共查询到20条相似文献，搜索用时 31 毫秒
1.
A. Koy M. Weinsheimer K.A.M. Pauls A.A. Kühn P. Krause J. Huebl G.-H. Schneider G. Deuschl R. Erasmi D. Falk J.K. Krauss G. Lütjens A. Schnitzler L. Wojtecki J. Vesper R. Korinthenberg V.A. Coenen V. Visser-Vandewalle L. Timmermann 《European journal of paediatric neurology》2017,21(1):136-146
Background
Data on paediatric deep brain stimulation (DBS) is limited, especially for long-term outcomes, because of small numbers in single center series and lack of systematic multi-center trials.Objectives
We seek to systematically evaluate the clinical outcome of paediatric patients undergoing DBS.Methods
A German registry on paediatric DBS (GEPESTIM) was created to collect data of patients with dystonia undergoing DBS up to the age of 18 years. Patients were divided into three groups according to etiology (group 1 inherited, group 2 acquired, and group 3 idiopathic dystonia).Results
Data of 44 patients with a mean age of 12.8 years at time of operation provided by 6 German centers could be documented in the registry so far (group 1 n = 18, group 2 n = 16, group 3 n = 10). Average absolute improvement after implantation was 15.5 ± 18.0 for 27 patients with pre- and postoperative Burke-Fahn-Marsden Dystonia Rating scale movement scores available (p < 0.001) (group 1: 19.6 ± 19.7, n = 12; group 2: 7.0 ± 8.9, n = 8; group 3: 19.2 ± 20.7, n = 7). Infection was the main reason for hardware removal (n = 6). 20 IPG replacements due to battery expiry were necessary in 15 patients at 3.7 ± 1.8 years after last implantation.Discussion
Pre- and postoperative data on paediatric DBS are very heterogeneous and incomplete but corroborate the positive effects of DBS on inherited and acquired dystonia. Adverse events including relatively frequent IPG replacements due to battery expiry seem to be a prominent feature of children with dystonia undergoing DBS. The registry enables collaborative research on DBS treatment in the paediatric population and to create standardized management algorithms in the future. 相似文献2.
A 22-year follow-up reveals a variable disease severity in early-onset facioscapulohumeral dystrophy
Rianne J.M. Goselink Caroline R. van Kernebeek Karlien Mul Richard J.L.F. Lemmers Silvère M. van der Maarel Oebele F. Brouwer Nicol Voermans George W. Padberg Corrie E. Erasmus Baziel G.M. van Engelen 《European journal of paediatric neurology》2018,22(5):782-785
Aim
To assess the long-term natural course of early-onset facioscapulohumeral dystrophy (FSHD), which is important for patient management and trial-readiness, and is currently lacking.Methods
We had the unique opportunity to evaluate 10 patients with early-onset FSHD after 22 years follow-up. Patients underwent a semi-structured interview, physical examination and additional genotyping.Results
Nine initial study participants (median age 37 years) were included, one patient died shortly after first publication. At first examination, one patient was wheelchair dependent, one patient walked aided, and eight patients walked unaided. After 22 years, four patients were wheelchair dependent, three walked aided, and two walked unaided. Systemic features, including hearing loss (56%), intellectual disability (44%), and a decreased respiratory function (56%), were frequent. Patients participated socially and economically with most patients living in a regular house (n = 6) and/or having a paid job (n = 4).Discussion
Patients with early-onset FSHD generally had a severe phenotype compared to classical onset FSHD. However, after 22 years of follow up they showed a wide variation in severity and, despite these physical limitations, participated socially and economically. These observations are important for patient management and should be taken into account in clinical trials. 相似文献3.
