Cervical Diastematomyelia: A Case Presentation and Systematic Review.

Diastematomyelia is a rare congenital disorder characterized by the separation of the spinal cord by an osseocartilaginous or fibrous septum. While diastematomyelia has been reported to be more common in the thoracic and lumbar regions, the true incidence of cervical diastematomyelia is currently unknown. In this study, we conducted the most comprehensive systematic review to date of all other case reports of diastematomyelia to better characterize the incidence of cervical diastematomyelia and provide comprehensive statistics on the clinical characteristics of diastematomyelia generally. Ninety-one articles were included in our study, which comprised 252 males (27.9%) and 651 females (72.0%) (and one patient with unspecified gender). In 507 cases, the vertebral level of the diastematomyelia was described, and we recorded those levels as either cervical ( n = 8, 1.6%), thoracic ( n = 220, 43.4%), lumbar ( n = 277, 54.6%), or sacral ( n = 2, 0.4%). In 719 cases, the type of diastematomyelia was specified as either Type I ( n = 482, 67.0%) or Type II ( n = 237, 33.0%). Our study found that diastematomyelia has been reported in the cervical region in only 1.6% of cases, and we provide comprehensive data that this disorder occurs in female-to-male ratio of approximately 2.6:1 and Type I versus Type II diastematomyelia in an estimated ratio of 2:1.

Bevacizumab and gamma knife radiosurgery for first-recurrence glioblastoma.

INTRODUCTION: Glioblastoma (GBM) is the most common central nervous system malignancy in adults. Despite decades of developments in surgical management, radiation treatment, chemotherapy, and tumor treating field therapy, GBM remains an ultimately fatal disease. There is currently no definitive standard of care for patients with recurrent glioblastoma (rGBM) following failure of initial management. OBJECTIVE: In this retrospective cohort study, we set out to examine the relative effects of bevacizumab and Gamma Knife radiosurgery on progression-free survival (PFS) and overall survival (OS) in patients with GBM at first-recurrence. METHODS: We conducted a retrospective review of all patients with rGBM who underwent treatment with bevacizumab and/or Gamma Knife radiosurgery at Roswell Park Comprehensive Cancer Center between 2012 and 2022. Mean PFS and OS were determined for each of our three treatment groups: Bevacizumab Only, Bevacizumab Plus Gamma Knife, and Gamma Knife Only. RESULTS: Patients in the combined treatment group demonstrated longer post-recurrence median PFS (7.7 months) and median OS (11.5 months) compared to glioblastoma patients previously reported in the literature, and showed improvements in total PFS (p=0.015), total OS (p=0.0050), post-recurrence PFS (p=0.018), and post-recurrence OS (p=0.0082) compared to patients who received either bevacizumab or Gamma Knife as monotherapy. CONCLUSION: This study demonstrates that the combined use of bevacizumab with concurrent stereotactic radiosurgery can have improve survival in patients with rGBM.

Postoperative Morbidity and Mortality in Lumbar Spine Surgery Patients With Chronic Kidney Disease and Chronic Steroid Use.

BACKGROUND: Perioperative steroids have traditionally been administered during lumbar spine surgery in order to decrease local inflammation and prevent scar tissue formation, which can otherwise contribute to significant, long-lasting postoperative pain due to the formation of epidural fibrosis around lumbar nerve roots. However, the use of steroids in lumbar spine patients has raised concerns of postoperative wound complications caused by corticosteroid-induced immunomodulatory effects and changes in collagen synthesis. Patients with chronic kidney disease (CKD) undergoing spine surgery are at a particularly elevated risk of various complications due to chronic CKD-related systemic inflammation and endothelial dysfunction. It is currently uncertain whether chronic steroid use in CKD patients exerts a protective effect postoperatively due to decreased systemic inflammation or instead is correlated with increased rates of wound complications. RESULTS: Using adjusted odds ratios to control for CKD-related comorbidities, our study of lumbar spine fusion patients who were chronic steroid users vs nonusers found no significant differences in rates of postoperative wound infections in later stage CKD patients. However, we also did not observe statistically significant reductions in hospital length of stay or rates of 30-day mortality, sepsis, or cardiac, pulmonary, and renal events. CONCLUSIONS: Our results indicate chronic steroid use neither contributes significantly to rates of wound infections nor exerts a protective effect against postoperative inflammatory complications in lumbar spine patients with CKD. CLINICAL RELEVANCE: Our findings do not support the practice of holding steroids in chronic users prior to lumbar spine surgery. Perioperative steroids do not appear to increase the risk of postoperative complications, but neither do they improve lumbar spine patient outcomes.

