Disseminated mycobacterium avium complex spinal osteomyelitis in a patient with interferon gamma receptor deficiency: A case report.

Disseminated mycobacterium avium complex (MAC) infection is rare and is classically associated with immunodeficient states. Osteomyelitis is a rare manifestation of disseminated MAC infection. The overwhelming majority of MAC infections occur in patients with human immunodeficiency virus (HIV). Disseminated MAC infection has been described in interferon gamma receptor deficiency, an immunodeficiency mechanistically linked to mycobacterial infection. We present a case of disseminated MAC vertebral osteomyelitis in a patient with interferon gamma receptor deficiency.

Sanford Larson, MD, PhD: a pioneering neurosurgeon, scientist, and educator.

Dr. Sanford Larson, MD, PhD (1929-2012), was an influential figure in spinal neurosurgery. Dr. Larson played a pivotal role in establishing neurosurgery's foothold in spinal surgery by serving as the inaugural chair of the Joint Section on Disorders of the Spine and Peripheral Nerves and as a president of the Cervical Spine Research Society. He made many advances in spine care, most notably the modification and popularization of the lateral extracavitary approach to the thoracolumbar spine. Dr. Larson established the neurosurgery residency program at the Medical College of Wisconsin; he also instituted the program's spine fellowship, the first in the United States for neurological surgeons. His mentorship produced numerous leaders in organized neurosurgery and neurosurgical education, including Edward Benzel, MD, Dennis Maiman, MD, PhD, Joseph Cheng, MD, Shekar Kurpad, MD, PhD, and Christopher Wolfla, MD. Dr. Larson was a prominent leader in spinal neurosurgery and his legacy carries on today through his contributions to research, education, and surgical technique.

Cerebellar anaplastic ganglioglioma in a septuagenarian.

Ganglioglioma is a rare neoplasm most common in children and adolescents. It is typically located in the supratentorial compartment, with the temporal lobe being the most common tumor location. Anaplastic ganglioglioma is a WHO grade III ganglioglioma, a rare subtype accounting for a small minority of ganglioglioma cases. Posterior fossa anaplastic ganglioglioma in an adult is incredibly rare; only 3 prior cases have been reported. Only 1 adult anaplastic ganglioglioma in the cerebellum has been reported. We present the second reported adult cerebellar anaplastic ganglioglioma.

Patterns and outcomes of intracranial pressure monitoring in traumatic brain injury: An analysis of the National Inpatient Sample.

INTRODUCTION: Elevated intracranial pressure (ICP) can cause progressive neurological deterioration following traumatic brain injury (TBI). ICP can be monitored to guide subsequent treatment decisions. However, there is conflicting data in the literature regarding the utility of ICP monitoring. We aim to describe patterns and outcomes of ICP monitoring in the United States with the use of a nationwide healthcare database. METHODS: We performed a 5-year analysis of the Nationwide Inpatient Sample database. We identified all adult TBI patients with a Glasgow Coma Scale (GCS) measuring 3-8 using International Classification of Diseases diagnostic codes. Propensity score matching (1:2 ratio) was performed to control for demographics, injury parameters and comorbidities. Outcome measures included inpatient mortality, length of stay (LOS), cost of care, and discharge disposition. RESULTS: After propensity score matching, a cohort of 1664 patients was obtained (monitored, 555; non-monitored, 1109). Index outcomes with respect to monitor and no-monitor are as follows: inpatient mortality (35.1%, 42.4%, P <0.01), median LOS (15 days, 6 days, P<0.001), median total charge (289,797 USD, 154,223 USD, P <0.001), discharge home (7.9%, 19.3%, P <0.001) and discharge to another facility (53.9%, 35.4%, P <0.001). DISCUSSION: ICP monitoring in TBI patients is associated with decreased inpatient mortality and discharge to home, and it is associated with an increased hospital LOS, total charge, and chance of discharge to another facility. CONCLUSION: The risks and benefits of ICP monitoring should be seriously considered when managing adults with severe TBI.

Differences in Responses of Immunosuppressed Kidney Transplant Patients to Moderna mRNA-1273 versus Pfizer-BioNTech.

