BackgroundFrontotemporal lobe disorders (FTD) are amongst the most common brain neurodegenerative disorders. Their relatively covert, frequently subtle presentations and diverse etiologies, pose major challenges in diagnosis and treatments. Recent studies have yielded insights that the etiology in the majority are due to environmental and sporadic causes, rather than genetic in origin.AimsTo retrospectively examine the cognitive and behavioral impairments in the veteran population to garner the range of differing syndrome presentations and etiological subcategories with a specific focus on frontotemporal lobe disorders.MethodologyThe design is a retrospective, observational registry, case series with the collection of epidemiological, clinical, cognitive, laboratory and radiological data on people with cognitive and behavioral disorders. Inclusion criteria for entry were veterans evaluated exclusively at Orlando VA Healthcare System, neurology section, receiving a diagnosis of FTD by standard criteria, during the observation period dated from July 2016 to March 2021. Frontotemporal disorders (FTD) were delineated into five clinical 5 subtypes. Demographic, cardiovascular risk factors, cognitive, behavioral neurological, neuroimaging data and presumed etiological categories, were collected for those with a diagnosis of frontotemporal disorder.ResultsOf the 200 patients with FTD, further cognitive, behavioral neurological evaluation with standardized, metric testing was possible in 105 patients. Analysis of the etiological groups revealed significantly different younger age of the traumatic brain injury (TBI) and Gulf War Illness (GWI) veterans who also had higher Montreal Cognitive Assessment (MOCA) scores. The TBI group also had significantly more abnormalities of hypometabolism, noted on the PET brain scans. Behavioral neurological testing was notable for the findings that once a frontotemporal disorder had been diagnosed, the four different etiological groups consistently had abnormal FRSBE scores for the 3 principal frontal presentations of (i) abulia/apathy, (ii) disinhibition, and (iii) executive dysfunction as well as abnormal Frontal Behavioral Inventory (FBI) scores with no significant difference amongst the etiological groups. The most common sub-syndromes associated with frontotemporal syndromes were the Geschwind-Gastaut syndrome (GGS), Klüver-Bucy syndrome (KBS), involuntary emotional expression disorder (IEED), cerebellar cognitive affective syndrome (CCA), traumatic encephalopathy syndrome (TES) and prosopagnosia. Comparisons with the three principal frontal lobe syndrome clusters (abulia, disinhibition, executive dysfunction) revealed a significant association with abnormal disinhibition FRSBE T-scores with the GGS. The regression analysis supported the potential contribution of disinhibition behavior that related to this complex, relatively common behavioral syndrome in this series. The less common subsyndromes in particular, were notable, as they constituted the initial overriding, presenting symptoms and syndromes characterized into 16 separate conditions.ConclusionBy deconstructing FTD into the multiple sub-syndromes and differing etiologies, this study may provide foundational insights, enabling a more targeted precision medicine approach for future studies, both in treating the sub-syndromes as well as the underlying etiological process.

INTRODUCTION:Traumatic brain injury (TBI) is a leading cause of death and disability, often resulting in prolonged coma and disordered consciousness. There are currently gaps in understanding the factors affecting rehabilitation location and outcome after TBI. OBJECTIVE:To identify the impact of demographics, comorbidities, and complications on discharge disposition in adults with prolonged coma following TBI. DESIGN:Retrospective cohort study. SETTING:Tertiary care hospitals and trauma centers in the United States. PARTICIPANTS:Patients 18 years of age or older with TBI and prolonged coma during the years 2008 to 2015. INTERVENTION:Not applicable. MAIN OUTCOME MEASURES:Demographics, clinical injury data, comorbidities, and complications were collected, and odds ratios (ORs) and descriptive analysis were calculated for mortality, long-term rehabilitation, and home discharge without services. RESULTS:A total of 6929 patients with TBI and prolonged coma were included in the final analysis; 3318 (47.9%) were discharged to rehabilitation facilities, 1859 (26.8%) died, and 1752 (25.3%) were discharged home. Older patients and those with higher injury severity scores had significantly higher ORs for mortality and rehab discharge. A total of 58.3% of patients presented with at least one comorbidity. Non-White ethnicities and self-pay/uninsured patients were significantly less likely to be discharged to a rehab facility. Furthermore, comorbidities including congestive heart failure (CHF) and diabetes were associated with a significantly increased OR for mortality and rehab discharge compared to home discharge without services. CONCLUSIONS:Comorbidities, age, and injury severity were the most significant risk factors for increased mortality and acute rehab discharge. Maximizing the treatment of comorbidities including CHF and diabetes has the potential to decrease mortality and adverse outcomes following TBI with prolonged coma.