Cervical spine flexion-extension radiography versus magnetic resonance imaging in pediatric patients following blunt traumatic injury.

Background: In pediatric trauma patients, 60-80% of spinal cord injuries involve the cervical vertebrae. While the American College of Radiology offers guidelines for best imaging practices in the setting of acute pediatric trauma, there is a lack of uniformity in imaging-decision protocols across institutions. MRI has been shown to demonstrate high sensitivity for both bony and ligamentous injuries while also avoiding unnecessary radiation exposure in the pediatric patient population. However, the efficacy of flexion-extension (FE) radiography following initial MRI has not been evaluated in children. Our hypothesis is that FE radiography conducted following an initial MRI does not contribute significant diagnostic information or reduce time to cervical collar removal and thus can be removed from institutional protocols in order to avoid unnecessary testing and reduce pediatric radiation exposure. Methods: Trauma data were collected for pediatric patients presenting with suspected acute cervical spine injury from 2014 to 2021. A total of 108 patients were subdivided into 41 patients who received "MRI Only" and 67 patients who received both "MRI and FE" diagnostic cervical spine imaging. Chi-square testing and t-tests were performed to determine differences between MRI and FE radiographic detection rates of bony and ligamentous injuries in the subgroups. Results: In patients for whom FE did not find any injury, MRI detected bony and ligamentous injuries in 9/63 and 12/65 cases, respectively. In 3/21 (14.3%) cases in which MRI detected a bony and/or ligamentous injury and FE did not, patients eventually required surgical intervention for c-spine stabilization. No patients required surgical fixation when FE radiography showed an abnormality and MRI was normal. Addition of follow-up FE radiography after initial MRI did not have a significant effect on overall hospital length of stay (MRI Only vs MRI+FE: 9.2±12.0 days vs 8.6±13.5 days, p=0.816) or on rates of collar removal at discharge or greater than 48 hours after imaging (MRI Only vs MRI+FE: 41.5% vs 56.7%, p=0.124). Conclusions: FE radiography following initial MRI did not have a significant effect on reducing time to cervical collar removal or overall hospital length of stay. In addition, in 3 of 6 cases (50.0%) in which surgical fixation was required, MRI detected ligamentous and/or bony injury while FE radiography was normal. Level of Evidence: This study contributes Level 2b scientific evidence consistent with a well-designed cohort or case-control analytic study.

Infantile-onset Pompe disease with neutropenia: Treatment decisions in the face of a unique phenotype.

Infantile-onset Pompe disease manifests with early signs of cardiomyopathy during the first few days to weeks of life. We present the case of a newborn born via emergency cesarean section with atrial flutter and moderate biventricular hypertrophy who was diagnosed with Pompe disease on New York State newborn screen. Diagnosis was confirmed with repeat leukocyte acid alpha-glucosidase (GAA) enzyme activity, GAA gene sequencing, urine Hex4, and evaluation of Cross-Reactive Immunological Material (CRIM) status. The patient was also found to be persistently neutropenic which to our knowledge has not been previously reported in the literature in association with Pompe disease. This report highlights the impact that newborn screening had on time to diagnosis and initiation of treatment with enzyme replacement therapy. We also discuss how our patient's concurrent neutropenia impacted decision making related to immune tolerance induction prior to starting enzyme replacement therapy.

Pan-Cancer Characterization of Intratumoral Autonomic Innervation in 32 Cancer Types in the Cancer Genome Atlas.

Over the past two decades, multiple studies have demonstrated the important role that the autonomic nervous system (ANS) plays in tumorigenesis and cancer progression. However, the mechanisms by which this process occurs have only recently begun to be elucidated. Further, the extent of autonomic innervation in various cancer types and its effects on tumor molecular, immunological, and histopathological features, as well as on patient outcomes, are not yet fully characterized. In this study, we analyzed intratumoral ANS gene expression signatures, including overall intratumoral neuron growth and sympathetic and parasympathetic markers, across 32 cancer types using tumor transcriptomic and clinical annotation data available from The Cancer Genome Atlas (TCGA). Our analysis revealed wide variations in intratumoral ANS expression both within and across cancer types. The association of ANS signatures with tumor histopathological characteristics and survival outcomes also varied by cancer type. We found intratumoral ANS expression to be commonly correlated with angiogenesis, TGF-ß signaling, and immunosuppression in the tumor microenvironment of many cancer types, which provide mechanistic insights into the involvement of intratumoral innervation in cancer development and progression. Our findings suggest that the potential benefits of cancer therapies targeting ß-adrenergic receptor-mediated stress signaling pathways are likely dependent on cancer type.