Michael L. Rinke Hardeep Singh Moonseong Heo Jason S. Adelman Heather C. O&#x;Donnell Steven J. Choi Amanda Norton Ruth E.K. Stein Tammy M. Brady Christoph U. Lehmann Steven W. Kairys Elizabeth Rice-Conboy Keri Thiessen David G. Bundy 《Academic pediatrics》2018,18(2):220-227
Objective
Diagnostic errors (DEs), which encompass failures of accuracy, timeliness, or patient communication, cause appreciable morbidity but are understudied in pediatrics. Pediatricians have expressed interest in reducing high-frequency/subacute DEs, but their epidemiology remains unknown. The objective of this study was to investigate the frequency of two high-frequency/subacute DEs and one missed opportunity for diagnosis (MOD) in primary care pediatrics.Methods
As part of a national quality improvement collaborative, 25 primary care pediatric practices were randomized to collect 5 months of retrospective data on one DE or MOD: elevated blood pressure (BP) and abnormal laboratory values (DEs), or adolescent depression evaluation (MOD). Relationships between DE or MOD proportions and patient age, gender, and insurance status were explored with mixed-effects logistic regression models.Results
DE or MOD rates in pediatric primary care were found to be 54% for patients with elevated BP (n = 389), 11% for patients with abnormal laboratory values (n = 381), and 62% for adolescents with an opportunity to evaluate for depression (n = 400). When examining the number of times a pediatrician may have recognized an abnormal condition but either knowingly or unknowingly did not act according to recommended guidelines, providers did not document recognition of an elevated BP in 51% of patients with elevated BP, and they did not document recognition of an abnormal laboratory value without a delay in 9% of patients with abnormal laboratory values.Conclusions
DEs and MODs occur at an appreciable frequency in pediatric primary care. These errors may contribute to care delays and patient harm. 相似文献4.
Aslihan Kara Metin Kaya Gurgoze Mustafa Aydin Erdal Taskin Unal Bakal Aysen Orman 《Pediatrics and neonatology》2018,59(4):375-379
Background
The aim of present study was to evaluate the indications, complications and outcomes of acute peritoneal dialysis (APD) in neonates at a referral university hospital during the previous 8 years.Methods
This retrospective analysis included a total of 52 newborn infants who underwent APD in a neonatal intensive care unit between January 2008 and March 2016. Demographic, clinical, laboratory and microbiological data were extracted from patients' medical files.Results
The primary causes for requiring APD were acute tubular necrosis (n = 36, 69.2%), inborn error of metabolism (n = 10, 19.2%), congenital nephrotic syndrome (n = 2, 3.9%), bilateral polycystic kidney (n = 2, 3.9%), renal agenesis (n = 1, 1.9%), and obstructive uropathy (n = 1, 1.9%). The mean duration of APD was 8.7 ± 15.87 days (range: 1–90 days). Procedural complications were mainly hyperglycemia (n = 16, 47.1%), dialysate leakage (n = 7, 20.6%), peritonitis (n = 3, 8.8%), catheter obstruction (n = 3, 8.8%), bleeding at the time of catheter insertion (n = 2, 5.9%), catheter exit site infection (n = 2, 5.9%), and bowel perforation (n = 1 2.9%). There were 40 deaths (76.9%), mainly due to underlying causes. Ten of the 12 survivors showed full renal recovery, but mild chronic renal failure (n = 1) and proteinuria with hypertension were seen (n = 1) in each of remaining patients.Conclusion
Peritoneal dialysis is an effective route of renal replacement therapy in the neonatal period for management of metabolic disturbances as well as renal failure. Although major complications of the procedure are uncommon, these patients still have a high mortality rate due to serious nature of the underlying primary causes. 相似文献5.
