Acute neurological diseases requiring hospitalization are relatively rare in women of childbearing age. However, during pregnancy and the postpartum period, several diseases increase in prevalence. Some are unique to the pregnant/postpartum state including preeclampsia and delivery-associated neuropathies. Others, although indirectly related to pregnancy, such as cerebral venous thrombosis, ischemic stroke, and intracerebral hemorrhage, increase in frequency and carry considerable risk of morbidity and mortality. In addition, treatment options are often limited. This review discusses the diseases more commonly seen during pregnancy and the postpartum period, with a focus on emergent neurological diseases and their management. Interventional therapies will also be discussed.

ImportanceIn patients with severe aortic valve stenosis at intermediate surgical risk, transcatheter aortic valve replacement (TAVR) with a self-expanding supra-annular valve was noninferior to surgery for all-cause mortality or disabling stroke at 2 years. Comparisons of longer-term clinical and hemodynamic outcomes in these patients are limited.ObjectiveTo report prespecified secondary 5-year outcomes from the Symptomatic Aortic Stenosis in Intermediate Risk Subjects Who Need Aortic Valve Replacement (SURTAVI) randomized clinical trial.Design, Setting, and ParticipantsSURTAVI is a prospective randomized, unblinded clinical trial. Randomization was stratified by investigational site and need for revascularization determined by the local heart teams. Patients with severe aortic valve stenosis deemed to be at intermediate risk of 30-day surgical mortality were enrolled at 87 centers from June 19, 2012, to June 30, 2016, in Europe and North America. Analysis took place between August and October 2021.InterventionPatients were randomized to TAVR with a self-expanding, supra-annular transcatheter or a surgical bioprosthesis.Main Outcomes and MeasuresThe prespecified secondary end points of death or disabling stroke and other adverse events and hemodynamic findings at 5 years. An independent clinical event committee adjudicated all serious adverse events and an independent echocardiographic core laboratory evaluated all echocardiograms at 5 years.ResultsA total of 1660 individuals underwent an attempted TAVR (n = 864) or surgical (n = 796) procedure. The mean (SD) age was 79.8 (6.2) years, 724 (43.6%) were female, and the mean (SD) Society of Thoracic Surgery Predicted Risk of Mortality score was 4.5% (1.6%). At 5 years, the rates of death or disabling stroke were similar (TAVR, 31.3% vs surgery, 30.8%; hazard ratio, 1.02 [95% CI, 0.85-1.22]; P = .85). Transprosthetic gradients remained lower (mean [SD], 8.6 [5.5] mm Hg vs 11.2 [6.0] mm Hg; P < .001) and aortic valve areas were higher (mean [SD], 2.2 [0.7] cm2 vs 1.8 [0.6] cm2; P < .001) with TAVR vs surgery. More patients had moderate/severe paravalvular leak with TAVR than surgery (11 [3.0%] vs 2 [0.7%]; risk difference, 2.37% [95% CI, 0.17%- 4.85%]; P = .05). New pacemaker implantation rates were higher for TAVR than surgery at 5 years (289 [39.1%] vs 94 [15.1%]; hazard ratio, 3.30 [95% CI, 2.61-4.17]; log-rank P < .001), as were valve reintervention rates (27 [3.5%] vs 11 [1.9%]; hazard ratio, 2.21 [95% CI, 1.10-4.45]; log-rank P = .02), although between 2 and 5 years only 6 patients who underwent TAVR and 7 who underwent surgery required a reintervention.Conclusions and RelevanceAmong intermediate-risk patients with symptomatic severe aortic stenosis, major clinical outcomes at 5 years were similar for TAVR and surgery. TAVR was associated with superior hemodynamic valve performance but also with more paravalvular leak and valve reinterventions.

Familial diabetes insipidus (FDI) is a syndrome of central vasopressin deficiency that is inherited in an autosomal dominant manner and that typically becomes clinically apparent in the first decade of life. Two novel mutations of the vasopressin gene have been identified in two previously unstudied kindreds with FDI. In each kindred, the inheritance of the FDI phenotype was consistent with an autosomal dominant mode of inheritance. In each proband, the diagnosis of central diabetes insipidus had been confirmed previously with a water deprivation protocol. After extraction of genomic DNA from each individual, the three exons of the vasopressin gene were separately amplified by PCR and directly sequenced using an automated dye termination method. In the proband and two other carriers of one kindred, a heterozygous C to T mutation was identified at nucleotide 1857. This is predicted to produce a serine to phenylalanine substitution at residue 56 of the vasopressin-related neurophysin peptide encoded by the mutated allele. The mutation also abolished an MspI site in the vasopressin sequence, and analysis of genomic DNA from eight members of the kindred (five with FDI) confirmed segregation of the mutation with the FDI phenotype. Another member of the kindred, a 13-month-old infant, also has the heterozygous C to T mutation, but a formal water balance study showed no evidence of diabetes insipidus. In the proband of the other kindred, a heterozygous G to A mutation was identified at nucleotide 1873. This mutation would be predicted to cause a cysteine to tyrosine substitution at residue 61 of the neurophysin encoded by the mutated allele. This heterozygous mutation was confirmed by the presence of an RsaI restriction site in one vasopressin allele in two members of the kindred. Therefore, two novel heterozygous mutations of the vasopressin gene have been identified in FDI kindreds. In one kindred, an asymptomatic carrier infant was identified and will require continued observation to determine whether she will develop clinical diabetes insipidus. The presence of these two novel mutations in a region of the vasopressin gene where other FDI mutations have been reported suggests that the part of the neurophysin peptide encoded by these sequences may be critically important in the appropriate expression of vasopressin.