Immunosuppressed kidney transplant (KT) recipients produce a weaker response to COVID-19 vaccination than immunocompetent individuals. We tested antiviral IgG response in 99 KT recipients and 66 healthy volunteers who were vaccinated with mRNA-1273 Moderna or BNT162b2 Pfizer-BioNTech vaccines. A subgroup of participants had their peripheral blood leukocytes (PBLs) evaluated for the frequency of T helper 1 (Th1) cells producing IL-2, IFN-γ and/or TNF-α, and IL-10-producing T-regulatory 1 (Tr) cells. Among KT recipients, 45.8% had anti-SARS-CoV-2 IgG compared to 74.1% of healthy volunteers (p = 0.009); also, anti-viral IgG levels were lower in recipients than in volunteers (p = 0.001). In terms of non-responders (≤2000 U/mL IgG), Moderna's group had 10.8% and Pfizer-BioNTech's group had 34.3% of non-responders at 6 months (p = 0.023); similarly, 15.7% and 31.3% were non-responders in Moderna and Pfizer-BioNTech groups at 12 months, respectively (p = 0.067). There were no non-responders among controls. Healthy volunteers had higher Th1 levels than KT recipients, while Moderna produced a higher Th1 response than Pfizer-BioNTech. In contrast, the Pfizer-BioNTech vaccine induced a higher Tr1 response than the Moderna vaccine (p < 0.05); overall, IgG levels correlated with Th1(fTTNF-α)/Tr1(fTIL-10) ratios. We propose that the higher number of non-responders in the Pfizer-BioNTech group than the Moderna group was caused by a more potent activity of regulatory Tr1 cells in KT recipients vaccinated with the Pfizer-BioNTech vaccine.

Sex differences in myocarditis hospitalizations: Rates, outcomes, and hospital characteristics in the National Readmission Database.

Inflammation of the myocardium, or myocarditis, presents with varied severity, from mild to life-threatening such as cardiogenic shock or ventricular tachycardia storm. Existing data on sex-related differences in its presentation and outcomes are scarce. Using the Nationwide Readmission Database (2016-2019), we identified myocarditis hospitalizations and stratified them according to sex to either males or females. Multivariable regression analyses were used to determine the association between sex and myocarditis outcomes. The primary outcome was in-hospital mortality, and the secondary outcomes included sudden cardiac death (SCD), cardiogenic shock (CS), use of mechanical circulatory support (MCS), and 90-day readmissions. We found a total of 12,997 myocarditis hospitalizations, among which 4,884 (37.6 %) were females. Compared to males, females were older (51 ± 15.6 years vs. 41.9 ± 14.8 in males) and more likely to have connective tissue disease, obesity, and a history of coronary artery disease. No differences were noted between the two groups with regards to in-hospital mortality (adjusted odds ratio [aOR] 1.20; confidence interval [CI] 0.93-1.53; P = 0.16), SCD (aOR:1.18; CI 0.84-1.64; P = 0.34), CS (aOR: 1.01; CI 0.85-1.20;P = 0.87), or use of MCS (aOR: 1.07; CI:0.86-1.34; P = 0.56). In terms of interventional procedures, females had lower rates of coronary angiography (aOR: 0.78; CI 0.70-0.88; P < 0.01), however, similar rates of right heart catheterization (aOR 0.93; CI:0.79-1.09; P = 0.36) and myocardial biopsy (aOR: 1.16; CI:0.83-1.62; P = 0.38) compared to males. Additionally, females had a higher risk of 90-day all-cause readmission (aOR: 1.25; CI: 1.16-1.56; P < 0.01) and myocarditis readmission (aOR:1.58; CI 1.02-2.44; P = 0.04). Specific predictors of readmission included essential hypertension, congestive heart failure, malignancy, and peripheral vascular disease. In conclusion, females admitted with myocarditis tend to have similar in-hospital outcomes with males; however, they are at higher risk of readmission within 90 days from hospitalization. Further studies are needed to identify those at higher risk of readmission.

Clinical and Radiological Features of Menetrier's Disease: A Case Report and Review of the Literature.

