Infant Stroke Associated With Left Atrial Thrombus and Supraventricular Tachycardia.

We report a rare case of cardioembolic stroke in the setting of supraventricular tachycardia (SVT) in an infant. After a week of irritability, a 10-week-old male presented to the emergency department with SVT requiring treatment with adenosine. He developed right-sided hemiparesis and focal motor seizures. Imaging of the brain showed ischemic infarct in the left middle cerebral artery (MCA) territory. Echocardiogram showed a newly formed large left atrial intracardiac thrombus. A coagulopathy workup was negative. He was treated with beta-blocker and anticoagulation therapy. He had mild residual right hemiparesis. During childhood, he developed medically refractory focal epilepsy from the left hemisphere, requiring epilepsy surgery at age 10. A child presenting with sustained SVT can be at increased risk for intracardiac thrombi and stroke.

Child neurology in the 21st century: More than the sum of our RVUs.

In September 2017, the Child Neurology Society (CNS) convened a special task force to review the practice of child neurology in the United States. This was deemed a necessity by our membership, as our colleagues expressed discouragement and burnout by the increase in workload without additional resources; reliance on work relative value units (wRVUs) as the sole basis of compensation; a push by administrators for providers to see more patients with less allotted time; and lack of administrative, educational, and research support. The CNS Task Force designed and distributed a survey to multiple academic divisions of various sizes, as well as to private practices. Our findings were strikingly similar across different practices, demonstrating high workloads, lack of resources, poor electronic medical record support, and high provider symptoms of fatigue and burnout. From the results, the CNS Task Force has concluded that wRVUs cannot be the sole basis of compensation for child neurology. We have also made several specific recommendations for alleviating the current situation, including innovative ways to fund child neurology as well as ways to enhance job satisfaction.

Tourette Syndrome and Comorbid Neuropsychiatric Conditions.

Tourette syndrome is a neuropsychiatric condition characterized by both motor and phonic tics over a period of at least 1 year with the onset in childhood or adolescence. Apart from the tics, most of the patients with Tourette syndrome have associated neuropsychiatric comorbidities consisting of attention deficit hyperactivity disorder, obsessive compulsive disorder, rage attacks, sleep issues, depression, and migraine. Patients may also have physical complications directly from violent motor tics which can rarely include cervical myelopathy, arterial dissection, and stroke. The purpose of this article is to review the associated neuropsychiatric comorbidities of Tourette syndrome with emphasis on recent research.

Variations in EEG discharges predict ADHD severity within individual Smith-Lemli-Opitz patients.

OBJECTIVE: We sought to examine the prevalence of EEG abnormalities in Smith-Lemli-Opitz syndrome (SLOS) as well as the relationship between interictal epileptiform discharges (IEDs) and within-subject variations in attentional symptom severity. METHODS: In the context of a clinical trial for SLOS, we performed cross-sectional and repeated-measure observational studies of the relationship between EEG findings and cognitive/behavioral factors on 23 children (aged 4-17 years). EEGs were reviewed for clinical abnormalities, including IEDs, by readers blinded to participants' behavioral symptoms. Between-group differences in baseline characteristics of participants with and without IEDs were analyzed. Within-subject analyses examined the association between the presence of IEDs and changes in attention-deficit/hyperactivity disorder (ADHD) symptoms. RESULTS: Of 85 EEGs, 43 (51%) were abnormal, predominantly because of IEDs. Only one subject had documented clinical seizures. IEDs clustered in 13 subjects (57%), whereas 9 subjects (39%) had EEGs consistently free of IEDs. While there were no significant group differences in sex, age, intellectual disability, language level, or baseline ADHD symptoms, autistic symptoms tended to be more prevalent in the "IED" group (according to Autism Diagnostic Observation Schedule-2 criteria). Within individuals, the presence of IEDs on a particular EEG predicted, on average, a 27% increase in ADHD symptom severity. CONCLUSIONS: Epileptiform discharges are common in SLOS, despite a relatively low prevalence of epilepsy. Fluctuations in the presence of epileptiform discharges within individual children with a developmental disability syndrome may be associated with fluctuations in ADHD symptomatology, even in the absence of clinical seizures.

Defective motion processing in children with cerebral visual impairment due to periventricular white matter damage.

AIM: We sought to characterize visual motion processing in children with cerebral visual impairment (CVI) due to periventricular white matter damage caused by either hydrocephalus (eight individuals) or periventricular leukomalacia (PVL) associated with prematurity (11 individuals). METHOD: Using steady-state visually evoked potentials (ssVEP), we measured cortical activity related to motion processing for two distinct types of visual stimuli: 'local' motion patterns thought to activate mainly primary visual cortex (V1), and 'global' or coherent patterns thought to activate higher cortical visual association areas (V3, V5, etc.). We studied three groups of children: (1) 19 children with CVI (mean age 9y 6mo [SD 3y 8mo]; 9 male; 10 female); (2) 40 neurologically and visually normal comparison children (mean age 9y 6mo [SD 3y 1mo]; 18 male; 22 female); and (3) because strabismus and amblyopia are common in children with CVI, a group of 41 children without neurological problems who had visual deficits due to amblyopia and/or strabismus (mean age 7y 8mo [SD 2y 8mo]; 28 male; 13 female). RESULTS: We found that the processing of global as opposed to local motion was preferentially impaired in individuals with CVI, especially for slower target velocities (p=0.028). INTERPRETATION: Motion processing is impaired in children with CVI. ssVEP may provide useful and objective information about the development of higher visual function in children at risk for CVI.

