Search Results (91)

The management of end-stage kidney disease (ESKD) poses a substantial clinical and economic challenge, characterized by a growing patient burden, rising healthcare costs, and persistent unmet needs to enhance survival outcomes and quality of life. Background/Objectives: Conventional high-flux hemodialysis (HD) remains the dominant form of renal replacement therapy for ESKD but is still associated with substantial morbidity and mortality. High-volume post-dilution online hemodiafiltration (HVHDF) offers a promising alternative by enhancing the convective removal of uremic toxins. Methods: We conducted a narrative review of randomized controlled trials, meta-analyses, real-world cohort studies, and registry analyses published between 2010 and 2024. Evidence was categorized into short-term, medium-term, and long-term outcomes, including hemodynamic stability, inflammation, anemia, infection risk, cardiovascular events, cognitive decline, quality of life, and survival. Results: HVHDF improves short-term outcomes by enhancing toxin clearance, stabilizing blood pressure, reducing inflammation and oxidative stress, and improving anemia management. Medium-term benefits include improved nutritional status, reduced hospitalizations related to infections, and improved neurological and immune function. Long-term data from major trials (e.g., ESHOL, CONVINCE) and large real-world studies show consistent reductions in all-cause and cardiovascular mortality, particularly with convection volumes ≥ 23 L/session. A clear dose–response relationship supports the clinical relevance of convection volume targets. HVHDF has also shown benefits in preserving cognitive function and enhancing health-related quality of life. Conclusions: Strong and converging evidence supports HVHDF as a superior dialysis modality. Given its survival benefits, better tolerance, and broader impact on patient outcomes, HVHDF should be considered the new standard of care in dialysis, especially in light of the recent regulatory approval of the machine that provides the ability to perform HDF in the United States. Full article

Hemolytic uremic syndrome (HUS), a thrombotic microangiopathy primarily affecting the kidneys, can also involve the central nervous system (CNS), often leading to significant morbidity and mortality. Neurologic manifestations are among the most severe extra-renal complications, particularly in children and during outbreaks of Shiga toxin-producing Escherichia coli (STEC)-associated HUS (typical (tHUS)). This review explores the clinical spectrum, pathophysiology, diagnostic workup, and age-specific outcomes of neurologic involvement in both typical (tHUS) and atypical (aHUS). Neurologic complications occur in up to 11% of pediatric and over 40% of adult STEC-HUS cases in outbreak settings. Presentations include seizures, encephalopathy, focal deficits, movement disorders, and posterior reversible encephalopathy syndrome (PRES). Magnetic resonance imaging (MRI) commonly reveals basal ganglia or parieto-occipital lesions, though subtle or delayed findings may occur. Laboratory workup typically confirms microangiopathic hemolytic anemia (MAHA), thrombocytopenia, and kidney damage, with additional markers of inflammation or metabolic dysregulation. Eculizumab is the first-line treatment for aHUS with CNS involvement, while its utility in STEC-HUS remains uncertain. Although many children recover fully, those with early CNS involvement are at greater risk of developing epilepsy, cognitive delays, or fine motor deficits. Adults may experience lingering neurocognitive symptoms despite apparent clinical recovery. Differences in presentation and imaging findings between age groups emphasize the need for tailored diagnostic and therapeutic strategies. Comprehensive neurorehabilitation and long-term follow-up are crucial for identifying residual deficits. Continued research into predictive biomarkers, neuroprotective interventions, and standardized treatment protocols is needed for improving outcomes in HUS patients with neurological complications. Full article