Background and Objectives Past studies on poststroke cognitive function have focused on the average performance or change over time, but few have investigated patterns of cognitive trajectories after stroke. This project used latent class growth analysis (LCGA) to identify clusters of patients with similar patterns of cognition scores over the first-year poststroke and the extent to which long-term cognitive outcome is predicted by the clusters ("trajectory groups"). Methods Data were sought from the Stroke and Cognition consortium. LCGA was used to identify clusters of trajectories based on standardized global cognition scores at baseline (T1) and at the 1-year follow-up (T2). One-step individual participant data meta-analysis was used to examine risk factors for trajectory groups and association of trajectory groups with cognition at the long-term follow-up (T3). Results Nine hospital-based stroke cohorts with 1,149 patients (63% male; mean age 66.4 years [SD 11.0]) were included. The median time assessed at T1 was 3.6 months poststroke, 1.0 year at T2, and 3.2 years at T3. LCGA identified 3 trajectory groups, which were characterized by different mean levels of cognition scores at T1 (low-performance, −3.27 SD [0.94], 17%; medium-performance, −1.23 SD [0.68], 48%; and high-performance, 0.71 SD [0.77], 35%). There was significant improvement in cognition for the high-performance group (0.22 SD per year, 95% CI 0.07–0.36), but changes for the low-performance and medium-performance groups were not significant (−0.10 SD per year, 95% CI −0.33 to 0.13; 0.11 SD per year, 95% CI −0.08 to 0.24, respectively). Factors associated with the low- (vs high-) performance group include age (relative risk ratio [RRR] 1.18, 95% CI 1.14–1.23), years of education (RRR 0.61, 95% CI 0.56–0.67), diabetes (RRR 3.78, 95% CI 2.08–6.88), large artery vs small vessel strokes (RRR 2.77, 95% CI 1.32–5.83), and moderate/severe strokes (RRR 3.17, 95% CI 1.42–7.08). Trajectory groups were predictive of global cognition at T3, but its predictive power was comparable with scores at T1. Discussion The trajectory of cognitive function over the first-year poststroke is heterogenous. Baseline cognitive function ∼3.6 months poststroke is a good predictor of long-term cognitive outcome. Older age, lower levels of education, diabetes, large artery strokes, and greater stroke severity are risk factors for lower cognitive performance over the first year.

Treatment of the chronic sequela that persist after a mild traumatic brain injury has been challenging with limited efficacy. The aim of this work was to report outcomes obtained from persons who met the criteria of persisting post-concussive symptoms (PPCS), utilizing a novel combination of modalities in a structured neurorehabilitation program. This work was designed as a retrospective, pre-post chart review of objective and subjective measures collected from 62 outpatients with PPCS a mean of 2.2 years post-injury, before and after a multi-modal 5-day treatment protocol. The subjective outcome measure was the 27-item modified Graded Symptom Checklist (mGSC). Objective outcome measures were motor speed/reaction time, coordination, cognitive processing, visual acuity, and vestibular function. Interventions included non-invasive neuromodulation, neuromuscular re-education exercises, gaze stabilization exercises, orthoptic exercises, cognitive training, therapeutic exercises, and single/multi-axis rotation therapy. Pre-post differences in measures were analyzed using the Wilcoxon signed-rank test, with effect size determined by the rank-biserial correlation coefficient. Pre-post treatment comparisons for the subjective mGSC overall, combined symptom measures, individual components of the mGSC, and cluster scores significantly improved for all items. Moderate strength relationships were observed for the mGSC composite score, number of symptoms, average symptom score, feeling in a "fog," "don't feel right," irritability, and physical, cognitive, and affective cluster scores. Objective symptom assessment significantly improved for trail making, processing speed, reaction time, visual acuity, and Standardized Assessment of Concussion. Patients suffering from PPCS ∼2 years after injury may have significant benefits with some moderate effect sizes from an intensive, multi-modal neurorehabilitation program.

