Journal of Child Neurology最新文献

Here, we describe the process of development of the methodology for an international multicenter natural history study of alternating hemiplegia of childhood as a prototype disease for rare neurodevelopmental disorders. We describe a systematic multistep approach in which we first identified the relevant questions about alternating hemiplegia of childhood natural history and expected challenges. Then, based on our experience with alternating hemiplegia of childhood and on pragmatic literature searches, we identified solutions to determine appropriate methods to address these questions. Specifically, these solutions included development and standardization of alternating hemiplegia of childhood-specific spell video-library, spell calendars, adoption of tailored methodologies for prospective measurement of nonparoxysmal and paroxysmal manifestations, unified data collection protocols, centralized data platform, adoption of specialized analysis methods including, among others, Cohen kappa, interclass correlation coefficient, linear mixed effects models, principal component, propensity score, and ambidirectional analyses. Similar approaches can, potentially, benefit in the study of other rare pediatric neurodevelopmental disorders.

Introduction: To investigate the challenges in the management of children and adolescents with epilepsy in China during the coronavirus disease (COVID-19) pandemic.

Methods: We conducted a cross-sectional survey among 845 patients with epilepsy using an online-based questionnaire. The questionnaire focused on sociodemographic characteristics, epilepsy-related conditions, health care access, COVID-19 vaccination, and the mental health of caregivers. Depression was assessed using Patient Health Questionnaire-9.

Results: During the pandemic, 24.73% of the patients had increased seizures. The majority of patients (68.89%) experienced difficulty obtaining antiseizure medications. In addition, 94.79% of the patients had difficulty consulting a doctor. A total of 52.78% of the patients selected telemedicine services, and most found these services to be helpful. Moreover, 76.11% of the patients failed to complete the COVID-19 vaccination. More than half of the caregivers had anxiety and depressive symptoms. The risk factors for depression comprised irregularity in taking antiseizure medications, difficulty in obtaining antiseizure medications, and failure to consult a doctor on time.

Conclusions: The COVID-19 pandemic presented a great challenge in the management of children and adolescents with epilepsy in China. The findings highlight the importance of improving health care systems and medication management and the mental health of their caregivers.

It is well established that extreme prematurity can be associated with cerebellar lesions potentially affecting the neurologic prognosis. One of the commonly observed lesions in these cases is pontocerebellar hypoplasia resulting from prematurity, which can pose challenges in distinguishing it from genetically caused pontocerebellar hypoplasia. This confusion leads to unacceptable and prolonged diagnostic ambiguity for families as well as difficulties in genetic counseling. Therefore, it is crucial to identify the clinical and neuroradiologic features allowing to differentiate between acquired and genetic forms of pontocerebellar hypoplasia in order to guide clinical practices and improve patient care. In this regard, we report in the present manuscript the clinical, developmental, and radiologic characteristics of 19 very premature children (gestational age <28 weeks, now aged 3-14 years) with cerebellar lesions and discuss the causal mechanisms. Our findings support the notion that a combination of specific clinical and radiologic criteria is essential in distinguishing between acquired and genetic forms of pontocerebellar hypoplasia.