Human parechoviruses, officially known as Parechovirus A (PeV-A), are more frequently reported as a significant cause of serious infections in newborns and young infants. We aimed to describe the clinical features and neurological outcomes of PeV-A encephalitis cases identified in Warsaw. Infants with suspected encephalitis were retrospectively identified in three hospitals in the summer of 2022. Cases of confirmed PeV-A infection had their comprehensive demographic, clinical, laboratory, imaging, and outcome data reviewed. The psychomotor development of the children up to the age of 2 years was assessed by using the standardized tools. We identified 18 cases of confirmed encephalitis with a PeV-A infection. Their median age was 16 days. Fourteen cases were included in the analysis, while one patient dropped out after the first visit. Most were boys (9/14), and one patient was born preterm. Three patients had white matter alterations on brain MRI at discharge. No significant neurologic sequelae were observed after acute illness. At the 24-month follow-up, based on the Bayley Scales of Infant and Toddler Development (BSID-IV) and the Brunet–Lézine Scale, the children showed no neurodevelopmental sequelae. Brain MRIs were obtained in all of the participants up to 12 months of age and revealed no significant lesions. Neurodevelopmental complications are not frequent in children after PeV-A encephalitis at 24 months of age. Continued follow-up in larger cohorts is needed to explore the predictors of long-term morbidity. Full article

Background: Cervical degenerative disease is a common condition associated with significant morbidity, often presenting as neck pain, radiculopathy, or myelopathy. Its growing incidence, particularly in the aging population, has led to an increased demand for surgical interventions aimed at relieving neural compression and restoring spinal stability. Objective: This study aims to evaluate surgical outcomes in patients with degenerative cervical conditions, with a particular focus on the role of intraoperative neurophysiological monitoring (IONM) in preventing adverse neurological events both immediately postoperatively and at long-term follow-up. Methods: A retrospective analysis was performed on patients who underwent cervical spine surgery for degenerative conditions between January 2021 and June 2024. Data collected included demographics, comorbidities, surgical details, and intraoperative neurophysiological monitoring. Surgical outcomes were assessed using the modified Rankin Scale (mRS), Odom’s Criteria, and the modified Japanese Association (mJOA) score. Results: Key findings demonstrated that advanced age and the presence of preoperative myelopathy were significantly associated with poorer postoperative outcomes across all evaluated measures. Conversely, factors such as gender, surgical approach, and the number of treated levels did not significantly influence recovery. Although intraoperative neurophysiological monitoring (IONM) did not show an immediate effect on postoperative outcomes, it was linked to prognostic value for long-term neurological status, suggesting a potential protective role in preserving neurological function. Conclusions: This study identifies age, preoperative functional status, and myelopathy as crucial predictors of postoperative recovery in cervical spine surgery for degenerative disease. These findings underscore the importance of early intervention in patients with myelopathy and highlight the complex role of IONM in improving long-term neurological outcomes. IONM changes may help identify patients at higher risk of poor recovery who could benefit from intensive postoperative rehabilitation. Further prospective studies are warranted to elucidate the complex interactions between patient characteristics and surgical factors in optimizing postoperative recovery. Full article

Background/Objectives: Aneurysmal subarachnoid hemorrhage (aSAH) remains a life-threatening cerebrovascular event with high rates of mortality and long-term morbidity. Among its complications, delayed cerebral ischemia (DCI) is a major contributor to poor clinical outcomes. Although cerebral vasospasm has traditionally been considered the primary mechanism underlying DCI, recent studies have revealed the multifactorial nature of this condition. This review aims to provide a comprehensive overview of the pathophysiology, preventive strategies, and current treatment options for DCI following aSAH. Methods: A narrative literature review was conducted using the PubMed database to identify peer-reviewed articles relevant to the prevention and treatment of DCI following aSAH. The search strategy employed the following terms: (“Subarachnoid Hemorrhage” [MeSH]) AND “Delayed Cerebral Ischemia” AND (“Prevention and Control” [Subheading] OR “Secondary Prevention” [MeSH]). This search strategy was designed to capture studies addressing both pharmacological and non-pharmacological preventive measures for DCI. Results: A comprehensive PubMed search identified a total of 113 relevant articles. Among these, 40 publications primarily addressed pharmacological interventions, while 22 focused on neuromonitoring techniques. An additional 20 articles explored the pathophysiological mechanisms underlying DCI, and 15 involved preclinical studies utilizing animal models. The remaining 16 articles encompassed diverse topics, including prophylactic endovascular therapies, newly proposed definitions of DCI, treatment algorithm development, functional outcome analyses, and entries in clinical trial registries. Emerging evidence highlights that vasospasm alone does not account for all cases of DCI. Pharmacological approaches such as nimodipine, clazosentan, and fasudil have shown varying degrees of efficacy. Circulatory management and removal of subarachnoid hematoma via CSF drainage or thrombolytics may reduce DCI risk, although their impact on long-term neurological outcomes remains controversial. Endovascular therapy and adjunctive agents such as cilostazol or anticoagulants have demonstrated potential but require further validation through large-scale trials. Conclusions: Effective DCI prevention and treatment require a multimodal approach targeting diverse pathological mechanisms beyond vasospasm. Improved risk stratification, early detection, and individualized therapy are essential for advancing the management of patients with aSAH. Full article

