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Background: The management of epilepsy is mainly based on antiseizure medications (ASMs). More than 20 ASMs have been introduced in clinical practice, providing a multitude of prescription choices. To date, there are no published data on the trends in ASMs prescriptions in Morocco. Therefore, we conducted a survey among practicing neurologists in seven tertiary referral hospitals in Morocco to study the current ASMs prescription preferences and their modifying factors. Methods: Our descriptive and analytical cross-sectional study was based on a survey sent between January and April 2022 to neurologists practicing in seven tertiary referral hospitals in Morocco. Information regarding the prescription of ASMs was collected using an exploitation form and analyzed using the SPSS version 13 software. Results: Based on questionnaire responses, our results showed that Valproic acid (96.3%) and Lamotrigine (59.8%) were the two most prescribed ASMs for generalized seizure types. For focal seizure types, Carbamazepine (98.8%) and Levetiracetam (34.1%) were the most commonly prescribed drugs, whereas for combined focal and generalized seizure types, the combination of Valproic acid and Carbamazepine (38.55%) was the most prescribed. Phenobarbital was the most commonly prescribed ASM for status epilepticus (40.2%). These prescription preferences were mainly due to seizure types, cost, health insurance coverage, years of experience, and additional epileptology training (p < 0.05). Conclusion: Our results show a shift in the prescription of ASMs in Morocco. Similar to many other countries, valproic acid and carbamazepine are considered the first-line treatments for generalized and focal seizure types. Some factors remain as major challenges in enhancing epilepsy management in Morocco.

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Castleman disease is a rare and atypical lymphoproliferative disorder characterized by diverse clinical manifestations. It has both unicentric and multicentric forms, the latter with further subdivisions, i.e., human herpesvirus 8-associated and idiopathic forms. The diagnosis of Castleman disease is often delayed, as it is rare, and because it shares clinical features with different autoimmune, inflammatory, and malignant lymphoproliferative disorders. The first-line treatment in unicentric form is mainly surgical, while in idiopathic Castleman disease, anti-interleukin-6 treatment is the therapy of choice. In virus-associated diseases, antiretroviral therapy and rituximab are recommended. In Hungary, only a few cases of Castleman disease have been published. This report presents our two decades of experience in the challenging diagnosis and management of this rare disorder, most properly underdiagnosed in Hungary. We provide insights into seven unicentric and five idiopathic multicentric Castleman disease cases, the latter ones especially highlighting the diagnostic and therapeutic challenges due to the variable and unique clinical features both of patients and diseases, e.g., bronchiolitis obliterans, stage IV diabetic renal failure, anti-HBc positivity, siltuximab treatment period, respectively.

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We present a case of pulmonary metastasis originating from renal angiomyolipoma (AML), as evidenced by whole-exome sequencing (WES) analysis. Although AML predominantly arises in the kidneys, it can emerge in various body parts, making it important to distinguish between multicentric development and metastasis. However, previous studies have not distinguished between these conditions. Our case features an 82-year-old woman with a history of renal AML who presented with multiple, randomly distributed, bilateral pulmonary nodules of varying size and pure fat densities. The patient's condition followed a benign course over 10 years. Through WES, we discovered shared mutations in pulmonary lesions that were absent in the patient's blood, including a pathological mutation in TSC2, suggesting a metastatic origin from renal AML. Knowledge of the pulmonary manifestations of AML and their distinctive imaging findings can help radiologists and clinicians diagnose and manage patients with similar presentations.

