Child Neurology Research Articles

Cerebral Palsy in Child Neurology and Neurodevelopmental Disabilities Training: An Unmet Need.

Cerebral palsy (CP) is the most common cause of childhood motor disability. However, there is limited guidance on training of child neurologists and neurodevelopmental disability specialists in the care of individuals with cerebral palsy. We sought to determine training program directors' impressions of the importance and adequacy of training in the diagnosis and management of cerebral palsy. In this cross-sectional study, all 82 child neurology and neurodevelopmental disability program directors were asked to complete a survey querying program characteristics, aspects of training in cerebral palsy, importance of cerebral palsy training, and perceived competence at graduation in cerebral palsy care. There were 35 responses (43% response rate). Nearly all program directors (91%) reported "learning to diagnose cerebral palsy" as very important, and most (71%) felt that "learning to manage cerebral palsy" was very important. Although most program directors reported trainees to be very or extremely competent in cerebral palsy diagnosis (77%), only 43% of program directors felt that trainees were very or extremely competent in cerebral palsy management. Time spent with cerebral palsy faculty was associated with higher reported competence in cerebral palsy diagnosis (P = .03) and management (P < .01). The presence of a cerebral palsy clinic was associated with higher reported competence in cerebral palsy management (P = .03). Child neurology and neurodevelopmental disability program directors reported that training in cerebral palsy is important for residents; however, a significant proportion felt that residents were not very well prepared to manage cerebral palsy. The development of cerebral palsy curricula and exposure to cerebral palsy clinics may improve training, translating to better care of individuals with cerebral palsy.

Pediatric Critical Care Neurologists in the United States and Canada: A Survey of Clinical Practice Experience.

To describe the characteristics of pediatric intensive care neurologists and their practice in the United States and Canada. We performed a survey-based study of child neurologists who self-identify as 'intensive care neurologists'. The survey included questions about demographics, training, pediatric neurocritical care service and job structure, teaching, academics, challenges, and views on the future of pediatric neurocritical care. We analyzed 55 surveys. Most respondents were 31-50 years of age with ≤10 years of practice experience. Fifty-four percent identified as female. Most completed subspecialty training after child neurology residency. The majority practice at highly resourced centers with >45 intensive care unit beds. Respondents cover a variety of inpatient (critical and noncritical care) services, at times simultaneously, for a median of 19.5 weeks/y and work >70 hours/wk when on service for pediatric neurocritical care. The top 3 challenges reported were competing demands for time, excess volume, and communication with critical care medicine. Top priorities for the "ideal pediatric neurocritical care service" were attendings with training in pediatric neurocritical care or a related field and joint rounding with critical care medicine. We report a survey-based analysis of the demographics and scope of practice of pediatric critical care neurologists. We highlight challenges faced and provide a framework for the further development of this rapidly growing field.

Masking for School-Age Children With Epilepsy: We Do Have Consensus!

This study was designed to assess current recommendations from child neurologists and epileptologists on masking for school-age children with epilepsy. A 7-item survey was created and sent out to members of the Child Neurology Society and Pediatric Epilepsy Research Consortium in August of 2021 to assess current practice and provider recommendations on masking. One hundred four individuals participated with representation from all regions of the United States. Masking was recommended by 95.1%, with 63.4% (n = 66) noting exception of those with severe intellectual disability, autism, and behavioral problems. Of those who write exemption letters, 54% write these <5% of the time. Only 3% reported potential adverse events associated with masking. Nearly all respondents recommended masking for school-age children with epilepsy. Potential risks of masking and adverse events were low. Improved guidance on masking is needed to ensure academic success of our patients with epilepsy.

Neurological, cognitive and learning evaluation of students who were born preterm.

ABSTRACTObjective: To evaluate the cognitive and academic profile of preterm newborns at school age and to determine the factors related to prematurity and sociodemographic profile that influence these results.Methods: Patients aged 6-14 years old that were assisted in the preterm follow-up clinic were recruited. The cognitive, academic, and neurological capacities were accessed through a detailed evaluation with a child neurologist, a neuropsychologist and a psychopedagogue. Neonatal data were collected from patient records.Results: 97 children were included and 14 were excluded from the study, resulting in 83 children. Gestational age (GA) was 30±3 weeks and weight at birth was 1138g (605 to 4185g). Poor performance was shown in 38.4% for writing, 57.5% for reading and 42.5% for mathematics. The mean total intelligence quotient (IQ) was 96±14.9 points, and 10.9% were considered altered. Children with unstructured families presented 78.3% of failure in reading tests (p=0.029). The multivariate analysis showed association between GA at birth and classic mini-mental score (p=0.043), total IQ (p=0.047), perceptual organization IQ (p=0.035), and processing speed IQ (p=0.036). There was also association between weight at birth and the classic (p=0.004) and adapted (p=0.007) mini-mental scores; invasive mechanic ventilation duration and classic mini-mental (p=0.049); and lower maternal age and processing speed IQ (p=0.033).Conclusions: Preterm infants at school age had high frequency of failure in cognitive and academic evaluation tests. Learning difficulties are high among them. Multiple neonatal variables are related with altered cognitive and students development.