A 59-year-old man presented with non-fluent aphasia and behavioral variant frontotemporal lobe degeneration (FTD), volunteered unusual visual symptoms that were best described as illusory visual spread (the image spreading over a larger area).This type of visual hyperfunction, related to the palinopsia syndromes, has not been reported in association with FTD.The syndrome may be best understood in terms of a visual variant of the environmental dependency syndrome, akin to the verbal variant of forced hyperphasia syndrome.

This case describes a patient who exhibits newfound superlative abilities in painting, music, philosophy, culinary, and performing arts after a traumatic brain injury (TBI) involving the frontal and temporal lobes. Such a dramatic change in de novo artistic behavior after brain injury is rare but has been reported in other patients with frontotemporal dementia, as well as other neurological diseases. Previous studies have shown that mild frontal cortical dysfunction likely plays a role in facilitating creative endeavors and that artistic circuitry is distributed throughout the brain. The neuronal reorganization which occurs after injuries enhances synapse formation and neural plasticity, which may contribute to the acceleration of artistic output after brain injury. This is likely an underdiagnosed phenomenon and a deeper understanding is required to allow clinicians to more effectively recognize and nurture newfound creativity in the setting of brain damage.

We describe a 34-year-old woman with an acute length-dependent smallfiber polyneuropathy (SFP) caused by Coxsackie B3 and B5 and Influenza A, H3N2 viruses.She presented with an acute onset of burning pain and distal sensory deficit in both legs shortly after the acute viral encephalopathy.This is the first report of the serologically confirmed length dependent SFP caused by Coxsackie and Influenza viruses.

We describe a 41 year-old man with painful hand and moving fingers (PHMF) syndrome. He presented with pain in the ulnar distribution of the left forearm and hand, and continuous abduction-adduction confined to the left IV finger. The symptoms started several months after trauma of the left cubital fossa with ulnar nerve injury. Response to therapy has remained suboptimal. We summarized the reported cases of the PHMF.

A 56-year-old woman had multiple, alternating attacks of Bell palsy, associated with lower labial edema and dysgeusia. The facial paresis and edema resolved only partially, and over the years she lost the ability to smile and kiss, and developed lacrimation while eating (figure). Otherwise, neurologic examination, dermal examination, and brain MRI and magnetic resonance angiography were unremarkable. Family history was negative. Notable were negative serology for Lyme, sarcoidosis, HIV, and HbA1C elevation. Melkersson-Rosenthal is a rare syndrome presenting with a triad of alternating or bilateral facial weakness, macroglossia, and, less commonly, fissured tongue, with features sometimes permanent.1,2

Evaluation of the multifaceted nature of frontal network syndromes is uniquely challenging and rarely tested in the acute/sub-acute stroke period. Field-dependent behavior such as imitation behavior, utilization behavior, and environmental dependency syndrome, as a component of altered environmental autonomy, may be a reliable bedside test. This research focused on the frequency of field-dependent behavior in stroke, the subtypes and relation to frontal lobe lesion location and stroke etiology. A validated frontal network score incorporating a 10-point imitation behavior scale was applied to alert patients without significant aphasia, encephalopathy, dementia, or substance abuse. Discriminative validity assessment with magnetic resonance imaging, diffusion weight imaging (MRI-DWI brain) was performed and correlational validity was established using standard neuropsychological tests. Of the stroke patients (n = 1436), those with frontal network symptoms (335/1203; 28%) were analyzed further. In the 73 patients with lesions restricted to the frontal lobes or the frontal subcortical circuits, 56 complied with the 10-point imitation behavior scale testing. Forty-five of 56 (80%) demonstrated imitation behavior (sensitivity 73% and specificity 94%). Correlational validity testing with four commonly used frontal lobe neuropsychological tests was good. The stroke etiology included 26 (59%) "other" causes, 9 (20%) intracerebral hemorrhages, 3 (7%) cardioembolic causes, 3 (7%) large vessel disease, 2 (4%) small vessel disease, and 2 (4%) unknown etiology. Field-dependent behaviour subtypes included imitation behavior (n = 45), utilization behavior (n = 9), environmental dependency syndrome (n = 4), and complex other forms of environmental dependence syndrome (n = 5). It was concluded that imitation behavior is a relatively common occurrence with lesions in the frontal lobes in the acute/sub-acute stroke period and is associated predominantly with non-mainstream (other) stroke causes and intracerebral hemorrhage.