Pediatric Neurology Research Articles

Evaluation of Patients Complaining of Staring Spells: Single Center Experience

Objective: This study aimed to analyze the demographic characteristics, spell semiology, and electroencephalographic characteristics of children with a complaint of staring spells and determine the factors that differentiate epileptic and non-epileptic etiology. Material and Methods: Fifty-six patients were included retrospectively between October 1, 2022, and December 1, 2023. The patients’ age, gender, co-morbidities, and other characteristics of the staring spells (duration, frequency, automatism, and presence of post-attack symptoms), access time to the pediatric neurologist, referring unit and access time to the final diagnosis were also recorded. Electroencephalography (EEG) was performed on all patients. Results: Fifty-six patients were divided into two according to epileptic and non-epileptic etiology. Thirty-three patients (59%) were diagnosed with non-epileptic staring spells, 15 (26.7%) were diagnosed with generalized epilepsy, and 8 (14.3%) were diagnosed with focal epilepsy. The non-epileptic group had a longer spell time and spell frequency, the presence of verbal stimulation response, and no post-attack symptoms (p<0.001). The access time to the pediatric neurologist was detected as 5.5 days, and the access time to the final diagnosis was 6.6 days. EEG was diagnostic in 100% of the epileptic group. Most of the patients were referred by pediatricians and family physicians (p<0.001). Conclusion: Identifying the cause of staring spells is crucial for further follow-up. In this study, we emphasized that history and routine EEG are important to determine the etiology. It has been observed that access time to pediatric neurologists and final diagnosis are shorter in our country compared to the literature. It can be concluded that pediatricians and family physicians have a high awareness of staring spells.

Impact of clinical clerkship integrated with clinical ladder on attending physicians’ teaching self-efficacy

BackgroundSelf-efficacy plays an important role in enhancing the teaching capabilities of attending physicians (APs). The clinical ladder (CL) is an educational approach developed in the field of nursing education that increases difficulty in an incremental manner. However, no previous study has confirmed the effectiveness of CL in medical education. Therefore, this study aimed to examine the effect of clinical clerkship integrated with clinical ladder (CC-CL) on the self-efficacy of APs.MethodsSixth-year medical students participated in CC-CL for 6 months starting from April 2023, and the changes in the self-efficacy of APs were retrospectively evaluated. The students were trained by the APs concurrently, and the achievement levels of each student were shared. The primary outcome measure was the physician teaching self-efficacy questionnaire (PTSQ) score. The PTSQ scores before and after CC-CL were analyzed using the Wilcoxon matched-pair signed-rank test.ResultsFifteen APs from the Department of Pediatric and Child Neurology were included in this study. No significant difference was observed in the total PTSQ scores of the APs before and after CC-CL. However, a significant increase was observed in the PTSQ score of APs who participated for at least 2 h per week over a period of more than 3 months (n = 8) after CC-CL (p = 0.022). Furthermore, APs who had received their pediatrician certification < 10 years ago (n = 8) showed a significant increase in the total PTSQ score after CC-CL (p = 0.022).ConclusionsCC-CL may play an important role in cultivating the self-efficacy of less experienced APs. Further comparative studies must be conducted in the future to validate the findings of this study.

Characteristics of and Treatment Patterns Among Pediatric Patients with Post-traumatic Headache in a Pediatric Neurology Setting (P4-12.009)

Characteristics of and Treatment Patterns Among Pediatric Patients with Post-traumatic Headache in a Pediatric Neurology Setting (P4-12.009)

Depictions of Pediatric Neurological Disabilities in Graphic Novels, 2010–2022 (P1-1.013)

Depictions of Pediatric Neurological Disabilities in Graphic Novels, 2010–2022 (P1-1.013)

An Improvement Project in Reducing After-Visit Phone Calls in a Community Pediatric Neurology Clinic: Too Much Communication?