An Article-Based Format for Medical Specialty and Subspecialty Maintenance of Certification.

This article describes an article-based alternative for maintenance of certification that the American Board of Psychiatry and Neurology developed and began pilot testing in 2019. The rationale for and components of the pilot program are presented along with data on participant performance and feedback from the first year of implementation in three primary specialties (neurology, child neurology, and psychiatry) and one subspecialty (child and adolescent psychiatry). Evaluation of the pilot program was guided by a widely used validity framework. Data were collected that addressed the five categories of validity evidence: content, response process, internal structure, relation to other variables, and consequences. Enrollment ranged from 66.7% for psychiatrists to 75.3% for child neurologists. For the 2019 cohort, the pass rates ranged from 92.6% for child and adolescent psychiatry to 98.7% for neurology, and very small numbers of diplomates failed or did not complete the process. For psychiatrists, there was a modest, but significant, relationship between performance on previous and subsequent maintenance of certification examinations. Ninety percent or more agreed that: the articles were easy to access and helpful to their practices; the mini-tests were a fair assessment of their understanding of the articles; and their test-taking experience was satisfactory. Most eligible diplomates participated in the article-based pilot project, and they strongly preferred this format to the traditional multiple-choice examinations. Most important, the pilot was perceived to be a meaningful and relevant learning activity that had a positive effect on patient care.

Role of clinical psychologists in child neurology

Role of clinical psychologists in child neurology

Neuroimaging Findings of the High-risk Neonates and Infants Referred to Mofid Children's Hospital.

Neuroimaging in high-risk neonates and infants is done to help child neurologists predict the future neurodevelopmental outcome of these children. In this study, we assessed high-risk neonates and infants admitted to the NICU or neonatal wards of Mofid children's Hospital, especially regarding clinical development and brain imaging. This cross-sectional study was conducted on 170 patients admitted to the neonatal and NICU ward of Mofid children's Hospital. Considering the inclusion criteria, 112 patients were included in this project. Brain ultrasonography was performed on almost all of these babies by a single radiologist. Some patients underwent a brain CT scan, and brain MRI without contrast was done on the others. These images were interpreted and compared by a single pediatric neuro-radiologist blinded to clinical data. All of these babies were followed up until 18 months of age. In this study, 57.1% of the patients were male and 42.9% were female. Of 44 patients who obtained Electroencephalogram (EEG) during the hospitalization period with probable seizure, 25 (56.8%) had normal EEGs. Of 89 babies who were examined by ultrasound, 19 (21.3%) had abnormal findings; ventriculomegaly and then germinal matrix hemorrhage (GMH) were the most common abnormalities. Also, 27 cases (71.1%) of 38 patients undergoing a CT scan had abnormal findings. The most common findings were a hypodense area in the white matter and ventriculomegaly. Of 41 patients who underwent MRI between 1 and 27 months, 34 cases (82.9%) had an abnormal MRI. The most common findings were periventricular hyperintensities in 17 cases (41.5%), mildly delayed myelination in 15 cases (36.6%), and severe brain atrophy or thinning of corpus callosum or white matter volume loss in seven cases (17.1%). During the follow-up period, which was 18.55 ± 6.56 months, 79 (70.5%) of the children had normal development and 33 (29.5%) were suffering from a global neurodevelopmental delay. More precisely, 49 (43.7%) and 35 (31.2%) patients had motor development delay and delayed verbal development, respectively. The abnormal findings of brain imaging in the ultrasound, CT scan, and MRI were all significantly associated with an adverse neurodevelopmental outcome (P <0.001, P = 0.02, and P <0.001, respectively). In this study, we showed that at any time before six months or after one year of age, the result of brain MRI was a strong predictor of the patient's outcome.

