Intricate Connection Among the Valsalva Maneuver, Gastrointestinal Tract, and Hemodynamics: A Rare Case Presentation.

A normal daily routine turns critical in seconds, and a biophysical maneuver that is instinctual leads to a rapid decline in a young healthy patient without any warning or precipitating signs. The Valsalva maneuver is a commonly used term for the act of bearing down that affects the vagus nerve, resulting in systemic changes primarily within the autonomic nervous system. This paper reviews a case in which a young man engaging in the Valsalva maneuver was later found unconscious and presented to the emergency department. Neuroimaging revealed a large middle cerebral artery stroke along with an undiagnosed temporal arachnoid cyst, without any significant historical medical records. The resulting course of this disease remained an interesting area of inquiry. This case highlights a rare but intricate interplay of several major physiological functions that collectively contribute to the unexpected demise of a young and healthy patient.

A Hematological Travesty in the Setting of Alcohol Use Disorder and Liver Cirrhosis.

Excessive ethanol consumption is associated with an increased risk of developing health complications, especially in individuals with pre-existing thrombocytopenia and cirrhosis. Here, we describe a case of a patient with a history of alcoholic cirrhosis, hypertension, and thrombocytopenia, presenting with significant decline following an incidence of binge drinking. Radiography identified a large non-lobar intraparenchymal hemorrhage. However, due to severe thrombocytopenia that was unresponsive to platelet therapy, the possibility of pursuing any form of surgical intervention was negated. Surgical contraindication and a lack of response to subsequent medical management contributed to the family's decision to opt for conservative medical treatment and comfort care. This case showcases the potential for liver cirrhosis in the setting of chronic alcohol use disorder to pave the way for terminal intracerebral hemorrhage.

Hepatic Transplantation Raises Concern for Vascular Infrastructure Compromise: A Case Study of Debilitating Arteriovenous Malformation.

The post-operative realm for hepatic transplant patients presents many challenges, but of them all, we take a deeper dive into an increased risk of associated cerebrovascular events. Cerebrovascular diseases, such as cerebral arteriovenous malformation (AVM), are a leading cause of death following a liver transplant. We present a unique case of a liver transplant patient who presented with no brainstem reflexes three months into the post-transplant period. Imaging studies revealed a ruptured AVM within the foramen magnum and cervicomedullary junction, as well as substantial cerebral hemorrhage. While establishing the exact cause of the AVM is not as trivial as it may appear, side effects associated with post-transplantation management regimens and possible congenital factors do shed some light on notable considerations. Given the potential damage associated with ruptured AVMs, poor patient outcomes are unfortunately not as rare as one would hope. This case highlights a rare but highly possible occurrence for cerebrovascular complications, specifically AVM rupture linked to liver transplantation and the systemic changes associated with a procedure as invasive as liver transplantation.

Utilization of Cerebral Blood Flow Study With Computed Tomography for Subdural Hematoma Management.

Stroke is among the leading causes of death in the United States, and with our aging population, it will remain a pertinent obstacle in the acute setting. While the field of neuroradiology has advanced tremendously over the years, particularly in improving what we can visualize and quantify, the phrase "time is brain" yet dominates acute stroke management. Optimizing diagnostic protocols for suspected stroke requires a careful balance of data acquisition and speed, as well as taking into account available resources. We present a case of a middle-aged patient with notable risk factors for stroke presenting to the emergency department with altered mental status and suspected stroke. Radiography revealed a large subacute subdural hematoma (SDH) with a mild mass effect on the surface of the brain. The evaluation was supplemented by a computed tomography (CT) and perfusion cerebral blood flow (CBF) study indicating cortical ischemia with penumbra from the SDH compression. SDH evacuation was successfully performed, and patient recovery was achieved within the intensive care unit (ICU). Rapid data acquisition via CBF with CT imaging is crucial for guiding treatment decisions for SDHs. While protocols for ischemic stroke are well-established, SDH protocols are not studied. Thus, we discuss the value of a multimodal CT imaging approach, including CBF studies, in SDH evaluation.

