Faculty Bibliography

BACKGROUND:Migraine treatment may mitigate migraine and associated pain in the perioperative period. OBJECTIVE:The aim of the study was to estimate the effect of perioperative acute and prophylactic migraine treatment on the risk of postoperative 30-day hospital readmission with an admitting diagnosis specifying any pain complaints among migraine patients. DESIGN/METHODS:Electronic health records were analysed for 21,932 adult migraine patients undergoing surgery between 2005 and 2017 at Beth Israel Deaconess Medical Center and Massachusetts General Hospital in Boston, Massachusetts, USA. METHODS:Perioperative abortive migraine treatment was defined as guideline-recommended medication (triptan, ergotamine, acetaminophen, nonsteroidal anti-inflammatory drug) prescription after surgery, within 30 days after discharge and prior readmission. Perioperatively continued prophylactic migraine treatment was defined as prescription both prior to surgery and perioperatively for recommended medications (beta-blockers, antidepressants, antiepileptics, onabotulinumtoxin A). RESULTS:Overall, 10,921 (49.8%) patients received a prescription for abortive migraine drugs. Of these, 1.2% and 1.5% of patients with and without such prescription were readmitted for pain, respectively. Patients with abortive treatment had lower odds of pain-related readmission (adjusted odds ratio 0.63 [95% confidence interval 0.49-0.81]). Prophylactic migraine treatment showed no effect on pain-related readmission independently of acute treatment (adjusted odds ratio 0.97 [95% confidence interval 0.72-1.32]). CONCLUSIONS:Migraine patients undergoing surgery with a perioperative prescription for abortive migraine drugs were at decreased risk of pain-related hospital readmission.

PURPOSE/OBJECTIVE:Close hemodynamic monitoring after craniotomy is routine given risk for post-operative hypertension, systemic and neurological complications. Patient and peri-operative variables associated with increased risk of post-craniotomy hypertension and complications are not well understood. Our analysis aims to estimate the incidence and prevalence of post-craniotomy hypertension, its time course, contributing factors, and post-craniotomy complications. MATERIAL AND METHODS/METHODS:This is a prospective study of patients admitted to the Neurosurgical Intensive Care Unit after an elective craniotomy. Variables associated with pre-surgical risk, demographics, and post-operative care were analyzed. RESULTS:A total of 282 patients were included in the final analysis, 44% had pre-existing hypertension. Post-craniotomy hypertension was seen in 21%, with a higher incidence in patients with pre-existing hypertension (p < .001), smaller craniotomies (p = .0035), and increased use of analgesic medications (p < .001). History of hypertension was the only independent risk factor for post-craniotomy hypertension in a multivariate regression model. Patients who developed post-craniotomy hypertension, showed a significant increase in length of stay, number and duration of antihypertensive treatment. However, post-craniotomy hypertension was not associated with a higher incidence of other post-operative complications. CONCLUSIONS:Development of hypertension after craniotomy is multi-factorial. In this prospective study, a prior history of hypertension was the only associated independent risk factor.

ADAMTSs (a disintegrin and metalloproteinase with thrombospondin motifs) are secreted metalloproteinases that play a major role in the assembly and degradation of the extracellular matrix (ECM). In this study, we show that ADAMTS18, produced by the epithelial cells of distal airways and mesenchymal cells in lung apex at early embryonic stages, serves as a morphogen in lung development. ADAMTS18 deficiency leads to reduced number and length of bronchi, tipped lung apexes, and dilated alveoli. These developmental defects worsen lipopolysaccharide-induced acute lung injury and bleomycin-induced lung fibrosis in adult Adamts18-deficient mice. ADAMTS18 deficiency also causes increased levels of fibrillin1 and fibrillin2, bronchial microfibril accumulation, decreased focal adhesion kinase signaling, and disruption of F-actin organization. Our findings indicate that ECM homeostasis mediated by ADAMTS18 is pivotal in airway branching morphogenesis.

