Effects of Fragmentation and the Case for Greater Cohesion in Neurologic Care Delivery.

GOALS: To define fragmentation in neurological care delivery; explain the positive and negative drivers in neurologic practice that contribute to fragmentation; illustrate situations that increase fragmentation risk; emphasize the costs and impact on both patients and providers; propose solutions that allow for more cohesive care. WORK GROUP: The Transforming Leaders Program (TLP) class of 2020 was tasked by American Academy of Neurology (AAN) leadership to identify the leading trends in inpatient and outpatient neurology and to predict their effects on future neurologic practice. METHODS: Research material included AAN data bases, PubMed searches, discussion with topic experts and AAN leadership. RESULTS: Trends in care delivery are driven by changes in the work force, shifts in health care delivery, care costs, changes in evidence-based care and patient factors. These trends can contribute to care fragmentation. Potential solutions to these problems are proposed based on care models developed in oncology and medicine. LIMITATIONS: This paper shares our opinions as there is a lack of evidence-based guidelines as to optimal neurological care delivery.

High Sleep Disturbance and Longer Concussion Duration in Repeat Concussions.

Objectives: The relationship between repeated concussions and sleep disturbance is yet to be fully understood. The objective of this study was to examine the relationship between sleep disturbance, concussion duration, and repeated concussions by assessing postconcussive symptoms and cognition. Methods: Subjects (ages 13-33 years) underwent postconcussion cognitive function evaluation and reported postconcussion symptoms including severity of disturbed sleep (drowsiness, trouble falling asleep, sleeping more than usual, and sleeping less than usual), mood disturbance (sadness, irritability, nervousness, and emotional lability), and headache. Data on cognitive function and concussive symptoms were collected for 430 subjects after first concussion, 192 subjects after second concussion, and 118 subjects after three or more concussions. A subset of subjects (119) were monitored longitudinally to assess concussion duration. Analyses included group comparisons, regression, and correlation; data were adjusted for age and gender. Results: Sleep disturbance differed significantly by group (mean[SEM]: 1st concussion = 2.56[0.2]; 2nd concussion = 3.65[0.34]; 3+ concussions = 4.32[0.43]). Concussion history predicts concussion duration (R2 = 0.20, F[1,116] = 27.33, p < 0.001). Furthermore, trouble falling asleep (ß = 0.15) and sleeping less than usual (ß = 0.15) predicted concussion duration (R2 = 0.062, F[1,116] = 3.15, p = 0.047). Reported sleep disturbance after repeated concussions was higher in patients with higher headache (F[2,732] = 3.15, p = 0.043) and mood disturbance (F[2,733] = 3.35, p = 0.036) severity. In addition, after repeated concussions, the positive correlation between sleep disturbance and cognitive dysfunction strengthened. Conclusions: History of repeated concussion is associated with longer concussion duration and higher reported sleep disturbance. Furthermore, those with sleep disturbance after repeated concussion exhibit more severe headaches, mood disturbance, and cognitive dysfunction.Abbreviations: ImPACT: Immediate Postconcussion Assessment and Cognitive Assessment (ImPACT); this is a computer-based test that assesses an individual's cognitive function and cumulatively documents current concussion symptoms. PCSS: Post Concussion Symptom Scale; this scale assesses the severity of concussion symptoms. SPSS: Statistical Package for Social Sciences. This is a statistical software package.

Executive dysfunction is associated with an altered executive control network in pediatric temporal lobe epilepsy.

OBJECTIVES: Children with temporal lobe epilepsy (TLE) exhibit executive dysfunction on traditional neuropsychological tests. However, there is limited evidence of neural network alterations associated with this clinical executive dysfunction. The objective of this study was to characterize working memory deficits in children with TLE via activation of the executive control network on functional magnetic resonance imaging (fMRI) and determine the relationships to fMRI behavioral findings and traditional neuropsychological tests. EXPERIMENTAL DESIGN: Functional magnetic resonance imaging was conducted on 17 children with TLE and 18 healthy control participants (age 8-16 years) while they performed the N-back task in order to assess activation of the executive control network. N-back accuracy, N-back reaction time, and traditional neuropsychological tests (Delis-Kaplan Executive Function System [D-KEFS] color-word interference and card-sort test) were also assessed. PRINCIPAL OBSERVATIONS: Children with TLE exhibited executive dysfunction on D-KEFS testing, reduced N-back accuracy, and increased N-back reaction time compared with healthy controls; D-KEFS and N-back behavioral findings were significantly correlated. Children with TLE also exhibited significant reduction in activation of the frontal lobe within the executive control network compared to healthy controls. These alterations were significantly correlated with N-back behavioral findings and D-KEFS testing. CONCLUSIONS: Children with TLE exhibit executive dysfunction, which correlates with executive control network alterations. This lends validity to the theory that the executive control network contributes to working memory function. The findings also indicate that children with TLE have network alterations in nontemporal brain regions.