The Aging Population Faces Increased Risk for Musculoskeletal Pathologies: The Problematic Atlas-Axis Instability.

Spinal cord injury (SCI), particularly of the traumatic variety, is a relatively common condition that disproportionately affects the elderly. Cases of SCI with nontraumatic etiologies in the geriatric population have increased over the last 20 years, however. Pannus formation, resulting from chronic inflammation of the spine, is one such etiology that may progress to SCI and potentially result in rapid neurological degeneration. Here we describe a case of an elderly woman who presented with a sudden onset of quadriplegia without a history of trauma. Radiography revealed upper cervical instability and fracture due to the presence of a large erosive pannus formation. Unfortunately, in the context of severe SCI, the reversibility of neurological decline is not always guaranteed. Additionally, surgical intervention is not always appropriate, especially among the elderly population, where medical management and end-of-life care are more often delivered.

COVID-19-Induced Hypoxia With Accompanying Syncope Event and Traumatic Injury.

The Centers for Disease Control and Prevention identified the first case of the novel coronavirus disease 2019 (COVID-19) on January 21, 2020 in the United States. Since its arrival, the virus has caused widespread havoc on the nation as a whole as well as on all individuals. The coronavirus family is not new to the field of medicine. In fact, the viral pathogenicity dates back to the early 1960s, with more information on the respiratory preference and the ability to cause acute respiratory pathology coming later in 2002. The novel coronavirus, severe acute respiratory syndrome coronavirus 2, causes a disease commonly referred to as COVID-19, which has a well documented course of severe respiratory pathology along with interesting systemic consequences that often complicate the clinical picture. This case presents an otherwise healthy young 35-year-old male who contracted the novel coronavirus, leading to multi-organ hypoxia and triggering a syncopal episode which resulted in physical trauma to the head leading to a minor subarachnoid hemorrhage.

The JAK Inhibitor Tofacitinib Rescues Intestinal Barrier Defects Caused by Disrupted Epithelial-macrophage Interactions.

BACKGROUND AND AIMS: Loss-of-function variants in protein tyrosine phosphatase non-receptor type-2 [PTPN2] promote susceptibility to inflammatory bowel diseases [IBD]. PTPN2 regulates Janus-kinase [JAK] and signal transducer and activator of transcription [STAT] signalling, while protecting the intestinal epithelium from inflammation-induced barrier disruption. The pan-JAK inhibitor tofacitinib is approved to treat ulcerative colitis, but its effects on intestinal epithelial cell-macrophage interactions and on barrier properties are unknown. We aimed to determine if tofacitinib can rescue disrupted epithelial-macrophage interaction and barrier function upon loss of PTPN2. METHODS: Human Caco-2BBe intestinal epithelial cells [IECs] and THP-1 macrophages expressing control or PTPN2-specific shRNA were co-cultured with tofacitinib or vehicle. Transepithelial electrical resistance and 4 kDa fluorescein-dextran flux were measured to assess barrier function. Ptpn2fl/fl and Ptpn2-LysMCre mice, which lack Ptpn2 in myeloid cells, were treated orally with tofacitinib citrate twice daily to assess the in vivo effect on the intestinal epithelial barrier. Colitis was induced via administration of 1.5% dextran sulphate sodium [DSS] in drinking water. RESULTS: Tofacitinib corrected compromised barrier function upon PTPN2 loss in macrophages and/or IECs via normalisation of: [i] tight junction protein expression; [ii] excessive STAT3 signalling; and [iii] IL-6 and IL-22 secretion. In Ptpn2-LysMCre mice, tofacitinib reduced colonic pro-inflammatory macrophages, corrected underlying permeability defects, and prevented the increased susceptibility to DSS colitis. CONCLUSIONS: PTPN2 loss in IECs or macrophages compromises IEC-macrophage interactions and reduces epithelial barrier integrity. Both of these events were corrected by tofacitinib in vitro and in vivo. Tofacitinib may have greater therapeutic efficacy in IBD patients harbouring PTPN2 loss-of-function mutations.