Cerebral Proliferative Angiopathy Presenting as Subdural Hematoma: A Case Report and Systematic Literature Review.

Cerebral proliferative angiopathy (CPA) is a rare cerebrovascular pathology that presents with unique clinical features due to distinct histologic, angiographic, and pathophysiologic characteristics that separate it from classical arteriovenous malformation. The disorder is characterized by uncontrolled angiogenesis in which functional brain parenchyma is interspersed with abnormal vascular channels without a distinct nidus. Common presenting symptoms include headache, seizures, and stroke-like symptoms. Hemorrhagic presentations are rare in contrast to the typical presentations of classical arteriovenous malformation. Here, we report a young woman with a history of a suspected connective tissue disorder who presented to the emergency department with worsening headaches in a delayed fashion after experiencing minor head trauma and was found to have a left-sided subdural hematoma. Angiography confirmed a diagnosis of CPA after abnormal cortical vasculature was noted during the patient's craniotomy. A systematic review of CPA cases described in the literature was performed using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, with the findings discussed.

Macroglossia Associated With Clobazam Administration: A Case Report and Literature Review.

Clobazam is a benzodiazepine derivative used as an antiepileptic agent for the treatment of focal and generalized seizures and drug-resistant epilepsy associated with Lennox-Gastaut Syndrome. While somnolence and mood-related side effects are commonly observed, acute macroglossia following initiation of Clobazam therapy has not been previously reported in the medical literature. In this case report, we present a female pediatric patient who developed significant tongue swelling with protrusion beyond the oral cavity after initiation of Clobazam for treatment-resistant epilepsy. Symptoms were unresponsive to antihistamines and steroids but resolved gradually in the days following discontinuation of Clobazam with no lingering sequelae.

Acute-Onset Psychosis Following Prolonged Hospitalization for COVID-19 Pneumonia.

BACKGROUND SARS-CoV-2 infection presents with a variety of clinical manifestations, from asymptomatic courses to prolonged hospitalizations with severe systemic inflammatory responses and multiorgan failure. One particular sequela of the disease that has gained wider attention over the past year is the sudden onset of neuropsychiatric symptoms in the weeks following recovery from COVID-19 pneumonia. While the pathophysiology for the development of this condition is uncertain, symptoms ranging from mild confusion and anxiety to florid psychosis with manic delusions and auditory and visual hallucinations have been rarely, but increasingly, reported in the literature. The acute development of such symptoms in the post-recovery period can be devastating for patients, their caregivers, and clinicians who may be unaware of effective management options. CASE REPORT In this case report, we present a 23-year old man who developed psychotic symptoms, including acute mania, delusions of grandeur, and auditory and visual hallucinations, 1 week following an extended hospitalization for COVID-19 pneumonia. The patient was admitted to our psychiatric unit and treated with a combination of antipsychotic and mood stabilizer medications. After 2 weeks of treatment, the patient's psychotic and mood-related symptoms resolved, with normal mental status maintained at last follow-up 1 month following discharge from our unit. CONCLUSIONS The acute development of neuropsychiatric symptoms is a rare but increasingly recognized sequela of COVID-19. Despite the severity of initial presentation, patients can be successfully treated with short courses of typical antipsychotic medications with complete return to baseline, unimpaired functioning, and no lingering psychiatric sequela.

Levetiracetam-induced aggression and acute behavioral changes: A case report and literature review.

