Neuropsychological Functioning in Primary Dystonia: Updated and Expanded Multidomain Meta-Analysis.

BACKGROUND: Primary dystonia is conventionally considered as a motor disorder, though an emerging literature reports associated cognitive dysfunction. OBJECTIVES: Here, we conducted meta-analyses on studies comparing clinical measures of cognition in persons with primary dystonia and healthy controls (HCs). METHODS: We searched PubMed, Embase, Cochrane Library, Scopus, and PsycINFO (January 2000-October 2020). Analyses were modeled under random effects. We used Hedge's g as a bias-corrected estimate of effect size, where negative values indicate lower performance in dystonia versus controls. Between-study heterogeneity and bias were primarily assessed with Cochran's Q, I2 , and Egger's regression. RESULTS: From 866 initial results, 20 studies met criteria for analysis (dystonia n = 739, controls n = 643; 254 effect sizes extracted). Meta-analysis showed a significant combined effect size of primary dystonia across all studies (g = -0.56, P < 0.001), with low heterogeneity (Q = 25.26, P = 0.15, I2  = 24.78). Within-domain effects of primary dystonia were motor speed = -0.84, nonmotor speed = -0.83, global cognition = -0.65, language = -0.54, executive functioning = -0.53, learning/memory = -0.46, visuospatial/construction = -0.44, and simple/complex attention = -0.37 (P-values <0.01). High heterogeneity was observed in the motor/nonmotor speed and learning/memory domains. There was no evidence of publication bias. Moderator analyses were mostly negative but possibly underpowered. Blepharospasm samples showed worse performance than other focal/cervical dystonias. Those with inherited (ie, genetic) disease etiology demonstrated worse performance than acquired. CONCLUSIONS: Dystonia patients consistently demonstrated lower performances on neuropsychological tests versus HCs. Effect sizes were generally moderate in strength, clustering around -0.50 SD units. Within the speed domain, results suggested cognitive slowing beyond effects from motor symptoms. Overall, findings indicate dystonia patients experience multidomain cognitive difficulties, as detected by neuropsychological tests. © 2022 International Parkinson and Movement Disorder Society.

Reappraisal of the anatomy of the frontotemporal branches of the facial nerve.

OBJECTIVE: The anatomy of the temporal branches of the facial nerve (FN) has been widely described in the neurosurgical literature because of its relevance in anterolateral approaches to the skull base and implication in frontalis palsies from these approaches. In this study, the authors attempted to describe the anatomy of the temporal branches of the FN and identify whether there are any FN branches that cross the interfascial space of the superficial and deep leaflets of the temporalis fascia. METHODS: The surgical anatomy of the temporal branches of the FN was studied bilaterally in 5 embalmed heads (n = 10 extracranial FNs). Exquisite dissections were performed to preserve the relationships of the branches of the FN and their relationship to the surrounding fascia of the temporalis muscle, the interfascial fat pad, the surrounding nerve branches, and their final terminal endpoints near the frontalis and temporalis muscles. The authors correlated their findings intraoperatively with 6 consecutive patients with interfascial dissection in which neuromonitoring was performed to stimulate the FN and associated twigs that were observed to be interfascial in 2 of them. RESULTS: The temporal branches of the FN stay predominantly superficial to the superficial leaflet of the temporal fascia in the loose areolar tissue near the superficial fat pad. As they course over the frontotemporal region, they give off a twig that anastomoses with the zygomaticotemporal branch of the trigeminal nerve, which crosses the superficial layer of the temporalis muscle, spanning the interfascial fat pad, and then pierces the deep temporalis fascial layer. This anatomy was observed in 10 of the 10 FNs dissected. Intraoperatively, stimulation of this interfascial segment yielded no facial muscle response up to 1 mA in any of the patients. CONCLUSIONS: The temporal branch of the FN gives off a twig that anastomoses with the zygomaticotemporal nerve, which crosses the superficial and deep leaflets of the temporal fascia. Interfascial surgical techniques aimed at protecting the frontalis branch of the FN are safe in their efforts to protect against frontalis palsy with no clinical sequelae when executed properly.

