Role of percutaneous CT-guided radiofrequency ablation in treatment of intra-articular, in close contact with cartilage and extra-articular osteoid osteomas: comparative analysis and new classification system.

PURPOSE: We investigated procedural safety, technical and clinical outcomes of the percutaneous image-guided radiofrequency ablation (PRFA) of intra-articular (IA), intra-articular close to cartilage (IACC), and extra-articular (EA) osteoid osteomas (OO). We proposed a new radiologic classification for osteoid osteoma depending on the degree and location of sclerosis which may correlate with technical failure and/or difficulties. MATERIAL AND METHODS: According to the inclusion criteria, we enrolled consecutive patients who were referred to the investigation center from June 2018 to January 2022. After clinical and CT imaging features were suggestive for the diagnosis of OO, all the patients were treated by percutaneous CT-guided RFA with a standardized technique. Biopsy of the lesion was not performed in all patients. A retrospective analysis was conducted to assess the procedure's technical, primary clinical, and secondary clinical successes, recurrence rate, and complications. We classified all the OOs according to a new proposed classification of the site and the amount of sclerosis. RESULTS: A total number of 55 patients were enrolled in our study according to the inclusion criteria. The mean age of the enrolled patients was 24.07 ± 14.71 years (ranges from 7 to 57 years). The M/F ratio was roughly 2:1. The mean follow-up was 20.18 ± 12.60 months (ranges from 2 to 44 months). EA group included 36 patients, IA included 5 and IACC included 14 patients. Technical success was achieved in all cases of IA and IACC groups. Technical success in the EA group was 97.22% (1 technical failure). Primary clinical success was 100%, 92.85%, and 91.66% for IA, IACC, and EA groups, respectively. Accordingly, the recurrence rate was 5.88% in EA, and 7.14% in IACC, while no recurrence occurred in the IA group. No complications occurred. The secondary success rate of the 3 cases of recurrence was 100%. CONCLUSIONS: PRFA proved to be a safe procedure with a high rate of success for OO treatment even in intra-articular lesions in close contact with cartilage. This study showed that the results in terms of technical and clinical success are comparable for IA OO, IACC OO, and EA OO, even if the recurrence rate was higher in EA OO. Our proposed new classification of the degree and location of sclerosis may correlate to technical failure, but further studies with a larger number of patients are needed for validation.

Hippocampal sclerosis and epilepsy surgery in neurofibromatosis type 1: case report of a 3-year-old child explored by SEEG and review of the literature.

PURPOSE: Epilepsy associated with neurofibromatosis type 1 (NF1) is infrequent and usually controlled with anti-epileptic drugs. However, in some drug-resistant patients a presurgical evaluation should be considered. Hippocampal sclerosis (HS) is one of the rare causes of epilepsy in neurofibromatosis type 1, which can lead to surgery. METHODS: We present a three-year-old child with refractory epilepsy associated with several structural brain abnormalities but normal hippocampi on brain MRI and a heterozygous variant in the NF1 gene (c.2542G > A). A complete presurgical evaluation was performed including stereo-electroencephalography (SEEG). RESULTS: Usual seizures were recorded, and the seizure onset zone was delineated in the anterior hippocampus. Pathological examination performed after a tailored mesio-temporal resection confirmed hippocampal sclerosis, and the child achieved seizure freedom with 2 years of follow-up. CONCLUSION: This rare pediatric case illustrates that NF1 may be associated with early-onset refractory epilepsy secondary to MRI-negative HS, supporting the major role of SEEG in the presurgical evaluation of patients with extended cortical malformations.

Risk factors and clinical outcomes of encapsulating peritoneal sclerosis: A case-control study from China.