Physicians strive to provide high-quality clinical care, yet after-visit patient telephone calls create extra demands on a clinician's time. Pediatric neurologists are particularly affected by this challenge given the number of patients with chronic illnesses they serve and the volume of worried parents they support. Added workload coupled with a busy office practice increases the likelihood of early physician burnout, which can have downstream effects on the quality of patient care and patient satisfaction. Using the IHI model for quality improvement (abbreviated QI moving forward), QI methodology was used to determine volume and key drivers of patient/family communications after a visit to a pediatric neurology clinic. Interventions aimed at reducing telephone messages by 15% over a 6-month period were put into place. A baseline audit of clinic phone calls was completed in 2019 to develop an overview of after-visit communications. After-visit telephone calls and web-based portal messages were then tracked for 3-week periods in 2019, 2020, and 2021 to understand key trends. A key driver diagram of patient/family communications after a clinic visit was created, and interventions aimed at reducing telephone messages were discussed. These interventions included optimizing MD-RN workflows, synchronous and asynchronous educational initiatives, and changes to our clinic's voicemail phone tree. Our primary outcome measure was the average monthly telephone call volume, and this measure was tracked monthly from November 2020 through December 2022. Similarly, electronic portal message volume was tracked and served as our balancing measure. Physicians, nurses, and patients were primary drivers of phone call volume. After interventions were in place, the average monthly call volume decreased by 30% from a baseline of 293 calls to 203 calls. This change was sustained for at least 1 year. The average monthly portal message volume remained consistent throughout the study period at 359 messages. Both physicians and nurses agree that after-visit patient communication affects their workload. This study illustrates that QI methodology can be used to plan and implement interventions aimed at decreasing after-visit telephone calls.

Understanding Health Care Provider Burnout When Caring for Patients With Refractory Epilepsy in the United States.

Among health care providers (HCPs), neurologists have one of the highest rates of burnout in the United States, compromising the quality and accessibility of patient care. Patients with refractory epilepsy are especially challenging to treat. This study aims to understand the burnout level in neurologists treating patients with refractory epilepsy and identify key contributing factors. US board-certified pediatric/adult neurologists who devote ≥50% of their time to clinical practice and treat ≥10 unique patients with refractory epilepsy annually were invited to take a noninterventional quantitative survey, designed to capture key elements of the HCP's background, burnout level, current practice, burden domains, and satisfaction with current antiseizure medications (ASMs). Burnout in 3 domains (emotional exhaustion, depersonalization, and personal accomplishment) was assessed by the validated Maslach Burnout Inventory-Human Services Survey. From March 11, 2022, to April 10, 2022, a total of 138 neurology-specialist HCPs participated in the survey, divided between adult epileptologists (n = 44), adult neurologists (n = 41), pediatric epileptologists (n = 36), and pediatric neurologists (n = 17). Of participating HCPs, 61% experienced at least some burnout (≥1 of 3 burnout domains categorized as high), and 4% experienced high burnout (3 of 3 burnout domains categorized as high). High burnout levels were driven by high pediatric and inpatient caseloads and unexpected pediatric patient reluctance to transition to adult care. HCPs with high burnout had a higher yearly caseload of patients with refractory epilepsy. Most HCPs (approximately 90%) indicated that patients with refractory epilepsy were more difficult to manage than those with nonrefractory epilepsy. The proportion of HCPs satisfied or extremely satisfied with ASMs was lower for patients with refractory epilepsy (20%) than that for patients with nonrefractory epilepsy (73%). Dissatisfaction was mostly due to workload and latency of the insurance approval process, out-of-pocket costs, and poor efficacy, safety, and tolerability. For 32% of HCPs, stopping practicing or moving to another practice within 5 years was probable or very probable. Some burnout is common among HCPs who treat patients with refractory epilepsy. However, management of refractory epilepsy is challenging, and satisfaction with available ASMs is low. Thus, addressing these contributing factors may help to alleviate HCP burnout.

Neuro-pediatric emergencies: clinical profile and outcomes.