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This study aimed to determine COVID-19 recovery time and identify predictors among hospitalized patients in the Dhanusha District of Madhesh Province, Nepal. This hospital-based longitudinal study involved 507 COVID-19 patients admitted to three distinct medical facilities for therapeutic intervention between April and October 2021. Data were collected for patient demography, symptoms, vital signs, oxygen saturation levels, temperatures, heart rates, respiratory rates, blood pressure measurements, and other health-related conditions. Kaplan-Meier survival curves estimated the recovery time, and a Cox proportional hazard model was used to identify the predictors of recovery time. For the total participants, mean age was 51.1 (SD = 14.9) years, 68.0% were males. Of the total patients, 49.5% recovered, and 16.8% died. The median for patient recovery was 26 days (95% CI: 25.1-26.7). Patients with severe or critical conditions were less likely to recover compared to those with milder conditions (hazard ratio (HR) = 0.34, 95% CI: 0.15-0.79; p = 0.012). In addition, an increase in oxygen saturation was associated with an elevated likelihood of recovery (HR = 1.09, 95% CI = 1.01-1.17, p = 0.018). This study underscores the need for early admission to hospital and emphasizes the targeted interventions in severe cases. Additionally, the results highlight the importance of optimizing oxygen levels in COVID-19 patient care.

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An osteoid osteoma is typically a benign bone tumor affecting young adult males, often presenting with nocturnal pain alleviated by nonsteroidal anti-inflammatory medications (NSAIDS). It usually manifests as a solitary nidus with surrounding sclerosis. An osteoid osteoma with a multicentric nidus, characterized by multiple nidi, is a rare variant. A 12-year-old girl presented with a one-year history of worsening, nighttime pain in her upper left leg. Plain radiographs revealed two lytic lesions with sclerosis. A computed tomography (CT) scan confirmed two well-defined sclerotic lesions with central lytic lesions. Magnetic resonance imaging (MRI) demonstrated two hypointense lesions with peripheral hyperintensity on short tau inversion recovery (STIR) sequences, suggestive of osteoid osteoma with a multicentric nidus. Differential diagnoses included osteomyelitis with Brodie's abscess, osteoblastoma, chondroblastoma, and malignant lesions. Due to the atypical presentation and lack of experience with radiofrequency ablation (RFA) for multicentric cases, surgical excision was performed. Histopathology confirmed osteoid osteoma. After rehabilitation, the patient was asymptomatic at six months with no recurrence on radiographs. This case highlights the unusual presentation of osteoid osteoma with a multicentric nidus in a young female. Radiological workup with plain films, CT, and MRI was crucial for diagnosis. While RFA is gaining popularity, surgical excision remains a valid option, especially for atypical cases.

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INTRODUCTION: Whole Exome Sequencing (WES) has emerged as an efficient tool in clinical cancer diagnostics to broaden the scope from panel-based diagnostics to screening of all genes and enabling robust determination of complex biomarkers in a single analysis. METHODS: To assess concordance, six formalin-fixed paraffin-embedded (FFPE) tissue specimens and four commercial reference standards were analyzed by WES as matched tumor-normal DNA at 21 NGS centers in Germany, each employing local wet-lab and bioinformatics. Somatic and germline variants, copy-number alterations (CNAs), and complex biomarkers were investigated. Somatic variant calling was performed in 494 diagnostically relevant cancer genes. The raw data were collected and re-analyzed with a central bioinformatic pipeline to separate wet- and dry-lab variability. RESULTS: The mean positive percentage agreement (PPA) of somatic variant calling was 76 % while the positive predictive value (PPV) was 89 % in relation to a consensus list of variants found by at least five centers. Variant filtering was identified as the main cause for divergent variant calls. Adjusting filter criteria and re-analysis increased the PPA to 88 % for all and 97 % for the clinically relevant variants. CNA calls were concordant for 82 % of genomic regions. Homologous recombination deficiency (HRD), tumor mutational burden (TMB), and microsatellite instability (MSI) status were concordant for 94 %, 93 %, and 93 % of calls, respectively. Variability of CNAs and complex biomarkers did not decrease considerably after harmonization of the bioinformatic processing and was hence attributed mainly to wet-lab differences. CONCLUSION: Continuous optimization of bioinformatic workflows and participating in round robin tests are recommended.