Acute Alcohol Intoxication With Accompanying Neurovascular Decline in Young Adults: A Sobering Case Series.

With an increase in both popularity and incidence, young adults continue to consume increasing amounts of ethanol-containing beverages at alarmingly short intervals, at times, far surpassing the threshold of binge drinking. From mixed beverages to party delights, high-concentration ethanol consumption continues to wreak havoc on the health of the younger generation. In this dual case series, we discuss two unique cases of alcohol-induced neurovascular compromise following episodes of high-volume ethanol consumption and acute neurovascular decline with varying outcomes. These cases highlight the hyperacute onset of severe pathology and the dire need for immediate medical intervention. While outcomes vary from case to case, our findings are congruent with those of vast medical literature that supports the consensus that immediate intervention to restore neurovascular flow is crucial for desired outcomes. At the end of the day, we cannot control the amount of alcohol that enters the mouths of our patients, but rather, we can educate them on safer practices while highlighting the risk and life-changing consequences of such risky behavior.

A Rare Form of Pott's Disease With Multifaceted Pathological Complications.

While tuberculosis is globally very prevalent, especially in the developing world, tuberculosis of the central nervous system (CNS) (including Pott's `disease) is an extremely rare occurrence for neurology/neurosurgery departments in the modern era. It is normally treated via rifampin, isoniazid, pyrazinamide, and ethambutol (RIPE) therapy with the need for surgical intervention deemed by the presence of neurological deficiency or abscess, spinal instability, or significant kyphosis. Here we describe a case of an elderly woman with Pott's disease and a history of HIV presenting with neurologic deficiencies in both legs and an infected mass causing mid-thoracic compression and kyphotic deformity. The presence of a compromised immune system greatly complicates treatment and worsens outcomes. The patient underwent trans-thoracic corpectomy for decompression and mass removal. Spinal realignment was accomplished with an anterior graft, using the patient's rib, preceding posterior stabilization with instrumentation. Postoperatively, the patient received RIPE therapy. Despite a compromised immune system, the full neurologic function of both legs was restored in four months.

Iatrogenic Acute Ischemic Necrosis Due to Emergent Bleeding Control in Ventral Foramen Magnum Meningioma with Spinal Instability.

Meningiomas are the most common benign intracranial tumors. They often require surgical resection and postoperative radiation/chemotherapy based on their histologic grade. While necrosis caused by preoperative embolization and spontaneous tumor infarction is appreciated by pathologists when staging meningiomas, intraoperative events including large bore artery occlusion may also alter the histopathologic picture of a benign meningioma. Hence, they should be considered when signs of unexpected ischemia and necrosis are found, as these same phenotypes are also hallmarks of a higher-grade disease. We describe a case of a man with a large ventral foramen magnum meningioma who underwent temporary intraoperative occlusion of the vertebral artery, leading to ischemic tumor necrosis with abundant neutrophil invasion when the tumor was eventually examined histologically.

Not Everything Requires a Psychiatry Consult: Subdural Hematoma as a Cause of Transient Acute Quadriplegia.