Magnesium Levels and Diastolic Blood Pressure (DBP) as a Vasospasm Prediction Metric in Patients With Aneurysmal Subarachnoid Hemorrhage (SAH).

Introduction Vasospasm is a significant cause of morbidity and mortality in patients with aneurysmal subarachnoid hemorrhage (SAH). The purpose of this study is to evaluate a possible link between vasospasm in patients with aneurysmal SAH and magnesium and blood pressure levels. Methods Subjects were selected based on chart review of patients presenting to a comprehensive stroke center in Southern California with aneurysmal SAH. 27 were included based on the following criteria: patients greater than 18 years of age, aneurysmal SAH, clinically symptomatic vasospasms and at least one diagnostic confirmation - either from a transcranial doppler (TCD) or digital subtraction angiogram (DSA). The following exclusion criteria also applied: 1) incomplete documentation in the medical record; 2) patients <18 years of age; and 3) patients without TCD measurements. Results In an overall analysis of all patients with or without vasospasm, it was found that the presence of vasospasm was significantly correlated with diastolic blood pressures (DBPs) on day of vasospasm with an r value of 0.418 and p<0.001. Average daily DBPs throughout hospital stay were also correlated with vasospasm with an r-value of 0.455 and p<0.001. Changes in magnesium overall were also significantly related to left Lindegaard ratios with an r value of -0.201 and p value of 0.032. Lindegaard ratios were significantly correlated with age with r values of 0.510, p<0.001, and r=-0.482, p<0.001 for left and right, respectively. A change in magnesium was inversely correlated to the left Lindegaard ratio with an n of 31 and p value of 0.014 (r= -0.439) in patients with vasospasm. We also found a lower incidence of vasospasm in patients older than 65. Conclusion Monitoring magnesium and increases in DBP might be effective as a prophylactic adjunct method in patients with SAH in an effort to predict clinical vasospasm.

Do social determinants of health impact access to neurosurgical care in the United States? A workforce perspective.

OBJECTIVE: This study attempts to use neurosurgical workforce distribution to uncover the social determinants of health that are associated with disparate access to neurosurgical care. METHODS: Data were compiled from public sources and aggregated at the county level. Socioeconomic data were provided by the Brookings Institute. Racial and ethnicity data were gathered from the Centers for Disease Control and Prevention Wide-ranging Online Data for Epidemiologic Research. Physician density was retrieved from the Health Resources and Services Administration Area Health Resources Files. Catchment areas were constructed based on the 628 counties with neurosurgical coverage, with counties lacking neurosurgical coverage being integrated with the nearest covered county based on distances from the National Bureau of Economic Research's County Distance Database. Catchment areas form a mutually exclusive and collectively exhaustive breakdown of the entire US population and licensed neurosurgeons. Socioeconomic factors, race, and ethnicity were chosen as independent variables for analysis. Characteristics for each catchment area were calculated as the population-weighted average across all contained counties. Linear regression analysis modeled two outcomes of interest: neurosurgeon density per capita and average distance to neurosurgical care. Coefficient estimates (CEs) and 95% confidence intervals were calculated and scaled by 1 SD to allow for comparison between variables. RESULTS: Catchment areas with higher poverty (CE = 0.64, 95% CI 0.34-0.93) and higher prime age employment (CE = 0.58, 95% CI 0.40-0.76) were significantly associated with greater neurosurgeon density. Among categories of race and ethnicity, catchment areas with higher proportions of Black residents (CE = 0.21, 95% CI 0.06-0.35) were associated with greater neurosurgeon density. Meanwhile, catchment areas with higher proportions of Hispanic residents displayed lower neurosurgeon density (CE = -0.17, 95% CI -0.30 to -0.03). Residents of catchment areas with higher housing vacancy rates (CE = 2.37, 95% CI 1.31-3.43), higher proportions of Native American residents (CE = 4.97, 95% CI 3.99-5.95), and higher proportions of Hispanic residents (CE = 2.31, 95% CI 1.26-3.37) must travel farther, on average, to receive neurosurgical care, whereas people living in areas with a lower income (CE = -2.28, 95% CI -4.48 to -0.09) or higher proportion of Black residents (CE = -3.81, 95% CI -4.93 to -2.68) travel a shorter distance. CONCLUSIONS: Multiple factors demonstrate a significant correlation with neurosurgical workforce distribution in the US, most notably with Hispanic and Native American populations being associated with greater distances to care. Additionally, higher proportions of Hispanic residents correlated with fewer neurosurgeons per capita. These findings highlight the interwoven associations among socioeconomics, race, ethnicity, and access to neurosurgical care nationwide.