Pediatric neurological emergencies are a significant concern, often leading to high rates of admission to pediatric intensive care units and increased mortality rates. In Saudi Arabia, the emergency department (ED) is the main entry point for most patients in the healthcare system. This study aimed to provide a comprehensive overview of pediatric neurology visits to the ED, analyzing patient demographics, clinical presentations, and outcomes. The retrospective study was conducted at a large tertiary care center and examined 960 pediatric patients with neurological emergencies out of 24,088 pediatric ED visits. The study population consisted mainly of male participants (56.5%) and 43.5% female participants, with a mean age of 5.29 ± 4.19 years. School-age children (6-12 years) represented the largest population group (29.1%), and over a third of patients were triaged as 'resuscitation' (n = 332, 34.6%). Seizures (n = 317, 33.0%) and postictal states (n = 187, 19.5%) were the most common reasons for seeking emergency care, accounting for over half of all cases. There were statistically significant differences in provisional diagnosis and chief complaints across different age groups (P >0.001 and P <0.001, respectively). The most common outcome was discharge (n = 558; 58.1%), and the mean length of stay was 10.56 ± 20.33 hours. Neuro-emergencies in pediatrics are a concern and a leading cause of mortality, morbidities, and increased hospital visits. The observed variations in presentation and outcomes across age groups further emphasize the importance of tailored approaches.

Pediatric Functional Neurological Disorder Triggered by Concussion: Physical Therapy Intervention with a 12-year-old male and 2-year follow up

Pediatric Functional Neurological Disorder Triggered by Concussion: Physical Therapy Intervention with a 12-year-old male and 2-year follow up

Clinical and Genetic Spectrum of Tubulinopathy: A Single-Center Study

Purpose: Tubulinopathy represents a group of disorders caused by variants in tubulin genes, which present with a wide spectrum of brain malformations. This study was conducted to provide insight into the phenotypic and genetic spectra of tubulinopathy within the Korean pediatric population. Methods: Among individuals who underwent genetic testing at a pediatric neurology clinic between June 2011 and December 2021, 15 patients with tubulin gene variants were retrospectively recruited. Clinical features, genetic information, and brain imaging findings were retrospectively reviewed.Results: The genetic spectra of the patients included TUBA1A (n=5, 33.3%), TUBB4A (n=6, 40.0%), TUBB3 (n=2, 13.3%), TUBB (n=1, 6.7%), and TUBB2A (n=1, 6.7%) variants. Two novel mutations were identified: a c.497A&gt;G; p.(Lys166Arg) variant in TUBA1A and a c.907G&gt;C; p.(Ala303Pro) variant in TUBB. All 15 patients exhibited developmental delays, with a broad spectrum of severity. Other common manifestations included microcephaly (n=10; 66.7%) and seizures (n=9; 60%). A review of the neuroimaging data revealed a range of findings that were both genotype-specific and overlapping across genotypes. In cases of TUBA1A mutation (n=5), four patients (80%) presented with pachygyria and polymicrogyria, while three (60%) displayed cerebellar hypoplasia and dysplasia. All patients with TUBB4A variants (n=6) exhibited hypomyelination, and three (50%) had cerebellar dysplasia.Conclusion: This study represents the first cohort analysis of tubulin gene mutations associated with tubulinopathy in a Korean pediatric population. It suggests that these mutations can produce a broad spectrum of neurodevelopmental and neuroimaging findings and should be considered within the differential diagnosis in relevant clinical scenarios.

Smith-Magenis Syndrome associated with Autism Spectrum Disorder with delayed diagnosis due to B12 deficiency: a case report