While subdural hematoma (SDH) is a commonly encountered emergent pathology that is often in the setting of trauma, its ability to present with a wide variety of symptoms, or no symptoms at all, may delay diagnosis. SDH symptoms progress in a stepwise manner, potentially resulting in rapid neurological degeneration and can result in irreversible damage. Here we describe a case of an elderly woman with bilateral chronic SDH with mass effect who initially presented with severe headaches and a mild altered mental status, notably without a history of head trauma. Diagnosis was achieved through radiographic imaging. Within 24 hours, the patient suddenly became quadriplegic. Emergent bilateral evacuation of SDH was performed. Full neurological recovery of both arms and legs was achieved without delay, demonstrating the ability of this approach to reverse the development of acute quadriplegia attributed to SDH in such patients. Comprehensive and timely medical screening on initial presentation accompanied by radiographic studies, especially of patients presenting with altered mental status is crucial for identifying any underlying pathology, such as SDH. Altered mental status without head trauma is not always psychologic in nature and a psychiatric consult is insufficient in identifying lesions of the central nervous system (CNS). Altered mental status encompasses a broad differential diagnosis that seeks to find organic causes of altered state. While mortality from symptomatic chronic SDH is high, especially in the geriatric patient population, our findings support the position that rapid diagnosis and intervention to reverse neurological deterioration is an essential component of improving patient outcomes.

Gliomatosis Cerebri: A Unique Presentation with Accompanying Clinical Nuance.

Gliomatosis cerebri (GC) has classically been considered a rare malignancy with a poor prognosis and is developmentally unique from solid tumors. More recently, GC has become better understood as a phenotype along the spectrum of gliomas and, most importantly, not mutually exclusive from the more common presentation of a tumor mass. The following case report illustrates not only the implications of the ontogeny of gliomas in clinical practice but also the successes that can accompany the early recognition of such a disease. Here, we report the presentation of a solid temporal lobe glioma, which, on presentation, was disseminating along well-defined mesolimbic white matter tracts. Once properly diagnosed and managed, the patient remarkably proceeded to achieve an impressive outcome given the extent of her pathology.

Recurrent Caterpillar-like Arachnoid Cysts Following Initial Resection: A Unique Presentation of a Disorder Where the Limits of Modern Medicine Are Reached.

Of the many emergent neurological cases presenting to the emergency department (ED) and operating room (OR) for resection, arachnoid cysts are amongst one of the rarer pathologies. The proper resection of arachnoid cysts has substantially decreased the risk of recurrence. Current medicinal and surgical approaches have been refined over the years and prove to be successful for many patients. Where current practices begin to fail is during the treatment of complex and rare cases, such as the one presented in this manuscript. The commonly accepted surgical practices that were utilized to aid in the management of our patient, who initially presented with a simple arachnoid cyst, unexpectedly resulted in the further development of additional arachnoid cysts, a very rare occurrence, and a complication that should be discussed amongst all specialists in the hope of identifying more focused, novel, and less-invasive approaches to cyst removal and recurrence prevention.

"Crimes against the Nervous System": Neurological References During the Nuremberg Doctors' Trials.

The Nuremberg Trials were a sequence of tribunal sessions held by the Allied Forces between November 1945 and October 1946 with the intent of prosecuting prominent representatives of the Nazi Party for crimes committed before and during the war. Because medical experiments in human prisoners were among the most heinous offenses, a specific series of court cases, known as the Doctor's Trials (the USA vs. Karl Brandt et al), was carried out. A considerable part of the official documents of the Nuremberg Trials has been recently made publicly available through the Nuremberg Trials Project, an initiative of the Harvard Law School Library. We performed a comprehensive analysis of the Doctors' Trials original documents (NMT 1: Medical Case) as well as other available academic and historical sources focusing on references to the nervous system, neurosurgical, and neurologic diseases. Besides providing a brief glance of a unique source of original historical documents, this historical vignette also attempts to fulfill, at least in some limited sense, the moral duty toward the Holocaust victims laid on our generation by remembering their fate.

Anti-coagulation Drug Warfarin Contributes to Severe Adverse Outcomes in Prolonged Unsupervised Use: A Double-edged Sword.