Atypical Neuroleptic Malignant Syndrome in the Setting of Quetiapine Overdose: A Case Report and Review of the Literature.

Neuroleptic malignant syndrome (NMS) is a rare and life-threatening emergency. The condition is largely iatrogenic and is often precipitated by medications such as antipsychotics. First-generation antipsychotics are more likely to cause NMS than second-generation antipsychotics. The literature lacks an objective measure for NMS diagnosis. Instead, the diagnosis relies largely on the recognition of characteristic symptoms in the presence of an inciting medication. Additional challenges exist with concomitant disease processes and toxicities that may distort the clinical picture. Here, we report a case of a 44-year-old Caucasian man who presented with atypical NMS in the setting of quetiapine overdose. The patient remained uncharacteristically afebrile throughout his admission. Although the patient recovered, extended delays in identification and management can contribute to an increased risk of morbidity and mortality.

Steroid-Resistant Double-Seronegative Optic Neuritis Responds Favorably to Plasma Exchange.

The clinical presentation of optic neuritis is quite characteristic, and the epidemiology, differential diagnosis, and treatment protocol are well established. However, when the presentation of optic neuritis is atypical, bilateral, and intravenous steroid-resistant, the treatment guidelines are quite nebulous. We present a case of bilateral severe double-seronegative optic neuritis with catastrophic vision loss and intravenous steroid resistance. After an exhaustive investigation, we empirically treated our patient with plasma exchange therapy and obtained a dramatic recovery of vision. When an immune etiology is suspected, this case is instructive vis-a-vis the utility of plasma exchange in refractory cases of optic neuritis despite seronegativity.

Isolated Double-Positive Optic Neuritis: A Case of Aquaporin-4 and Myelin Oligodendrocyte Glycoprotein Antibody Seropositivity.

Optic neuritis (ON) causes acute vision loss with typical and atypical profiles, serological markers, imaging findings, and clinical outcomes depending on the associated underlying pathophysiology. Neuromyelitis optica (NMO) and myelin oligodendrocyte glycoprotein antibody disease (MOGAD) are the usual causes of acute severe sequential or simultaneous bilateral optic neuritis. These conditions are usually accompanied by multi-level spinal cord demyelination, and notably, they are typically positive for either NMO or Myelin oligodendrocyte glycoprotein (MOG) autoantibodies, but rarely both. We present a case of isolated sequential bilateral optic neuritis that was seropositive for both NMO and MOG antibodies.

Myeloneuropathy in the Setting of Hypocupremia: An Overview of Copper-Related Pathophysiology.

A posterior cord or dorsal column myelopathy due to neurosyphilis presenting as a tabetic gait is a classic neurological vignette and is taught to all medical students. Its clinical presentation is so graphic that its simulacrum with diseases other than neurosyphilis is labeled as pseudotabes. The latter can be seen with vitamin B12 deficiency as a subacute combined degeneration (SCD) of the spinal cord, another neurology classic. However, not all cases of pseudotabes are due to posterior cord myelopathy as some can arise with other deafferentation syndromes such as polyganglioneuropathies as seen with paraneoplastic syndromes, Sjogren's syndrome, idiopathic autoimmune diseases, and post-viral neuronopathies. A unique and interesting cause of pseudotabes is due to copper deficiency; copper being a metallic trace element that is fundamental to cellular life. Herein, we present a case of copper deficiency manifesting as pseudotabes and review the biochemical properties of copper and its effects on the nervous system.

Nonmissile penetrating head injury with a wooden table leg: An illustrative case.

Penetrating head injuries are relatively uncommon and require a unique approach. This report highlights a previously unreported mechanism of injury with a table leg and the steps required to evaluate and promptly treat the patient.

Thoracic Osteomyelitis and Eustachian Valve Endocarditis: A Case Report and Literature Review.