Perspective on transition from pediatric to adult health care for patients with neurological disease: current situation and issues

An improvement in efficacy treatment and development of the social support system has led to many patients with neurological disease being able to reach adulthood. Therefore health care for life from pediatrics to adulthood has become necessary. The Special Committee for Measures Against Transition from Pediatric to Adult Health Care of the Japanese Society of Neurology officially started to examine the current situation and issues of transition from pediatric to adult health care in July 2020. Pediatric neurologists and adult neurologists have an awareness of this issue of constructing a better transition from pediatric to adult health care. However, there are some tasks that need to be resolved in the medical system. We intend to improve the understanding of transition and assessment of medical service fees for transition in cooperation with the Japanese Society of Neurology and the Japanese Society of Child Neurology.

Anything But Typical.

A graduating child neurology resident reflects upon how her first neurology “patient” single-handedly taught her an entire textbook worth of knowledge and became the guiding force that led her to leave general pediatrics.

To the further development of medical safety in the field of child neurology

To the further development of medical safety in the field of child neurology

Epilepsy in Angelman syndrome

Angelman's syndrome (AS) is accompanied by specific changes in the EEG and genetically determined epilepsy. To analyze the neurological status, changes on EEG, MRI, the course of epilepsy in patients with Angelman syndrome (observed at the Svt. uca`s Institute of Child Neurology and Epilepsy). 47 patients with a genetically verified diagnosis of AS (aged 2 to 20 years, mean age 8.5 years; 26 boys and 21 girls) were included. The diagnosis was established by DNA methylation in 32 patients and sequencing in 15 patients (12 cases of deletion and 3 cases of nucleotide substitution were identified). Of the 47 patients, 45 have epilepsy. The seizures start up to 5 years of age, inclusive. For treatment, patients received various antiepileptic drugs. Long-term follow-up of epilepsy was followed in 40 of 47 patients, and 36 of 40 achieved drug remission. After several years without seizures, 24 out of 30 had a relapse, which was quickly stopped in 23 out of 30 patients. The severity of the disease is influenced by the nature of the mutation and the length of the deletion, as well as persistent epileptic seizures. The most effective AEDs in patients in our study are: in monotherapy, valproic acid, levetiraceiam, ethosuximide; in duotherapy, valproic acid in combination with levetiracetam or ethosuximide, less often levetiracetam with ethosuximide. Early genetic diagnosis of AS facilitates the selection of AET.

The impact of birthweight on the development of cerebral palsy: A population-based matched case-control study

BackgroundCerebral palsy (CP) is a common cause of physical impairment in children, especially in newborns who are small for gestational age (SGA). AimsThe aim of our study was to investigate the association between birth weight and the risk of developing CP, controlling for gestational age and plurality. Study designThis retrospective, observational, case-control study was based on Slovenian Registry of Cerebral Palsy (SRCP) and Slovenian National Perinatal Information System (NPIS) data for the period 2002 to 2010. SubjectsFor each pregnancy that resulted in the birth of the newborn(s) who later developed CP (n = 254), three pregnancies with newborns who did not develop CP (n = 762) were selected and matched for gestational age and plurality. Outcome measuresDiagnosis of CP was made at age 5 years or older by a developmental pediatrician trained in child neurology or a child neurologist using standard measures. ResultsRisk of CP increased progressively as birth weight percentiles fell below the 50th centile, with children in the lowest percentiles at greatest risk. Birth weight percentiles traditionally classified as SGA were an independent risk factor for developing CP, with an odds ratio of 2.43 (95% confidence interval 1.57, 3.73). ConclusionsThe results of this study suggest that the risk for developing CP is inversely related to birth weight, even at birth weights that do not meet the standard definitions of SGA. Synopsis - study questionDoes birth weight represent a potential risk factor for the development of cerebral palsy (CP) when controlling for gestational age and plurality? What's already knownNewborns who are small for gestational age (SGA) are at higher risk of developing CP according to published studies. However, different definitions of SGA (birth weight below the 10th, 5th, or 3rd percentile for gestational age) have been used by researchers and clinicians, making it difficult to compare studies. What this study addsThis study suggests that the risk of developing CP is inversely related to birth weight, even at birth weights that do not meet standard definitions of SGA.

