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Systemic light chain amyloidosis is a rare and severe disorder characterized by amyloid fibril deposition in various tissues, often leading to organ failure. Early diagnosis is crucial but challenging due to diverse clinical manifestations. Our case report presents a complex case of systemic light chain amyloidosis in a 62-year-old patient with cardiac, renal, neurological, and gastrointestinal involvement. The patient's treatment with cyclophosphamide, bortezomib, dexamethasone, and intravenous daratumumab yielded significant improvement, aligning with recent studies. Following treatment, the patient improved from stage IV to stage II systemic light chain amyloidosis per the National Comprehensive Cancer Network (NCCN) guidelines, indicating a more favorable prognosis. Hence, the successful integration of daratumumab in our case underscores its potential as a valuable addition to the treatment regimen for advanced systemic light chain amyloidosis, showcasing significant improvements across multiple organ systems.
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The vein of Rolando, also known as the central sulcal vein, is a critical superficial cerebral vein located in the central sulcus, playing a pivotal role in the venous drainage of the motor and sensory cortices. Named after the Italian anatomist Luigi Rolando, this vein serves as a crucial anatomical landmark in neurosurgery, guiding surgeons to approach critical brain structures with minimal damage. This article explores the anatomy and clinical significance of the vein of Rolando, emphasizing its role in neurosurgery and neuroimaging. Advanced imaging techniques such as functional MRI (fMRI), Magnetic Resonance Venography (MRV), and CT Angiography have enhanced the ability to diagnose and preserve this vein, reducing surgical risks. The article also discusses the interconnectedness of the vein of Rolando with other cerebral veins like the vein of Trolard and underscores the importance of understanding venous variations and drainage patterns for successful surgical outcomes. Preventive measures to protect the vein during neurosurgery are essential to prevent complications such as venous congestion and intracranial pressure. This overview highlights the necessity for precise anatomical knowledge and advanced diagnostic tools in optimizing neurosurgical procedures and patient care.
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Cerebral Veins , Neurosurgeons , Neurosurgical Procedures , Humans , Cerebral Veins/surgery , Cerebral Veins/anatomy & histology , Cerebral Veins/diagnostic imaging , Magnetic Resonance Imaging , Neurosurgical Procedures/methodsABSTRACT
INTRODUCTION: The recurrent artery of Heubner (RAH), also known as the medial striate artery, is the most clinically important perforator of the anterior cerebral artery. RAH aneurysm is relatively rare, with 11 cases found in the present literature review, but poses significant clinical challenges due to potential impact on cognitive and motor functions. This systematic review explored available case reports to comprehensively understand clinical presentation, diagnosis, management and outcome in RAH aneurysm. MATERIALS AND METHODS: Following PRISMA guidelines, this systematic review extensively explored RAH aneurysms, covering demographics, symptoms, diagnosis, treatments and outcomes. Comprehensive searches on PubMed, Scopus, Google Scholar, and Science Direct employed keywords such as "recurrent artery of Heubner aneurysm" and "Heubner's artery." RESULTS: After extensive screening, 9 qualifying studies were identified, with 11 patients diagnosed with rare RAH aneurysm. Median age was 55 years (standard deviation, 15.3 years), with 54.5% males. 45.5% of patients presented risk factors, including Moyamoya disease in 2 patients. The majority were classified as grade I/II on the Hunt and Hess (H&H) and World Federation of Neurological Societies (WFNS) systems. Aneurysms were predominantly located in the A1 segment, with a mean size of 4.7 mm. Treatments varied, with clipping being the most frequent (63.6%). The mortality rate was 18.2%. Clipping was associated with favorable outcomes but higher rates of infarction. CONCLUSION: This analysis highlighted the various symptoms, therapy methods and outcomes of RAH aneurysm, with A1 being the predominant origin. Future research should explore potential genetic predisposition factors and novel therapeutic interventions to address gaps in our knowledge.
