Risk factors following first spontaneous epileptic seizure in children below 3 years of age

Aim of the study: To assess the recurrence rate after the first spontaneous epileptic seizure in pediatric patients, and determine the related risk factors. Materials and Methods: Data from 190 infants (aged between one month and three years) admitted after first spontaneous epileptic seizure to Soochow University Affiliated Children's Hospital between April 2009 and April 2011 were retrospectively analyzed; patients were followed up until April 2014. The recurrence rate and risk factors of recurrence were analyzed based on central nervous system imaging data (computed tomography or magnetic resonance imaging), electroencephalogram (EEG) and developmental quotient assessment. Results: Of the 190 infants, 11 cases (5.79%) were lost to follow-up. The cumulative recurrence rate of spontaneous epileptic seizure after five years was 52%. A total of 82% recurrence cases occurred within the first year. Age > 24 months (odds ratio (OR) = 0.498?95% confidence interval (CI): 0.285–0.869) was an independent protective factor, while symptomatic seizure (OR = 1.624, 95% CI: 1.020–2.587), seizure during sleep (OR = 2.779, 95% CI: 1.757–4.397) and epileptic discharge in EEG (OR=3.269, 95% CI: 2.049–5.217) were independent risk factors. Conclusion: Recurrence rate is high in infants after the first spontaneous epileptic seizure, and recurrence is more likely to occur within one year.     

Validation of the parent-proxy version of the pediatric Charcot-Marie-Tooth disease quality of life instrument for children aged 0–7 years

Objective

To evaluate the parent-proxy version of the pediatric Charcot Marie Tooth specific quality of life (pCMT-QOL) outcome instrument for children aged 7 or younger with CMT. We have previously developed and validated the direct-report pCMT-QOL for children aged 8–18 years and a parent proxy version of the instrument for children 8–18 years old. There is currently no CMT-QOL outcome measure for children aged 0–7 years old.

Methods

Testing was conducted in parents or caregivers of children aged 0–7 years old with CMT evaluated at participating INC sites from the USA, United Kingdom, and Australia. The development of the instrument was iterative, involving identification of relevant domains, item pool generation, prospective pilot testing and clinical assessments, structured focus group interviews, and psychometric testing. The parent-proxy instrument was validated rigorously by examining previously identified domains and undergoing psychometric tests for children aged 0–7.

Results

The parent-proxy pCMT-QOL working versions were administered to 128 parents/caregivers of children aged 0–7 years old between 2010 and 2016. The resulting data underwent rigorous psychometric analysis, including factor analysis, internal consistency, and convergent validity, and longitudinal analysis to develop the final parent-proxy version of the pCMT-QOL outcome measure for children aged 0–7 years old.

Conclusions

The parent-proxy version of the pCMT-QOL outcome measure, known as the pCMT-QOL (0–7 years parent-proxy) is a valid and sensitive proxy measure of health-related QOL for children aged 0–7 years with CMT.     

Behavioral,cognitive, and adaptive development in infants with autism spectrum disorder in the first 2?years of life

Background

To delineate the early progression of autism spectrum disorder (ASD) symptoms, this study investigated developmental characteristics of infants at high familial risk for ASD (HR), and infants at low risk (LR).

Methods

Participants included 210 HR and 98 LR infants across 4 sites with comparable behavioral data at age 6, 12, and 24 months assessed in the domains of cognitive development (Mullen Scales of Early Learning), adaptive skills (Vineland Adaptive Behavioral Scales), and early behavioral features of ASD (Autism Observation Scale for Infants). Participants evaluated according to the DSM-IV-TR criteria at 24 months and categorized as ASD-positive or ASD-negative were further stratified by empirically derived cutoff scores using the Autism Diagnostic Observation Schedule yielding four groups: HR-ASD-High, HR-ASD-Moderate (HR-ASD-Mod), HR-ASD-Negative (HR-Neg), and LR-ASD-Negative (LR-Neg).

Results

The four groups demonstrated different developmental trajectories that became increasingly distinct from 6 to 24 months across all domains. At 6 months, the HR-ASD-High group demonstrated less advanced Gross Motor and Visual Reception skills compared with the LR-Neg group. By 12 months, the HR-ASD-High group demonstrated increased behavioral features of ASD and decreased cognitive and adaptive functioning compared to the HR-Neg and LR-Neg groups. By 24 months, both the HR-ASD-High and HR-ASD-Moderate groups demonstrated differences from the LR- and HR-Neg groups in all domains.

Conclusions

These findings reveal atypical sensorimotor development at 6 months of age which is associated with ASD at 24 months in the most severely affected group of infants. Sensorimotor differences precede the unfolding of cognitive and adaptive deficits and behavioral features of autism across the 6- to 24-month interval. The less severely affected group demonstrates later symptom onset, in the second year of life, with initial differences in the social-communication domain.