BACKGROUND: Encapsulating peritoneal sclerosis (EPS) is an uncommon, but serious complication in patients with continuous ambulatory peritoneal dialysis (PD) who have a considerable mortality rate. This study aimed to identify risk factors and outcomes of EPS in Chinese patients on PD. METHODS: Sixteen patients on PD who met the International Society for Peritoneal Dialysis criteria for diagnosis of EPS in the First Affiliated Hospital of Sun Yat-Sen University from 1997 to 2018 were included. Patients without EPS were matched for age, sex and the duration of PD and selected at a 1:3 ratio for the controls. A case-control study was conducted to analyse the clinical profile and risk factors associated with EPS in patients. RESULTS: The prevalence of EPS in patients on PD in our centre was 0.55%. The percentage of EPS significantly increased with the duration of PD. In univariate regression analysis, a history of peritonitis (odds ratios (OR): 2.83; 95% confidence interval (CI): 0.82-9.68; p = 0.08), peritoneal glucose exposure (OR: 1.12; 95% CI: 1.03-1.22; p < 0.01) and a high peritoneal transport status (OR: 14.70; 95% CI: 1.85-117.02; p < 0.01) were associated with EPS in patients on PD. However in the multivariate model, only a high peritoneal transport status (adjusted odds ratios (aOR): 13.65; 95% CI: 1.69-109.96; p = 0.01) was independently associated with EPS. CONCLUSION: The rate of EPS significantly increases with the duration of PD. Progressive peritoneal dysfunction, especially a high peritoneal transport status, is associated with a higher risk of EPS in this population.

Contribution of podocyte inflammatory exosome release to glomerular inflammation and sclerosis during hyperhomocysteinemia.

The nucleotide-binding oligomerization domain-like receptor containing pyrin domain 3 (NLRP3) inflammasome has been implicated in podocyte injury and glomerular sclerosis in response to hyperhomocysteinemia (hHcy). However, it remains unknown how the products of NLRP3 inflammasome in cytoplasm are secreted out of podocytes. In the present study, we tested whether exosome release serves as a critical mechanism to mediate the action of NLRP3 inflammasome activation in hHcy-induced glomerular injury. By various approaches, we found that hHcy induced NLRP3 inflammasome activation and neutrophil infiltration in glomeruli of WT/WT mice. Lysosome-MVB interaction in glomeruli remarkably decreased in WT/WT mice fed with FF diet, leading to elevation of urinary exosome excretion of these mice. Podocyte-derived exosomes containing pro-inflammatory cytokines increased in urine of WT/WT mice in response to hHcy. The release of inflammatory exosomes from podocytes was prevented by Smpd1 gene deletion but enhanced by podocyte-specific Smpd1 gene overexpression (Smpd1 encodes Asm in mice). Pathologically, hHcy-induced podocyte injury and glomerular sclerosis were blocked by Smpd1 gene knockout but amplified by podocyte-specific Smpd1 gene overexpression. Taken together, our results suggest that Asm-ceramide signaling pathway contributes to NLRP3 inflammasome activation and robust release of inflammatory exosomes in podocytes during hHcy, which together trigger local glomerular inflammation and sclerosis.

Tuberculosis lymphadenopathy: A rare etiology of the superior vena cava syndrome.

Superior vena cava syndrome is the clinical expression of the obstruction of the superior vena cava reducing the blood flow. Malignant etiologies are the most common. Its management is multidisciplinary and despite the progress of endovascular procedures, conventional surgery retains its place in certain indications. Mediastinal fibrosis secondary to tuberculosis lymphadenopathy may be associated with superior vena cava syndrome. In the presence of symptomatic SVCS associated with extensive mediastinal fibrosis compressing the superior vena cava with sub occlusive thrombosis, conventional surgery remains a treatment option, with cavo-venous derivation by prosthetic bypass.

A quantitative MRI index for assessing the severity of hippocampal sclerosis in temporal lobe epilepsy.