Objective: This study aimed to explore diagnostic performance of 3D-NERVE as an adjunct to electromyography for the assessment of brachial plexus injury in infants. Methods: Imaging of infants with brachial plexus injury using 3D-NERVE and/or 3D-STIR from 2019 to 2022 were reviewed. Images were evaluated between the 2 sequences for nerve-to-fat ratio, nerve-to-muscle ratio, muscle-to-fat ratio, fat suppression homogeneity, and display rate of brachial plexus branches. Results: This study included 37 infants who were referred for a clinical diagnosis of brachial plexus injury. A total of 21 infants accepted 3D-NERVE sequence scanning, and 16 infants accepted 3D-NERVE and 3D-STIR sequences scanning. The results of examination were generally consistent with electromyography. The 2 sequences were compared, yielding the following results. There were no pulsation artifacts (0/16), and 1 case with heterogeneous fat saturation (1/16) was seen on 3D-NERVE. There were no pulsation artifacts (0/16), and 5 cases with heterogeneous fat saturation (5/16) were seen on 3D-STIR. 3D-NERVE performed better (P < .05) for nerve-to-fat and nerve-to-muscle ratios compared with 3D-STIR, and no significant difference in the muscle-to-fat ratio (P > .05). The 3D-NERVE and STIR helped depict 100% (16/16) of the brachial roots and brachial plexus trunk. Brachial plexus bundles and brachial plexus branches were observed in 93.75% (15/16) and 68.75% (11/16) of the 3D-NERVE and 93.75% (15/16) and 62.5% (10/16) of the 3D-STIR, respectively. The differences were not statistically significant (P > .05). Conclusion: Nerve trauma was better visualized with the 3D-NERVE, which is an effective adjunct to electromyography for doctors to assess brachial plexus injury and consequently helps in better treatment planning.

Background / objective: Seizures are a complication for pediatric patients requiring extracorporeal membrane oxygenation (ECMO). There are no standardized guidelines regarding continuous electroencephalography (EEG) monitoring to detect seizures in these patients, and the impact of protocolized monitoring has not been evaluated. Here we examined the effects of continuous EEG protocol implementation in our pediatric ECMO population.

Methods: Retrospective chart reviews were conducted on 57 patients who underwent extracorporeal membrane oxygenation and concurrent continuous EEG out of 165 patients supported on extracorporeal membrane oxygenation. Timing of continuous EEG initiation and seizures detected by continuous EEG was determined for 5 years prior to and 15 months after protocol implementation.

Results: Protocol implementation was associated with increased ECMO-supported patients who were concurrently monitored by continuous EEG. Time from ECMO cannulation to continuous EEG initiation was shorter (median 7 hours after versus 16.2 hours before; P < .001). Patients who had ongoing seizures at the start of continuous EEG recording decreased from 64% preprotocol to 0% postprotocol (P < .001), and there was an associated earlier time to break in status epilepticus postprotocol. Seizures were detected past 48 hours after cannulation in 50% of patients in the postprotocol group.

Conclusions: Protocol implementation resulted in earlier continuous EEG initiation and more EEGs initiated before seizure onset with evidence of altered seizure dynamics. Although current recommendations suggest that continuous EEG duration of 24-48 hours results in seizure detection for >90% of critically ill adults, longer monitoring may be needed to reliably detect seizures in children supported with ECMO, particularly if monitoring is initiated earlier in the post-cannulation period.

Background: Obesity among youth with epilepsy has multifactorial etiology, yet socioecologic obesity risk factors (eg, neighborhood factors) have not been examined in this population. This study examined (1) the prevalence of obesity adjusting for relevant covariates and (2) socioecologic correlates of obesity in adolescents with epilepsy aged 10-17 years.

Methods: This cross-sectional study used 2017-2018 National Survey of Children's Health data (total n = 27,094; epilepsy n = 184). Chi-square tests compared weighted prevalence of obesity with relevant covariates among all adolescents and adolescents with epilepsy. Weighted multiple logistic regression models were conducted to adjust for covariates.

Results: The prevalence of obesity in adolescents with epilepsy was 27.8% (95% confidence interval [CI] 15.4%-40.3%) vs 15.1% (95% CI 14.1%-16.2%) for the non-epilepsy group. Adolescents with epilepsy also had higher odds of obesity after adjusting for age, gender, race/ethnicity, household income, physical activity, and medical home (odds ratio [OR] 2.1, 95% CI 1.2-3.8). Adjusting for sociodemographics, anxiety (OR 4.5, 95% CI 1.3-15.6), 2 or more adverse childhood experiences (OR 7.3, 95% CI 1.6-33.4), neighborhood detracting elements (eg, OR 5.2, 95% CI 1.5-18.5 for 1 detracting element), and forgone care (ie, unmet health care needs) (OR 22.4, 95% CI 3.8-132.8) were associated with obesity in adolescents with epilepsy. Adjusting for multiple comparisons, neighborhood detracting elements (P < .0001) and forgone care (P < .0007) remained significant.

