Demons syndrome with pericardial effusion followed by intensive care unit-acquired weakness: A case report and literature review

Demons syndrome is defined by hydrothorax and ascites associated with a benign genital tumor that resolves after resection of the tumor. However, Demons syndrome with pericardial effusion has never been reported. Intensive care unit–acquired weakness is a neurological sequela to sepsis/systemic inflammatory response syndrome, or multi-organ failure. A 47-year-old, nulligravid, Japanese woman, was transferred to our hospital for refractory heart failure and a ruptured ovarian tumor. She had an 11-cm left ovarian tumor with ascites, hydrothorax, and pericardial effusion; she was intubated for pulmonary hypertension and admitted to the intensive care unit for septic shock. Four days later, a left salpingo-oophorectomy was performed for Demons syndrome with pericardial effusion. The histological diagnosis indicated a serous cystadenoma with fibrotic changes. Following surgery, ventilator weaning was delayed due to intensive care unit–acquired weakness. The association between Demons syndrome and pericardial effusion should be recognized to ensure early treatment and for preventing sequalae from the disease.

Neurological Sequela of Acute Pesticide Poisoning Among Adults in Central Taiwan

Background and Purpose: Cases of acute pesticide poisoning account for significant morbidity and mortality in developing countries; however, its burden in Taiwan remains unknown. The study examined acute pesticide poisoning (APP) involving adults in the central region of Taiwan, which is a mainly agricultural sub-urban area.Methods: The retrospective study evaluated the outcome and neurological sequelae of patients with APP in a Taiwanese cohort between April 2002 and February 2019. The pesticides were classified according to the Insecticide Resistance Action Committee Mode of Action (MoA) classification. The clinical characteristics, duration of hospitalization (days), follow-up duration (years), in-hospital mortality, neurological sequela, and imaging findings were recorded. Furthermore, multivariate logistic regression analyses were performed.Results: We identified 299 patients with APP comprising 206 (68.9%) adult men with a mean exposure age of 56.4 ± 16.8 years. Paraquat, organophosphates, pyrethroids, carmabates, and phosphinic acid were the most commonly known reported poisoning agents. The mortality rate was highest in users with paraquat (77.1%), followed by phosphinic acid (22.2%), carbamates (16.7%), and organophosphates (15.8%). After a mean follows up of 3.69 ± 2.26 years, the most common neurological sequela was a cognitive decline (56 among 225 survivors, 24.89%), peripheral neuropathy (11 among 225 survivors, 4.89%), tremor (10 among 225 survivors, 4.44%), ataxia (3/225, 1.33%), and parkinsonism feature (2/225, 0.89%). Brain imaging studies revealed basal ganglion lesions on CT or hyperintensity on T2-weighted MRI images in 26 among 46 patients (56.5%). The basal ganglion lesions on brain imaging had a positive correlation with neurological sequelae.Conclusion: Acute pesticide poisoning (APP)-related mortality is high especially paraquat intoxication, and cognitive decline, as well as peripheral neuropathy, were the most common neurological sequelae among survivors, which is highly correlated with basal ganglia lesions on brain imaging.

CD11c+CD163+ Cells and Signal Transducer and Activator of Transcription 3 (STAT3) Expression Are Common in Melanoma Leptomeningeal Disease

Leptomeningeal disease (LMD) in melanoma patients is associated with significant neurological sequela and has a dismal outcome, with survival measured typically in weeks. Despite the therapeutic benefit of targeted therapies and immunotherapies for Stage IV melanoma, patients with LMD do not typically benefit. A deeper understanding of the tumor microenvironment (TME) of LMD may provide more appropriate therapeutic selection. A retrospective analysis of subjects who underwent surgical resection with LMD (n=8) were profiled with seven color multiplex staining to evaluate the expression of the global immune suppressive hub - the signal transducer and activator of transcription 3 (STAT3) and for the presence of CD3+ T cells, CD68+ monocyte-derived cells, CD163+ immune suppressive macrophages, and CD11c+ cells [potential dendritic cells (DCs)] in association with the melanoma tumor marker S100B and DAPI for cellular nuclear identification. High-resolution cellular imaging and quantification was conducted using the Akoya Vectra Polaris. CD11c+ cells predominate in the TME (10% of total cells), along with immunosuppressive macrophages (2%). Another potential subset of DCs co-expressing CD11c+ and the CD163+ immunosuppressive marker is frequently present (8/8 of specimens, 8%). Occasional CD3+ T cells are identified, especially in the stroma of the tumor (p=0.039). pSTAT3 nuclear expression is heterogeneous in the various immune cell populations. Occasional immune cluster interactions can be seen in the stroma and on the edge. In conclusion, the TME of LMD is largely devoid of CD3+ T cells but is enriched in immune suppression and innate immunity.

