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Background and Objectives: Longitudinal outcomes in anti-NMDA receptor encephalitis (anti-NMDARe) are still not fully understood and may not be adequately captured with the modified Rankin Scale (mRS), often the sole reported outcome. We aim to characterize longitudinal outcomes in anti-NMDARe using multiple outcome measures., Methods: This single-center, retrospective, observational study examined outcome measures (mRS and Clinical Assessment Scale in Autoimmune Encephalitis [CASE]) in adults with NMDA receptor-IgG in CSF at short- and long-term follow-ups using linear and logistic regression modeling. Patients with evaluations for cognitive impairment (Montreal Cognitive Assessment/Mini-Mental State Examination), depression (Patient Health Questionnaire-9), and anxiety (General Anxiety Disorder-7) >6 months from symptom onset were correlated with final CASE scores., Results: Thirty-eight patients (76% female, median disease onset age = 28 years, range = 1-75 years) were included. The majority received first-line immunosuppressants (97%) at a median of 3.9 weeks (interquartile range [IQR] = 2.1-9.7) from symptom onset and 68% received second-line therapies. At baseline, median/mean mRS and CASE were 4 (IQR = 3-5) and 12.9 (SD = 7.2), respectively. At short-term follow-up (median = 10 weeks, IQR = 6-17), factors associated with higher CASE and mRS included dysautonomia, coma/lethargy, seizures/status epilepticus, and intensive care unit admission ( p < 0.05). At long-term follow-up (median = 70 weeks, IQR = 51-174), median/mean mRS and CASE were 2 (IQR = 1-3) and 4.4 (SD = 4.2), respectively. Only weakness at symptom onset predicted higher mRS scores (odds ratio = 5.6, 95% confidence interval 1.02-30.9, p = 0.047). Despite both mRS and CASE improving from baseline ( p < 0.001), only 9 patients (31%) returned to their premorbid function. Among patients with cognitive and mood evaluations >6 months from onset, moderate-severe cognitive impairment (42%), depression (28%), and anxiety (30%) were frequent. Cognitive and depression measures were associated with final CASE subscores (including memory, language, weakness, and psychiatric)., Discussion: Multiple clinical factors influenced short-term outcomes, but only onset weakness influenced long-term mRS, highlighting that mRS is predominantly affected by global motor function. Although mRS and CASE improved over time for most patients, these outcome measures did not capture the full extent of long-term functional impairment in terms of mood, cognition, and the ability to return to premorbid function. This emphasizes the need for increased utilization of more nuanced cognitive and mood outcome measures.

Background and Objectives: Most acute symptomatic seizure (ASyS) patients stay on antiseizure medications (ASM) long-term, despite low epilepsy development risk. The Post-Acute Symptomatic Seizure (PASS) clinic is a transition of care model for ASyS patients who individualize ASM management with the goal of a safe deprescription. We evaluated patients discharged on ASMs after a witnessed or suspected ASyS to analyze their PASS clinic visit attendance and its predictors., Methods: A single-center, retrospective cohort study of adults without epilepsy who were discharged from January 1, 2019, to September 30, 2019, on first-time ASMs due to witnessed or suspected ASyS (PASS clinic-eligible). We fit a cause-specific Cox proportional hazards model to analyze factors associated with PASS clinic attendance, which depends on survival in this patient population that has a high early postdischarge mortality (a competing risk). We checked for multicollinearity and the assumption of proportional hazards., Results: Among 307 PASS clinic-eligible patients, 95 (30.9%) attended the clinic and 136 (44.3%) died during a median follow-up of 14 months (interquartile range = 2-34). ASyS occurred in 60.2% (convulsive 47%; electrographic 26.7%) of patients. ASMs were continued in the absence of ASyS or epileptiform abnormalities (EAs) in 27% of patients. Multivariable analysis revealed that the presence of EAs (HR = 1.69, 95% CI 1.10-2.59), PASS clinic appointments provided before discharge (HR = 3.39, 95% CI 2.15-5.33), and less frequently noted ASyS etiologies such as autoimmune encephalitis (HR = 2.03, 95% CI 1.07-3.86) were associated with an increased clinic attendance rate. Medicare/Medicaid insurance (HR = 0.43, 95% CI 0.24-0.78, p = 0.005) and the presence of progressive brain injury (i.e., tumors; HR = 0.55, 95% CI 0.32-0.95, p = 0.032) were associated with reduced rate of PASS clinic attendance., Discussion: Our real-world data highlight the need for appropriate postdischarge follow-up of ASyS patients, which can be fulfilled by the PASS clinic model. Modest PASS clinic attendance can be significantly improved by adhering to a structured discharge planning process whereby appointments are provided before discharge. Future research comparing patient outcomes, specifically safe ASM discontinuation in a PASS clinic model to routine clinical care, is needed., Competing Interests: The authors report no relevant disclosures. Full disclosure form information provided by the authors is available with the full text of this article at Neurology.org/cp., (© 2024 American Academy of Neurology.)