We present a case report describing the diagnosis and management of a patient who presents with a rare diagnosis of Menetrier's disease. This condition poses a diagnostic challenge to clinicians due to its nonspecific clinical presentation and is oftentimes misdiagnosed for more common gastric disorders. Menetrier's disease is characterized by gastric mucosal hypertrophy and subsequent protein loss, resulting in gastric symptoms and widespread edema. While the etiology remains unclear, notable associations have been observed with Helicobacter pylori (H. pylori) infection and overexpression of transforming growth factor-alpha (TGF-a). The management often involves supportive measures with medical and surgical interventions for refractory cases and when necessary. This report includes a comprehensive review of the literature on the clinical presentation, diagnostic approach, and management of this rare disease. By documenting such cases in the medical literature, we aim to enhance the clinician's ability to recognize and manage this disorder, thereby preventing the development of more severe manifestations such as gastric carcinoma.

Persistent Hiccups after Subthalamic Nucleus Deep Brain Stimulator Implantation for Parkinson's Disease: Case Report and Literature Review.

Hiccups are mediated by a reflex arc that consists of afferent, central, and efferent components. The structures involved in the central component have not been fully elucidated, although several brainstem structures have been implicated, including the subthalamic nucleus (STN). Accordingly, Parkinson's disease (PD), a disease defined by the loss of dopaminergic neurons in the STN, has an interesting, although unspecified, relationship to hiccups. Hiccups have been described in association with PD and the use of dopaminergic medications. Interestingly, deep brain stimulation (DBS) of the STN appears to be implicated in the pathogenesis of hiccups as well. There have been sporadic reports of hiccups occurring in conjunction with STN-DBS. We present a case of hiccups occurring after STN-DBS for PD in a 65-year-old man. STN-DBS significantly improved his PD symptoms; however, he developed persistent and daily hiccups. As of writing, 24 months after surgery, the patient experiences hiccups several times per day with no associated gastrointestinal complaints and with significant improvement in his PD symptoms. This report describes the case details and summarizes the existing literature describing hiccups in patients undergoing surgical treatment for PD.

Marchiafava-Bignami disease: Case presentation and radiological imaging.

Marchiafava-Bignami disease (MBD) is a rare vitamin B deficiency classically associated with alcoholism. MBD damages the corpus callosum and presents with nonspecific neurological symptoms. Radiological imaging is critical for diagnosing MBD and commencing subsequent treatment, which often consists of vitamin B supplementation. We present a case of MBD in a 56-year-old male with alcohol use disorder, epilepsy, schizophrenia, post-traumatic stress disorder, and cardiovascular risk factors. The patient presented with general neurological symptoms, and there were several potential diagnoses to consider based on the patient's history. Radiological imaging was necessary for diagnosis. This case demonstrates the role radiological imaging plays in the workup of MBD.

Incomplete Anterior Spinal Artery Syndrome Responsive to Intrathecal Baclofen.

Anterior cord syndrome (ACS) occurs as a result of ischemia in the territory of the anterior spinal artery (ASA). Although spinal cord strokes are rare, the ASA is the most commonly affected vessel in the spinal cord. The typical presentation of an ASA stroke is paraparesis or paraplegia, bilateral loss of pain and temperature sensation, and fecal or urinary incontinence; the underlying neural structures responsible for these symptoms include the corticospinal tracts and anterior horns, anterolateral spinothalamic tracts, and lateral horns, respectively. ACS is a feared complication of aortic procedures and has been well-documented to occur during or after endovascular abdominal aortic aneurysm revascularization (EVAR). We report a case of incomplete or partial ACS presenting with delayed-onset spasticity and instability several months following EVAR, who was subsequently treated with intrathecal baclofen. We hypothesize that this patient's ischemia selectively damaged descending white matter tracts responsible for modulating the stretch receptor reflex, including damage to the corticospinal tract, which likely also impaired positional stability.

Radiological Diagnosis and Surgical Treatment of Gallstone Ileus.

We report on the diagnosis and treatment of a patient who presented with a small bowel obstruction due to gallstone ileus. This condition is an infrequent complication of cholelithiasis that presents with non-specific and intermittent findings, including bloating, early satiety, constipation, nausea, and vomiting. Contrast-enhanced CT features the classic imaging finding, called Rigler's triad, which includes small bowel distension, gas in the gallbladder, and an ectopic gallstone. Laparoscopic enterolithotomy is employed to prevent further erosion through the gallbladder wall and into the adjacent gastrointestinal structures. The early diagnosis and treatment of gallstone ileus results in decreased morbidity and mortality.

