Predictors of Life Expectancy in Autopsy-Confirmed Alzheimer’s Disease

Background: Life expectancy (LE) following Alzheimer’s disease (AD) is highly variable. The literature to date is limited by smaller sample sizes and clinical diagnoses. Objective: No study to date has evaluated predictors of AD LE in a retrospective large autopsy-confirmed sample, which was the primary objective of this study. Methods: Participants (≥50 years old) clinically and neuropathologically diagnosed with AD were evaluated using National Alzheimer’s Coordinating Center (N = 1,401) data. Analyses focused on 21 demographic, medical, neuropsychiatric, neurological, functional, and global cognitive predictors of LE at AD dementia diagnosis. These 21 predictors were evaluated in univariate analyses. Variables found to be significant were then entered into a forward multiple regression. LE was defined as months between AD diagnosis and death. Results: Fourteen predictors were significant in univariate analyses and entered into the regression. Seven predictors explained 27% of LE variance in 764 total participants. Mini-Mental State Examination (MMSE) score was the strongest predictor of LE, followed by sex, age, race/ethnicity, neuropsychiatric symptoms, abnormal neurological exam results, and functional impairment ratings. Post-hoc analyses revealed correlations of LE were strongest with MMSE ≤12. Conclusion: Global cognitive functioning was the strongest predictor of LE following diagnosis, and AD patients with severe impairment had the shortest LE. AD patients who are older, male, white, and have more motor symptoms, functional impairment, and neuropsychiatric symptoms were also more likely have shorter LE. While this model cannot provide individual prognoses, additional studies may focus on these variables to enhance predictions of LE in patients with AD.

Guillain-Barré Syndrome with Rapid Onset and Autonomic Dysfunction Following First Dose of Pfizer-BioNTech COVID-19 Vaccine: A Case Report

Guillain-Barre syndrome (GBS) is an immune-mediated, often post-infectious illness manifesting as an acute, characteristically monophasic, polyradiculoneuropathy. We present a case of GBS with autonomic involvement following an mRNA-based vaccine against SARS-COV2 (Pfizer/BioNTech mRNA-BNT162b2). A 58-year-old woman presented with fatigue, distal extremity paresthesias, and severe back pain within 3 days after receiving her first vaccine dose. She developed worsening back pain and paresthesias in distal extremities which prompted her initial presentation to the hospital. By the third week post-vaccine, she developed increasing gait unsteadiness, progression of paresthesias, and new autonomic symptoms including presyncopal episodes and constipation. Neurological exam showed bilateral distal predominant lower extremity weakness, decreased sensation in a length-dependent pattern, and areflexia. EMG/NCS showed a diffuse sensorimotor polyneuropathy with mixed demyelinating and axonal features consistent with GBS. She was treated with 2 g/kg of IVIG over 3 days and also received prednisone 60 mg daily for 3 days for severe back pain, with improvement of symptoms. This possible association with mRNA-based vaccination expands the potential triggers for an autoimmune-based attack on the peripheral nervous system.

Abstract 14218: Post-Cardiac Arrest Care Delivery Varies Widely Across Institutions Belonging to the PediRES-Q Collaborative

Introduction: Despite national pediatric post-cardiac arrest care (PCAC) guidelines to improve survival and neurological outcomes, there are limited studies describing PCAC delivery in pediatric institutions. The objective of this study was to describe reported PCAC delivery in pediatric institutions. We hypothesized that there would be variability in PCAC processes across institutions. Methods: An IRB-approved REDCap survey was distributed electronically to the lead resuscitation investigator at each institution belonging to the international Pediatric Resuscitation Quality Improvement collaborative (PediRES-Q). Data were summarized using descriptive statistics. A chi-square test was used to compare categorical data. Results: Twenty-four of 47 centers completed the survey (51%). Most respondents (58%) belonged to large centers (≥1000 annual PICU admissions). Two-third (67%) of centers reported using no specific selection criteria to initiate PCAC with the other third employing order sets, paper forms, or institutional guidelines. Smaller centers (<1000 annual PICU admissions) more frequently reported the use of attending-directed care for PCAC initiation/delivery (80%) versus larger centers (57%), p = 0.04. Common PCAC targets included temperature (96%), glucose (75%), and age-based blood pressure (88%). Most PCAC included EEG (75%) but neuroimaging was only included at 46% of centers. Duration of PCAC was either tailored to clinical improvement and neurological exam (54%) or time-based (45%). Only 25% of centers reported having a mechanism for evaluating PCAC adherence. Common barriers to effective PCAC implementation included lack of time and limited training opportunities (Figure 1). Conclusions: There is wide variation in PCAC delivery among surveyed pediatric institutions. Targeting common themes such as standardization of PCAC initiation and bundle components and implementing adherence evaluation could improve PCAC.

