid-cystic, enhancing lesion of dimension located in retrorectal-presacral space. The surgical specimen was reported as ARMS after pathological evaluation. INTERVENTIONS: The tumor was complete surgical resection, and after surgery, the patient was treated with combination chemotherapy. OUTCOMES: At 23 months follow up, the patient was asymptomatic with no evidence of metastases or local recurrence. LESSONS: Improvements in imaging in addition to early surgical intervention and chemotherapy treatment are crucial to improve survival chances against RMS.

Anatomical study based on 3D-CT image reconstruction of the hip rotation center and femoral offset in a Chinese population: preoperative implications in total hip arthroplasty.

BACKGROUND: Several anatomical studies regarding the value of hip rotation center (HRC) and femoral offset (FO) have been performed in Western populations. However, there are a few data on hip morphological values in the Chinese population based on CT scans. This study measured the values of the hip and pelvis, especially HRC and FO, in a Chinese population and compared them with the published values obtained from Western populations. PATIENTS AND METHODS: One hundred patients (50 females and 50 males) were included in the present study, and 3D-CT reconstructions of the hip and pelvis were generated. The mean age was 51.4 ± 8.9 years and mean body mass index (BMI) was 23.5 ± 2.6 kg/m2. All the morphologic measurements were compared between genders and sides, and the relationships between different parameters were analyzed. RESULTS: The mean FO values were 38.4 ± 4.7 mm and 35.6 ± 4.4 mm for the males and females, respectively. A significant negative correlation was noted between FO and neck shaft angle (NSA) in both genders (r = - 0.262, P = 0.009 for the males, r = - 0.350, P ≤ 0.001 for the females). A significant positive correlation was found between horizontal distance (HD) and diameter of the femoral head (DFH) in both genders (r = 0.734, P ≤ 0.001 for the males, r = 0.658, P ≤ 0.001 for the females). A significant positive correlation was noted between HD and pelvic width (PW) in males (r = 0.455, P ≤ 0.001). A significant positive correlation was also noted between HD and pelvic height (PH) in males (r = 0.318, P ≤ 0.001). A significant positive correlation was observed between FO and pelvic cavity height (PCH) in males (r = 0.411, P ≤ 0.001), and a significant positive correlation was observed between VD and PCH in females (r = 0.497, P ≤ 0.001). The tip of the greater trochanter was, on average, 7.0 mm higher than the femoral head center. Relationships between DFH and pelvic morphometric parameters were also observed. CONCLUSION: The present morphological data and the relationships between them can be applied to design better ethnic-specific THA prostheses and preoperative plans.

Total hip arthroplasty following failure of tantalum rod implantation for osteonecrosis of the femoral head with 5- to 10-year follow-up.

BACKGROUND: Total hip arthroplasty (THA) with failure of tantalum rod implant for osteonecrosis of the femoral head (ONFH) will be the only choice for patients. However,it remains unknown whether tantalum rod implantation has an adverse effect on the survival time of implants following conversion to THA. The aim of this study was to retrospectively evaluate the clinical and radiographic outcomes of conversion to THA in patients who were previously treated with implantation of a tantalum rod. METHODS: This study included 31 patients (39 hips), who underwent conversion to THA due to failure of core decompression with an implanted tantalum rod. Among these 31 patients, 26 patients were male and five patients were female. The mean age of these patients was 49.3 years old (range: 36-64 years old). The control group included 33 patients (40 hips), who underwent total hip replacement without tantalum rod implantation. The hip Harris score, implant wear, osteolysis, radiolucencies and surgical complications were recorded during the follow-up. The distribution of tantalum debris in the proximal, middle and distal periprosthetic femoral regions, radiolucent lines and osteolysis were analyzed on post-operative radiographs. RESULTS: There were no significant differences in Harris score, liner wear and complications between the two groups (P > 0.05). Osteolysis and radiolucent lines more likely occurred in patients with tantalum debris distributed in three regions than in one or two regions (P < 0.05). CONCLUSIONS: The mid-term clinical outcome of patients who underwent THA with tantalum rod implantation was not different from those without a tantalum rod, suggesting that tantalum debris did not increase the liner wear rate. However, the distribution of periprosthetic tantalum debris in the proximal, middle and distal femoral regions may increase the risk of femoral osteolysis and radiolucent lines.