Anti-coagulation medications are widely used in clinical practice, especially in the United States as cardiac-related emergencies are on the rise. An overarching caveat in using anti-coagulation drugs as a long-term treatment regimen, such as warfarin, is that patients are closely monitored by their primary care provider. Routine monthly laboratory examinations are strictly required to assess the international normalized ratio (INR) which measures prothrombin time (PT), providing insight into how long it takes for blood to clot. A 58-year-old Caucasian male presented with non-traumatic acute quadriplegia with cord compression starting at the 3rd cervical (C3) level and continuing throughout the entire spinal cord. Radiological studies revealed severe cervical stenosis from C3 to C7 resulting from a posterior spinal mass, thoracic and lumbar spine studies revealed a compressive posterior acute epidural hematoma (EDH) compressing the thoracic spinal cord from T6 to T10 with signal change, as well as a L4-S1 posterior acute epidural hematoma compressive of the cauda equina. Upon detailed review of our patient's history it became evident that our patient had been on a prolonged and unsupervised warfarin (Coumadin) regimen for congestive heart failure without primary care follow-up or routine laboratory testing. Our patient was a prime surgical candidate for an immediate two-stage decompression, hematoma evacuation and instrumentated fusion surgery. Post-operatively, our patient regained leg strength to 2/5 and arm strength to 4+/5.

Trauma-induced Acute Epidural Hematoma: The Rising Sun in a Progressively Lethargic Man.

A young adult, 18 years of age, presented to the emergency department with severe traumatic brain injury (TBI) resulting from a bicycle versus vehicle head-on collision. The patient initially presented in a promising condition but quickly deteriorated into a state of unconsciousness with no meaningful responses to stimuli or coordinated voluntary movement. Stat computed tomography (CT) revealed a large, right-sided, acute epidural hematoma (EDH) with mass-effect and a severe midline shift indicative of immediate surgery. This case highlights the importance of closely monitoring traumatic brain injury patients regardless of initial presentation and neurological exam results, as the patient's condition may drastically and rapidly change without much warning. Additionally, it is key to utilize regular radiological studies on these patients, to detect any neurological changes as close to onset as possible. Lastly, it is imperative that neurosurgeons closely monitor the patients/ state of consciousness as a rapid decline serves as a key diagnostic indicator of the need for immediate surgery.

Concomittant fibrous dysplasia with aneurysmal bone cyst formation within the skull, humerus and rib.

Polyostotic fibrous dysplasia (FD) is a rare pathology characterized by the abnormal and gradual replacement of normal bone (calcium hydroxylapatite of osteoid matrix) with fibrous connective tissue. Aneurysmal bone cyst (ABC) is a tumor-like benign lesion with blood-filled cavities that can affect virtually any bone in the body. We report on a 20-year-old male presenting with an extremely rare pathology of FD with ABC formation of the skull, fourth rib and humerus. Our case report represents a novel literary addition to rare FD with ABC pathologies. Optimal diagnosis of this rare pathology can be achieved by a full body evaluation for clinical and radiographic FD with or without ABC, and optimal treatment for this rare pathology is the maximum surgical excision of the tumor and/or soft tissue.

Management Strategies and Outcomes for VHL-related Craniospinal Hemangioblastomas.

Hemangioblastomas are rare and benign tumors accounting for less than 2% of all central nervous system (CNS) tumors. The vast majority of hemangioblastomas occur sporadically, whereas a small number of cases, especially in younger patients, are associated with Von Hippel-Lindau (VHL) syndrome. It is thought that loss of tumor suppressor function of the VHL gene results in stabilization of hypoxia-inducible factor alpha with downstream activation of cellular proliferative and angiogenic genes that promote tumorigenesis. VHL-related hemangioblastomas predominantly occur in the cerebellum and spine. Lesions are often diagnosed on contrast-enhanced craniospinal MRIs, and the diagnosis of VHL occurs through assessment for germline VHL mutations. Surgical resection remains the primary treatment modality for symptomatic or worrisome lesions, with excellent local control rates and neurological outcomes. Stereotactic radiotherapy can be employed in patients who are deemed high risk for surgery, have multiple lesions, or have non-resectable lesions. Given the tendency for development of either new or multiple lesions, close radiographic surveillance is often recommended for asymptomatic lesions.