BACKGROUND: Hippocampal sclerosis (HS) is associated with post-surgery outcome in patients with temporal lobe epilepsy (TLE), and an automated method that quantifies HS severity is still lacking. Here, we aim to propose an MRI-based HS index (HSI) that integrates hippocampal volume and FLAIR signal to measure the severity of HS. METHODS: Forty-two pre-surgery TLE patients were included retrospectively, with T1-weighted (T1W) and FLAIR images acquired from each subject. Two experienced neurosurgeons (W.D. and C.S.) and one neurologist (Q.L.) rated HS severity with a four-class grading scale (normal, mild, moderate and severe) based on both hippocampal volume loss and increased FLAIR signal. A consensus of HS severity for each subject was made by voting among the three visual rating results. Regarding the automatic quantification, the hippocampal volume was quantified by AccuBrain on T1W image, and the FLAIR signal of hippocampus was calculated as the mean intensity of hippocampal region on the FLAIR image (normalized by the mean intensity of gray matter). To fit the HSI from visual rating, we applied ordinal regression with the voted visual rating as the dependent variable, and hippocampal volume and FLAIR signal as the independent variables. The HSI was calculated by weighting the predicted probabilities of the four-class grading scales from ordinal regression. RESULTS: The intra-class correlation coefficient (single measure) of the three raters was 0.806. The generated HSI was significantly correlated with the visual rating scales of the three raters (W.D.: 0.823, Q.L.: 0.817, C.S.: 0.717). HSI scores well differentiated the different HS categories as defined by the agreed HS visual rating (normal vs. mild: p < 0.001, mild vs. moderate: p < 0.001, moderate vs. severe: p = 0.001). CONCLUSIONS: The proposed HSI was consistent with visual rating scales from epileptologists and sensitive to HS severity. This MRI-based index may help to evaluate HS severity in clinical practice. Further validations are needed to associate HSI with post-surgery outcomes.

Combined sclerosis of the spinal cord revealing Biermer's anemia: about a case.

Biermer's anemia is an auto immune disease that can lead to neurological manifestations. Medullary combined sclerosis accounts for only 10% of neurological complications. There are few reports of documented bone marrow involvement by magnetic resonance imaging (MRI). Observation: We report a case of combined sclerosis of the cervical spinal cord assessed by cerebro-medullary MRI in a context of vitamin B12 deficiency in a 36-year-old patient who was hospitalized in the intensive care unit for septic shock with respiratory starting point, requiring the use of mechanical ventilation for 13 days and the discovery in post-extubation of pancytopenia with macrocytic anemia and flasquo-spasmodic tetraparesis prompting an emergency MRI showing combined sclerosis of the spinal cord , with vitamin B12 deficiency and megaloblastic anemia at the myelogram. The patient had benefited of vitamin therapy combined with a physical rehabilitation and the evolution was marked by a recovery of walking and normalization of biology after 2 months. Conclusion:    The advantage of medullary MRI in a severe clinical form with neurological impairment of vitamin B12 deficiency to guide the diagnosis. The treatment is based on vitamin therapy with a good evolution.

[Suspected hippocampal sclerosis in an european shorthair cat diagnosed with polycythemia vera]. / Verdacht auf Hippocampussklerose bei einer Europäisch-Kurzhaar-Katze mit der Diagnose Polycythaemia vera.

A 7-year-old male castrated cat was presented because of an acute onset of lethargy and vestibular ataxia. The cat was diagnosed with polycythemia vera. Later the patient developed additional clinical signs including orofacial twitching, aggressivity, hypersalivation, circling and a head tilt. A magnetic resonance imaging was performed and revealed hippocampal alterations compatible with hippocampal sclerosis. The presented case report describes the clinical signs and hematologic findings in a cat with polycythemia vera and the response of treatment. Another part is the discussion about the hypothesis that the hippocampal alterations were due to a cerebral hypoperfusion caused by the polycythemic condition.

Long-term outcomes after childhood convulsive status epilepticus.