Electronic supplementary material

The online version of this article (doi:10.1186/s11689-015-9117-6) contains supplementary material, which is available to authorized users.     

Long-term use of temozolomide: Could you use temozolomide safely for life in gliomas?

Temozolomide (TMZ) is an alkylating agent used in the management of gliomas. Although TMZ is generally safe and acute toxicity is well documented, there are limited data on long-term toxicities. We present three patients with glioma; all patients started on TMZ after having progressed following primary treatment. These patients have continued TMZ for 5 years, 7 years and 8 years respectively. So far they have had no serious side effects. We discuss these patients while raising the question of prolonged TMZ use.     

Brain tissue oxygenation changes in children during the first 24 h following head injury

Aim  The aim of this study is to assess the changes of brain tissue oxygen levels in children during the first 24 h following head injury and its correlation with changes of intracranial pressure and clinical outcome. Method  Invasive monitoring of partial brain tissue oxygen tension (PbtO₂) using the Licox (Integra Neurosciences, Plainsboro, NJ, USA) oxygen probe was performed in children with severe head injury requiring ventilation, during the years 2002–2005. The study focused in the recordings of the first 24 h following injury. Results  There were four patients (three males, one female) with an age range of 2–12 years. All injuries were due to motor vehicle accidents. The Glasgow Coma Score ranged from 5 to 9. All patients had diffuse axonal injuries on Computed Tomography scan. One patient underwent a bilateral decompressive craniectomy. The total duration of monitoring was 567.84 h. During the first 24 h, the mean PbtO₂ was 4.2 mmHg, 12.7 mmHg, 21.8 mmHg, and 25.1 mmHg in each patient. Fifteen episodes of ICP > 20 mmHg were seen in the first 24 h of monitoring. Nine of these episodes were accompanied by a reduction in PbtO₂ levels. The Glasgow Outcome Score at 1 year was good recovery (GOS 3) in three patients and severe disability in one patient. There were no complications from the monitoring. Conclusions  In children with head injury, rise in ICP may be accompanied by fall in PbtO₂. However, low brain oxygen levels during the first 24 h following head injury may not correlate necessarily with poor outcome.     

Multimodality treatment of cerebral AVMs in children: a single-centre 20 years experience

Purpose  

The purpose of this study was to review our experience with a multimodality treatment approach in the management of cerebral arteriovenous malformation (AVM) in children.     

Further evidence of validity of the Modified Melbourne Assessment for neurologically impaired children aged 2 to 4 years

Aim This paper reports the second phase of a study to extend the Melbourne Assessment for use with children with neurological impairment aged 2 to 4 years. The aim was to establish if (1) children’s scores on the Modified Melbourne Assessment (MMA) and the Quality of Upper Extremity Skills Test (QUEST) showed a moderate to high, positive relation, (2) children had comparable behaviours for task and time demands on both tools, and (3) scores on the MMA could discriminate between children with mild, moderate, and severe levels of upper limb impairment. Method An observational study of 30 children (19 males, 11 females) with neurological impairment aged 2 to 4 years. Twenty‐four children had spasticity (20 with a unilateral and four with a bilateral impairment) and two children presented with athetosis, two with ataxia, and two with hypotonia. Results A high, positive relation was found between children’s scores on the MMA and the QUEST (ρ=0.90; p=0.001). The clinical use of the MMA was comparable to the QUEST. MMA scores were able to discriminate between children’s levels of upper limb impairment as determined by clinicians’ ratings (F_2,27=67.76, p=0.001). Interpretation These findings suggest the MMA can be clinically useful for children as young as 2.5 years and has the advantage of being valid for use with older children. Scores from the tool can also provide therapists with a quantitative means of consistently reporting level of upper limb impairment.     

Utility of a rotation–suction microdebrider for tumor removal in endoscopic endonasal skull base surgery

The microdebrider is a common tool used in endoscopic sinus surgery for removing polypoid and sinonasal tissue. It uses rotating blades and an integrated suction device for controlled removal of tissue under video–endoscopic visualization. To our knowledge, the application of the microdebrider for endoscopic removal of skull base tumors has not been reported. This study aimed to investigate the utility of the rotation–suction microdebrider as a tool for endoscopic endonasal removal of solid and fibrous skull base tumors. Thirty-two patients underwent endoscopic endonasal skull base surgery where the rotation–suction microdebrider was used as the primary tool for tumor removal and debulking. Pathologies included a variety of anterior skull base meningiomas, sinonasal skull base malignancies, juvenile nasopharyngeal angiofibromas, schwannomas, and other skull base lesions. Gross total and near total removal was achieved in 87.5% (28/32) of patients, and subtotal removal was performed in 12.5% (4/32) of patients. The microdebrider allowed efficient debulking and removal of solid and fibrous tumors, such as meningiomas, that were not responsive to standard ultrasonic aspiration. There were no complications of orbital or neurovascular injury, or thermal injury to the nostril. The rotation–suction microdebrider is a useful tool for endoscopic endonasal removal of skull base tumors. This is particularly useful for solid and fibrous tumors that are not responsive to standard ultrasonic aspiration. For intracranial tumors, it is critical to remain inside the tumor capsule during debulking so as to avoid injury to the surrounding neurovascular structures.     