IntroductionSmith-Magenis syndrome (SMS) is a complex genetic disorder characterised by distinctive physical features, developmental delay, cognitive impairment and a typical behavioural phenotype. SMS is caused by interstitial 17p11.2 deletions (90%) involving multiple genes, including the retinoic acid-induced 1 gene (RAI1), or by pathogenic variants in RAI1 itself (10%).ObjectivesIn this case report, we present a case of Smith-Magenis syndrome with Autism Spectrum Disorder with karyotype 46,XX, 17p 11.2 gene deletion confirmed by Autism Spectrum Disorder, who was followed up in a paediatric neurology outpatient clinic with neuromotor developmental delay and whose diagnosis was delayed due to B12 deficiency. We also update scientific developments in Smith-Magenis syndrome.MethodsWe describe an 18-month-old male with Smith-Magenis syndrome and Autism Spectrum Disorder who was seen in our paediatric psychiatric outpatient clinic and who received B12 replacement with developmental delay.ResultsThe patient was followed up in the paediatric neurology outpatient clinic with delay in neuromotor developmental milestones and this delay was thought to be due to B12 deficiency (B12&lt;100 ng/L). The initial examination revealed delay in neuromotor and behavioural milestones, speech delay, wide and high nasal bridge and hypertelorism. Further physical examination revealed syndactyly of the second and third toes bilaterally and crossed lower teeth. Clinical and psychometric testing (Ankara Developmental Screening Inventory) by 2 consultants and 1 research assistant resulted in a diagnosis of intellectual disability and an additional diagnosis of Autism Spectrum Disorder due to social deficits that could not be explained by intellectual disability.ConclusionsSmith-Magenis syndrome is a well-known disorder involving the deletion of chromosome 17p11.2, which contains the RAI1 gene. This condition is associated with neuromotor and behavioural delay, as well as distinctive dysmorphic features. Clinicians should consider Smith-Magenis syndrome in the differential diagnosis for patients with delayed neuromotor and behavioural milestones, even in the presence of documented blood parameters (such as B12 deficiency) that may account for the delay.Disclosure of InterestNone Declared

COVID-19 vaccination uptake in children with epilepsy and vaccine hesitancy among their parents: a survey.

This study explored the coronavirus disease 2019 (COVID-19) vaccination coverage among children with epilepsy (CwE), factors affecting vaccination coverage, and the effect of COVID-19 vaccines on epilepsy after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. A questionnaire was administered to CwE and their parents at the Pediatric Neurology Clinic of the Second Affiliated Hospital of Xi'an Jiaotong University between December 12, 2022, and February 28, 2023. Data were analyzed using the t-tests, chi-square tests, and logistic regression. The analysis included 250 CwE who responded to the survey; of these, 152 (60.8%) had been vaccinated against COVID-19. COVID-19 vaccine hesitancy in parents whose CwE were not vaccinated was mostly due to concerns of vaccine-related exacerbation of seizures and of vaccine-related adverse reactions (44.30% and 41.90% of the respondents, respectively). Univariate analysis showed that vaccination and number of doses of vaccine did not affect seizure incidence within 1 month of SARS-CoV-2 infection. Logistic regression analysis showed that CwE below primary school age, and those taking two or more antiseizure medication (ASMs) were less likely to be vaccinated (p = 0.007). Conclusion: The primary reasons for vaccine hesitancy among parents of unvaccinated CwE were concerns regarding seizure exacerbation and adverse reactions following COVID-19 vaccination. CwE who were below primary school age and those who took two or more ASMs were less likely to be vaccinated. Addressing parents' concerns is necessary to build their confidence in COVID-19 vaccines and ensure that CwE are vaccinated. What is Known: • People with epilepsy have a higher risk of severe and fatal COVID-19 than those without epilepsy but, despite this, COVID-19 vaccination coverage is considerably lower in people with epilepsy than in people without epilepsy. What is New: •In unvaccinated children with epilepsy, the foremost reasons for COVID-19 vaccine hesitancy among parents were concerns about seizure exacerbation and vaccine-related adverse reactions. •Vaccination and number of doses of vaccine did not exacerbate seizures in children with epilepsy, those below primary school level and those taking two or more antiseizure medications were less likely to be vaccinated.