PURPOSE OF REVIEW: It is widely accepted that childhood convulsive status epilepticus (CSE) has associated short-term and long-term mortality and morbidity. However, the role of CSE itself on subsequent adverse outcomes is still debated. In addition, whether prolonged seizures cause any long-term hippocampal injury and developmental or memory impairment is uncertain. In this review, we aim to provide an overview of long-term outcomes after childhood CSE, highlighting data from recent literature on this subject. RECENT FINDINGS: Long-term outcome after childhood CSE is favorable in previously normal children, with low incidence of epilepsy, motor and intellectual disability, behavioral impairment and need for special educational provision. Mesial temporal sclerosis is uncommon in children after prolonged febrile seizures. There is substantial morbidity after childhood CSE, but this is seen primarily in children with symptomatic causes and preexisting neurological abnormalities. Cause is the primary determinant of outcomes after childhood CSE and the additional effect of CSE characteristics such as seizure duration seems to be less than previously believed. SUMMARY: Childhood CSE is associated with substantial neurological, cognitive and behavioral morbidity. Early identification of these difficulties and appropriate intervention are likely to have a major positive impact on their quality of life.

Multimodality Imaging of Focal and Diffuse Fibrosing Mediastinitis.

Fibrosing mediastinitis is a rare benign but potentially life-threatening process that occurs because of proliferation of fibrotic tissue in the mediastinum. The focal subtype is more common and typically is associated with an abnormal immunologic response to Histoplasma capsulatum infection. Affected patients are typically young at presentation, but a wide age range has been reported, without a predilection for either sex. The diffuse form may be idiopathic or associated with autoimmunity, usually affects middle-aged and/or elderly patients, and is more common in men. For both subtypes, patients present with signs and symptoms related to obstruction or compression of vital mediastinal structures. The most common presenting signs and symptoms are cough, dyspnea, recurrent pneumonia, hemoptysis, and pleuritic chest pain. Patients with the diffuse subtype may have additional extrathoracic symptoms depending on the other organ systems involved. Because symptom severity is variable, treatment should be individualized with therapies tailored to alleviate compression of the affected mediastinal structures. Characteristic imaging features of fibrosing mediastinitis include infiltrative mediastinal soft tissue (with or without calcification) with compression or obstruction of mediastinal vascular structures and/or the aerodigestive tract. When identified in the appropriate clinical setting, these characteristic features allow the radiologist to suggest the diagnosis of fibrosing mediastinitis. Careful assessment is crucial at initial and follow-up imaging for exclusion of underlying malignancy, assessment of disease progression, identification of complications, and evaluation of treatment response. Online supplemental material is available for this article. ©RSNA, 2019.

Estimating risk of encapsulating peritoneal sclerosis accounting for the competing risk of death.

BACKGROUND: Risk of encapsulating peritoneal sclerosis (EPS) is strongly associated with the duration of peritoneal dialysis (PD), such that patients who have been on PD for some time may consider elective transfer to haemodialysis to mitigate the risk of EPS. There is a need to determine this risk to better inform clinical decision making, but previous studies have not allowed for the competing risk of death. METHODS: This study included new adult PD patients in Australia and New Zealand (ANZ; 1990-2010) or Scotland (2000-08) followed until 2012. Age, time on PD, primary renal disease, gender, data set and diabetic status were evaluated as predictors at the start of PD, then at 3 and 5 years after starting PD using flexible parametric competing risks models. RESULTS: In 17 396 patients (16 162 ANZ, 1234 Scotland), EPS was observed in 99 (0.57%) patients, less frequently in ANZ patients (n = 65; 0.4%) than in Scottish patients (n = 34; 2.8%). The estimated risk of EPS was much lower when the competing risk of death was taken into account (1 Kaplan-Meier = 0.0126, cumulative incidence function = 0.0054). Strong predictors of EPS included age, primary renal disease and time on PD. The risk of EPS was reasonably discriminated at the start of PD (C-statistic = 0.74-0.79) and this improved at 3 and 5 years after starting PD (C-statistic = 0.81-0.92). CONCLUSIONS: EPS risk estimates are lower when calculated using competing risk of death analyses. A patient's estimated risk of EPS is country-specific and can be predicted using age, primary renal disease and duration of PD.