Background: The transition of care from an aggressive disease-directed plan to comfort focused hospice care is a difficult choice for patients with devastating stroke and their family members particularly in the absence of advance directives. However, it is not clear if preexisting Do Not Resuscitate (DNR) status influences the timing of this decision. Objectives: To evaluate factors associated with transfer time to hospice for patients with devastating stroke and, particularly, the influence of defined code status prior to admission. Methods: A retrospective analysis of patients with stroke admitted to inpatient hospice from January 2013-December 2014 at our institution was conducted yielding 71 patients. The group was dichotomized to those with a preexisting DNR (n=19) or not (n=52). Clinical variables and sociodemographic factors were collected and compared using chi-square tests of proportion and t-tests for independent groups. Specifically, median time from admission to inpatient hospice for both groups was compared using a Wilcoxon Ranked Sum test set at 0.05 to test statistical significance. Results: For all patients, the mean age was 82.01 +/- 9.64 years, 60.6% female and 84.5% Caucasian. Approximately 66% of strokes were ischemic and 34% hemorrhagic with a mean NIH score of 21.45 +/- 6.03. Age (83.7 +/- 9.8 vs. 81.4 +/- 9.6; p=0.377 ), ethnicity (% Caucasian: 84.2 vs. 84.6; p= 0.967 ), stroke subtype (% ischemic: 73.7 vs. 61.5; p= 0.343 ), severity (NIH: 21.2 +/- 8.8 vs. 20.9 +/- 4.6; p=0.852 ), insurance status (% Medicare Plus: 73.7 vs. 57.7; p=0.219 ), history of prior stroke (26.3% vs. 23.1%; p=0.777 ), dementia (21.1% vs. 28.9%; p=0.511 ), malignancy (10.53% vs. 13.46%; p=0.742 ), and living arrangement (% living with family: 73.7 vs. 51.9; p=0.132 ) were not statistically different in the DNR and no DNR cohorts respectively. The median time from admission to inpatient hospice for preexisting DNR vs. no DNR did not differ (3.0 vs. 4.5 days ; p=0.176 ). Conclusion: In our study, there were no significant factors, including preexisting DNR status, that influenced transition time to inpatient hospice. Code status on admission is not an indicator of goals of care. Future studies are needed to validate these findings

Background: Acute stroke management in the ED is complex requiring a team-based approach, particularly as the therapeutic window for ischemic strokes has been extended to 24 hours from symptom onset. Objectives: To train and educate neurology, emergency medicine (EM) residents and nursing staff on acute stroke management in the ED using a case simulation based approach. Methodology: We designed five case studies (4 standardized patients and 1 high-fidelity mannequin) exhibiting signs and symptoms of acute stroke. Two anterior circulation large vessel occlusions (one eligible for IV thrombolytic and one eligible for mechanical thrombectomy in the extended time window), one basilar thrombosis requiring intervention, one hypertensive hemorrhage and one stroke mimic were included. A total of 17 PGY 1 EM, 7 PGY 2 neurology, 1 stroke fellow, 5 ED nurses, 3 ED and 2 Neurology faculty members divided into 5 teams rotating through all cases. All residents completed didactic education, a 7 item survey (rated 1-5) and a 10 item quiz on acute stroke management pre- and post-simulation. Each group was allotted 15 minutes to assess the patient, interpret results and advice management followed by a10 minute focused debriefing and feedback session. Objective improvements were assessed using paired T-test analysis of pre and post testing. Results: Pre vs. post simulation self-assessment survey of: I. Knowledge, understanding and management of acute stroke (2.83 +/- 0.82 vs. 3.63 +/- 0.6, p < 0.0001); II. Use of IV-tPA (3.13 +/- 0.85 vs. 4.17 +/- 0.70, p < 0.0001); III. Mechanical thrombectomy (3.00 +/- 0.83 vs. 4.13 +/- 0.74, p < 0.0001); IV. Blood pressure management (3.21 +/- 0.83 vs. 4.12 +/- 0.76, p < 0.0001); V. Team member responsibilities (2.73 +/- 0.98 vs. 4.08 +/- 0.83, p < 0.0001) and VI .Asking for help (3.71 +/- 1.04 vs. 4.58 +/- 0.58, p = 0.0003) improved. The role of neurology in stroke management from the ED perspective remained unchanged (3.75 +/- 0.99 vs. 4.13 +/- 0.74, p = 0.1191), however all quiz results improved (7.33 +/- 1.83 vs. 8.58 +/- 1.35, p = 0.0048). Conclusion: Case based simulation was successful in training and educating neurology and EM residents and ED nursing staff on acute stroke management, improving medical knowledge, communication and effective teamwork.