Movement disorders in MCT8 deficiency/Allan-Herndon-Dudley Syndrome

Background and objectivesMCT8 deficiency is a rare genetic leukoencephalopathy caused by a defect of thyroid hormone transport across cell membranes, particularly through blood brain barrier and into neural cells. It is characterized by a complex neurological presentation, signs of peripheral thyrotoxicosis and cerebral hypothyroidism. Movement disorders (MDs) have been frequently mentioned in this condition, but not systematically studied. MethodsEach patient recruited was video-recorded during a routine outpatient visit according to a predefined protocol. The presence and the type of MDs were evaluated. The type of MD was blindly scored by two child neurologists experts in inherited white matter diseases and in MD. Dystonia was scored according to Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS). When more than one MD was present, the predominant one was scored. Results27 patients were included through a multicenter collaboration. In many cases we saw a combination of different MDs. Hypokinesia was present in 25/27 patients and was the predominant MD in 19. It was often associated with hypomimia and global hypotonia. Dystonia was observed in 25/27 patients, however, in a minority of cases (5) it was deemed the predominant MD. In eleven patients, exaggerated startle reactions and/or other paroxysmal non-epileptic events were observed. ConclusionMDs are frequent clinical features of MCT8 deficiency, possibly related to the important role of thyroid hormones in brain development and functioning of normal dopaminergic circuits of the basal ganglia. Dystonia is common, but usually mild to moderate in severity, while hypokinesia was the predominant MD in the majority of patients.

An Initiative to Increase Residency Program Diversity

A physician workforce that reflects the patient population is associated with improved patient outcomes and promotes health equity. Notwithstanding, racial and ethnic disparities persist within US medical schools, making some individuals underrepresented in medicine (URM). We sought to increase the percentage of URM residents who matched into our pediatric residency programs from a baseline of 5% to 35% to achieve demographic parity with our patients. We developed a multifaceted approach using multiple iterative tests of change, with the primary strategy being increased visibility of URM trainees and faculty to residency applicants. Strategies included applicant interviews with URM faculty, interview dinners with URM residents, visibility at academic conferences for URM trainees, development of targeted marketing materials, and a visiting student program supported by networking with URM residents. The primary outcome measure was the percentage of matched residents in the categorical pediatrics, child neurology, and medical genetics training programs who identified as URM. The percentage of URM residents increased to 16% (6 of 37) in 2018, 26% (11 of 43) in 2019, 19% (8 of 43) in 2020, and 21% (9 of 43) in 2021 (a four-year average of 22% URM residents; P = .0002). This progress toward a more representative residency program was met by challenges, such as pipeline concerns, the minority tax, and recruitment during a pandemic. We were able to implement small, low-resource strategies that had a large cumulative impact and could be implemented in other residency programs. Specific tactics and challenges encountered are discussed in this special article.

Association of Child Neurology (AOCN) Consensus Statement on the Diagnosis and Management of Febrile Seizures

Febrile seizures are quite common in children but there are controversies in many aspects of their diagnosis and management. An expert group consisting of pediatric neurologists and pediatricians was constituted. The modified Delphi method was used to develop consensus on the issues of definitions and investigations. The writing group members reviewed the literature and identified the contentious issues under these subheadings. The questions were framed, pruned, and discussed among the writing group members. The final questions were circulated to all experts during the first round of Delphi consensus. The results of the first round were considered to have arrived at a consensus if more than 75% experts agreed. Contentious issues that reached a 50-75% agreement was discussed further in online meetings and subsequently voting was done over an online platform to arrive at a consensus. Three rounds of Delphi were conducted to arrive at final statements. The expert group arrived at a consensus on 52 statements. These statements pertain to definitions of febrile seizures, role of blood investigations, urine investigations, neuroimaging, electroencephalography (EEG), cerebrospinal fluid analysis and screening for micronutrient deficiency. In addition, role of rescue medications, intermittent anti-seizure medication and continuous prophylaxis, antipyretic medication and micronutrient supplementation have been covered. This consensus statement addresses various contentious issues pertaining to the diagnosis and management of febrile seizures. Adoption of these statements in office practice will improve and standardize the care of children with this disorder.

Outcomes of Inpatient Child Neurology Consultations

Child neurology covers a wide range of relevant topics. Both doctors and parents are concerned when a child complains of being unable to walk or having a headache, and they are afraid of postponing the diagnosis. The goal of this study was to assess pediatric neurology inpatient consults. A total of 1,669 requests for pediatric neurology inpatient consultations were reviewed retrospectively during a one-year period. Seizure and febrile seizure were the most common reasons for child neurology consultations (60,6% and 12.2%, respectively). Neuromotor developmental delay (5.7%), syncope (3.4%), headache (2.6%), altered mental status (2.1%), acute complaint of inability to walk (1.8%), facial nerve paralysis (1.4%), ataxia (1.2%), and monoparesis/hemiparesis (1.2%) were among the other reasons for child neurology consultations (1%). Fifty-seven patients (8.8%) who were consulted owing to a seizure were not diagnosed as having seizures. The most common cause of changed mental status was meningoencephalitis. Nine (47.4%) of the patients with acute inability to walk were evaluated as viral myositis. In a patient with acute neurological symptoms, there may be a simple underlying cause or a serious cause.