Use of the O-arm® for skull base resection in a sphenoorbital meningioma

Intraoperative imaging during skull base surgery allows the surgeon to evaluate surgical results and direct further bone resection prior to closure, avoiding the potential morbidity of inadequate surgical therapy or reoperation. Intraoperative CT (iCT) scanning has become widely available in recent years, but its neurosurgical applications have been limited mostly to spinal and functional operations. We report a patient with a sphenoorbital meningioma with adjacent hyperostosis causing proptosis and optic canal stenosis in which a portable iCT scanner (O-arm®; Medtronic, Fridley, MN, USA) was used to guide further resection. Postoperatively, the patient experienced resolution of her proptosis, and her vision remains clinically normal. The O-arm® can be easily incorporated into standard operating rooms and is useful in tailoring bony skull base resections.     

Alterative application of five anticonvulsants according to the half life for the treatment of status epilepticus in children with severe viral encephalitis

BACKGROUND: Traditional subhibernation therapy may easily cause complications, such as respiratory depression and hyportension because of application of chlorpromazine hydrochloride and promethazine in a large dosage. OBJECTIVE: To observe therapeutic effect of modified subhibernation therapy (alterative application of five anticonvulsants according to the half life) on status epilepticus in children with severe viral encephalitis (VE). DESIGN: Contrast observation. SETTING: Department of Pediatrics, the First Hospital of Jilin University. PARTICIPANTS: The participants in present study were 96 patients with severe viral encephalitis including 52 boys and 44 girls who received treatment in the Department of Pediatrics, the First Hospital of Jilin University from February 2000 to March 2006. All children met the diagnostic criteria of Zhufutong Practice Pediatrics (the seventh edition). Two weeks ago, they ever got upper respiratory infection or enteronitis and so on before the onset, spirit abnormal, behavior disorder, limbs act disorder, vomit, headache, convulsion, nervous system masculine signs such as limbs act disord, autonomic nerve damage manifestation, brain nerve palsy, dysreflexia, meningeal irritation sign, cerebrospinal fluid and electroencephalography (EEG) abnormity. All parents provided the confirmed consent. The patients were randomly divided into control group (n =40) and experimental group (n =56). METHODS: Patients in the control group received anticonvulsion, ice compress and routine treatment. The convulsion was treated with five drugs: 0.5 mg/kg wintermin and phenergan, respectively, 100 g/L chlorpromazine hydrochloride (0.5 mL/kg), 5 mg/kg luminal, 0.3 mg/kg ansiolin. When convulsion attacked, those five drugs were given alternatively; however, those were not given if the convulsion did not attack. Children in the experimental group were treated with improved subhibernation therapy based on routine treatment. The dosages of anticonvulsants were as the same as those in the control group. Based on the half life, every drug was alternated every 4–6 hours. In addition, anticonvulsants administrated for 2 successive days whether tic attacked or not. Then the hypnotic was removed gradually. MAIN OUTCOME MEASURES: Therapeutic efficacy, time of disappeared clinical symptoms and physical sign, and security of administration. RESULTS: All the 96 patients were involved in the final analysis. ① Total effective rate and reliability: Total effective rate was higher in the treatment groups than the control group (χ2=5.871 7, P < 0.05). All patients did not have respiratory depression and side effects. ② Time of disappeared clinical symptoms and physical sign: Recovery time of convulsion, fever, headache and vomit was shorter in the treatment group than that in the control group, and there was significant difference (t =17.612 1–34.330 7, P < 0.05); in addition, symptoms of status epilepticus were relieved obviously. Meanwhile, recovery time of paralysis, coma and anepia was shorter in the treatment group than that in the control group, and there was significant difference (t =10.660 8–24.700 8, P < 0.05). CONCLUSION: Therapeutic effect of improved subhibernation therapy on status epilepticus induced by severe viral encephalitis is positively and safer.     