Despite the common occurrence left ventricular assist device (LVAD)-associated intracranial hemorrhage, the etiology of intracranial hemorrhage is uncertain. We aim to explore the impact of infection and international normalized ratio (INR) on intracranial hemorrhage in a case-crossover study. We reviewed consecutive patients with intracranial hemorrhage in a prospectively collected data of LVAD patients from a single, tertiary center from October 2004 to December 2016. Information on infection and INR values were collected at the time and 1 month before the intracranial hemorrhage as controls. Of 477 persons with LVAD, 47 (10%) developed intracranial hemorrhage (27 intracerebral, 14 subarachnoid, and 6 subdural hemorrhages). Of 47 (median age 58; 39 males), 27 (54%) persons had active infection at the time of intracranial hemorrhage; seven (21%) of 44 LVADs had infection at 1 month before intracranial hemorrhage. The relative risk of intracranial hemorrhage because of active infection compared with the infections at 1 month was 2.3 (95% CI: 1.5-3.4; p < 0.0001). The mean INRs at the time of intracranial hemorrhage were also significantly higher at the time of hemorrhage compared with those at 1 month (2.6 ± 1.9 vs. 1.8 ± 0.8; p = 0.01). Of 13 persons with cerebral angiogram (seven subarachnoid and six intracerebral hemorrhages), four (57%) infectious intracranial aneurysms were identified only in patients with subarachnoid hemorrhage (SAH) who also had bloodstream infections. Active infection and elevated INR were associated with LVAD-associated intracranial hemorrhage. The occurrence of both bloodstream infection and subarachnoid hemorrhage may indicate the presence of infectious intracranial aneurysm in LVAD.

Background: We describe patient-reported outcomes (PROs) in adults with CNS tumors and evaluate their correlation with physician-reported functional status., Methods: We completed a retrospective cohort study of patients managed at a high-volume CNS tumor institute between September 2013 and September 2014. PROs were measured using 6 domains from the PROs Measurement Information System (PROMIS): anxiety, physical function, pain interference, sleep disturbance, fatigue, and satisfaction with social roles. Physician-reported outcomes were measured using the Eastern Cooperative Oncology Group Scale of Performance Status (ECOG). We compared differences in PROMIS scores across tumor types using analysis of variance and measured the correlation between PROMIS scores and ECOG scores using spearman correlations. Finally, we compared the range of PROMIS physical function scores within each ECOG level., Results: In a cohort of 2,828 patients, 1,284 (45.4%) completed all 6 PROMIS domains. There were significant differences in PROMIS scores across tumor types for all domains except anxiety. The strength of the correlation between PROMIS and ECOG scores was weak to moderate for all PROMIS domains (all p < 0.001). The correlation was the strongest between the physical function domain and ECOG score (ρ = -0.54), although there was a broad distribution of physical function scores within ECOG level, with scores spanning nearly 5 SDs within most ECOG levels., Conclusions: Symptom burden was associated with tumor type. There were only weak to moderate correlations between PROMIS and ECOG scores, underscoring the importance of integrating PROs into clinical practice for patients with CNS tumors.