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Background: Understanding pathways to dual diagnosis (DD) care will help organize DD services and facilitate training and referral across healthcare sectors. Aim: The aim of our study was to characterize the stepwise healthcare and other contacts among patients with DD, compare the characteristics of the first contact persons with common mental disorder (CMD) versus severe mental illness (SMI), and estimate the likelihood of receiving appropriate DD treatment across levels of contacts. Methods: This cross-sectional, descriptive study in eight Indian centers included newly enrolled patients with DD between April 2022 and February 2023. The research spans varied geographic regions, tapping into regional variations in disease burden, health practices, and demographics. The study categorized healthcare contacts by using the WHO Pathways Encounter Form. Results: The sample (n = 589) had a median age of 32 years, mostly males (96%). Alcohol was the most common substance; SMI (50.8%) and CMD were equally represented. Traditional healers were a common first contact choice (18.5%); however, integrated DD care dominated subsequent contacts. Assistance likelihood increased from the first to the second contact (23.1% to 62.1%) but declined in subsequent contacts, except for a significant rise in the fifth contact (97.4%). In the initial contact, patients with CMD sought help from public-general hospitals and private practitioners for SUD symptoms; individuals with SMI leaned on relatives and sought out traditional healers for psychiatric symptoms. Conclusion: Recognizing the cultural nuances, advocating for integrated care, and addressing systemic challenges pave the way to bridge the gap in DD treatment.

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Diffuse large B-cell lymphoma (DLBCL) is the most prevalent subtype of non-Hodgkin lymphoma (NHL) in domestic dogs, with many similarities to its human counterpart. The progression of the disease is rapid, and treatment must be initiated early to achieve cancer remission and extend life. This study examined the relationship between progression-free survival (PFS) and microRNA (miRNA) expression in dogs with DLBCL. miRNAs are small non-coding RNA molecules that typically regulate gene expression post-transcriptionally. They are involved in several pathophysiological processes, including the growth and progression of cancer. Based on the analysis of small RNA sequencing (sRNA-seq) data, we validated a group of miRNAs in lymph nodes from 44 DLBCL-affected dogs with known outcomes. We used quantitative PCR to quantify their expression and report a specific subset of miRNAs is associated with decreased PFS in dogs with DLBCL. The miR-192-5p and miR-16-5p expression were significantly downregulated in dogs with increased PFS. These results indicate that miRNA profiling may potentially identify dogs with DLBCL that will experience poor outcomes following treatment. Identifying specific miRNAs that correlate with the progression of canine DLBCL could aid the development of individualized treatment regimens for dogs.

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Castleman disease (CD) comprises a rare spectrum of disorders characterized by benign lymphoepithelial proliferation, classified into unicentric and multicentric forms. The idiopathic multicentric Castleman disease (iMCD) subtype, specifically, is challenging to diagnose and treat due to its variable manifestations and unpredictable disease course. We report a case of a 23-year-old female with a history of iron deficiency anemia presenting with concurrent antiphospholipid syndrome (APS) and human herpesvirus-6 (HHV-6) positivity. Investigations revealed a gastric mass, with a biopsy suggestive of the plasma cell variant of CD. This case report aims to understand the possible association of HHV-6 positivity with CD and the significance of diagnosing APS early in patients with the disease. Treatment with siltuximab and tocilizumab proved effective, highlighting the role of interleukin 6 (IL-6) in the elusive etiology of this condition.

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Castleman's disease is a rare lymphoproliferative disease that usually presents as a solitary mass in the mediastinal or cervical region. Castleman's disease can be usually of two types: unicentric type (which involves only one site of lymph nodes) and multicentric type (which involves multiple sites of lymph nodes). We report the case of a 26-year-old female with multiple sclerotic bone lesions in unicentric Castleman's disease. The definitive diagnosis was made by excisional biopsy with immunohistochemistry, 18F-fluoro-2-deoxy-D-glucose positron emission tomography (FDG-PET) study, and MRI scan. This case report emphasizes the need for proper workup for systemic manifestations in unicentric Castleman's disease.