Postconcussive Symptoms After Single and Repeated Concussions in 10- to 20-Year-Olds: A Cross-Sectional Study.

The objective was to characterize cognitive deficits and postconcussive symptoms in a pediatric population with no concussion, a single concussion, and ≥2 concussions, using a cross-sectional design. Cognitive function and postconcussive symptoms were assessed in participants (age 10-20) with no concussion (n = 1118), single concussion (n = 368), and repeated (≥2) concussions (n = 252). Analyses were adjusted for age and gender. Individuals with ≥2 concussions exhibited more total postconcussive symptoms; more loss of consciousness, amnesia and confusion; more headaches; and poorer cognitive function compared to no concussion and single concussion. Postconcussive symptoms may play a modulatory role in cognitive dysfunction after repeated concussions as those with loss of consciousness, amnesia, confusion, or headaches exhibited worse verbal memory, visual memory, visual-motor processing, and poorer impulse control compared to those without these symptoms. This analysis demonstrates that repeated concussions is associated with poorer cognitive function and postconcussive symptoms compared to a single concussion.

Gender differences in sleep symptoms after repeat concussions.

OBJECTIVES: There are notable gender differences in concussion as well as sleep. Sleep disturbance is a major symptom of post-concussive syndrome. The interplay between sleep disturbance, concussion, and gender has yet to be examined. The objective of this study was to determine whether sleep disturbance plays a role in the known gender differences associated with concussion. METHODS: Males and females (ages 10-35 years) completed a pre-concussion and post-concussion evaluation. Levels of sleep disturbance were assessed at baseline (N = 1284), after first concussion (N = 432) and after repeated concussions (one or more concussions, N = 296). Sleep disturbance levels were also compared among males and females with headaches, mood changes, and cognitive dysfunction. Analyses included group comparisons and correlations of post-concussive symptoms and cognitive dysfunction, respectively. Data was adjusted for age. RESULTS: Compared to males, females reported higher levels of sleep disturbance after a single concussion; however, levels of sleep disturbance equalized after repeated concussions. Females with higher levels of sleep disturbance had more headaches and more mood changes requiring medical treatment, compared to males. In addition, sleep disturbance was more strongly correlated with cognitive dysfunction in females compared to males. CONCLUSION: Gender differences in concussion recovery after repeat concussions may be primarily due to a difference in sleep disturbance between the genders. This difference in sleep appears to moderate the levels of other post-concussive symptoms. This indicates that sleep disturbance should be closely monitored and treated in females after a concussion. Further studies are required to determine the underlying reasons for these gender differences.

Gender Differences in Concussion and Postinjury Cognitive Findings in an Older and Younger Pediatric Population.

BACKGROUND: Studies have documented gender differences associated with concussion. The purpose of this study was to determine if these gender differences are also noted within a pediatric population. METHODS: This prospective study analyzed 1971 patients who had completed preconcussion and postconcussion neuropsychological testing within the Washington, DC, area. RESULTS: Our results showed that children and adolescents with concussion exhibit gender differences with respect to risk factors, recovery, and symptomatology. Females are more likely to present with a concussion (P < 0.001), experience more discomfort from a concussion (P < 0.001), and seek treatment for postconcussive headaches (P < 0.001). On the other hand, males are more likely to sustain a concussion from a contact sport (P < 0.001) and experience loss of consciousness, confusion, and amnesia with a concussion more frequently than females (P < 0.001). Postconcussive cognitive function also differs by gender. Both males and females exhibit a decline in cognitive testing compared with baseline (P < 0.001); however, visual memory (P = 0.02) is more affected in females than in males. These findings remain unchanged among pediatric patients aged ≥14 years; however, no gender differences were noted in individuals aged ≤13 years. CONCLUSION: It is important for health care providers, schools, athletic trainers, and coaches to be aware of these gender differences associated with concussion in order to provide adequate surveillance and appropriate monitoring and support during the recovery period.