Prevalence and characteristics of significant social anxiety in children aged 8–13 years

Background  Social anxiety has been frequently studied in both population- and clinical-based adult and adolescent samples. Corresponding research in children is scarce and is dominated by clinical studies. The aim of the present population-based study was to examine the prevalence of significant social anxiety (SSA) in preadolescent children and compare their characteristics with those of children without SSA. The spectrum of social anxiety is explored by comparing children with different levels of social anxiety, as defined by 1–2 versus 3–5 social situations feared. Method  The sample consisted of 14,497 parents and their 3rd–7th grade children (8–13 years old) who participated in a health profile study, including questions covering DSM-IV criteria A–D for social anxiety disorder (SAD). Socio-demographic data, social and school functioning, somatic complaints, parent–child relationships, and use of health services were added to a logistic regression model to explore characteristics associated with children with, and without SSA. Associated emotional and behavioural problems were measured by the Strengths and Difficulties Questionnaire (SDQ) using parent and self-report. Results  Parents described 2.3% of all children as significantly socially anxious and 0.9% feared at least three social situations. The majority of children with SSA managed their everyday life well. However, compared with children without SSA, children with SSA struggled more often in different areas of life and showed a significantly higher prevalence of associated emotional and behavioural symptoms. Our findings also support the notion of social anxiety as a spectrum concept. Conclusions  Social anxiety problems start in childhood and can be impairing, even in non-clinical populations and in reasonably young age groups. Increased awareness of different aspects of social anxiety is needed to identify children who are at risk and to devise appropriate interventions to improve the immediate and long-term outcome.     

Improvement of health-related quality of life in relapsing remitting multiple sclerosis patients after 2 years of treatment with intramuscular interferon-beta-1a

In patients with relapsing remitting multiple sclerosis (RRMS), the effect of interferon-beta (INFb) on health-related quality of life (HR-QoL) is not firmly documented. The objective of this study is to assess HR-QoL during 2 years of treatment with intramuscular INFb and its correlation with disability. In 36 neurological practices in the Netherlands (17), Belgium (16), United Kingdom (2) and Luxemburg (1), 284 RRMS patients were treated with intramuscular INFb-1a. Physical and mental domains of HR-QoL were measured by the MS54 Quality of Life (MS54QoL) questionnaire, and disability was assessed by the Multiple Sclerosis Functional Composite (MSFC) (Timed 25-Foot Walk Test [Timed 25-FWT], 9 Hole Peg Test [9-HPT], Paced Auditory Serial Addition Test [PASAT]) at baseline and at months 3, 6, 12, 18 and 24. Expanded Disability Status Scale (EDSS) score was assessed at baseline and month 24. Pearson’s correlation coefficients were determined and predefined factors were analyzed for relation to HR-QoL after baseline by stepwise regression analyses on physical and mental scores. 204 patients (71.8%) completed 2 years of treatment. Mean values for MS54QoL increased from 56.6 to 61.0 for physical (p < 0.05) and from 57.2 to 61.1 for mental domain (p = 0.07). Correlations between physical domain and MSFC was −0.40 (p < 0.05), and between mental domain and MSFC −0.24 (p < 0.05). MSFC and EDSS did not change. Increase of physical MS54QoL was associated with lower age, lower EDSS, less time for Timed 25-FWT, and higher PASAT score at baseline. Increase of mental MS54QoL was associated with higher PASAT and lower EDSS. Patients who discontinued INFb had lower physical or mental HR-QoL at baseline. In RRMS patients, 2 years of treatment with intramuscular INFb-1a is associated with an increase in HR-QoL, especially in younger patients with low disability.     

Does the Movement Assessment Battery for Children-2 at 3 years of age predict developmental coordination disorder at 4.5 years of age in children born very preterm?

BackgroundVery preterm children (VPT) are at high risk for developmental coordination disorder (DCD). The Movement Assessment Battery for Children-2 (MABC-2) Test is commonly used to identify children with DCD, but little is known about the predictive validity of this assessment in this population.AimsThe aims of this study were to determine if MABC-2 scores at 3 years can predict DCD at 4.5 years and if DCD can be reliably identified in VPT children at 3 years.Methods and proceduresIn a retrospective sample of 165 VPT children, logistic regression was used to determine if the MABC-2 score at 3 years is predictive of DCD at 4.5 years. Cross-tabulations were used to determine the relationship between scores at 3 years and DCD at 4.5 years.Outcomes and resultsMABC-2 scores at age 3 were a significant predictor (OR = 0.82, p = 0.001) of DCD diagnosis at 4.5 years. The MABC-2 has excellent sensitivity (90%), moderate specificity (69%), small to moderate positive predictive value (38%) and high negative predictive value (97%).Conclusions and implicationsThe MABC-2 is highly sensitive in identifying VPT children with DCD, but also has many false positives. MABC-2 scores can reliably predict VPT children who are not at risk of DCD.