Proventricular dilatation disease (PDD) is a neurologic syndrome of birds caused by the infectious agent Psittacine Bornavirus (PaBV). Clinical disease may be based on the T-cell-mediated immune response to PaBV within the central and peripheral nervous system, similar to Borna disease virus, a closely related mammalian virus. Lymphoplasmacytic infiltrations may occur in ganglia, nerve plexuses, peripheral nerves and the central nervous system of the infected bird. Clinical disease may result in multiple neurologic disorders and life-threatening morbidity. Treatment of PDD with antivirals and non-steroidal anti-inflammatories has thus far been non-curative and unsuccessful long-term. Cyclosporine is an immunosuppressant drug that decreases cell-mediated immune responses by inhibiting T-cell proliferation and decreasing cytokine production. In avian species, cyclosporine is a potent immunosuppressant with T-cell-specific action. A pilot study performed in PaBV-infected cockatiels showed increased weight gain and a lack of morbidity or mortality following experimental PaBV infection and cyclosporine treatment at 10 mg/kg orally every 12 h. In this case series of six psittacine birds affected by PDD, cyclosporine at this dose alleviated or reduced clinical signs in multiple birds without severe sequelae. Itraconazole was used concurrently in these cases to prevent secondary fungal infections during immunosuppression but may have had a synergetic effect when used in combination with cyclosporine. Further prospective research is indicated to better evaluate cyclosporine use in birds with PDD. However, these preliminary clinical findings suggest that cyclosporine and itraconazole administration is a treatment option for palliation of PDD in psittacine patients, especially those refractory to other treatments. Full article

Background. Polytrauma is one of the three leading causes of mortality worldwide and a major contributor to psychosocial morbidity and disability. Concomitant traumatic brain injuries further increase this risk. Methods. We present the case of a 6-year-old child who sustained significant trauma in a road accident, resulting in injuries to multiple anatomical regions, including the central nervous system. Results. Two years after the accident, the child experiences brief episodes of decreased visual acuity, dizziness, nausea, pallor, and headaches, along with occasional migraines that resolve spontaneously. Additionally, the child struggles with school adjustment. Brain injuries associated with polytrauma are crucial prognostic factors in both the short and long term, influenced by the brain’s reactivity and recovery capacity. There is also an increased risk of developing syncopal episodes, seizures, and other neurological manifestations. Conclusions. The direct and secondary effects of the trauma have contributed to psychosocial disabilities, necessitating complex medical care and family-centered interventions. Full article

Mild traumatic brain injury (mTBI) is a leading cause of morbidity in children with both short- and long-term neurological, cognitive, cerebrovascular, and emotional deficits. These deficits have been attributed to ongoing pathophysiological cascades that occur acutely and persist post-injury. Given our limited understanding of the transcriptional changes associated with these pathophysiological cascades, we studied formalin-fixed paraffin-embedded (FFPE) tissues from the frontal cortex (FC) and the hippocampus + amygdala (H&A) regions of swine (N = 40) after a sagittal rapid non-impact head rotation (RNR). We then sequenced RNA to define transcriptional changes at 1 day and 1 week after injury and investigated the protective influence of cyclosporine A (CsA) treatment. Differentially expressed genes (DEGs) were classified into five temporal patterns (Early, Transient, Persistent, Intensified, Delayed, or Late). DEGs were more abundant at 1 week than 1 day. Shared significant gene ontology annotations in both regions included terms associated with neuronal distress at 1 day and neurorecovery at 1 week. CsA (20 mg/kg/day) infused for 1 day (beginning at 6 h after injury) accelerated 466 DEGs in the FC and 2794 DEGs in the H&A, such that the CsA-treated transcriptional profile was associated with neurorecovery. Overall, our data reveal the effects of anatomic region and elapsed time on gene expression post-mTBI and motivate future studies of CsA treatment. Full article