OBJECTIVE:To determine time trends and distinguishing autopsy findings of sudden unexpected death in epilepsy (SUDEP) in the U.S. METHODS:We identified the decedents where epilepsy/seizure was listed as cause/contributor to death, or comorbid condition on death certificate among all decedents who underwent medico-legal investigation at three medical examiner (ME) offices across the country: New York City (2009-2016), San Diego County (2008-2016), and Maryland (2000-2016). After reviewing all available reports, deaths classified as definite/probable/near SUDEP or SUDEP plus were included for analysis. Mann-Kendall trend test was used to analyze temporal trends in SUDEP rate for 2009-2016. Definite SUDEPs were compared to sex- and age ± 2 years-matched non-SUDEP deaths with a history of epilepsy regarding autopsy findings, circumstances, and comorbidities. RESULTS:1086 SUDEP cases were identified. There was a decreasing trend in ME-investigated SUDEP incidence between 2009-2016 (z= -2.2 S= -42 p= 0.028) among three regions. There was a 28% reduction in ME-investigated SUDEP incidence from 2009-2012 to 2013-2016 (CI: 17%-38%, p<0.0001). We found no correlation between SUDEP rates and the month of year or day of week. There was no difference between SUDEP and non-SUDEP deaths regarding neurodevelopmental abnormalities, pulmonary congestion/edema, and myocardial fibrosis. CONCLUSIONS:There was a decreasing monotonic trend in ME-investigated SUDEP incidence over eight years, with a 28% reduction in incidence from 2009-2012 to 2013-2016. Unlike SIDS and sudden cardiac death, we found no correlation between SUDEP and the season of year or day of week. No autopsy findings distinguished SUDEP from non-SUDEP deaths.

The COVID-19 pandemic dramatically affected the operations of New York City hospitals during March and April of 2020. This article describes the transformation of a neurology division at a 450-bed tertiary care hospital in a multi-ethnic community in Brooklyn during this initial wave of COVID-19. In lieu of a mass redeployment of staff to internal medicine teams, we report a novel method for a neurology division to participate in a hospital's expansion of care for patients with COVID-19 while maintaining existing team structures and their inherent supervisory and interpersonal support mechanisms.

OBJECTIVE:To determine the prevalence of cerebrospinal fluid (CSF) markers associated with inflammation (i.e., elevated white blood cell count, protein concentration, and CSF-specific oligoclonal bands) in patients with early active autoimmune encephalitis (AE). METHODS:CSF characteristics, including WBC count, protein concentration, and oligoclonal banding, were analyzed in patients diagnosed with AE at two tertiary care centers. RESULTS:(range: 0-544) and the median CSF protein concentration was 0.42 g/L (range: 0.15-3.92). CONCLUSIONS:White blood cell counts and protein levels were within normal limits in the CSF of a substantial proportion of patients with early active AE. Inclusion of CSF oligoclonal banding identified a higher proportion of patients with an inflammatory CSF profile, especially when CSF was sampled early in the disease process.

There is considerable interest in the long-term brain health of retired contact and collision sport athletes; however, little is known about possible underlying changes in functional brain connectivity in this group. We evaluated whole-brain functional connectivity patterns using multi-voxel pattern analysis (MVPA) to determine whether alterations in functional connectivity distinguish retired professional athletes from a matched group of healthy community control subjects. Thirty-two retired athletes with a history of multiple self-reported sport-related concussions and 36 healthy community control subjects who were similar in age and education, completed functional magnetic resonance imaging. We identified brain regions with abnormal functional connectivity patterns using whole-brain MVPA as implemented in the Conn toolbox. First-level MVPA was performed using 64 principal component analysis (PCA) components. Second-level F test was performed using the first three MVPA components for retired athletes > controls group contrast. Post hoc seed-to-voxel analyses using the MVPA cluster results as seeds were performed to characterize functional connectivity abnormalities from brain regions identified by MVPA. MVPA revealed one cluster of abnormal functional connectivity located in cerebellar lobule V. This region of lobule V corresponded to the ventral attention network. Post hoc seed-to-voxel analysis using the cerebellar MVPA cluster as a seed revealed multiple areas of cerebral cortical hyper-connectivity and hypo-connectivity in retired athletes when compared with controls. This initial report suggests that cerebellar dysfunction might be present and clinically important in some retired athletes.

Background:Parkin mutations are suspected in early-onset Parkinson's disease with early motor complications, and in pedigrees showing an autosomal recessive pattern. Some compound heterozygous mutations can present with various uncommon phenotypes. Case Report:Two siblings with the same mutations, one with atypical postural and action tremor, and the other with an axonal motor autonomic neuropathy. A woman with a 45-year history of slowly progressive parkinsonism with no motor complications. Discussion:Due to the variability of phenotypes of Parkin mutations, testing should also be warranted in patients with atypical tremor syndromes or axonal polyneuropathy when more common causes have been ruled out. Highlights:We report three patients with extremely atypical parkin mutation phenotypes: an atypical tremor syndrome, an axonal motor autonomic neuropathy, and a remarkably slowly progressive parkinsonism. This shows that parkin mutations may present with a highly variable phenotype, and should be considered in patients with such manifestations.