Patrones de seguimiento y manejo clínico multidisciplinario en pacientes con Mucopolisacaridosis tipo IVA en Colombia

Background: There is limited data on the follow-up and management of patients with mucopolysaccharidosis IVA (MPS-IVA) undergoing enzyme replacement therapy (ERT) in Colombia. Objective: We aim to assess the real-world data of patients with MPS-IVA undergoing ERT in Colombia to understand patient profiles, follow-up patterns, and treatment dynamics. Methods: A convenience sample of geneticists and pediatric neurologists were recruited from November-2020 to January-2021. The physicians responded to a questionnaire-based report for each patient under their care regarding the patient profiles, follow-up patterns, and treatment dynamics. Results: A total of 24 physicians (geneticists [92%] and pediatric neurologists [8%]) provided data on 107 patients with MPS-IVA. Patients were diagnosed through molecular (36%) and/or enzymatic assays (97%). The mean age of patients was 19.5 years and 51% were male. The average time from first symptom to diagnosis was 7.1 years. The mean age at first symptoms was 4 years and at diagnosis was 11 years, further, the mean age at first ERT was 15 years. There was an increase in assessments after ERT initiation; parameters most frequently evaluated were weight, height, and echocardiography; quality of life, 6MWT, and 3-minute stair-climb test were the least frequent. ERT interruptions &gt;2 months were observed in 63% patients. Conclusions: Monitoring of MPS-IVA patients receiving ERT remains suboptimal in Colombia. Establishing national management guidelines and implementing centralized reference centers, where patients can receive comprehensive care are warranted for ensuring appropriate systems, services, and support as a priority, with a potential positive effect in the course of the disease.

Atypical electroclinical presentation of Landau Kleffner syndrome: a case report of an 8 year old Nigerian child

Epileptic seizures account for common neurologic presentations in paediatric neurology units in developing countries. Atypical presentation of syndromic epilepsy may be missed especially sensory, psychic or autonomic manifestations. An 8 year old male Nigerian child presented with recurrent vomiting and delayed motor milestones, regression of speech (acquired aphasia) with unremarkable birth history or any underlying medical or surgical condition. He had delayed gross motor development and continuous bilateral polyspike waves of slow sleep on EEG. Self-limited autonomic epileptic seizure syndrome which was a strong differential of autonomic seizures in early childhood was not suggestive as his EEG findings failed to demonstrate occipital spike wave pattern nor did photic stimulation evoke any epileptiform waves. Child made significant clinical improvement with anticonvulsants, neuro vitamins, and behavioural therapy, evidenced by regain of non-verbal communication, resolution of autonomic symptoms and temper tantrums. A high index of suspicion should be applied in patients with acquired aphasia with subclinical or recurrent autonomic symptoms.

Prevalence, Clinical Profiles, and Prognosis of CIDP in Japanese Nationwide Survey: Analyses of 1,257 Diagnosis-Confirmed Patients.

To investigate the current epidemiology, clinical profile, and treatment of chronic inflammatory demyelinating polyneuropathy (CIDP) using a nationwide survey in Japan. We conducted a nationwide survey using an epidemiologic method established in 2021. Questionnaire sheets were sent to the hospital departments of neurology and pediatric neurology throughout Japan. A primary questionnaire was used to determine the number of patients and their prevalence, and a second questionnaire was used to collect detailed clinical information. The primary survey showed that the estimated number of patients with CIDP was 4,180, with a prevalence of 3.3 per 100,000 persons. In the secondary survey, detailed clinical data were available for 1,257 patients. The male-to-female ratio was 1.5:1, and the median age at onset was 52 years. Typical CIDP was the most frequent subtype (52%), followed by distal (17%) and multifocal/focal CIDP (17%). Initial treatments included immunoglobulin therapy (72%), corticosteroids (15%), and others (13%). Among patients with CIDP, 78% had a progressive/relapsing course, 14% did not respond to first-line treatments, and 18% could not walk independently at the last visit. Among the subtypes, typical CIDP had the most severe disability before treatment (44% of patients could not walk independently). However, they showed a more favorable response to treatment than those with distal or multifocal CIDP. In the subgroup analyses, logistic regression analyses showed that younger age at onset, no muscle atrophy, and abnormal median-normal sural sensory nerve responses were associated with a higher probability of independent walking. Our study represents the largest cohort study on CIDP to demonstrate the current epidemiologic and clinical status of CIDP in Japan. Clinical subtypes seem to be associated with different treatment responses and outcomes; therefore, an appropriate treatment strategy according to the pathophysiology of each subtype is required to improve the prognosis of CIDP.