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BACKGROUND: Chronic rhinosinusitis with nasal polyps (CRSwNP) is a prevalent chronic inflammatory condition affecting the nose and paranasal sinuses, posing a significant socio-economic impact with substantial challenges in management. Biologics targeting type 2 inflammation such as dupilumab, have emerged as promising options. This study addresses a critical knowledge gap by comprehensively evaluating the 3-year impact of sustained dupilumab therapy in CRSwNP. METHODS: A multicentric, retrospective collection of real-world data from five tertiary referral centers in Germany was conducted, enrolling 150 adult patients. The study investigated patient-reported outcomes, disease-specific indices and clinical measures, focusing on therapeutic response persistence, adverse events, and factors influencing treatment continuity. RESULTS: Results indicate significant improvements in clinical parameters from baseline (n = 150) with sustained effectiveness after 36 months (n = 138) as indicated in mean score ± standard deviation. Dupilumab treatment significantly improved overall disease-related impairment (VAS score: 7.5 ± 2.5 to 1.6 ± 1.3) and rhinosinusitis symptoms (SNOT-22: 59.4 ± 19.4 to 18.0 ± 15.0). Nasal Polyp Scores (NPS) decreased (5.3 ± 1.8 to 0.7 ± 1.1), and olfactory function improved (3.2 ± 2.5 to 8.4 ± 2.8), with three out of four patients achieving normosmia or hyposmia after 36 months. An "Excellent" treatment response according to EUFOREA23 criteria was observed in 76.5% of patients after 36 months. Sixteen patients discontinued Dupilumab, 12 permanently. Adverse events totaled 69 in 48 patients, commonly self-limiting. CONCLUSION: The study highlights the enduring effectiveness and lack of habituation to dupilumab after a sustained therapy of 3 years, providing valuable insights into its long-term therapeutic implications for CRSwNP patients.

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Canine lymphoma, the most prevalent haematopoietic tumour in dogs, presents significant challenges in veterinary oncology. This study investigates the prognostic value of the neutrophil-to-lymphocyte ratio (NLR) in small-sized dogs (≤10 kg) with multicentric lymphoma. In this retrospective study, we examined medical records and haematological data from 35 dogs to assess the association between NLR and two key outcomes: time-to-progression (TTP) and lymphoma-specific survival (LSS) using Cox proportional hazards models. Our findings revealed a significant correlation between elevated NLR and a worse prognosis, as evidenced by TTP (p = 0.005) and LSS (p = 0.001). NLR is linked to increased hazard ratios (HRs) for the time-to-progression rate (TTPR) at 180, 360 and 540 days (p = 0.001, p = 0.003 and p = 0.005, respectively) and the lymphoma-specific survival rate (LSSR) at the same intervals (p = 0.016, p = 0.001 and p = 0.001, respectively). Cutoff value of 3.764 for NLR was established, above which there is a significantly increased risk of early disease progression and decreased survival. Additionally, our analysis indicates that dogs with substage b exhibited earlier progression than those with substage a, evident in overall (p = 0.026) and TTPR at 180 days (p = 0.004), 360 days (p = 0.018), 540 days (p = 0.026) and LSSR at 180 days (p = 0.033). The results underscore the potential of NLR as a prognostic marker in cases of dogs ≤10 kg with multicentric lymphoma, suggesting that higher NLR is associated with a poorer prognosis.

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A 55-year-old man was admitted to the hospital with vomiting, diarrhoea, and chest pain. Upon examination, he exhibited signs of increased inflammatory response, acute kidney injury, and thrombocytopenia, leading to a diagnosis of TAFRO syndrome, which was supported by the clinical evidence of generalized lymphadenopathy, pleural effusion, and hepatosplenomegaly. Despite receiving intensive multimodal immunosuppressive therapy, including glucocorticoid pulse therapy (methylprednisolone 1,000 mg/day), tocilizumab, and cyclosporine in the intensive care unit, the patient showed minimal response and succumbed to the disease on the seventh day of hospitalization. Histopathological analysis of the lymph nodes revealed idiopathic multicentric Castleman disease (iMCD)-like features, and Epstein-Barr virus-encoded RNA (EBER) in situ hybridization identified multiple EBER-positive cells. These findings highlight the elusive pathogenic mechanism of TAFRO syndrome and the potential resistance of some patients to standard treatments such as tocilizumab. The presence of EBER-positive cells in lymph nodes or bone marrow may serve as an indicator of disease severity and treatment resistance. Therefore, histopathological detection of EBER-positive cells may help predict responsiveness to conventional treatments, disease severity, and prognosis in patients with TAFRO syndrome.