The utility of infarct volume measurement in pediatric ischemic stroke.

This study aims to determine if stroke volume as measured on diffusion-weighted imaging is associated with neurologic outcome in children with acute arterial ischemic stroke. A cohort of patients presenting to a tertiary care children's hospital with acute ischemic stroke were studied. The relationship between stroke volume, clinical characteristics, and neurologic outcome utilizing the Glasgow Outcome Scale were analyzed. In children with poor outcome, the median volume of infarction on diffusion-weighted imaging was larger when compared with children who had a good outcome. Children with stroke volume >10% of total brain volume were more likely than patients with stroke volume <5% total brain volume to have a poor outcome. Seizures were associated with a 10.5-fold increase in the risk of a poor outcome. Stroke volume, in conjunction with clinical characteristics, can assist practitioners in identifying a subset of patients with acute ischemic stroke who might benefit from aggressive medical and/or surgical management.

Seizures in acute childhood stroke.

OBJECTIVES: To describe the risk of seizures in children with acute stroke and identify factors predicting their later risk of epilepsy. STUDY DESIGN: Data for patients >3.5 years of age at a tertiary care children's hospital with acute stroke were collected and reviewed. RESULTS: Seventy-seven patients were identified (mean age, 8.4 years); 21% had clinical seizures. An additional 10% of patients had a clinical seizure during the acute hospitalization. Status epilepticus was common in infants and patients with cortical strokes. Non-convulsive status epilepticus was captured only in patients with prolonged electroencephalograms and always within 24 hours of monitoring. Six months after their stroke, 24% of our patients had epilepsy, all of whom experienced seizures at initial presentation with stroke. CONCLUSION: In our series of pediatric patients with stroke, most of the clinical seizures occurred within the first 24 hours of presentation and did not vary in stroke subtype. Status epilepticus was common, especially in infants. Epilepsy had a high likelihood of developing in the next 6 months in children with seizures in the first 24 hours of stroke onset. Prolonged electroencephalogram monitoring was useful in detecting non-convulsive status epilepticus, but not in predicting the risk of epilepsy at 6 months.

Adolescent with Rhabdomyolysis due to Undiagnosed Hypothyroidism.

Exercise-induced rhabdomyolysis has been described in military recruits, trained athletes and daily runners. Statin use, quail ingestion, infection by Epstein-Barr virus (EBV), and hypothyroidism, though rare, are risk factors for the development of rhabdomyolysis. We describe the case of a 15-year-old female who presented with myalgias, weakness, and pigmenturia following marching band practice. Laboratory tests confirmed an elevated creatine kinase (CK) level as well as a profound hypothyroid state. Muscle biopsy revealed severe muscle necrosis and myositis. Treatment with levothyroxine resulted in obtaining an euthyroid state and regain of muscle strength as well as decrease in CK levels. Although rare, hypothyroidism should be considered as a potential cause of rhabdomyolysis in pediatric patients undergoing a myopathy workup.

Neurometabolic disorders and dysfunction in autism spectrum disorders.

The cause of autism remains largely unknown because it is likely multifactorial, arising from the interaction of biologic, genetic, and environmental factors. The specific role of metabolic abnormalities also is largely unknown, but current research may provide insight into the pathophysiologic underpinnings of autism, at least in some patients. We review a number of known neurometabolic disorders identified as having an autistic phenotype. We also discuss the possible involvement of mitochondrial disorders and dysfunction as well as a theory regarding an increased vulnerability to oxidative stress, by which various environmental toxins produce metabolic alterations that impair normal cellular function. Finally, we review various strategies for metabolic work-up and treatment. Accurate diagnosis of neurometabolic disorders and a broader understanding of underlying metabolic disturbance even in the absence of known disease have important implications both for individual patients and for research into the etiology of autism.