Peripheral Vaso-Occlusive Events Following Trauma in Patients With Sickle Cell Trait.

Sickle cell trait (SCT) is a common hemoglobin variant, particularly in patients of African descent. Patients with SCT have an increased risk of adverse ocular events following trauma with subsequent elevated intraocular pressure (IOP). The authors describe three cases of young males with peripheral vaso-occlusive events following ocular trauma with subsequent elevated IOP. These cases demonstrate the importance of careful peripheral exams in young patients with SCT following trauma. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:e84-e87.].

Evaluation of dual pathology among drug-resistant epileptic patients with hippocampal sclerosis.

PURPOSE: Dual pathology (DP) is defined as simultaneous presence of hippocampal sclerosis (HS) and any other pathology in the brain. Since this is a less probed concept, we aimed to evaluate the frequency and characteristics of DP among drug-resistant epileptic patients with HS. METHODS: This is a cross-sectional study conducted during 2007-2016 in Kashani Comprehensive Epilepsy Center, Isfahan, Iran. Patients with diagnosis of drug-resistant epilepsy and HS were enrolled in the study, and demographic data, seizure semiology, EEG findings, and MRI findings were collected. We compared these variables between three groups of DP, unilateral HS, and bilateral HS. RESULTS: Of the 200 enrolled cases, 29 patients (14.5%) had DP and 21 patients (10.5%) had bilateral HS; the remaining patients had unilateral HS. The average age of patients with DP was 30.03, and 65.5% of them were male. Patients with DP had more EEG discharges from regional and multi-focal sites compared to unilateral HS (P value < 0.001). Also, complex partial seizure (CPS) was more commonly presented in patients with unilateral HS (96.8%). Comparison of disease characteristics between DP and bilateral HS showed no difference in most categories (P > 0.05). CONCLUSIONS: We found DP among 14.5% of our drug-resistant epileptic patients with HS. DP patients mostly presented with CPS and had high proportion of ictal and interictal EEG discharges from regional and multi-focal areas. Gliosis and focal cortical dysplasia were the most common pathologies among DP patients. Patients with DP showed a similar behavior to bilateral HS in many features.

Neurocysticercosis as a probable risk factor for hippocampal sclerosis.

OBJECTIVE: Neurocysticercosis is one of the most common risk factors for epilepsy but its association with drug-resistant epilepsy remains uncertain. Conjectures of an association with drug-resistant epilepsy have been fueled by reports of an association between calcific neurocysticercosis lesions (CNL) and hippocampal sclerosis (HS) from specialized epilepsy centers in Taenia solium-endemic regions. METHODS: The debate arising from these reports is whether the association is causal. Evidence for the association is not high quality but sufficiently persuasive to merit further investigation with longitudinal imaging studies in population-based samples from geographically-diverse regions. The other controversial point is the choice of a surgical approach for drug-resistant epilepsy associated with CNL-HS. RESULTS: Three approaches have been described: standard anteromesial temporal lobectomy, lesionectomy involving a CNL alone and lesionectomy with anteromesial temporal lobectomy (for dual pathology); reports of the latter two approaches are limited. CONCLUSIONS: Presurgical evaluation should consider possibilities of delineating the epileptogenic zone/s in accordance with all three approaches.