Background: Thoracic schwannomas are benign nerve sheath tumors that can cause neurological and respiratory symptoms depending on their location and extension. The optimal surgical approach remains debated, particularly regarding resection extent, complication rates, and postoperative morbidity. Methods: This retrospective multicenter study analyzed 106 patients treated between 2011 and 2024, classifying tumors according to the Eden system and comparing surgical strategies. Surgical variables, including operative time, blood loss, resection extent, recurrence rates, and complications classified by Clavien–Dindo, were analyzed. Results: Eden I and II schwannomas were treated with laminectomy (LCT) or hemilaminectomy (HLCT) and transpedicular approaches (TPD), achieving high gross total resection (GTR) rates with minimal complications. Eden III dumbbell tumors benefited from a combined neurosurgical–thoracic approach (LCT + VATS), which resulted in higher GTR rates (100% vs. 62%, p < 0.01) and lower dural complications compared to neurosurgical resection alone. Eden IV extraforaminal schwannomas were best managed with VATS, which was associated with lower intraoperative blood loss (p = 0.018), shorter surgical duration (p = 0.027), and reduced postoperative complications compared to open thoracotomy. Our findings confirm that minimally invasive techniques, particularly VATS and combined neurosurgical–thoracic approaches, optimize tumor resection while reducing morbidity. However, feasibility depends on institutional resources and multidisciplinary collaboration. Conclusions: This study provides a stratified comparison of surgical approaches tailored to Eden classification, aiming to identify the most effective and least morbid strategies for each lesion type. Future prospective studies should validate these findings, integrating preoperative functional assessments and long-term follow-up to better stratify surgical risk, personalize operative planning, and refine surgical decision making for thoracic schwannomas. Full article

Background: Squamoid eccrine ductal carcinoma (SEDC) is an exceedingly rare and aggressive cutaneous adnexal malignancy, with fewer than 100 reported cases. Its histopathologic overlap with squamous cell carcinoma (SCC) frequently leads to misdiagnosis, delaying appropriate management. Unlike SCC, SEDC exhibits biphasic differentiation, deep infiltration, and a high rate of perineural invasion, contributing to significant morbidity and poor long-term outcomes. Given the absence of standardized treatment protocols, managing SEDC remains a challenge. Case Presentation: We report an unusual case of an 80-year-old female presenting with progressive numbness, nasal deviation, and a subcutaneous indurated lesion in the left nasofacial region. The early neurological symptoms were an atypical feature, suggesting perineural invasion (PNI) before visible tumor progression. Initial histopathologic evaluation was inconclusive, raising suspicion of SCC, necessitating immunohistochemical analysis, which confirmed ductal differentiation, leading to the final diagnosis of SEDC. The patient underwent radical resection with intraoperative margin assessment (Mohs micrographic surgery; MMS) followed by adjuvant radiotherapy (62 Gy/31 fractions) due to high-risk features, including perineural and perivascular invasion. Despite initial disease control, a local recurrence involving the left orbit and nasal bone occurred 20 months postoperatively, demonstrating the aggressive nature of SEDC despite clear surgical margins and adjuvant therapy. Due to disease progression and refusal of further surgery, only palliative care was provided. During follow-up, the patient contracted COVID-19, further complicating her clinical status and contributing to her demise. While COVID-19 was not directly linked to SEDC progression, its impact on patient management was significant. Conclusions: This case underscores the diagnostic and therapeutic challenges of SEDC, emphasizing the need for early suspicion, extensive histopathologic assessment, and aggressive multimodal treatment. The importance of multidisciplinary management—particularly in elderly and immunocompromised patients—and long-term surveillance due to high recurrence risk and PNI is crucial. Full article