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Canine multicentric lymphoma (CML) is a prevalent hematopoietic neoplasm that initially responds well to treatment but often relapses due to chemotherapy resistance. Evaluation of treatment response is essential for effective management. Ultrasound (US) can differentiate between benign and lymphomatous lymph nodes (LLNs). However, its utility in monitoring LLNs post chemotherapy is limited. This study aimed to compare US parameters of LLNs during the first 3 weeks post treatment and evaluate their diagnostic performance compared with the conventional method for assessing treatment response. This study included 95 LLNs from 15 dogs with CML and 60 normal lymph nodes (NLNs) from 15 healthy dogs. US, including B-mode and elastography, was performed pre-treatment and weekly for 3 weeks post treatment, and compared with the results of NLNs. LLNs were categorized into partial response and stable disease groups using the conventional method. US scores were established by combining B-mode and elastography parameters. The results showed significantly higher values of LLNs in the short-to-long axis ratio, elastographic scales, and blue-to-green color histogram compared with NLNs. Additionally, LLNs at pre-treatment had significantly higher values than LLNs post treatment. US scores significantly differed among the healthy, partial response, and stable disease groups. In conclusion, B-mode US, elastography, and US scores demonstrated changes during chemotherapy consistent with the conventional method and can be used in conjunction with the conventional method to evaluate the treatment response of CML.

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Multicentric reticulohistiocytosis (MRH) is the most frequent entity in the group of reticulohistiocytoses. It is usually accompanied by a symmetrical erosive polyarthritis and is frequently associated with cancer and autoimmune disorders. Autoimmune syndrome induced by adjuvants (ASIA) is an inflammatory syndrome triggered by adjuvants such as those contained in vaccines or by silicone implants. Here we report a 71-years old female with a history of breast cancer treated with surgery and subsequent prosthesis who developed a systemic hyperinflammatory syndrome including seronegative symmetric polyarthritis, multiple skin lesions and two large nodular lesions in the oral cavity and larynx. Clinical picture was consistent with a clinical diagnosis of ASIA, with breast implant rupture and/or vaccination against SARS-CoV-2 as possible triggers. Histopathology of skin, oral and laryngeal nodules revealed cutaneous/mucous and submucosal infiltration of large epithelioid mononuclear or binucleated cells with fine granular ground glass-like cytoplasm and round to kidney-shaped nuclei with prominent nucleoli, without atypical features or relevant pleomorphism, accompanied by sparse giant cells and lymphocytes. These cells stained positive for CD68 and CD45 and negative for S100, CD1a, and markers of epithelial or neural/melanocytic differentiation, altogether consistent with a diagnosis of reticulohistiocytosis. Clinic-pathological correlation allowed the final diagnosis of MRH. To our knowledge, this is the first report of a co-occurrence of MRH with ASIA and this is relevant to broaden the spectrum of those both rare diseases.

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The optimal treatment endpoints and duration of continuous therapy for multicentric Castleman disease (MCD) remain controversial. We retrospectively analyzed data from 123 patients with Human Herpesvirus (HHV)-8 negative MCD. We demonstrated that continuous therapy significantly enhanced progression-free survival (PFS) in patients who achieved an optimal response after initial treatment. These findings underscore the critical role of continuous therapy in HHV-8 negative MCD. Further studies with larger cohorts are required to validate these findings.