Infective endocarditis and vertebral osteomyelitis are rare infections, most commonly caused by methicillin-sensitive Staphylococcus aureus (MSSA). The eustachian valve is an embryological remnant of the inferior vena cava that has the potential to harbor a nidus leading to infective endocarditis. Eustachian valve endocarditis has been documented in the literature on less than 50 occasions and has yet to be documented in the presence of concomitant vertebral osteomyelitis. In this case, we present a 43-year-old male presenting with vertebral osteomyelitis caused by methicillin-resistant Staphylococcus aureus (MRSA). Persistent bacteremia prompted the identification of vegetative growth on a eustachian valve remnant. This case helps mend the gap in the literature by documenting the treatment considerations in a patient with eustachian valve endocarditis in the presence of osteomyelitis caused by MRSA.

Branchial Cleft Cyst Harbors Metastatic Papillary Thyroid Carcinoma.

Branchial cleft cysts are congenital anomalies which develop in utero, most commonly arising from the second branchial cleft. They are often asymptomatic lateral neck masses but can enlarge and become symptomatic in the setting of infection. The cystic cavity can form a potential space which can harbor infection and, in rare cases, malignant spread of primary tumors. Herein, we present a rare case of a 28-year-old male with an enlarging branchial cleft cyst of six months duration following an upper respiratory infection. Routine post-surgical histopathological examination of the excised mass revealed metastatic papillary thyroid carcinoma. To our knowledge, this case is one of five cases reported within the primary literature. This case draws attention to the occult nature of papillary thyroid carcinoma and the importance of routine histopathological examination of seemingly benign surgically excised lesions.

Perioperative Positioning in Neurosurgery: A Technical Note on Park Bench Positioning for the Obese Patient Using the "Arrowhead" Technique.

Complex neurosurgical procedures, such as those traversing the posterior fossa, require optimization of the operative corridor with advanced patient positioning methods. Even seemingly small changes in the location of intracranial mass lesions can require a more dramatic operative trajectory. Modifications of traditional lateral, semi-sitting, and park-bench approaches have been described in the literature to access these lesions; however, technical considerations with respect to enlarged body habitus have yet to be fully explored. Herein, we describe a technique for positioning obese patients in the park bench position, which is referred to as the "Arrowhead technique," along with a literature review of positional complications and considerations in the setting of obesity.

Reliability of self-report versus the capacity to consent to treatment instrument to make medical decisions in brain metastasis and other metastatic cancers.

OBJECTIVE: To evaluate the ability of persons with metastatic cancer to self-assess their medical decision-making capacity (MDC). To investigate this, we compared an objective measure of MDC with self-ratings and evaluated predictors of agreement. METHODS: Data were obtained from a cross-sectional study of metastatic cancer patients at a large academic medical center. Across all standards of MDC, sensitivity, specificity, and reliability using Gwet's AC1 statistic were calculated using the objective measure as the gold standard. Logistic regression was used to evaluate predictors of agreement between the measures across all MDC standards. RESULTS: In those with brain metastases, high sensitivity (greater than 0.7), but low specificity was observed for all standards. Poor reliability was observed across all standards. Higher age resulted in higher odds of disagreement for Standard 3 (appreciation) (OR: 1.07, 95% CI: 1.00, 1.15) and Standard 4 (reasoning) (OR: 1.05, 95% CI: 1.00, 1.10). For Standard 3, chemotherapy use and brain metastases compared to other metastases resulted in higher odds of disagreement (Chemotherapy: OR: 5.62, 95% CI: 1.37, 23.09, Brain Metastases: OR: 5.93, 95% CI: 1.28, 27.55). For Standard 5 (understanding), no predictors were associated with disagreement. CONCLUSIONS: For less cognitively complex standards (e.g., appreciation), self-report may be more valid and reliable than more cognitively complex standards (e.g., reasoning or understanding). However, overall, MDC self-report in the current sample is suboptimal. Thus, the need for detailed assessment of MDC, especially when patients are older or used chemotherapy, is indicated. Other studies should be conducted to assess MDC agreement longitudinally.

Predictive Value of Credit Score on Surgery Resident and Fellow Academic and Professional Performance.