A.I. Buizer P.E.M. van Schie E.A.M. Bolster W.J. van Ouwerkerk R.L. Strijers L.A. van de Pol A. Stadhouder J.G. Becher R.J. Vermeulen 《European journal of paediatric neurology》2017,21(2):350-357
Background
In non-walking children with severe spasticity, daily care can be difficult and many patients suffer from pain. Selective dorsal rhizotomy (SDR) reduces spasticity in the legs, and therefore has the potential to improve daily care and comfort.Aim
To examine effects of SDR on daily care and comfort in non-walking children with severe spasticity due to different underlying neurological conditions.Methods
Medical history, changes in daily care and comfort and satisfaction with outcome were assessed retrospectively in non-walking children who underwent SDR in our center, with a mean follow-up of 1y 7m (range 11m–4y 3m). All eligible patients (n = 24, years 2009–2014) were included.Results
Mean age at SDR was 12y 4m (SD 4y 3m, range 2y 8m–19y 3m). Associated orthopaedic problems were frequent. Seven patients underwent scoliosis correction in the same session. Most improvements were reported in dressing (n = 16), washing (n = 12) and comfort (n = 10). Median score for satisfaction was 7 on a scale of 10 (range 1–9). SDR resulted in reduction of spasticity in leg muscles. In nine patients dystonia was recorded post-operatively, mainly in children with congenital malformations and syndromes.Interpretation
SDR is a single event intervention that can improve daily care and comfort in non-walking children with severe spasticity, and can safely be combined with scoliosis correction. Despite the improvements, satisfaction is variable. Careful attention is necessary for risk factors for dystonia, which may be unmasked after SDR. 相似文献6.
Jacob Brodsky Karampreet Kaur Talia Shoshany Sophie Lipson Guangwei Zhou 《European journal of paediatric neurology》2018,22(4):667-673
Introduction
Migraine variant disorders of childhood include benign paroxysmal torticollis of infancy (BPTI) and benign paroxysmal vertigo of childhood (BPVC). This study aimed to review our experience with BPTI and BPVC and determine the incidence of children transitioning between each of these disorders and to vestibular migraine (VM).Methods
We retrospectively reviewed the medical records of patients seen at the Balance and Vestibular Program at Boston Children's Hospital between January 2012 and December 2016 who were diagnosed with BPTI, BPVC, and/or VM.Results
Fourteen patients were diagnosed with BPTI, 39 with BPVC, and 100 with VM. Abnormal rotary chair testing was associated with progression from BPTI to BPVC (n = 8, p = 0.045). Eight (57.1%) patients with BPTI and 11 (28.2%) with BPVC had motor delay. Eleven (78.6%) patients with BPTI and 21 (53.8%) with BPVC had balance impairment. Six BPTI patients developed BPVC (42.9%), six BPVC patients developed VM (15.4%), and two patients progressed through all three disorders (2%). One BPTI patient progressed directly to VM.Discussion
Most patients with BPTI will experience complete resolution in early childhood, but some will progress to BPVC, and similarly many patients with BPVC will progress to VM. Parents of children with these disorders should be made aware of this phenomenon, which we refer to as “the vestibular march.” Children with BPTI and BPVC should also be screened for hearing loss, otitis media, and motor delay. 相似文献7.
Mirjam N. Landgraf Lucia Albers Birte Rahmsdorf Katharina Vill Lucia Gerstl Michaela Lippert Florian Heinen 《European journal of paediatric neurology》2018,22(3):507-515
Objective
The objective of our study was to evaluate the knowledge about fetal alcohol spectrum disorders (FASD) and the implementation of the German guideline for FASD among different professionals in the health and social system and among parents with children with FASD.Methods
A questionnaire about FASD, containing 20 items, was sent by post to all children's hospitals (n = 287), all hospitals for child and adolescent psychiatry (n = 173), all social paediatric centres (n = 162), all neuropaediatricians (n = 129) and all youth welfare offices (n = 672) in Germany. Furthermore a link to the questionnaire as online version was put in the member's newsletter by 14 relevant professional societies. Besides, the questionnaire was distributed personally to the attendees of the annual national FASD conference (n = 363).Results
Altogether 428 persons took part in the survey. 273 participants were professionals and 155 parents of children with FASD. More than 95% of the professionals and parents knew that alcohol consumption during pregnancy constitutes a risk for the child. The prevalence of maternal alcohol consumption and of FASD was underestimated. Although approx. 70% of the professionals knew which disorders belong to FASD just a few could tell their specific deficits. Questions regarding effective intervention for children with FASD and the long-term outcome were only partially answered correctly.Discussion
Professionals in the German health and social system are aware of FASD but underestimate the level of damage and the impact on every day functioning of the affected people. 相似文献8.