Background: Patient-reported outcome measures are increasingly being utilized in clinical care and research to evaluate outcomes following stroke. To optimize the clinical utility of these measures, we aimed to quantify meaningful change by establishing minimal important differences (MIDs), or responder definitions, for 4 domains affected in ischemic and hemorrhagic stroke patients., Methods and Results: We performed a retrospective cohort study of stroke patients seen in the Cleveland Clinic cerebrovascular center between September 2, 2012 and November 7, 2017. Four Patient-Reported Outcome Measurement Information System (PROMIS) scales were completed within 1 month poststroke and again at 6 months. MIDs were estimated using an anchor-based approach based on a global impression of change question and supported using 3 distribution-based methods. Cumulative distribution functions assessed responder thresholds. MIDs were additionally derived across sex, race, and varying levels of severity as defined by the modified Rankin Score and baseline PROMIS score. During the study period, 337 incident stroke patients completed at least 1 PROMIS domain scale at both time points (average age 61±14, 56% female). Estimates from the 4 methods were triangulated to provide a MID range across PROMIS domain: 2.5 to 6.5 T-score points for physical function and fatigue, 2.5 to 7.5 for social role satisfaction, and 3.0 to 8.0 for anxiety. Cumulative distribution functions plots identified between 30% and 40% of patients as having meaningful improvement based on the anchor-based estimates across all 4 domains. Meaningful change thresholds remained consistent across categories of sex and race. Anchor-based MIDs increased with increasing severity, whereas distribution-based MIDs remained consistent across severity levels., Conclusions: Our study is the first to evaluate interpretability of changes in PROMIS scores for stroke survivors. Future studies can utilize these thresholds to identify responders of stroke interventions. Based on our estimated MID ranges, researchers and clinicians can choose a responder threshold for comparing change in domain score at the group level, individual level, or by severity.

Objective: To determine the incidence and predictors of acute cerebral ischemia and neurologic deterioration in intracerebral hemorrhage (ICH) patients after an institutional protocol change in systolic blood pressure (SBP) target from <160 to <140 mm Hg., Methods: We retrospectively compared persons admitted with primary ICH before and after a protocol change in SBP target from <160 to <140 mm Hg. The primary outcomes were presence of acute cerebral ischemia on MRI completed within 2 weeks of ICH and acute neurologic deterioration., Results: Of 286 persons with primary ICH, 119 underwent MRI and met inclusion criteria. Sixty-two had a target SBP <160 mm Hg (group 1) and 57 had a target SBP <140 mm Hg (group 2). There were no differences between the 2 groups in baseline clinical and radiographic characteristics, but over the first 24 hours of hospitalization, group 2 had lower mean SBP (134 vs 143 mm Hg, p < 0.001) and lower minimum SBP over 72 hours (106 vs 112 mm Hg, p = 0.02). Acute cerebral ischemia was more frequent in group 2 than in group 1 (32% vs 16%; p = 0.047) as was acute neurologic deterioration (19% vs 5%; p = 0.022). A minimum SBP ≤120 mm Hg over 72 hours was associated with cerebral ischemia, while no patient with a minimum SBP ≥130 mm Hg had cerebral ischemia. Acute cerebral ischemia was significantly associated with worse discharge NIH Stroke Scale score, while SBP target was not., Conclusions: Intensive lowering of SBP <140 mm Hg in acute ICH, particularly allowing SBP <120 mm Hg, is associated with increased remote cerebral ischemic lesions and acute neurologic deterioration., (© 2018 American Academy of Neurology.)

Objectives: (1) Examine 8 patient-reported domains of health across levels of disability compared to the US general population; and (2) identify factors associated with domain scores in patients with ischemic stroke., Methods: Observational cohort study of 1,195 patients in a cerebrovascular clinic from February 17, 2015, to January 27, 2017, who completed Neuro-QoL (Quality of Life in Neurological Disorders) executive function or the following PROMIS (Patient-Reported Outcomes Measurement Information System) scales as part of routine care: physical function, satisfaction with social roles, fatigue, anxiety, depression, pain interference, and sleep disturbance., Results: Mean age was 62 (±15) years, and 81% were white. Median modified Rankin Scale (mRS) score at the clinic visit was 1 (interquartile range 0-2). Percentage of patients with scores meaningfully worse than the general population ranged from 28% (sleep disturbance) to 63% (physical function). Scores were worse in patients with higher mRS levels, although correlation between scores and mRS level varied (sleep disturbance r = 0.16 to physical function r = 0.52). Most affected domains were physical function (T score = 58.8), satisfaction with social roles (T score = 55.4), and executive function (T score = 53.4). Disability, lower income, and female sex were associated with worse scores in multiple domains. Age was associated with worse physical function but lower anxiety, depression, and sleep disturbance., Conclusions: Patients with ischemic stroke reported symptoms in multiple domains that increase to variable degrees at higher levels of disability. Physical function, satisfaction with social roles, and executive function were most affected. This information improves our understanding of the well-being of patients with ischemic stroke and brings attention to the importance of social roles and executive function for stroke survivors., (© 2018 American Academy of Neurology.)