Introduction Surgical specialties consistently remain among the most competitive residency and fellowship programs with some of the highest rates of unmatched applicants. Attrition in surgical specialties is as high as 30% and particularly problematic given the extended duration of training and limited number of positions. Applicants are traditionally evaluated using a streamlined set of objective metrics, such as board scores, class rank, leadership, letters of recommendation, research productivity, and volunteerism. Consumer credit scores have been shown to be predictors of personality and work performance, however, the literature has yet to explore consumer credit histories in the context of surgical resident and fellow performance. This study aims to determine whether consumer credit scores of surgery residents and fellows are predictive of academic and professional performance. Methods This is a multi-institutional observational survey study across all American Council of Graduate Medical Education and Royal College of Physicians and Surgeons accredited surgical residency and fellowship programs in the United States and Canada. Ninety-nine surgical residents and fellows with educational status of post-graduate year two or higher participated in this study. Dichotomous (yes or no) survey items were formulated to assess performance indicators in the domains of notable achievements and awards, research output, written examination performance, professionalism, and surgical/technical skills. Three-digit Fair Isaac Corporation (FICO) credit scores, a widely accepted consumer reporting score, were collected to avoid calculation variability between algorithms.  Results Surgical residents and fellows reported credit scores between 611( fair) and 853 (exceptional) with a median (interquartile range) of 774 (715-833). The majority of participants 51.5%(51) reported very good credit scores. Those with higher credit scores (very good/exceptional) were 377% more likely to have one or more positive performance indicators OR (95% CI) = 3.77 (1.43-9.97). Similarly, residents with lower credit scores (fair/good) were only 40% more likely to have one or more negative performance indicators. The credit score has a moderate ability to distinguish between the presence and absence of positive performance indicators (area under the curve {AUC} = 0.70, p = 0.001). The use of 753 as a credit score cutoff is 78.9% sensitive and 52.4% specific for discerning surgery residents and fellows with one or more positive performance indicators. The credit score did not significantly discern those with negative performance indicators. Conclusions While credit score was significantly functional in discerning those with and without positive performance indicators, sensitivity and specificity rates leave much to be desired. This study suggests credit score may have a utility as a companion to traditional metrics used in identifying candidates for surgery residencies and fellowships who will have positive performance in the domains of research productivity, written examination performance, and professional awards and recognition. Additional studies are needed to assess this utility on a larger scale.

Correction: Predictive Value of Credit Score on Surgery Resident and Fellow Academic and Professional Performance.

[This corrects the article DOI: 10.7759/cureus.15946.].

The Impact of the COVID-19 Pandemic on Resident Physicians Well-Being in the Surgical and Primary Care Specialties in the United States and Canada.

PURPOSE:  The COVID-19 pandemic disrupted the professional, social, and spiritual activities of resident physicians around the world, impacting wellness and personal relationships. Moreover, social distancing caused significant limitations or shutdown of places of worship, including churches, synagogues, mosques, etc. Our goal was to survey resident physicians in primary care and surgical subspecialties in the United States (U.S.) and Canada and to examine the effect of the COVID-19 pandemic on their well-being. METHODS:  An international cross-sectional study was performed in November 2020, using an anonymous survey of programs in the U.S. and Canada, containing 20 questions to assess the impact of the pandemic on resident participation in social and spiritual activities and the effects on their wellness, and personal relationships. The emails with survey links attached were sent to individual program coordinators from accredited residency training programs in the United States and Canada. This consisted of programs accredited by the American Osteopathic Association (AOA), The Royal College of Physicians and Surgeons of Canada (RCPSC), and the Accreditation Council of Graduate Medical Education (ACGME). The survey was evenly divided among surgical programs (General Surgery, Neurological Surgery, Orthopedic Surgery, Urological Surgery, and Integrated Surgical Residency Programs such as Plastic Surgery, Cardiothoracic Surgery, Pediatric Surgery, and Vascular Surgery) as well as primary care programs (Internal Medicine and Family Medicine). RESULTS:  A total of 196 residents, 60 primary care residents, and 136 surgery residents participated in the study. Ninety-six participants (49%) were female, and 98 of the participants (50%) were male, with the remainder two residents identifying as "Other." Of the primary care residents, the majority (39, 65%) were female. Conversely, the majority (77, 57%) of surgery residents were male. CONCLUSION:  The COVID-19 pandemic has affected the social lives, relationships, and spiritual well-being of both surgical and primary care resident physicians. However, primary care residents reported significantly greater engagement in personal relationships and were more likely to express feelings of mental and physical exhaustion, prohibiting social attendance.