Cognitive and EEG fluctuation in benign childhood epilepsy with central-temporal spikes: a case series.

Aware of parental reports of academic variability, we investigated month-to-month fluctuations in cognitive abilities and EEG status by repeated measures testing in six children with benign epilepsy with central-temporal spikes (BECTS). All showed greater than normal test-retest variability. Daytime EEG abnormalities were also variable. Short-term fluctuations in cognitive function appear common in children with BECTS, potentially impacting academic performance.

Impact of age and strain on ischemic brain injury and seizures after carotid ligation in immature mice.

Stroke is an important cause of neurologic injury in the neonatal period and frequently results in lifelong neurologic impairments. We reported previously that unilateral carotid ligation on postnatal day (P)12 in CD1 mice causes acute behavioral seizures and unilateral brain injury and provides a model for neonatal stroke in human infants. In the present study we confirmed that behavioral seizures observed after ligation on P12 in the CD1 strain are associated with rhythmic ictal discharges that show temporal progression on electrocorticograms. We also examined the effects of carotid ligation performed at different ages in CD1 mice or performed in the C57Bl/6 strain. The right common carotid was ligated at P7, P10, P12 or P21 in CD1 mice or at P12 in C57Bl/6 mice. Littermate controls received sham surgery. Seizures were rated for 4h after surgery; brain injury was scored one week later. In a separate group of P12 CD1 mice, electrocorticographic activity was recorded continuously for 4h after carotid ligation or sham surgery. Brain injury and cumulative seizure score varied significantly with age (p<0.001) and strain (p<0.001). In CD1 mice, injury was greatest after ligation on P10 to P12 and seizure score was maximal at P12. Seizure scores were significantly correlated with injury after ligation on P10 or P12. C57Bl/6 mice, like C3Heb/FeJ mice examined previously, were much less vulnerable to seizures and injury than CD1 mice after ligation on P12. This study demonstrates that carotid ligation in the CD1 mouse on P12 causes acute electrographic rhythmic discharges that correlate with behavioral seizures. We also found that the age at which ligation is performed and genetic strain have a strong influence on the severity of injury.

Cortical processing of respiratory afferent stimuli during sleep in children with the obstructive sleep apnea syndrome.

STUDY OBJECTIVES: Children with the obstructive sleep apnea syndrome (OSAS) have blunted upper airway responses to negative pressure, but the underlying cause remains unknown. Cortical processing of respiratory afferent information can be tested by measuring respiratory-related evoked potentials (RREPs). We hypothesized that children with OSAS have blunted RREP responses compared to normal children during sleep. DESIGN: During sleep, RREPs were obtained from EEG electrodes Fz, Cz, Pz during stage 2 sleep, slow wave sleep (SWS), and REM sleep. RREPs were produced with multiple short occlusions of the upper airway. SETTING: Sleep laboratory. PARTICIPANTS: 9 children with OSAS and 12 normal controls. MEASUREMENTS AND RESULTS: Children with OSAS had significantly decreased evoked K-complex production in stage 2 sleep and slow wave sleep and significantly reduced RREP N350 and P900 components in slow wave sleep. There were no significant differences in any of the measured RREP components in stage 2 sleep, and the only REM difference was decreased P2 amplitude. CONCLUSIONS: Results indicate that in children with OSAS, cortical processing of respiratory-related information measured with RREPs persists throughout sleep; however, RREPs during SWS are blunted compared to those seen in control children. Possible causes for this difference include a congenital deficit in neural processing reflective of a predisposition to develop OSAS, or changes in the upper airway rendering the airway less capable of transducing pressure changes following occlusion. Further research is required to evaluate RREPs after effective surgical treatment of OSAS in children, in order to distinguish between these alternatives.

Cortical auditory dysfunction in benign rolandic epilepsy.

PURPOSE: To evaluate cortical auditory function, including speech recognition, in children with benign rolandic epilepsy (BRE). METHODS: Fourteen children, seven patients with BRE and seven matched controls, underwent audiometric and behavioral testing, simultaneous EEG recordings, and auditory-evoked potential recordings with speech and tones. Speech recognition was tested under multiple listening conditions. RESULTS: All participants demonstrated normal speech recognition abilities in quiet, as well as normal peripheral and subcortical auditory function. BRE patients performed significantly worse than controls when speech recognition was tested under adverse listening conditions, including background noise. Five BRE patients who were impaired on two or more tests had centrotemporal spiking on awake EEG. There were no significant group differences in the latency or amplitude of early N100 cortical responses to speech or tones. Conversely, the mismatch negativity, a preattentive index of cortical processing that is elicited passively, was absent or prolonged for speech, but not tones, in BRE patients as compared to controls. DISCUSSION: Children with BRE demonstrated specific speech recognition impairments. Our evoked potential findings indicate that these behavioral impairments reflect dysfunction of nonprimary auditory cortex and cannot be attributed solely to attention difficulties. A possible association between auditory impairments and centrotemporal spiking (>1/min) on awake EEG was identified. The pattern of speech recognition impairments observed is a known risk factor for academic difficulties in school-age children. Our results underscore the importance of comprehensive auditory testing, using behavioral and electrophysiological measures, in children with BRE.