Genetic causes of infection induced encephalitis

IntroductionPatients with encephalitis following a viral infection are often thought to have a para infectious, inflammatory, or autoimmune cause for their presentation. These diagnoses usually result in treatments with immunosuppressant therapies which can have side effects. However, there is an increasing body of evidence demonstrating that patients can have a direct genetic cause mediating viral infection triggered encephalitis, where inflammation is a secondary response. These patients may benefit not from immunosuppressive therapies, but from protection from infection through dedicated immunisation programs and early antiviral therapies at times of infection. MethodsA small case series of paediatric neurology patients (n = 2) from a single institution with infection induced encephalitis and an underlying genetic cause, is presented. Patients with a confirmed genetic cause of infection induced encephalitis were identified and consented by their treating neurologist for inclusion in this case series. Ethics approval was gained for this case series and review of the surrounding literature. ConclusionA case of both DBR1 and NUP214 genetic changes resulting in infection induced encephalitis is presented. This case series raises awareness of this rare group of disorders and provides clues to their identification. Features to prompt clinician consideration of such genetic conditions are also highlighted. Although rare, identification of these patients is important due to implications on treatment, prognosis, and family planning.

Metabolic etiologies in children with infantile epileptic spasm syndrome: Experience at a tertiary pediatric neurology center

ObjectiveInfantile epileptic spasm syndrome (IESS), including West syndrome (WS) and infantile spasm (IS), causes a challenging prognosis, particularly when associated with metabolic etiologies. MethodsThis study, conducted at a tertiary pediatric neurology center, explored the prevalence and clinical features of inborn errors of metabolism in 112 children with IESS over 10 years. ResultsMost patients presented with seizures, primarily flexor spasms, and the median age at onset was 5 months. Comprehensive clinical evaluation and neuroimaging revealed structural-acquired causes as the most common etiology. Notably, inborn errors of metabolism were identified in 5.4 % of cases, with six distinct diagnoses including nonketotic hyperglycinemia, pyridoxine-dependent epilepsy, primary coenzyme Q10 deficiency 7, congenital disorder of glycosylation type IIM, 6-pyruvoyl tetrahydrobiopterin synthase deficiency, and argininosuccinate lyase deficiency. The prevalence of inborn errors of metabolism in this cohort was consistent with global variations reported in the literature. Genetic testing, including karyotype analysis and whole exome sequencing, was performed in a subset of cases with no clear diagnosis, revealing abnormalities in approximately 50 % of cases. Adrenocorticotropic hormone emerged as the most frequently prescribed antiseizure medication. ConclusionThis study provides insight into the diagnostic challenges associated with IESS and highlights the importance of metabolic investigations, especially in cases without a clear etiology. The findings emphasize the need for further genetic and metabolic studies to enhance prognostic accuracy and guide potential treatment options for children with IESS, particularly in populations with high rates of consanguinity.

Gender Differences in the Pediatric Neurosurgical Workforce: Professional Practice, Work-Life Balance, and Beyond.

Evidence suggests that female neurosurgeons experience unique challenges in the workplace including lack of academic advancement, challenges with work-life balance, harassment, and discrimination. How these factors influence the gender gap in neurosurgery remains unclear. This analysis investigated gender differences in pediatric neurosurgeons in professional and nonprofessional activities and responsibilities. A survey examining professional activities, work-life balance, family dynamics, career satisfaction, and workplace discrimination and harassment was administered to 495 pediatric neurosurgeons. Response rate was 49% (n = 241). One-third of the pediatric neurosurgical workforce is female. There were no gender differences in race/ethnicity, American Board of Neurological Surgery/American Board of Pediatric Neurological Surgery certification rates, or pediatric neurosurgery fellowship completion. No gender differences were found in operative caseload, weekly hours worked, or working after 8 pm or weekends. Women took call more frequently than men ( P = .044). Men were more likely to work in academia ( P = .004) and have salary subsidization from external sources ( P = .026). Women were more likely to anticipate retirement by age 65 years ( P = .044), were less happy with call commitments ( P = .012), and worked more hours at home while off ( P = .050). Women more frequently reported witnessing and experiencing racial discrimination ( P = .008; P < .001), sexual harassment ( P = .002, P < .001), and feeling less safe at work ( P < .001). Men were more likely married ( P = .042) with 1 ( P = .004) or more children ( P = .034). Women reported significantly greater responsibility for child and domestic care ( P < .001). There were no gender differences in work-life balance, feeling supported at work, or having enough time to do things outside of work. Despite little difference in workload and professional responsibilities, women held more domestic responsibilities and experienced and witnessed more racial and sexual discrimination in the workplace. Surprisingly, there were no reported differences in work-life balance or feeling supported at work between genders. These findings suggest that factors unique to female neurosurgeons may contribute to continued gender disparity in the field.