Neurocysticercosis as a probable risk factor for hippocampal sclerosis / Neurocisticercose como provável fator de risco para esclerose hipocampal

ABSTRACT Neurocysticercosis is one of the most common risk factors for epilepsy but its association with drug-resistant epilepsy remains uncertain. Conjectures of an association with drug-resistant epilepsy have been fueled by reports of an association between calcific neurocysticercosis lesions (CNL) and hippocampal sclerosis (HS) from specialized epilepsy centers in Taenia solium-endemic regions. The debate arising from these reports is whether the association is causal. Evidence for the association is not high quality but sufficiently persuasive to merit further investigation with longitudinal imaging studies in population-based samples from geographically-diverse regions. The other controversial point is the choice of a surgical approach for drug-resistant epilepsy associated with CNL-HS. Three approaches have been described: standard anteromesial temporal lobectomy, lesionectomy involving a CNL alone and lesionectomy with anteromesial temporal lobectomy (for dual pathology); reports of the latter two approaches are limited. Presurgical evaluation should consider possibilities of delineating the epileptogenic zone/s in accordance with all three approaches.

RESUMO A neurocisticercose é um dos mais comuns fatores de risco para a epilepsia, mas sua associação com a epilepsia resistente a medicamentos (DRE) permanece incerta. Conjecturas de uma associação com a DRE têm sido alimentadas por relatos de uma associação entre lesões de neurocisticercose calcária (CNL) e esclerose hipocampal (HS) de centros especializados em epilepsia em regiões endêmicas de Taenia solium. O debate que surge desses relatórios é se a associação é causal. Se bem as evidências para a associação não são de alta qualidade, são suficientemente persuasivas para merecer mais investigação com estudos longitudinais de imagens em amostras de base populacional de regiões geograficamente diversas. O outro ponto controverso é a escolha da abordagem cirúrgica para a DRE associada à CNL-HS. Três abordagens têm sido descritas: lobectomia temporal ântero-mesial padrão, lesionectomia envolvendo apenas CNL e lesionectomia com lobectomia temporal ântero-mesial (para patologia dupla); os relatórios das duas últimas abordagens são limitados. A avaliação pré-cirúrgica deve considerar as possibilidades de delinear a (s) zona (s) epileptogênica (s) de acordo com as três abordagens.

Increased expression of WNK3 in dispersed granule cells in hippocampal sclerosis of mesial temporal lobe epilepsy patients.

Granule cell dispersion (GCD) is a common neuropathological feature of hippocampal sclerosis (HS) in patients with temporal lobe epilepsy (TLE). However, the underlying molecular mechanism of GCD formation remains unclear. The present study aimed to investigate the expressional changes of With No Lysine protein kinase subtype 3 (WNK3), a molecule upstream of cation-chloride cotransporters with reciprocal expression in sclerosed hippocampus of TLE patients. Using immunofluorescence staining, we analyzed WNK3 immunoreactivity in hippocampal specimens from histologically normal controls and TLE patients with HS. Our results showed that WNK3 expression was significantly increased in dispersed granule neurons in hippocampal tissues from patients with TLE compared with histologically normal hippocampus. These findings demonstrate a potential association between an increased expression of WNK3 and GCD formation during the chronic phase of epilepsy. Controlling WNK3 expression may thus be a novel therapeutic target in epileptogenesis.

Some Peculiarities of Local Distribution of Multipotent Mesenchymal Stromal Cells after Their Injection into Intact Muscle Tissue in Experiment.

Changes in the muscular tissue after subcutaneous injection of autologous bone marrow multipotent mesenchymal stromal cells transfected with GFP gene and additionally stained with cell membrane dye Vybrant CM-Dil in the projection of ligated femoral vein were studied by light microscopy with luminescence. Stromal cells injected through the skin can appear not only in the damaged tissue where acceleration of regeneration processes is required, but also in intact structures located in superficial or deeper layers. In intact muscular tissue, stromal cells spreading in the perivascular tissue initiate inflammation and migration of macrophages, activate and even trigger sclerotic processes due to differentiation into connective tissue cells (fibroblasts) and stimulation of proliferation and collagen synthesis by host fibroblasts. Injected multipotent mesenchymal stromal cells are gradually phagocytized by macrophages.