Shiri Shinar Tally Lerman-Sagie Monica Echevarria Telleria Fernando Viñals Raquel García Hector Quiroga Carmina Bermejo Liat Ben-Sira Zvika Leibovitz Joseph Har-Toov Gustavo Malinger 《European journal of paediatric neurology》2018,22(6):929-934
Introduction
Pericallosal lipomas (PCL) are congenital soft masses of adipose cells encapsulated by a thin layer of fibrous tissue, appearing adjacent to the corpus callosum (CC). The lipomas are usually diagnosed prenatally during the third trimester. The purpose of this study was to identify 2nd trimester ultrasound findings that may hint to a later diagnosis of PCL and to evaluate their MRI evolution.Methods
A multicenter study of fetuses diagnosed during the 3rd trimester with a PCL in 7 fetal ultrasound units between 2001 and 2017. We reevaluated the ultrasound and MRI images starting from the referral examination and until the time of diagnosis, searching for clues that could have prompted an earlier diagnosis. Parents were contacted at the end of the study period to obtain information regarding development and neurological examination.Results
Fifteen patients with PCL were diagnosed during the study period; fourteen had second trimester scans. A 2nd trimester diagnosis was established in only 2 (13.3%). Anomalies of the CC were evident in 8/14 patients during the 2nd trimester scan and included: short length (n = 4), increased thickness (n = 1), complete agenesis (n = 2) and partial agenesis (n = 1). Third trimester ultrasound scans were considered diagnostic of PCL in all 12 remaining cases, in eight it was considered an isolated finding. Postnatal neurological evaluation in the isolated cases revealed normal development in all children. One child was diagnosed with attention deficit disorder.Conclusions
Non visualization of a PCL during the 2nd trimester is common, and should not be considered a diagnostic error. An underlying PCL should be included in the differential diagnosis of CC anomalies during this time period, necessitating further follow up into the 3rd trimester. 相似文献9.
Andreea Nissenkorn Tomer Erlich Dorit E. Zilberman Ifat Sarouk Alexander Krauthammer Noam D. Kitrey Gali Heimer Bruria BenZeev Yoram Mor 《European journal of paediatric neurology》2018,22(6):1118-1123
Background
Ataxia telangiectasia (AT) is a neurodegenerative cerebellar disorder, caused by mutations in the ATM gene, involved in DNA repair. Radiosensitivity, progressive ataxia, immune deficiency and malignancies, are well known symptoms, but urological manifestations are scarcely described.Objective
To characterize urologic manifestations in a large cohort of AT patients.Methods
Retrospective cross-sectional chart study comprising 52 AT patients followed at a National AT Center.Results
25% of the cohort (13 patients/8 males) had urologic symptoms, which presented at 11 ± 4.3 years. The most common symptom was secondary enuresis affecting 15% of the patients (8 children/4 males). Incontinence appeared at 8 ± 6.2 years of age, and resolved spontaneously within 15 ± 8.3 months in 6 patients. It preceded loss of ambulatory capacity by 1–2 years in 7 patients. Lumbosacral MRI were normal (4 children) and urine cultures (all) were negative. Urodynamic evaluation that was performed in only one patient revealed overactive bladder. Additional manifestations were macroscopic hematuria due to bladder telangiectasia in a 12-year-old, and renal cell carcinoma in a 22-year-old. Other manifestations unrelated to AT were neprolithiasis, vesico-ureteral reflux and scrotal pain, each in 1 patient.Discussion
Transient secondary enuresis is a frequent finding in AT patients, heralding loss of ambulatory capacity, tough it's pathophysiological mechanism is largely no understood. 相似文献10.