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Central nervous system tumors are abnormal proliferations of neuronal cells within the brain and spinal cord. They can be primary or secondary and place a heavy financial, psychological, and physical burden on individuals. The highly selective blood-brain barrier, which only permits specific molecules to flow into the brain parenchyma, inhibits the efficacy of pharmacological medicines. Treatment options include surgery, chemoradiotherapy, and targeted therapy. Despite advances in therapy over the past few decades, the overall morbidity and mortality rates are still high, emphasizing the need for improved therapeutic choices to improve survival and quality of life further. Nano pharmaceuticals have demonstrated encouraging outcomes in in vivo trials using microscopic particles to enhance bioavailability and selectivity. The most successful clinical results to date have been achieved by liposomes, extracellular vesicles, and biomimetic nanoparticles; nevertheless, clinical trials are required to confirm their safety, efficacy, affordability, longterm impact, and success in patients from various demographics. Nano pharmaceuticals have the potential to change the paradigm of therapy for brain tumors, allowing better outcomes as primary and adjunctive therapy.
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Chronic myelomonocytic leukemia (CMML) presents as a complex hematologic malignancy with myelodysplastic and myeloproliferative features. Our case report explores the rare coexistence of CMML with immune thrombocytopenic purpura (ITP) in a 63-year-old female patient. CMML diagnosis followed World Health Organization criteria, and the patient was classified as having high-risk myelodysplastic syndrome (MDS)-CMML stage 2. Initial treatment with subcutaneous azacytidine for CMML proved partially effective, highlighting persistent severe thrombocytopenia. Subsequent investigations revealed secondary ITP associated with Crohn's disease. Conventional ITP therapies, including high-dose steroids and intravenous immunoglobulin, showed limited efficacy. Eltrombopag, a thrombopoietin receptor agonist, was initiated, resulting in the normalization of platelet counts within six weeks. Our case emphasizes the diagnostic challenges and intricate treatment landscape of CMML-associated ITP, suggesting eltrombopag as a potential therapeutic option in refractory cases. The study contributes to the evolving understanding of the complex interplay between myeloid disorders and immune-mediated hematological conditions, calling for personalized and multidisciplinary approaches to enhance patient outcomes.
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Proteus mirabilis is a Gram-negative bacterium with exclusive molecular and biological features. It is a versatile pathogen acclaimed for its distinct urease production, swarming behavior, and rapid multicellular activity. Clinically, P. mirabilis is a frequent pathogen of the human urinary system where it causes urinary tract infections (UTIs) and catheter-associated urinary tract infections (CAUTIs). This review explores the epidemiology, risk factors, clinical manifestations, and treatment of P. mirabilis infections, emphasizing its association with UTIs. The bacterium's genome analysis revealed the presence of resistance genes against commonly used antibiotics, an antibiotic-resistant phenotype that poses a serious clinical challenge. Particularly, the emergence of extended-spectrum ß-lactamases (ESBLs) and carbapenemases resistant P. mirabilis strains. On a molecular level, P. mirabilis possesses a wide array of virulence factors including the production of fimbriae, urease, hemolysins, metallophores, and biofilm formation. This review thoroughly tackles a substantial gap in understanding the role of metallophores in shaping the virulence factors of P. mirabilis virulence. Siderophores, iron metal chelating and transporting metallophores, particularly contribute to the complex pathogenic strategies, displaying a potential target for therapeutic intervention.