Nitrous Oxide Induced Posterior Cord Myelopathy: Beware of the Methyl Folate Trap.

Posterior cord myelopathy and subacute combined degeneration of the spinal cord are well-known complications of nitrous oxide abuse. Supplementation with vitamin B12 is an effective treatment strategy to correct low serum B12 levels or normal serum levels of dysfunctional vitamin B12 associated with this pathology. In this case, we report a patient with a one-year history of heavy nitrous oxide consumption; anywhere from eight to 30 canisters of 8 grams of nitrous oxide daily. The patient's symptoms worsened after the institution of megadoses of vitamin B12, despite normal serum folate levels. This can be explained by the phenomenon of the methyl folate trap and warrants prompt supplementation with synthetic folate. We discuss the biochemistry of nitrous oxide toxicity and the underlying mechanisms contributing to the methyl folate trap.

Association Between Treatment-Resistant Sarcoid Myopathy and Inclusion Body Myositis.

The association between sarcoid myopathy and inclusion body myositis is a rare phenomenon that is not well understood. In this case, we present a 46-year-old female with a five-year history of sarcoidosis who became refractory to treatment, experiencing progressive deterioration and muscle wasting. The patient's distribution of muscle weakness did not follow characteristic patterns of inclusion body myositis. Yet, a subsequent deltoid biopsy revealed diagnostic findings typical of inclusion body myositis. This case report reveals an association between treatment-resistant sarcoid myopathy and the evolution of inclusion body myositis in the absence of characteristic clinical findings.

Bilateral Orbital Metastases Masquerading as Ocular Myasthenia Gravis: A Case Report and Review of the Literature.

Ocular myasthenia gravis and orbital metastases have overlapping symptoms but divergent diagnostic and treatment strategies. Here, we present a 58-year-old female, with a 20-year history of advanced metastatic breast cancer, who presented to the neurology clinic with fatigue, muscle weakness, bilateral ptosis, and diplopia that worsened throughout the day. While the initial presentation was consistent with ocular myasthenia gravis, a subsequent evaluation revealed bilateral metastatic lesions of breast origin within the orbits. This case highlights the variable nature of metastatic disease and underscores the importance of a comprehensive neoplastic workup in patients with new-onset symptomatology and a prior history of advanced cancer despite purported remission status.

Long-term Survival in a Patient with Butterfly Glioblastoma: A Case Report.

Butterfly glioblastoma (bGBM) is a malignant glioma that crosses the corpus callous with bilateral cerebral hemisphere involvement. Literature reports are scarce and highlight a dismal prognosis with limited successful treatment options. We describe a patient who survived more than five years from the initial diagnosis. A 44-year-old woman presented to the emergency room for evaluation one day after a motor vehicle collision at the insistence of her husband, with four weeks of confusion, behavioral changes, and increased fatigue. Magnetic resonance imaging (MRI) of the brain revealed an enhancing, heterogeneous mass with significant necrosis, centered in the septum pellucidum and corpus callosum with intraventricular extension. She underwent a stereotactic biopsy of the lesion. Pathology was consistent with glioblastoma, WHO grade IV. She underwent standard radiation treatment and adjuvant temozolomide, demonstrating a near-complete disappearance of the tumor on imaging for the subsequent two years. Upon recurrence, she underwent additional chemotherapy with limited response. A repeat biopsy was positive for a BRAF mutation and she was treated with lomustine. After two cycles, she developed thrombocytopenia and shortly after elected to discontinue treatment. She succumbed to the progression of disease five years and two months after the initial presentation. bGBMs are uncommon and highly aggressive brain tumors. A tailored treatment protocol may improve survival. This case marks an unusually long survival of a patient with bGBM and may prompt further research to better understand the behavior of these tumors and how to improve treatment response and survival.