Michael R. Pranzatelli Tyler J. Allison Elizabeth D. Tate 《European journal of paediatric neurology》2018,22(4):586-594
Introduction
Flow cytometric cerebrospinal fluid (CSF) lymphocyte subset analysis has improved the diagnosis of neuroinflammation and identified multiple markers of inflammation in opsoclonus-myoclonus syndrome (OMS). The aim of this exploratory, retrospective study was to analyze the effect of immunotherapy on these markers to determine which agents are disease modifying.Methods
Cross-sectional immunological observations were made in an IRB-approved case–control study, and patients were treated empirically. Ten different CSF lymphocyte subpopulations from 18 children with persistent OMS had been measured by flow cytometry before and after clinical treatment with cyclophosphamide/ACTH/IVIG combination (n = 7) or ACTH/IVIG alone (n = 11). Clinical severity of OMS was scored from videotapes by a blinded observer using the OMS Evaluation Scale.Results
Only cyclophosphamide combination therapy (mean dose 26 ± 3 mg/kg or 922 ± 176 mg/m2 x 6 cycles) significantly decreased the percentage of CSF B cells. The mean reduction was 65%, with CSF B cell frequency normalized at 7–8 months in 70%. Other abnormalities of the CSF immunophenotype, such as the low CD4/CD8 T cell ratio, persisted, and there were no therapeutic changes in T cell activation/maturation markers. Effects on relative and absolute size of PBMC subsets were similar. Clinical improvement was 70% and 55% in respective treatment groups. The relapse rates of the two groups did not significantly differ.Discussion
The main effect of cyclophosphamide combination therapy on neuroinflammation in OMS was moderate reduction in CSF B cell expansion. Though exploratory, it may provide a steroid sparer option in partially-responsive OMS. 相似文献11.
Po-Cheng Hung Huei-Shyong Wang Ming-Liang Chou Kuang-Lin Lin Meng-Ying Hsieh I-Jun Chou Alex M-C. Wong 《Pediatrics and neonatology》2018,59(6):573-580
Background
The aim of this study was to evaluate the clinical, and neuroimaging features, outcomes, and other associated systemic disorders in children with schizencephaly at a single medical center in Taiwan.Methods
We retrospectively reviewed the medical records and magnetic resonance images (MRI) of children with schizencephaly between January 2000 and December 2014. The MRI findings of schizencephaly were recorded along with the presence of associated cerebral disorders. Clinical, electroencephalographic and additional systemic disorders were also recorded.Results
A total of 21 patients (13 males and 8 females) were included in the study. According to the location of schizencephaly, the patients were classified into two groups: unilateral (n = 16) and bilateral (n = 5). The majority of the patients with neurological deficits were detected before 1 year of age, especially in bilateral clefts. The most common initial presentation was hemiparesis in unilateral schizencephaly, and seizures in bilateral schizencephalies. Ventriculomegaly was the most common associated cerebral disorder, and the most common additional systemic disorders included congenital heart disease, hydronephrosis, and strabismus. Seventeen patients suffered from epileptic seizures with generalized tonic-clonic seizures being the most common. Eight patients developed refractory epilepsy. The majority of the patients had motor deficits, intellectual disabilities, and language deficits, especially in bilateral clefts.Conclusions
This study demonstrates that the clinical features of schizencephaly vary widely, with their severity closely related to the cleft. Determining the type, size, and extent of schizencephaly is useful to plan management and predict the prognosis. 相似文献12.
Daniel Tibussek Felix Distelmaier Michael Karenfort Stefani Harmsen Dirk Klee Ertan Mayatepek 《European journal of paediatric neurology》2017,21(2):280-285
Background
Cerebrospinal fluid (CSF) opening pressure (OP) of ≥28 cm H2O is now considered a diagnostic criterion for Pseudotumor cerebri syndrome (PTCS) in children. However, it has been proposed that a diagnosis of “probable” PTCS can be made with an OP < 28 cm H2O if other diagnostic criteria are met. We report a group of children with probable PTCS.Methods
Retrospective analysis of 25 children diagnosed with PTCS but with a CSF OP below 28 cm H2O. Eleven patients were identified during a nation-wide, prospective, active hospital-based surveillance, and additional 14 patients from our own institution. An extensive chart review of these cases was performed in order to identify signs and symptoms supportive of PTCS.Results
Of these 25 patients 23 were treated with acetazolamide. Five children required escalation of medical treatment. Findings supportive of PTCS in the absence of an abnormal OP were: papilledema (n = 24), abducens nerve palsy (n = 7), without papilledema in one of them, headache (n = 15). Six patients had a relapse. A second lumbar puncture (LP) documented an opening pressure of >30 cm H2O in seven children. MRI findings supportive of PTCS were seen in eight patients.Conclusions
The diagnosis of probable PTCS as a subgroup of PTCS can be convincingly made in children with an OP < 28 cm H2O. Results of opening pressure measurement always need to be interpreted within the whole clinical context. Treatment decisions in patients with “probable” PTCS should follow the same stage-based principles as for “proven” PTCS. 相似文献13.