Readmissions in Patients with Cerebral Cavernous Malformations (CCMs): A National Readmission Database (NRD) Study

BACKGROUNDCerebral cavernous malformations (CCMs) are microvascular CNS lesions prone to hemorrhage leading to neurological sequela such as stroke and seizure. A subset of CCM patients have aggressive disease leading to multiple bleeding events, likely resulting multiple hospitalizations. Hospital admission rates are an important metric that has direct financial impact on hospitals and an indicator of overall disease burden. Furthermore, analysis of hospital readmissions can lead to early identification of high-risk patients and provides insight into the pathogenesis of CCM lesions. The purpose of this study is to identify high risk CCM patients with increased all cause readmission and comorbidities associated with increased readmissions.METHODSAll US hospital admissions due to CCMs were searched using the 2017 National Readmission Database (NRD). Patients with readmissions within 30 days of discharge from index hospitalization were identified and analyzed, relative to the remaining population.RESULTSAmong all patients hospitalized for CCM, 14.9% (13.7-16.2%) required all cause readmission within 30 days. Multivariate logistical regression analysis showed that substance abuse (p=0.003), diabetes (p=0.018), gastrointestinal bleed (p=0.002), renal failure (p=0.027), and coronary artery disease (p=0.010) were predictive of all cause readmissions, while age group 65-74 (p=0.042), private insurance (p<0.001), and treatment at a metropolitan teaching institution (p=0.039) were protective. Approximately half of all readmissions are caused by neurological (33.9%) and infectious (14.6%) etiologies. The 30-day lesion bleeding rate after index hospitalization is 0.8% (0.5-1.2%).CONCLUSIONSAll identified comorbidities associated with increased risks of readmission contribute to vascular stress, suggesting its role in lesion pathogenesis. This is the first and only study to analyze readmission metrics for CCMs in order to identify high risk patient factors to date.

Murine Models of Central Nervous System Disease following Congenital Human Cytomegalovirus Infections

Human cytomegalovirus infection of the developing fetus is a leading cause of neurodevelopmental disorders in infants and children, leading to long-term neurological sequela in a significant number of infected children. Current understanding of the neuropathogenesis of this intrauterine infection is limited because of the complexity of this infection, which includes maternal immunological responses that are overlaid on virus replication in the CNS during neurodevelopment. Furthermore, available data from human cases are observational, and tissues from autopsy studies have been derived from only the most severe infections. Animal models of this human infection are also limited by the strict species specificity of cytomegaloviruses. However, informative models including non-human primates and small animal models have been developed. These include several different murine models of congenital HCMV infection for the study of CMV neuropathogenesis. Although individual murine models do not completely recapitulate all aspects of the human infection, each model has provided significant information that has extended current understanding of the neuropathogenesis of this human infection. This review will compare and contrast different murine models in the context of available information from human studies of CNS disease following congenital HCMV infections.

Rare Case of Young Patient with Intraventricular Angiomatous Meningioma

Pediatric meningiomas are rare and account for only 2.2% of CNS tumors. In this age group, they are more frequently located in atypical sites, such as, mainly, the ventricular system, with a frequency of 8.8 to 13.6%. Adding this to the fact that the angiomatous subtype constitutes only 2.1% of all meningiomas, the rarity of the case reported here i s corroborated. We report a 17-year-old female patient diagnosed with intraventricular angiomatous meningioma; she underwent surgical resection of the tumor in the body and frontal horn of the right lateral ventricle, and there was no neurological sequela. With a follow-up of 4 years, there was no recurrence and the patient had clinical stability. Intraventricular tumors usually have slow growth and reach considerable size until they cause symptoms and then are diagnosed. In addition, the tumor’s deep location and proximity to eloquent areas make such tumors an neurosurgical challenge. The angiomatous subtype, due to the presence of hypervascularization (consisting of more than 50% of vascular components), may, in some cases, hinder surgical resection as well as be erroneously diagnosed. However, surgical treatment aimed at total resection of the lesion remains the conduct of choice in the case reported here, especially in patients in the first two decades of life, in which the use of radiation is avoided. Specifically when it comes to the surgery, we chose a transcallosal approach that allows a good transoperative visualization of the lesion when located in the body and frontal horn of the lateral ventricle.