INNV-38. COVID-19 INDUCED TELEMEDICINE LESSONS FOR CLINICIANS CARING FOR PATIENTS WITH PRIMARY BRAIN TUMORS

BACKGROUND The COVID-19 pandemic triggered a rapid conversion from in-person to video-visits for new patient consultations and follow-up visits. Now with available vaccines and declining case rates efforts are underway to return to in-person visits as they provide more revenue and are viewed as best for patients and clinicians. This abstract reviews these assumptions. METHODS Electronic medical records from seven full-time faculty neuro-oncologists at Johns Hopkins Hospitals were retrospectively reviewed from 4/1/20 to 3/31/21 to examine the use of video visits over time and their patient demographics. RESULTS From 4/1/20 to 3/31/21, 279 new patients were seen (57% video-visits) with a median age of 52 years for both video and in-person visits. Patients came from 15 states for video and 17 states for in-person visits. There were also 2247 follow-up visits (85% video-visits) with a median age of 47 yrs for video and 50 yrs for in-person visits. Patients came from 28 states for video and 14 for in-person visits. No show visits were more frequent for in-person visits. During early months of the pandemic, few patients were seen in the clinics. Thereafter, video-visits rose sharply comprising 93% of follow-up visits in June 2020 and 62% of new patient consultations in September 2020. These rates have remained high (in March 2021, 72% of all follow-up visits and 59% of new patient consultations). CONCLUSIONS Despite reductions in COVID-19 infection rates, our neuro-oncologists continue to favor video-visits for new patient consultations and follow-up visits. Video-visits save patients and caregivers travel time, parking costs, and time away from work. They also allow an acceptable history and neurological exam, participation by many family members, easy sharing of MRI scans and laboratory data, and discussions unencumbered by face masks and shields. These advantages to video-visits remain significant even as the pandemic recedes.

Migraine Headache as a Presenting Feature of Multiple Sclerosis: A Case Report

Multiple sclerosis (MS) is a chronic inflammatory disease, causing neuronal demyelination and axonal damage in the central nervous system. Symptoms of MS vary widely because of different grades of sensory, motor, and cognitive dysfunctions. Although headache as the initial symptom of MS is rare, it is a common comorbidity that affects most patients. However, it is unclear that the headache manifestation in newly diagnosed people with MS should be considered as an MS attack or merely a comorbid condition. We report the case of a 31-year-old woman with newly diagnosed MS who presented with exacerbation of headache episodes without any abnormal neurological exam findings. The headaches did not respond to nonsteroidal anti-inflammatory drugs and triptans. After administration of methylprednisolone, the headaches were significantly improved, and during 3 months of follow-up receiving glatiramer acetate, no episode of headache has occurred. This case demonstrates the possible relationship between migraine and MS in newly diagnosed patients. New-onset headaches, a change in the pattern of previous episodes, and inadequate clinical drug response to headache treatment should all be taken seriously and warrant further investigation. Thereby, early diagnosis and proper treatment for patients with MS could improve their quality of life.

Association of Network Connectivity via Resting State Functional MRI with Consciousness, Mortality, and Outcomes in Neonatal Acute Brain Injury