B.J. van der Knoop K.J. Oostrom I.A. Zonnenberg M.M. van Weissenbruch R.J. Vermeulen J.I.P. de Vries 《European journal of paediatric neurology》2018,22(5):845-853
Background
Maternal trauma complicates pregnancy in approximately 7%. Long-term development of children exposed to maternal trauma is unknown.Aim
To determine neurobehavioural outcome of children (6–18 years) born after maternal trauma in pregnancy compared to a matched control group.Study design
Case-control study performed at a tertiary medical centre.Subjects
All consecutive children born after maternal hospitalization for trauma during pregnancy between 1995 and 2005. Controls were children born at the same hospital and period after an uneventful pregnancy.Outcome measures
Trauma type and severity (Injury Severity Score, ≥9: severe); information from medical files at admission (cases). All mothers filled out two questionnaires about the infant; 1. concerning health, motor development and educational level, 2. concerning behavioural development through the validated Dutch version of the Child Behavior Checklist (CBCL).Results
Questionnaires were returned by 34 cases and 28 controls. The traumas concerned mainly motor vehicle accidents and falls, and 3/34 had severe injuries. No differences in health, motor development, educational level and CBCL was found between the cases and controls, except for more hospitalization in the cases (p = 0.009).Conclusion
Long-term follow-up of a limited population of children 6–18 years after exposure of mainly non-severe trauma before birth is similar to a control population except for unexplained more hospitalization in the cases. 相似文献14.
Tamara Dostanic Breda Sustersic Darja Paro-Panjan 《European journal of paediatric neurology》2018,22(4):682-689
Background
The quality of general movements (GMs) has proven to have predictive value for the developmental outcome, but this has not yet been studied in twins.Aims
Our aim was to analyse the quality of GMs and neurological and developmental outcome in relation to the gestational age (GA), mode of conception and other perinatal risk factors in a group of twins.Study design
The documentation of twins referred for follow-up in the period from 1998 to 2016 was studied retrospectively. Data concerning the quality of GMs, perinatal risk factors and developmental outcome were analysed.Subjects
Eighty-nine twin pairs, GA from 24 to 38 weeks (median 35.0; IQR 3), birth weight 670 g–3820 g (median 2323; IQR 645) were included.Outcome measures
Results of neurological, psychological and speech/language development and school outcome were analysed.Results
GMs at term age and at three to four months postterm age did not differ with regard to the mode of conception. Preterm birth was significantly related to GMs at three to four months postterm age. At term age, GMs were significantly related to neurological outcome, while at three to four months postterm age, GMs were related to both the neurological and psychological outcome. Difficulties in speech/language development were diagnosed in almost half of the children, more frequently in boys and children with lower Apgar scores.Conclusion
The study highlights the value of GMs for predicting the developmental outcome in twins and indicates the importance of developmental, especially speech/language, follow-up in twins. 相似文献15.