Cervical Artery Dissection and Sports

Cervical artery dissection (CeAD) occurring in the context of sports is a matter of concern for CeAD patients. They seek advice on the role of sports in CeAD and on the safety of resuming sports after CeAD. The scarcity of studies and guidelines addressing these issues poses a challenge. We aimed at summarizing the current knowledge about CeAD and sports in order to provide an informed, comprehensive opinion for counseling CeAD patients. We took into account pathophysiological considerations, observations of cases reports, series, and registries, and conclusions by analogy from aortic dissection or inherited connective tissue syndromes. In summary, practicing active sports as the cause of CeAD seems uncommon. It seems recommendable to refrain from any kind of sports activities for at least 1 month, which can be extended in case of an unfavorable clinical or neurovascular course. We recommend starting with sport activities at low intensity—preferably with types of endurance sports—and to gradually increase the pace in an individually tailored manner, taking into circumstances of the occurrences of the CeAD in the individual patient (particularly in relation to sports), the meaning of sports activities for the individual well-being, the presence or absence of comorbidities and of neurological sequela, neurovascular findings, and whether there are signs of an underlying connective tissue alteration. Major limitations and several forms of bias are acknowledged. Still, in the absence of any better data, the summarized observations and considerations might help clinicians in advising and counseling patients with CeAD in clinical practice.

Whole Exome Sequencing as a Diagnostic Tool of Primary Complement Component Deficiencies: A Multicenter Experience of Three Novel Mutations

Diagnosis of primary complement deficiencies requires a high index of suspicion. Thus, susceptible patients are often underdiagnosed and untreated. Here, we present a multi-center experience with three novel inborn errors of the classical complement system. This is a retrospective multicenter analysis of computerized medical records of children (> 18 years) admitted in the period between 2003 and 2018 at Shaare Zedek Medical Center in Jerusalem and Edmond and Lily Safra Children's Hospital, Tel-Hashomer Medical Center, in Ramat Gan, Israel. Patients were genetically diagnosed by a complimentary immune work-up. We identified 5 patients (3 males) from four different consanguineous families harboring three novel mutations in the complement components C6-C8. Genetic mutations were identified by whole exome sequencing. Clinical manifestations consisted of meningitis with or without meningococcemia. The immune work-up demonstrated nearly absent levels of CH50, compatible with a complement pathway defect. The mean diagnosis delay was 10.56 (0–30) years. Conclusion: Invasive meningococcal infections may be life-threatening and cause severe neurological sequela. Awareness of risk factors for primary complement deficiencies, even at the firs infectious episode, should facilitate prompt immune and genetic investigations, commencing diagnosis and proper treatment.

Radiologic Assessment of the Sinonasal Tract, Nasopharynx and Mastoid Cavity in Patients with SARS-Cov-2 Infection Presenting with Acute Neurological Symptoms

Background: Acute neurological sequela in patients with COVID-19 infection include acute thromboembolic infarcts related to cytokine storm and post infectious immune activation resulting in a prothrombotic state. Radiologic imaging studies of the sinonasal tract and mastoid cavity in patients with COVID-19 infection are sparse and limited to case series. In this report, we investigate the radiologic involvement of nasal cavity, nasopharynx, paranasal sinuses, and mastoid cavity in patients with SARS-CoV-2 infection who presented with acute neurological symptoms. Methods: Retrospective review of medical records and neuroradiologic imaging in patients diagnosed with acute COVID-19 infection who presented with acute neurological symptoms to assess radiologic prevalence of sinus and mastoid disease and its correlation to upper respiratory tract symptoms. Results: Of the 55 patients, 23 (42%) had partial sinus opacification, with no evidence for complete sinus opacification. The ethmoid sinus was the most commonly affected (16/55 or 29%). An air fluid level was noted in 6/55 (11%) patients, most commonly in the maxillary sinus. Olfactory recess and mastoid opacification were uncommon. There was no evidence of bony destruction in any of the studies, Cough, nasal congestion, rhinorrhea, and sore throat were not significantly associated with any radiological findings. Conclusion: In patients who present with acute neurological symptoms, COVID-19 infection is characterized by limited and mild mucosal disease within the sinuses, nasopharynx and mastoid cavity. Level of Evidence: 4