Conclusion: Variables related to mental health, family functioning, built environment, and forgone care were associated with obesity in adolescents with epilepsy, but the association was not fully explained by these factors. Obesity interventions for this population should consider multiple levels of influence including the community and special health care needs of this population.

Introduction: Periventricular leukomalacia occurs in up to 25% of very preterm infants resulting in adverse neurodevelopmental outcomes. In its acute phase, periventricular leukomalacia is clinically silent. Although ultrasonography is widely available, its sensitivity in the early detection of periventricular leukomalacia is low. Case Report and Published Literature: We identified a preterm infant with early diffusion-weighted imaging changes that later evolved to periventricular leukomalacia. Thirty-two cases of abnormal diffusion-weighted imaging reliably heralding severe periventricular leukomalacia in the preterm infant have been published in the literature. Notable features include the following: (1) infants were more mature preterm infants (29-36 weeks' gestation); (2) findings were often serendipitous with benign clinical courses; (3) diffusion-weighted imaging changes only were evident in the first weeks of life with later evolution to more classical abnormalities on conventional magnetic resonance imaging (MRI) or ultrasonography. Conclusion: Diffusion-weighted imaging in the first week of life may be a reliable early marker of severe periventricular leukomalacia injury in more mature preterm infants.

The deep gray nuclei are paired interconnected gray nuclei comprising the basal ganglia and thalami. Injury to the deep gray nuclei secondary to hypoxic-ischemic injury is associated with poor short- and long-term clinical outcomes. The signal changes following hypoxic-ischemic injury are dynamic and evolve over a period of time from injury to resolution. Radiologically relevant events following hypoxic-ischemic injury include the onset of anaerobic metabolism immediately following hypoxic-ischemic injury, increase in cytotoxic edema followed by its resolution, and the onset and progression of neuronal necrosis and gliosis. Appearance of lactate peak on proton spectroscopy is the initial radiologic evidence of hypoxic-ischemic injury. Diffusion-weighted imaging has the highest prognostic value and pseudo-normalizes following 1 week of hypoxic-ischemic injury. Recommended timing for magnetic resonance imaging (MRI) is between 4 and 7 days. MR imaging performed between 1 and 6 months underestimates the extent of injury because radiologic changes are subtle. This review provides a detailed timeline of radiologic abnormalities in the deep gray nuclei following hypoxic-ischemic injury.

Aim: We reviewed the clinical features of a sample of pediatric acquired demyelinating syndromes with the purpose of determining the appropriate protocol for follow-up after the first episode.

Methods: A multicenter retrospective observational study was conducted on a cohort of 40 children diagnosed with a first episode of acquired demyelinating syndrome over the period 2012-2021. Patients were evaluated with clinical and neuroradiologic assessment after 3, 6, and 12 months, with a median follow-up of 4.0 years.

Results: At the first acquired demyelinating syndrome episode, 18 patients (45%) were diagnosed with acute disseminated encephalomyelitis, 18 (45%) with clinical isolated syndrome, and 4 (10%) with multiple sclerosis. By month 12, 12 patients (30%) had progressed from an initial diagnosis of acute disseminated encephalomyelitis (2) or clinical isolated syndrome (10) to multiple sclerosis. Of these, 6 had clinical relapse and 6 radiologic relapse only. The first relapse occurred after a median of 3 months. Among the patients who had evolved toward multiple sclerosis, there was a prevalence of females (P = .014), higher oligoclonal bands positivity (P = .009), and older median age (P < .001) as compared with those who had remained stable.

Interpretation: Both clinical and radiologic follow-up of children with acquired demyelinating syndromes is crucial, especially during the first year after acute onset, for early identification of multiple sclerosis and prompt initiation of disease-modifying treatment to delay axonal damage and to limit disability.