J. Toulouse M. Hully D. Brossier M.-L. Viallard L. de Saint Blanquat S. Renolleau M. Kossorotoff I. Desguerre 《European journal of paediatric neurology》2019,23(1):171-180
Background
In Pediatric Intensive Care Unit (PICU) two types of population require the intervention of neuropediatricians (NP): chronic brain diseases’ patients who face repetitive and prolonged hospitalizations, and patients with acute brain failure facing the risk of potential neurologic sequelae, and both conditions may result in a limitation of life-sustaining treatments (LLST) decision.Objective
To assess NP's involvement in LLST decisions within the PICU of a tertiary hospital.Method
Retrospective study of medical reports of patients hospitalized during 2014 in the Necker-Hospital PICU. Patients were selected using keywords (“cardiorespiratory arrest”, “death”, “withdrawal of treatment”, “palliative care”, “acute brain failure”, or “chronic neurological disease”), and/or if they were assessed by a NP during the hospitalization. Demographic and medical data were analysed, including the NP's assessment and data about Collaborative Multidisciplinary Deliberation (CMD) to discuss potential LLST.Results
Among 1160 children, 274 patients were included and 142 (56%) were assessed by a NP during their hospitalization for diagnosis (n = 55) and/or treatment (n = 95) management. NP was required for 59%–100% of patients with neurological acute failure, and for 14–44% of patients with extra neurological failure. A LLST decision was taken after a CMD for 27 (9.8%) of them, and a NP was involved in 19/27 (70%) of these decisions that occurred during the hospitalization (n = 19) or before (n = 8).12 patients died thereafter the LLST decision (40% of the 30 dead patients).Conclusion
NP are clearly involved in the decision-process of LLST for patients admitted in PICU, claiming for close collaboration to improve current practices and the quality of the care provided to children. 相似文献16.
Stephanie Garcia-Tarodo Alexandre N. Datta Gian P. Ramelli Fabienne Maréchal-Rouiller Christian G. Bien Christian M. Korff 《European journal of paediatric neurology》2018,22(3):396-403
Objective
The role of autoimmunity and neural antibodies is increasingly recognized in different forms of seizures and epilepsy. Their prevalence in new-onset epilepsy has also recently been the focus of several clinical cohorts in the adult and pediatric population, with positive titers in 10–11% of cases. Our aim was to determine the seropositivity at the first seizure onset in a non-selective group of children.Method
We conducted a prospective multicenter cohort study recruiting children aged 0–16 years with new-onset seizures presenting at the In- and Outpatient Pediatric Neurology Departments of three Children's Hospitals in Switzerland between September 2013 and April 2016. Neural antibodies were screened within the first 6 months of a first seizure and when positive, repeated at 1 month and 6 months follow-up.Results
A total of 103 children were enrolled with a mean age at presentation of 5 years (range 1 day–15 years 9 months). The majority (n = 75) presented with generalized seizures and 6 had status epilepticus lasting > 30 min. At the time of onset, 55% of patients had fever, 24% required emergency seizure treatment and 27% hospitalization. Epilepsy was diagnosed at follow-up in 18%. No specific antibody was found. Serum antibodies against the VGKC complex, without binding to the specific antigens LGI1 and CASPR2, were found in two patients. Four patients harbored not otherwise characterized antibodies against mouse neuropil.Interpretation
Specific neural antibodies are rarely found in an unselected population of children that present with a first seizure. Applying an extensive neuronal antibody profile in a child with new-onset seizures does not appear to be justified. 相似文献17.
Juliana T. Pacheco Timo Siepmann Jessica Barlinn Simon Winzer Ana Isabel Penzlin Volker Puetz Maja von der Hagen Kristian Barlinn 《European journal of paediatric neurology》2018,22(6):1035-1041
Background and purpose
Safety and efficacy of intravenous (IV) thrombolysis and endovascular therapy in children with acute ischemic stroke (AIS) are unknown to date. We aimed to review and synthesize currently available evidence on these acute recanalization therapies in pediatric stroke patients.Methods
We performed a systematic review and meta-analysis of all available data on safety and efficacy of acute treatment including thrombolysis and endovascular therapy in pediatric AIS patients aged <18 years. We searched the electronic databases Medline and Cochrane Library for eligible studies published from the earliest date available until August 31, 2016. Safety outcomes included intracerebral hemorrhage (ICH) post-treatment and in-hospital mortality. Efficacy outcomes included functional outcome 3–6 months after index stroke.Results
We identified 222 records, of which 3 studies with a total of 16,987 pediatric stroke patients met our eligibility criteria of whom 181 received IV thrombolysis. No data exists from randomized trials and no data is available on endovascular thrombectomy. Risk of any ICH was increased in children receiving thrombolysis (risk ratio = 3.48, 95%CI: 1.66–7.29; p = 0.001) compared with controls, with no evidence of heterogeneity (I2 = 0%). None of the included studies reported complete data on symptomatic ICH. In-hospital mortality was similar between pediatric stroke patients treated with thrombolysis and controls (risk ratio = 1.44, 95%CI: 0.39–5.40; p = 0.586), with evidence of heterogeneity (I2 = 62%). Efficacy of revascularization therapies could not be analyzed due to lack of outcome data.Conclusions
Our analyses demonstrate a substantial lack of data on efficacy and safety of acute recanalization therapies in children with AIS.PROSPERO Registration Information
URL: http://www.crd.york.ac.uk/PROSPERO. Unique identifier: CRD42016047140. 相似文献18.