Introduction: This study presents long-term data of pediatric AIS patients with a favorable initial clinical presentation who were followed by a tertiary pediatric neurology clinic with a well-organized stroke team. Method: Patients who were diagnosed with AIS at pediatric age (28 days–18 years) and followed for at least 5 years by the same clinic were included in this study. The clinical and demographical characteristics of the patients were retrospectively collected from their medical records. At their last visit, the modified Rankin scale (mRS) and Pediatric Stroke Outcome Measure Short Neuro Exam (PSOM-SNE) were administered, and a neurological examination was performed. Results: A total of 32 patients (20 of whom were male, 62.5%) were included in this study. Their mean age at the time of the study was 162.62 ± 64.4 (62–300) months. The mean age at first ischemic stroke was 77.39 ± 61.93 (0.5–180) months, and the mean follow-up duration was 85.44 ± 20.52 (60–121) months. Seventeen patients (53.3%) reported normal daily functions at the last visit. A younger presentation age (≤60 months) was related to a longer hospital admission duration (24 h vs. 9 h) and worse long-term functional outcomes (p = 0.023). The affected vascular territory did not have any significant effect on long-term clinical outcomes (p = 0.550). Anticoagulant treatment alone was consistent with a worse prognosis compared to antithrombotic treatment alone or the combination of both (p = 0.026). PSOM-SNE scores were helpful in detecting some mild cognitive and language dysfunctions in patients with favorable mRS scores and subtle neurological sequelae. Conclusions: Pediatric AIS with a mild presentation has some degree of long-term morbidity, even when handled at well-organized stroke centers. A younger presentation age has the highest risk of long-term neurological sequelae. Full article

Background: Subarachnoid hemorrhage (SAH) is a severe neurological condition with high mortality and morbidity rates, often exacerbated by secondary complications such as inflammation, cerebral vasospasm, and apoptosis. Proinflammatory cytokines, including interleukin-1 (IL-1), tumor necrosis factor-alpha (TNF-α), and interleukin-6 (IL-6), play critical roles in these pathological processes. Anakinra, an IL-1 receptor antagonist, has demonstrated significant anti-inflammatory effects in various disease models. This study aimed to evaluate the efficacy of anakinra in mitigating inflammation, vasospasm, and apoptosis in an experimental rat model of SAH. Methods: Thirty-two male Sprague Dawley rats were divided into four groups: Control (healthy), SAH (no treatment), Saline (0.2 mL saline subcutaneously), and Anakinra (50 mg/kg subcutaneously, twice daily). Proinflammatory markers (CRP, TNF-α, IL-1, IL-6, and fibrinogen) were measured in serum and cerebrospinal fluid (CSF) at 3, 7, and 10 days post-SAH. Basilar artery diameter was evaluated histopathologically, and Caspase-3 expression was assessed immunohistochemically to determine apoptotic activity. Results: SAH significantly increased levels of CRP, TNF-α, IL-1, IL-6, and fibrinogen in both serum and CSF, reduced basilar artery diameter, and elevated Caspase-3 expression compared to the Control group. Saline treatment provided limited improvements, with inflammatory markers and histopathological parameters remaining elevated. Anakinra treatment significantly reduced inflammatory markers, restored basilar artery diameter, and lowered Caspase-3 expression, highlighting its efficacy in mitigating inflammation, vasospasm, and apoptosis. Conclusions: Anakinra effectively suppresses inflammation, alleviates cerebral vasospasm, and inhibits apoptosis in an experimental model of SAH. These findings suggest its potential as a therapeutic agent for managing SAH and its complications. Further research is needed to explore its clinical applicability and long-term effects. Full article