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INTRODUCTION: Anosognosia, a neurological condition, involves a lack of awareness of one's neurological or psychiatric deficits. Anton Syndrome (AS), an unusual form of anosognosia, manifests as bilateral vision loss coupled with denial of blindness. This systematic review delves into 64 studies encompassing 72 AS cases to explore demographics, clinical presentations, treatments, and outcomes. MATERIALS AND METHODS: The study rigorously followed PRISMA guidelines, screening PubMed, Google Scholar, and Scopus databases without timeframe limitations. Only English human studies providing full text were included. Data underwent thorough assessment, examining patient demographics, etiological variables, and treatment modalities. RESULTS: Sixty-four studies met the stringent inclusion criteria. Examining 72 AS cases showed a median age of 55 (6 to 96 years) with no gender preference. Hypertension (34.7 %) and visual anosognosia (90.3 %) were prevalent. Stroke (40.3 %) topped causes. Management included supportive (30.6 %) and causal approaches (30.6 %). Improvement was seen in 45.8 %, unchanged in 22.2 %, and deterioration in 11.1 %. Anticoagulation correlated with higher mortality (p < 0.05). DISCUSSION: AS, an unusual manifestation of blindness, stems mainly from occipital lobe damage, often due to cerebrovascular incidents. The syndrome shares features with Dide-Botcazo syndrome and dates back to Roman times. Its causes range from strokes to rare conditions like multiple sclerosis exacerbation. Accurate diagnosis involves considering clinical presentations and imaging studies, distinguishing AS from similar conditions. CONCLUSION: This comprehensive review sheds light on AS's complex landscape, emphasizing diverse etiologies, clinical features, and treatment options. Tailored treatments aligned with individual causes are crucial. The study's findings caution against blanket anticoagulation therapy, suggesting a nuanced approach. Further research is pivotal to refine diagnostics and optimize care for AS individuals.
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Agnosia , Blindness , Humans , Agnosia/etiology , Agnosia/diagnosis , Blindness/etiology , Adult , Aged , Middle Aged , Young Adult , Aged, 80 and over , Adolescent , Child , Female , Male , SyndromeABSTRACT
OBJECTIVES: Traumatic brain injury (TBI) represents a major health concern worldwide with a large impact in the Middle East and North Africa (MENA) region as a consequence of protracted wars and conflicts that adversely affect the general population. Currently, systematic TBI studies in the MENA region are lacking, nonetheless they are immensely needed to enhance trauma management and increase survival rates among TBI patients. This systematic review aims to characterize TBI in the MENA region to guide future policy choices and research efforts and inform tailored guidelines capable of improving TBI management and patient treatment and outcome. Furthermore, it will serve as a road map to evaluate and assess knowledge of trauma impact on regional health systems that can be adopted by health-care providers to raise awareness and improve trauma care. METHODS: We conducted a comprehensive search strategy of several databases including MEDLINE/Ovid, PubMed, Embase, Scopus, CINAHL, Google Scholar, and the grey literature in accordance with the PROSPERO systematic review protocol CRD42017058952. Abstracts were screened, and selected eligible studies were reviewed independently by 2 reviewers. We collected demographics information along with TBI characteristics, mortality rates, and regional distribution. Data were extracted using REDCap and checked for accuracy. RESULTS: The search strategy yielded 23,385 citations; 147 studies met the eligibility criteria and were included in this review. Motor vehicle accident (MVA) was the leading cause of TBI (41%) in the MENA region, followed by the military- (15.6%) and fall- (8.8%) related TBI. Males predominantly suffer from TBI-related injuries (85%), with a high prevalence of MVA- and military-related TBI injuries. The TBI mortality rate was 12.9%. The leading causes of mortality were MVA (68%), military (20.5%), and assault (2.9%). The vast majority of reported TBI severity was mild (63.1%) compared to moderate (10.7%) and severe TBI (20.2%). Patients mainly underwent a Glasgow Coma Scale assessment (22.1%), followed by computed tomography scan (8.9%) and surgery (4.1%). CONCLUSIONS: Despite its clinical, social, and economic burden, the evidence of TBI research in the MENA region is scarce. Further research and high-quality epidemiological studies are urgently needed to gain a deep understanding of the TBI burden in the region, facilitate the allocation of adequate resources, implement effective preventive and intervention strategies and advise on the TBI patient management as reflective on the TBI patterns and modes.