Background An accurate and comprehensive test of integrated brain network function is needed for neonates during the acute brain injury period to inform on morbidity. This retrospective cohort study aimed to assess whether integrated brain network function by resting state functional MRI, acquired during the acute period in neonates with brain injury, is associated with acute exam, neonatal mortality, and 5-month outcomes. Methods This study included 40 consecutive neonates with resting state functional MRI acquired 1-22 days after suspected brain insult from March 2018 to July 2019 at Phoenix Childrens Hospital. Acute period exam and test results were assigned ordinal scores based on severity as documented by respective treating specialists. Analyses (Fisher exact, Wilcoxon-rank sum test, ordinal/multinomial logistic regression) examined association of resting state networks with demographics, presentation, neurological exam, electroencephalogram, anatomical MRI, magnetic resonance spectroscopy, passive task functional MRI, and outcomes of discharge condition, outpatient development, motor tone, seizure, and mortality. Results Subjects had a mean (standard deviation) gestational age of 37.8 (2.6) weeks, a majority were male (63%), with diagnosis of hypoxic ischemic encephalopathy (68%). Other findings at birth included mild distress (48%), moderately abnormal neurological exam (33%), and consciousness characterized as awake but irritable (40%). Significant associations after multiple testing corrections were detected for resting state networks: basal ganglia with outpatient developmental delay (odds ratio [OR], 14.5; 99.4% confidence interval [CI], 2.00-105; P<.001) and motor tone/weakness (OR, 9.98; 99.4% CI, 1.72-57.9; P<.001); language/frontal-parietal network with discharge condition (OR, 5.13; 99.4% CI, 1.22-21.5; P=.002) and outpatient developmental delay (OR, 4.77; 99.4% CI, 1.21-18.7; P=.002); default mode network with discharge condition (OR, 3.72; 99.4% CI, 1.01-13.78; P=.006) and neurological exam (P=.002 (FE); OR, 11.8; 99.4% CI, 0.73-191; P=.01 (OLR)); seizure onset zone with motor tone/weakness (OR, 3.31; 99.4% CI, 1.08-10.1; P=.003). Resting state networks were not detected in only three neonates, who died prior to discharge. Conclusions This study provides level 3 evidence (OCEBM Levels of Evidence Working Group) that the degree of abnormality of resting state networks in neonatal acute brain injury is associated with acute exam and outcomes. Total lack of brain network detection was only found in patients who did not survive.

Heterogeneous development of children with Congenital Zika Syndrome-associated microcephaly

Objective To describe the neurological and neurodevelopmental outcomes of children with Congenital Zika Syndrome (CZS) associated microcephaly beyond 2 years of age. Method We followed children with CZS-associated microcephaly in an outpatient clinic in Salvador, Brazil. Neurological and neurodevelopmental assessments were performed using the Hammersmith Infant Neurological Examination (HINE) and Bayley Scales of Infant and Toddler Neurodevelopment (Bayley-III) respectively. Results Of the 42 children included, 19 were male (45.2%); median (interquartile range) age at neurological evaluation was 28 (25–32) months, and 36 (85.7%) had severe microcephaly. HINE and Bayley-III results were completed for 35/42 (83.3%) and 33/42 (78.5%) children respectively. Bayley-III identified a severe developmental delay in 32/33 (97.0%) children while 1/33 (3.0%) had only a mild delay. In the multivariable analysis, we found that Bayley-III and HINE scores were correlated. Better HINE scores were associated with higher Bayley-III cognitive raw scores (β = 0.29; CI 95% = 0.02–0.57) and motor raw scores (β = 0.43; CI 95% = 0.04–0.82) after adjusting for head circumference, prematurity, and age at neurodevelopmental evaluation. Furthermore, we found that greater head circumference at follow up was associated with higher cognitive (β = 1.27; CI 95% = 0.01–2.53) and motor raw scores (β = 2.03; CI 95% = 0.25–3.81). Conclusion Children with CZS-associated microcephaly demonstrate severe neurodevelopmental delays and slower growth rates than their peers over time. Still, they have remarkably heterogeneous neurodevelopmental profiles according to neurological exam scores which correlate with their long-term outcomes. We found that HINE scores effectively captured the heterogeneity of neurological capabilities among these children and could be predictive of cognitive and motor development progress.

Fatigue and cognitive impairment in post-COVID syndrome: possible treatment approaches