Levetiracetam is a second-generation antiepileptic medication used to treat a wide range of partial and generalized seizure disorders. While Levetiracetam is generally well-tolerated, mild mood-related side effects (e.g., anxiety, agitation, and depression) have been observed in a minority of patients in the days following initiation of therapy or changes in dosing. The development of acute aggression requiring termination of Levetiracetam therapy has been rarely reported in the medical literature but poses a limiting effect on treatment options for refractory epilepsy in pediatric patients. In this report, we present a teenage male patient with a history of seizure disorder who developed sudden, severe behavioral abnormalities and aggression following increases in his Levetiracetam dosing. His symptoms resolved rapidly after return of his medication dosing to baseline, with no further sequelae noted. Our observations suggest that Levetiracetam remains a safe and effective first-line antiepileptic whose adverse behavioral side effect profile can be properly managed with close patient monitoring and dose titration.

Treatment of Morgellons disease with doxycycline.

Morgellons disease (MD) is a rare dermatopathy characterized by nonspecific symptoms and the production of multicolored fibers and granular tissue from diffuse skin ulcerations which are described as being either pruritic or painful. The etiology of MD is currently unknown; previous studies have suggested both psychiatric and infectious causes, with increasing interest over the previous decade in elaborating a possible pathogenesis for the disease secondary to infection by Borrelia species. We report a middle-aged Caucasian female who developed symptoms of MD in the days following exposure to a tick bite after spending an afternoon hiking through a wooded area. She was subsequently treated with a course of Doxycycline and found on two-week follow-up to have complete remission of her symptoms. This case report further supports the theory for an infectious etiology of MD and encourages future studies into its pathophysiology.

Radial first or patient first: a case series and meta-analysis of transradial versus transfemoral access for acute ischemic stroke intervention.

BACKGROUND: Few studies have compared technical success and effectiveness of transradial access (TRA) versus transfemoral access (TFA) for mechanical thrombectomy (MT) for acute ischemic stroke (AIS). We compared the two approaches for technical success, effectiveness, and outcomes. METHODS: We retrospectively compared TRA with TFA for AIS MT at our institute. We additionally performed a systematic review and meta-analysis of studies describing the use of TRA alone or in comparison with TFA for MT. Primary outcomes included rate of successful reperfusion (thrombolysis in cerebral infarction (TICI) >2b), number of passes, access-site complications, and 3- month mortality and favorable functional outcomes (modified Rankin Scale (mRS) score 0-2). RESULTS: A total of 222 consecutive patients (TRA=93, TFA=129) were included in our case series. The rate of successful reperfusion was significantly higher for the TFA cohort (91.4% vs 79.6%, P=0.01) with lower mean number of passes (1.8±1.2 vs 2.4±1.6, P=0.014). Three-month mortality in the TFA group was lower (22.1% vs 40.9% for the TRA cohort (P=0.004), with a higher rate of favorable functional outcomes (51.3% vs 34.1%, P=0.015). A meta-analysis of 10 studies showed significant heterogeneity in rates of successful reperfusion (57.1% to 95.6%, heterogeneity=67.55%, P=0.001). None of the previous comparative studies reported 3-month mortality and functional outcomes. CONCLUSIONS: This case series demonstrate a higher successful reperfusion rate, fewer passes, lower 3-month mortality, and improved 3-month functional outcomes with TFA. The systematic review highlights the inadequacy of existing evidence. Prospective comparative studies are needed before a 'radial-first' approach can be adopted for stroke intervention.

Optimal experimental design for predator-prey functional response experiments.

Functional response models are important in understanding predator-prey interactions. The development of functional response methodology has progressed from mechanistic models to more statistically motivated models that can account for variance and the over-dispersion commonly seen in the datasets collected from functional response experiments. However, little information seems to be available for those wishing to prepare optimal parameter estimation designs for functional response experiments. It is worth noting that optimally designed experiments may require smaller sample sizes to achieve the same statistical outcomes as non-optimally designed experiments. In this paper, we develop a model-based approach to optimal experimental design for functional response experiments in the presence of parameter uncertainty (also known as a robust optimal design approach). Further, we develop and compare new utility functions which better focus on the statistical efficiency of the designs; these utilities are generally applicable for robust optimal design in other applications (not just in functional response). The methods are illustrated using a beta-binomial functional response model for two published datasets: an experiment involving the freshwater predator Notonecta glauca (an aquatic insect) preying on Asellus aquaticus (a small crustacean), and another experiment involving a ladybird beetle (Propylea quatuordecimpunctata L.) preying on the black bean aphid (Aphis fabae Scopoli). As a by-product, we also derive necessary quantities to perform optimal design for beta-binomial regression models, which may be useful in other applications.