Background: We sought to assess neurologic provider satisfaction with the systematic electronic collection of patient-reported outcome measures (PROMs) for both disease-specific measures and depression screening (Patient Health Questionnaire [PHQ-9])., Methods: A web-based survey was sent to 299 staff physicians and advanced practice providers on the staff email list of a large group neurologic practice, of whom 206 used the PROM system. The survey consisted of 11 questions with Likert response options regarding perceived usefulness of PROM collection; usefulness of PROM data for clinical care, quality, and research activities according to provider age group and type; and perceived usefulness between disease-specific information and the PHQ-9 depression screen., Results: Of those who use the PROM system, 73.3% (151/206) responded. PROM collection was useful for patient care (strongly agree or agree 59.6%), research (strongly agree or agree 68.5%), and to a lesser extent, quality improvement (strongly agree or agree 48.6%). Providers aged 66-75 years believed PROM data were less useful for research ( p < 0.01). PROM collection affected patient interactions or clinical management (always or usually 34.6% for disease-specific information and 31.3% for the PHQ-9). Responses were similar concerning perceived clinical usefulness (strongly agree or agree 67.3%) for center-selected disease-specific PROMs and the mandated PHQ-9 (69.8%)., Conclusions: Providers favorably viewed systematic electronic collection of PROMs in neurologic patients. A mandated depression screening was perceived as favorably as center-selected disease-specific information and should be considered when implementing PROMs in neurologic practice.

Creutzfeldt-Jakob disease (CJD) and other prion diseases are rapidly progressive spongiform encephalopathies that are invariably fatal. Clinical features and magnetic resonance imaging, electroencephalogram, and cerebrospinal fluid abnormalities may suggest prion disease, but a definitive diagnosis can only be made by means of neuropathologic examination. Fluorodeoxyglucose positron emission tomography (FDG-PET) is not routinely used to evaluate patients with suspected prion disease. This study includes 11 cases of definite prion disease in which FDG-PET scans were obtained. There were 8 sporadic CJD cases, 2 genetic CJD cases, and 1 fatal familial insomnia case. Automated FDG-PET analysis revealed parietal region hypometabolism in all cases. Surprisingly, limbic and mesolimbic hypermetabolism were also present in the majority of cases. When FDG-PET hypometabolism was compared with neuropathologic changes (neuronal loss, astrocytosis, spongiosis), hypometabolism was predictive of neuropathology in 80.6% of cortical regions versus 17.6% of subcortical regions. The odds of neuropathologic changes were 2.1 times higher in cortical regions than subcortical regions (P=0.0265). A similar discordance between cortical and subcortical regions was observed between FDG-PET hypometabolism and magnetic resonance imaging diffusion weighted imaging hyperintensity. This study shows that there may be a relationship between FDG-PET hypometabolism and neuropathology in cortical regions in prion disease but it is unlikely to be helpful for diagnosis.

Background: Recently, van Walraven developed a weighted summary score (VW) based on the 30 comorbidities from the Elixhauser comorbidity system. One of the 30 comorbidities, cardiac arrhythmia, is currently excluded as a comorbidity indicator in administrative datasets such as the Nationwide Inpatient Sample (NIS), prompting us to examine the validity of the VW score and its use in the NIS., Methods: Using data from the 2009 Maryland State Inpatient Database, we derived weighted summary scores to predict in-hospital mortality based on the full (30) and reduced (29) set of comorbidities and compared model performance of these and other comorbidity summaries in 2009 NIS data., Results: Weights of our derived scores were not sensitive to the exclusion of cardiac arrhythmia. When applied to NIS data, models containing derived summary scores performed nearly identically (c statistics for 30 and 29 variable-derived summary scores: 0.804 and 0.802, respectively) to the model using all 29 comorbidity indicators (c=0.809), and slightly better than the VW score (c=0.793). Each of these models performed substantially better than those based on a simple count of Elixhauser comorbidities (c=0.745) or a categorized count (0, 1, 2, or ≥ 3 comorbidities; c=0.737)., Conclusions: The VW score and our derived scores are valid in the NIS and are statistically superior to summaries using simple comorbidity counts. Researchers wishing to summarize the Elixhauser comorbidities with a single value should use the VW score or those derived in this study.