Post-COVID syndrome can develop in all patients who have had COVID-19, regardless of the disease severity. Clinical manifestations postCOVID syndrome vary greatly, but the most common symptoms include fatigue, anxiety and depression disorders (ADDs), and cognitive impairment (CI).Objective: to evaluate the efficacy and safety of Cholytilin (choline alfoscerate) and the combined drug MexiB 6 in patients with post-COVID syndrome and fatigue, ADDs, and CI.Patients and methods. The study included 100 patients aged 22 to 71 years who have had COVID-19 5.4 months ago. Inclusion criterion: cognitive complaints, fatigue, and emotional disturbances. The evaluation included neurological exam, Montreal Cognitive Assessment Scale (MoCA), Frontal Assessment Battery (FAB), 10-words list task, Multidimensional Fatigue Inventory (MFI-20), Hospital Anxiety and Depression Scale (HADS). Study participants were divided into two groups. Patients who had ADDs (anxiety/depression level according to HADS ≥8 points; n=50) were prescribed with MexiB 6 (1 tablet three times per day). Patients with CI (mean MoCA score ≤25 points; n=50) were prescribed with Cholytilin (2 capsules (800 mg) in the morning and 1 capsule (400 mg) at lunchtime). The follow-up period was 60 days.Results and discussion. According to MoCA scores, a decrease in cognition was observed in 58% of participants, while 28% did not notice CI earlier. ADD fere present in 51%, and fatigue — in 100% of patients. We observed a significant reduction in fatigue severity (from 62.42±7.18 to 52.32±16.36 points; p<0.05) in patients prescribed with MexiB 6. The majority of patients noted a significant increase in physical activity, decreased fatigue, improvement of attention and physical well-being, and increased workplace efficiency. We also found a significant decrease in ADDs severity: ADDs either regressed completely (in 42% of participants) or became subclinical (in 48%; р<0.001). CI severity also reduced according to mean МоСА (from 26.60±1.31 to 27.28±1.39 points; p<0.05) and FAB (from 16.98±1.06 to 17.20±0.90 points; p<0.05) scores. In a subgroup of patients with mild CI treated with Cholytilin mean МоСА (from 23.50±0.99 to 26.36±1.34; р<0.001) and FAB (from 16.02±0.91 to 16.96±0.99; р<0.001) scores significantly increased. Complete regression of CI was observed in 74% of participants (р<0.001). We also found a decrease in ADDs (р<0.001) and fatigue (mean MFI-20 scores decreased from 42.28±10.73 to 35.60±8.10; р<0.001) severity in all study participants.Conclusion. Patients who have had COVID-19, regardless of the disease severity, have a high prevalence of fatigue, ADDs and CI, and MexiB 6 and Cholytilin have a potential in their treatment.

NUMERICAL RATING SCALE AS A PREDICTOR OF AN UNDERLYING PATHOLOGY IN PEDIATRIC BACK PAIN USING MAGNETIC RESONANCE IMAGE AS DIAGNOSTIC TOOL

Background & Objectives: Recently, constant and night pain has been discarded as adequate clinical markers to predict the presence of an underlying pathology in pediatric back pain. The pain intensity has been recognized as an important domain in the pain assessment. Numerical Rating Scale (NRS) is one of the most common validated tools to assess pediatric pain intensity in children above 8 years of age. The aim of this study is to assess NRS as a predictor of underlying pathologies found by magnetic resonance image (MRI) in pediatric back pain. We hypothesize that a higher NRS score is associated with a high sensitivity, specificity and likelihood ratio to identify the present of organic pathology in pediatric chronic back pain. Methodology: After obtaining Institutional Review Board approval, a retrospective electronical medical record review was conducted. All pediatric patients who reported back pain lasting > 4 weeks between 2009 to 2018 were enrolled in the study. As per regular protocol, a pediatric orthopedic surgeon evaluated all patients who presented with back pain. After a non-diagnostic history, physical examination and spinal x-ray; spine MRI was order. Pain was graded with the use of NRS from 0 to 10. Patients were divided in two groups: NRS (1-5) & NRS (6-10). Variables such as gender, age, pain frequency, night pain, neurological exam, and the presence of an underlying pathology were compared between both groups. Patients that presented with injury due to trauma, previous diagnosis of back pain or cervical pain were excluded. Results: A total of 467 patients were evaluated in the study. Mean age of subjects was 15 years; 69% being female. An underlying pathology was identified in 131/315 (41.6%) patients with NRS (6-10), and 55/152 (36.2%) patients with NRS (1-5) (P=0.27). Patients with NRS (6-10) had two times more probability of suffering constant pain (P<0.03) and three times more likely of having an abnormal neurological examination (P<0.05). See table 1. Conclusion: Evaluation and treatment of children and adolescent with chronic back pain is challenging. Our study shows a strong association between NRS high (6-10) and constant pain and/or abnormal neurological exam. However, the use of NRS of (6-10) was not found as adequate predictor for the presence of an underlying organic pathology in children and adolescent patients. Therefore, physicians should not rely only high NRS score to recommend advance imaging study to assess chronic back pain in children and adolescent patients. Summary [Table: see text]