Glioblastoma in pregnant patient with pathologic and exogenous sex hormone exposure and family history of high-grade glioma: A case report and review of the literature.

Background: Glioblastoma (GBM) incidence is higher in males, suggesting sex hormones may influence GBM tumorigenesis. Patients with GBM and altered sex hormone states could offer insight into a relationship between the two. Most GBMs arise sporadically and heritable genetic influence on GBM development is poorly understood, but reports describing familial GBM suggest genetic predispositions exist. However, no existing reports examine GBM development in context of both supraphysiologic sex hormone states and familial predisposition for GBM. We present a case of isocitrate dehydrogenase (IDH)-wild type GBM in a young pregnant female with polycystic ovary syndrome (PCOS), history of in vitro fertilization (IVF), and significant family history of GBM and further discuss how unique sex hormone states and genetics may affect GBM development or progression. Case Description: A 35-year-old pregnant female with PCOS and recent history of IVF treatment and frozen embryo transfer presented with seizure and headache. Imaging revealed a right frontal brain mass. Molecular and histopathological analysis of the resected tumor supported a diagnosis of IDH-wild type GBM. The patient's family medical history was significant for GBM. Current literature indicates testosterone promotes GBM cell proliferation, while estrogen and progesterone effects vary with receptor subtype and hormone concentration, respectively. Conclusion: Sex hormones and genetics likely exert influence on GBM development and progression that may compound with concurrence. Here, we describe a unique case of GBM in a young pregnant patient with a family history of glioma and atypical sex hormone exposure due to endocrine disorder and pregnancy assisted by exogenous IVF hormone administration.

Ventriculoureteral shunt: Narrative review of contemporary cases and its historical role in the development of renal transplantation.

Ventriculo-ureteral (VU) shunting is a little-known method of managing hydrocephalus. This paper reviews contemporary uses of this shunting technique and describes its historical significance to the field of organ transplantation. The ureter may serve as a possible backup, or alternative, distal drainage site compared to the more common peritoneum, atrium, and pleural space. Sporadic contemporary uses of the VU shunt have been reported in unique situations, demonstrating a possible utility in modern neurosurgery. Interestingly, the VU shunt played an important role in the development of kidney transplantation. In the late 1940s and early 1950s, David Hume, a general surgery resident, and colleagues at the PBBH undertook a series of human kidney transplantations. Concurrently, Donald Matson, a pediatric neurosurgeon at Peter Bent Brigham, was utilizing the VU shunt in hydrocephalic patients. Dr. Matson's VU shunt technique involved total nephrectomy, and some of the kidneys harvested from Dr. Matson's were used by his general surgery colleagues in their transplantation trials. Although none of the transplanted kidneys from this series were successful, the transplant team in Boston, minus David Hume, went on to perform the world's first kidney transplant a few years later. This relatively unfamiliar procedure may be applicable to specific situations, and it is of historical importance to the field of transplantation.

Inferior mesenteric vein thrombophlebitis secondary to acute diverticulitis.

Inferior mesenteric vein thrombophlebitis is an uncommon condition. Most cases of portal-mesenteric thrombophlebitis affect either the portal vein or superior mesenteric vein; it is not known why the inferior mesenteric vein is less affected. Thrombophlebitis typically occurs following inflammatory intra-abdominal processes, such as diverticulitis. Diverticulitis is a common condition in the Western world, with several common complications, such as fistula formation and bowel wall perforation. However, although diverticulitis is a common cause of portal-mesenteric thrombophlebitis, thrombophlebitis is still a rare complication of diverticulitis. We present a case of diverticulitis complicated with interior mesenteric vein thrombophlebitis with gas extension into the portal vein.

Leukoencephalopathy, calcifications, and cysts: Labrune syndrome.

Labrune syndrome is an extremely rare disorder characterized by a radiological triad of leukoencephalopathy, cerebral calcifications, and cysts. The condition is the result of an autosomal mutation in the SNORD118 gene, a non-protein encoding gene that mediates rRNA synthesis. The mutation results selectively in cerebral microangiopathy through an unknown mechanism. Radiological imaging is central to diagnosing the condition, but, because the condition is so rare, there is no standard treatment paradigm. We describe the longitudinal progression of a case of Labrune syndrome, including the radiological diagnosis and imaging and surgical management.