Introduction: Traumatic brain injury (TBI) in pediatric population is responsible for significant mortality and morbidity, particularly among children aged 0–4 and young adults aged 15–24. The developing brain’s unique characteristics may increase vulnerability to injuries, potentially leading to long-term cognitive and motor deficits. Current therapeutic options for neuronal regeneration post-TBI are limited, although neurotrophins, especially nerve growth factor (NGF), show promise in enhancing recovery. NGF can mitigate excitotoxicity and promote neuroprotection, particularly by intranasal administration, which is attractive because of its non-invasive nature. Case Presentation: A three-year-old boy suffered from severe TBI due to a car accident, leading to multiple complications, including a basilar skull fracture and cerebral venous sinus thrombosis. Initial assessments revealed significant neurological impairments. After intensive care and rehabilitation, the child exhibited gradual improvements in consciousness and motor functions but continued to face challenges, particularly with left-sided hemiparesis. Nine months post-injury, he began intranasal administration of human-recombinant NGF (hr-NGF) as part of a clinical trial. Discussion: Following hr-NGF treatment, the child demonstrated notable advancements in motor function, achieving independent standing and walking. Cognitive assessments indicated improvements in various domains, including verbal comprehension and executive functioning. EEG results showed reduced epileptiform activity. These findings suggest that hr-NGF may facilitate recovery in pediatric TBI cases by enhancing both motor and cognitive outcomes. Conclusions: This case highlights the potential role of intranasal hr-NGF administration as a therapeutic strategy for improving neurological recovery in children with severe TBI. The positive clinical outcomes support further exploration of NGF as a viable treatment option to mitigate long-term sequelae associated with pediatric brain injuries. Full article

Background/Objectives: Surgery for adolescent idiopathic deformities is often aimed at improving aesthetic appearance, striving for the best possible correction. However, severe and rigid scoliotic curves not only present aesthetic issues but can also compromise cardiopulmonary health and cause early neurological impairment due to spinal cord compression, posing significant risks of morbidity and mortality if untreated. Conservative treatments are ineffective for severe curves, defined by scoliotic angles over 70° and flexibility below 30% on lateral bending X-rays. Treatment often requires invasive interventions, such as osteotomies and vertebral resections. In particular, posterior vertebral column resection (PVCR) has shown effectiveness in realigning vertebral structures in complex cases. This study describes the efficacy and risks of PVCR through a series of cases treated at our institution. Methods: This case series was conducted at the Rizzoli Orthopedic Institute in Bologna, involving eight pediatric patients with severe, rigid spinal deformities, operated upon between 2018 and 2023. The underlying pathologies included idiopathic kyphoscoliosis, neurofibromatosis type 1, Pott’s disease, and other congenital anomalies. Preoperative assessment included standard radiographs, magnetic resonance imaging, and computed tomography. During PVCR, motor and sensory evoked potentials were monitored to minimize neurological injury risk. Postoperative management included blood transfusions, antibiotic support, and early physiotherapy. Results: PVCR resulted in an average reduction in the Cobb angle from 86.3° preoperatively to 22.4° postoperatively, with a mean correction of 64%. The mean duration of the procedures was 337.4 min. Three patients had an uneventful postoperative course, while five developed complications, including infections and temporary neurological deficits, which were successfully managed. One patient developed an epidural hemorrhage that required emergency surgery for hematoma evacuation, with partial recovery. This study demonstrates the potential of PVCR for correcting rigid spinal deformities, highlighting the importance of postoperative management to minimize the associated risks. Conclusions: Posterior vertebral resection techniques offer significant promise in the correction of pediatric spinal deformities. Although ours is a small case series, it can provide important data for such treatment. Long-term monitoring is needed to fully understand the impact of these procedures and to further refine surgical techniques. Full article

Neonatal intraventricular hemorrhage is a serious condition associated with significant acute and long-term morbidity and mortality. Neurosurgical intervention aims to relieve life-threatening raised intracranial pressure and prevent neurological deterioration. In recent years, advancements in disease understanding have paved the way for clinicians to re-evaluate conventional approaches in the management of affected patients. Examples include various neurosurgical techniques to actively reduce blood products with a view to avoid the consequences of complex hydrocephalus and intraparenchymal injury in the developing brain. In this entry paper, we aim to provide an overview of the current perspectives, pathophysiology and management strategies for this difficult condition. Full article