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PURPOSE: Neurocytomas represent 0.25 to 0.5% of primary brain tumours and are mainly found in young adults. These tumours have neuronal differentiation. The cornerstone treatment is neurosurgery. The efficacy of other therapies, including radiotherapy, is still unclear. The objective of this study was to evaluate the management of central neurocytomas and the role of radiotherapy. MATERIALS AND METHODS: All adult patients (age 18 years or older) newly diagnosed with a histologically confirmed neurocytoma between 2006 and 2015 in France were included. RESULTS: One hundred and sixteen patients were diagnosed with a central neurocytoma during the study period. All patients underwent surgical resection, and six received adjuvant radiotherapy. Eleven patients received radiotherapy due to progression. After a median follow-up of 68.7 months, local failure occurred in 29 patients. The 5-year local control rate was 73.4%. According to univariate analysis, marker of proliferation Ki67 index greater than 2% (hazard ratio [HR]: 1.48; confidence interval [CI]: 1.40-1.57; P=0.027) and subtotal resection (HR: 8.48; CI: 8.01-8.99; P<0.001) were associated with an increase in local failure. Gross total resection was associated with a higher risk of sequelae epilepsy (HR: 3.62; CI: 3.42-3.83; P<0.01) and memory disorders (HR: 1.35; CI: 1.07-1.20; P<0.01). Ten patients (8.6%) died during the follow-up. The 10-year overall survival rate was 89.0%. No prognostic factors for overall survival were found. CONCLUSION: The analysis showed that patients who underwent subtotal surgical resection, particularly when the tumour had a Ki67 index greater than 2%, had an increased risk of local recurrence. These patients could benefit from adjuvant radiotherapy.

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Antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a condition characterized by vessel inflammation and may have a variety of etiologies. Among these, cocaine and its common adulterant, levamisole, have been described to contribute to the development of AAV with distinct cutaneous manifestations. Classically, these manifestations involve purpuric or necrotic lesions involving the ears, nose, and extremities. However, we present a case of cocaine-induced AAV presenting with violaceous nodules on the dorsal hands in order to demonstrate that this condition may not always present with retiform purpura and skin necrosis.

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As described throughout this book, different triggers can elicit a variety of different cytokine storm disorders that share overlapping clinical features (Fig. 31.1). Even within a particular cytokine storm disorder, multiple different triggers can elicit the syndrome. Like HLH, multicentric Castleman disease (MCD) serves as a great example of this as it can be caused by a viral infection, neoplastic cell population, or an unknown cause. Furthermore, the idiopathic subtype of MCD (iMCD) provides one of the first examples of a cytokine storm disorder that could be abrogated with targeted neutralization of a single cytokine when inhibition with the anti-interleukin-6 (IL-6) receptor monoclonal antibody tocilizumab was shown to effectively treat iMCD in the 1990s. Of course, this "iMCD treatment," tocilizumab, has been used in a variety of cytokine storm settings over the last 30+ years.

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BACKGROUND: Extreme oncoplasty is a breast-conserving operation using oncoplastic techniques in a patient who does not meet the traditional criteria for breast conservation and in whom most physicians would suggest a mastectomy. These tumors are generally multicentric and/or multifocal, they span more than 50 mm, or they can be large recurrences in a previously irradiated breast. METHODS: A prospective single institution database was queried from 2008 through mid 2023 for patients who met the criteria for extreme oncoplasty and were treated with excision plus whole-breast radiation therapy (WBRT) or mastectomy without WBRT. Patients with recurrent breast cancer were excluded. Endpoints were local, regional, and distant recurrence as well as overall and breast-cancer-specific survival. RESULTS: 272 patients were treated with oncoplastic mammaplasty, using a standard or split reduction excision followed by postoperative WBRT. An additional 101 patients elected to be treated with mastectomy without postoperative radiation therapy. With a median follow-up of 7 years, there were no significant differences in local, regional, or distant recurrence, nor in breast-cancer-specific survival or overall survival. CONCLUSIONS: We strongly support extreme oncoplasty plus WBRT as the default procedure of choice for patients with large multifocal/multicentric lesions amenable to reconstruction with volume displacement mammaplasty.