Respiratory-related evoked potentials during sleep in children.

STUDY OBJECTIVES: The respiratory related evoked potential (RREP) has been previously recorded in children and adults during wakefulness and in adults during sleep. However, there have been no data on RREP during sleep in children. We thus examined children during sleep to determine whether early RREP components would be maintained during all sleep DESIGN AND PARTICIPANTS: Twelve healthy, nonsnoring children, aged 5-12 years, screened by polysomnography and found to have no sleep disorders were assessed during stage 2 sleep, slow wave sleep, and REM sleep. Brief occlusions were presented via an occlusion valve at the inspiratory port of a non-rebreathing valve as interruptions of inspiration. EEG responses were averaged and assessed for the presence of early and late RREP components. RESULTS: Robust early components were seen in the majority of subjects in all sleep stages. Late components were also present, although with some apparent differences compared to those previously reported in adults (using the same recording protocol and an almost identical method of stimulus presentation). Specifically, N350 and N550 were less readily differentiated as separate components, and the N550 did not display the clear anterior-posterior amplitude gradient that is ubiquitous in adults. CONCLUSION: Cortical processing of respiratory-related information persists throughout sleep in children. The pattern of activation in the late components appear to reflect differences in the structure of the developing brain prior to the process of dendritic pruning associated with adolescence.

Children with seizures exhibit preferences for foods compatible with the ketogenic diet.

Although highly effective for the treatment of intractable epilepsy, the ketogenic diet is not always included in the treatment option hierarchy presented to families, in part due to perceptions that children will find the high-fat/low-carbohydrate regimen unpalatable. This study assessed if children with seizures exhibit food preferences compatible with the diet, as well as if caregivers were accurate in predicting preferences. Children aged 2-17, with (n=29) and without (n=30) a history of seizures, participated in a paired choice food preference assessment while parents estimated child preferences verbally. Children with seizures exhibited significantly higher preferences for fat versus carbohydrate foods compared with controls, and parents demonstrated low accuracy. Future studies could use similar assessment methods to prospectively track whether such preferences predict diet compliance and/or efficacy. Research into the underlying metabolic basis for this preference and possible related neurophysiological mechanisms in seizure etiology and treatment is warranted.

Social behavior profile in young males with fragile X syndrome: characteristics and specificity.

The present study characterizes distinctive and specific features of social behavior impairment, termed social behavior profile (SBP), in young males with fragile X syndrome (FraX). Fourteen males with FraX and autism (FraX+Aut), ages 3-8 years, were compared with either 41 FraX boys without autism (Aut), 7 age-matched males with developmental language delay and autism (DLD+Aut), or with 11 boys with non-selected (for language delay) idiopathic autism (IA), on several standardized instruments assessing social behavior and autistic features (i.e., autism diagnostic interview-revised, ADI-R). We found that FraX+Aut subjects displayed more impairment in overall cognition, problem/aberrant behavior, and adaptive behavior than the rest of the FraX cohort, even when individuals with pervasive developmental disorder (PDD) were included in the latter. Compared to both DLD+Aut and IA, FraX+Aut males were less impaired in ADI-R reciprocal social interaction (RECS) domain. However, boys with FraX+Aut were in general comparable to DLD+Aut subjects in problem/aberrant and adaptive behaviors. Based on the contrast between FraX+Aut and non-autistic FraX and DLD+Aut, we were able to identify measures (e.g., child behavior checklist (CBCL) withdrawn subscale) that better define social interaction impairment in FraX. Comparisons with DLD+Aut and IA led to the conclusion that communication impairment (COMM) and stereotypic behavior contribute relatively more to the diagnosis of autism in FraX+Aut. In agreement with recent studies, our data suggest that FraX+Aut, and more generally SBP, is a distinctive subphenotype among boys with FraX, which may share some pathophysiological mechanisms with IA.

Secondary narcolepsy in children with brain tumors.

We report two cases of children with disabling daytime sleepiness associated with suprasellar tumors and hypothalamic obesity. Multiple sleep latency testing demonstrated features consistent with severe narcolepsy, with sleep latencies of 0.25 and 0.75 minutes, and REM latencies of 2.1 and 1.5 minutes, respectively. An additional patient with hypothalamic damage secondary to a brain tumor, who was thought to be in a vegetative state, had features of narcolepsy on polysomnography. All children responded well to treatment with stimulants. We speculate that secondary narcolepsy associated with hypothalamic tumors is due to damage or loss of hypothalamic hypocretin-containing neurons. In view of the good response to treatment, we recommend that all children with excessive daytime sleepiness and hypothalamic damage be evaluated for narcolepsy.