Access to Pediatric Neurology Training and Services Worldwide: A Survey by the International Child Neurology Association.

Access to Pediatric Neurology Training and Services Worldwide: A Survey by the International Child Neurology Association.

The utility of lidocaine in neonatal conviction syndrome

BACKGROUND: Lidocaine is a drug belonging to the local anesthetics; it is rapidly distributed in the body and metabolized in the liver. At commonly recommended doses, the therapeutic index of lidocaine remains very high and plasma concentrations remain largely below neurotoxic, cardiotoxic and other thresholds. It is used during various medical procedures, and in the nervous system it acts by binding to a specific site which is the sodium channel receptor of the neural membrane and blocking the passage of ions. The present study is of interest to know the potential usefulness of lidocaine in neonates with convulsive syndrome. In order to implement improvements in the treatment of those who need it. In this way, pediatric neurologists can achieve better results in the interventions to treat this pathology. Methods: In the present systematic review we try to answer and perform a literature search through pubmed and others to analyze the usefulness of lidocaine in neonatal convulsive syndrome and its potential benefit. Results: After the application of the inclusion and exclusion criteria, we will be able to identify the groups of newborns most at risk and the efficacy of lidocaine treatment. Conclusion: It is concluded that lidocaine is the third drug used in neonatal convulsive syndrome for maintenance where it is received as a continuous infusion and is effective in suppressing electroclinical seizures; excessive doses should be avoided as it can also produce convulsions and other adverse effects in children.

Assessment of Barriers to Referral and Appointment Wait Times for the Evaluation of Spinal Muscular Atrophy (SMA): Findings from a Web-Based Physician Survey.

Spinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disease characterized by progressive muscle weakness and atrophy. Clinical trial data suggest early diagnosis and treatment are critical. The purpose of this study was to evaluate neurology appointment wait times for newborn screening identified infants, pediatric cases mirroring SMA symptomatology, and cases in which SMA is suspected by the referring physician. Approaches for triaging and expediting referrals in the US were also explored. Cure SMA surveyed healthcare professionals from two cohorts: (1) providers affiliated with SMA care centers and (2) other neurologists, pediatric neurologists, and neuromuscular specialists. Surveys were distributed directly and via Medscape Education, respectively, between July 9, 2020, and August 31, 2020. Three hundred five total responses were obtained (9% from SMA care centers and 91% from the general recruitment sample). Diagnostic journeys were shorter for infants eventually diagnosed with SMA Type 1 if they were referred to SMA care centers versus general sample practices. Appointment wait times for infants exhibiting "hypotonia and motor delays" were significantly shorter at SMA care centers compared to general recruitment practices (p = 0.004). Furthermore, infants with SMA identified through newborn screening were also more likely to be seen sooner if referred to a SMA care center versus a general recruitment site. Lastly, the majority of both cohorts triaged incoming referrals. The average wait time for infants presenting at SMA care centers with "hypotonia and motor delay" was significantly shorter when initial referrals were triaged using a set of "key emergency words" (p = 0.036). Infants directly referred to a SMA care center versus a general sample practice were more likely to experience shorter SMA diagnostic journeys and appointment wait times. Triage guidelines for referrals specific to "hypotonia and motor delay" including use of "key emergency words" may shorten wait times and support early diagnosis and treatment of SMA.