Co-phasing experiment of a segmented mirror using a combined broadband and two-wavelength algorithm.

In this paper, a broadband phasing algorithm is combined with a two-wavelength phasing algorithm to detect the piston error of a segmented mirror with the advantages of long range, high precision, and fast detection. Moreover, an active optics co-phasing experimental system of the segmented mirror is built to verify the algorithm's effectiveness. The segmented mirror consists of four hexagonal segments, with flat-to-flat lengths of 100 mm and radii of curvature of 2000 mm. First, a Shack-Hartmann sensor and piezoelectric actuators are used to finely co-focus the segmented mirror. Then, the broadband phasing algorithm is used to reduce the piston error of the segmented mirror to several micrometers. Finally, the two-wavelength phasing algorithm is used to reduce the piston error of the segmented mirror to zero. The experimental results show that the measurement accuracy is better than 26 nm, and the adjustment accuracy is approximately 55 nm, which demonstrates that the combined algorithm is valuable for segmented-mirror co-phasing measurement and adjustment.

Primary intracerebral osteosarcoma: a rare case report and review.

BACKGROUND: Primary intracranial osteosarcoma is a extremely rare disease entity. We describe a case of primary intracerebral osteosarcoma in an adult brain. CASE DESCRIPTION: A patient who presented with a 1-week history of headaches, and MRI examination was performed. The immunohistochemical diagnosis confirmed primary intracerebral osteosarcoma. The patient was treated with a surgical resection of the tumor. CONCLUSION: Primary osteosarcomas occurring in the brain are extremely rare. The MRI images did not provide a specific pretreatment diagnosis, and the histopathology was the mainstay in establishing the diagnosis.

Inflammatory pseudotumor of the pineal region: First reported case.

Inflammatory pseudotumors originating in the central nervous system (CNS) are quite rare. To the best of our knowledge, the present study reports the first case of a inflammatory pseudotumor that developed in the pineal region, and describes the neuroimaging, morphological and immunohistochemical findings. A 53-year-old male presented with hearing loss that had been apparent for 1 year and blurred vision that had persisted for 10 months. Magnetic resonance imaging (MRI) demonstrated a homogeneously-enhanced mass in the pineal region and obstructive hydrocephalus. A pathological examination revealed that the lesion was comprised predominantly of spindled cells in a collagenous background, with dense infiltrates of small lymphocytes, plasma cells and uninucleated histiocytes. Immunopositivity for cluster of differentiation (CD)138 was noted. Immunohistochemical staining showed that the cells were immunonegative for glial fibrillary acidic protein, S-100, placental alkaline phosphatase, neurofilament and Pit-Oct-Unc class 5 homeobox 1. The Ki-67 labeling index was <5%. The lymphocytic infiltrates consisted of CD3- and CD43-positive T-cells, and CD20- and CD79A-positive B-cells. Plasma cells displayed polytypic reactivity for immunoglobulin κ and λ light chains. Based on the MRI and the morphological and immunohistochemical analysis, a diagnosis of an inflammatory pseudotumor was formed. The patient underwent a surgical resection of the tumor and currently has a good prognosis.

Abnormal coexistence of unipolar, bipolar, and threshold resistive switching in an Al/NiO/ITO structure.

This paper reports an abnormal coexistence of different resistive switching behaviors including unipolar (URS), bipolar (BRS), and threshold switching (TRS) in an Al/NiO/indium tin oxide (ITO) structure fabricated by chemical solution deposition. The switching behaviors have been strongly dependent on compliance current (CC) and switching processes. It shows reproducible URS and BRS after electroforming with low and high CC of 1 and 3 mA, respectively, which is contrary to previous reports. Furthermore, in the case of high-forming CC, TRS is observed after several switching cycles with a low-switching CC. Analysis of current-voltage relationship demonstrates that Poole-Frenkel conduction controlled by localized traps should be responsible for the resistance switching. The unique behaviors can be dominated by Joule heating filament mechanism in the dual-oxygen reservoir structure composed of Al/NiO interfacial layer and ITO. The tunable switching properties can render it flexible for device applications.