Spasticity and Dystonia are Underidentified in Young Children at High Risk for Cerebral Palsy.

Early spasticity and dystonia identification in cerebral palsy is critical for guiding diagnostic workup and prompting targeted treatment early when it is most efficacious. However, differentiating spasticity from dystonia is difficult in young children with cerebral palsy. We sought to determine spasticity and dystonia underidentification rates in children at high risk for cerebral palsy (following neonatal hypoxic-ischemic encephalopathy) by assessing how often child neurologists identified hypertonia alone versus specifying the hypertonia type as spasticity and/or dystonia by age 5 years. Of 168 children, 63 developed cerebral palsy and hypertonia but only 19 (30%) had their hypertonia type specified as spasticity and/or dystonia by age 5 years. Child neurologists did not specify the type of hypertonia in a majority of children at high risk of cerebral palsy. Because early tone identification critically guides diagnostic workup and treatment of cerebral palsy, these results highlight an important gap in current cerebral palsy care.

Oral dyspraxia in self-limited epilepsy with centrotemporal spikes: a comparative study with a control group.

self-limited epilepsy with centrotemporal spikes, previously considered benign focal childhood epilepsy with centrotemporal spikes show clinical signs of involvement of Rolandic areas, mainly lower area, which may affect the planning and execution of motor sequences. This study aimed to evaluated oral praxis in children with self-limited epilepsy with centrotemporal spikes and compare to the age-matched control group. This was a descriptive study with 74 children with self-limited epilepsy with centrotemporal spikes, with the classical forms according to International League Against Epilepsy, and between 4 and 15 years of age, selected from the child neurology outpatient clinic of the Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil, and 239 age-matched and educational level-matched (convenience sampling) control children. All children were submitted to the battery of oral volitional movements, which consisted of 44 tests for oral movement (tongue, lip, cheek, jaw, and palate) and 34 phonemes and consonant cluster tasks, with simple and sequenced oral movements. The mean age and standard deviation (SD) of children with epilepsy was 9.08 years (SD 2.55) and of controls 9.61 years (SD 3.12). The results showed significant differences between the groups with a poorer performance of children with epilepsy compared to children without epilepsy in simple and particularly in sequenced movements. These findings can be attributed to the genetically determined immaturity of cortical structures related to motor planning in children with self-limited epilepsy with centrotemporal spikes.

Multisystemic inflammatory syndrome in children associated with covid - 19. A case Report

Pediatric inflammatory multisystem syndrome (PIMS) associated with asymptomatic COVID-19 has been reported in children of various ages from infancy onwards. The dominant symptoms of PIMS are fever above 38.5 C lasting more than three days, elevated inflammatory markers, and the occurrence of symptoms from at least two systems; the digestive system and the cardiovascular system. Early diagnosis and treatment improve prognosis and may reduce life-threatening complications. We present a case of a 7-month-old infant hospitalized in the Department of Infectious Diseases and Child Neurology in Poznan.

Pediatric Acute Stroke Protocols in the United States and Canada

To describe existing pediatric acute stroke protocols to better understand how pediatric centers might implement such pathways within the context of institution-specific structures. We administered an Internet-based survey of pediatric stroke specialists. The survey included questions about hospital demographics, child neurology and pediatric stroke demographics, acute stroke response, imaging, and hyperacute treatment. Forty-seven surveys were analyzed. Most respondents practiced at a large, freestanding children's hospital with a moderate-sized neurology department and at least 1 neurologist with expertise in pediatric stroke. Although there was variability in how the hospitals deployed stroke protocols, particularly in regard to staffing, the majority of institutions had an acute stroke pathway, and almost all included activation of a stroke alert page. Most institutions preferred magnetic resonance imaging (MRI) over computed tomography (CT) and used abbreviated MRI protocols for acute stroke imaging. Most institutions also had either CT-based or magnetic resonance-based perfusion imaging available. At least 1 patient was treated with intravenous tissue plasminogen activator (IV-tPA) or mechanical thrombectomy at the majority of institutions during the year before our survey. An acute stroke protocol is utilized in at least 41 pediatric centers in the US and Canada. Most acute stroke response teams are multidisciplinary, prefer abbreviated MRI over CT for diagnosis, and have experience providing IV-tPA and mechanical thrombectomy. Further studies are needed to standardize practices of pediatric acute stroke diagnosis and hyperacute management.