M. Blankenburg J. Junker G. Hirschfeld E. Michel F. Aksu J. Wager B. Zernikow 《European journal of paediatric neurology》2018,22(3):470-481
Introduction
Many patients with cerebral palsy (CP) suffer chronic pain as one of the most limiting factors in their quality of life. In CP patients, pain mechanisms are not well understood, and pain therapy remains a challenge. Quantitative sensory testing (QST) might provide unique information about the functional status of the somatosensory system and therefore better guide pain treatment.Objectives
To understand better the underlying pain mechanisms in pediatric CP patients, we aimed to assess clinical and pain parameters, as well as QST profiles, which were matched to the patients' cerebral imaging pathology.Patients and methods
Thirty CP patients aged 6–20 years old (mean age 12 years) without intellectual impairment underwent standardized assessments of QST. Cerebral imaging was reassessed. QST results were compared to age- and sex-matched controls (multiple linear regression; Fisher's exact test; linear correlation analysis).Results
CP patients were less sensitive to all mechanical and thermal stimuli than healthy controls but more sensitive to all mechanical pain stimuli (each p < 0.001). Fifty percent of CP patients showed a combination of mechanical hypoesthesia, thermal hypoesthesia and mechanical hyperalgesia; 67% of CP patients had periventricular leukomalacia (PVL), which was correlated with mechanic (r = 0.661; p < 0.001) and thermal (r = 0.624; p = 0.001) hypoesthesia.Conclusion
The combination of mechanical hypoesthesia, thermal hypoesthesia and mechanical hyperalgesia in our CP patients implicates lemniscal and extralemniscal neuron dysfunction in the thalamus region, likely due to PVL. We suspect that extralemniscal tracts are involved in the original of pain in our CP patients, as in adults. 相似文献19.
René Kurz Julia Huemer Elvira Muchitsch Martha Feucht 《European journal of paediatric neurology》2018,22(5):803-806
Objective
To evaluate prospectively the effectiveness of cognitive behavioral therapy (CBT) in children with autism spectrum disorder (ASD).Methods
Drug-naïve children who met the DSM-V criteria for a diagnosis of ASD were recruited from a day care center, specialized in long-term treatment of children and adolescents with ASD. Symptom assessment was performed using the Aberrant Behavior Checklist (ABC) before (base-line) and after 12 months (follow-up) of CBT.Results
Nine boys with a mean age of 6 (±2.0) years were included. Compared to baseline, significant improvements of symptoms of irritability (p = 0.012), hyperactivity (p = 0.008) and lethargy (p = 0.008) were observed at follow-up.Conclusion
Results indicate that CBT is an effective therapy for children with ASD. Larger studies are needed to give more details about which symptoms respond best in these patients. 相似文献20.
Zvi Leibovitz Hanna Mandel Tzipora C. Falik-Zaccai Shani Ben Harouch David Savitzki Karina Krajden-Haratz Liat Gindes Mordechai Tamarkin Dorit Lev William B. Dobyns Tally Lerman-Sagie 《European journal of paediatric neurology》2018,22(3):525-531