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OBJECTIVE: Infants of diabetic mothers (IDM) are at higher risk of perinatal morbidities and glycemic instability, but the impact of maternal diabetes on neonatal and neurological short-term outcomes of neonates with hypoxic-ischemic encephalopathy (HIE) remains poorly described. Our objective was to determine the impact of maternal diabetes on neonatal and neurological short-term outcomes following neonatal HIE. STUDY DESIGN: This was a retrospective single-center study including 102 term neonates with HIE who received therapeutic hypothermia (TH) treatment between 2013 and 2020. Multiple regression analysis was used to assess the relationship between the presence of maternal diabetes and short-term outcomes. RESULTS: Neonates with HIE and maternal diabetes exposure had a significantly lower gestational age at birth (38.6 vs. 39.7 weeks of gestation, p = 0.005) and a significantly higher mean birth weight (3,588 ± 752 vs. 3,214 ± 514 g, p = 0.012). IDM with HIE were ventilated for longer duration (8 vs. 4 days, p = 0.0047) and had a longer neonatal intensive care unit (NICU) stay (18 vs. 11 days, p = 0.0483) as well as took longer time to reach full oral feed (15 vs. 7 days, p = 0.0432) compared with neonates of nondiabetic mother. Maternal diabetes was also associated with an increased risk of death or abnormal neurological examination at discharge in neonates with HIE (odds ratio: 6.41 [1.54-26.32]). CONCLUSION: In neonates with HIE, maternal diabetes is associated with an increased risk of death or short-term neonatal morbidities, such as longer duration of ventilation, prolonged neonatal stay, greater need for tube feeding, and being discharged with an abnormal neurological examination. Strategies to prevent, reduce, or better control maternal diabetes during pregnancy should be prioritized to minimize complications after perinatal asphyxia. KEY POINTS: · Maternal DB is associated with unfavorable outcomes.. · IDM have longer ventilatory support and tube feeding.. · IDM have higher risk of abnormal neurological examination..
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OBJECTIVE: We investigated how electroencephalography (EEG) quantitative measures and dysglycemia relate to neurodevelopmental outcomes following neonatal encephalopathy (NE). METHODS: This retrospective study included 90 neonates with encephalopathy who received therapeutic hypothermia. EEG absolute spectral power was calculated during post-rewarming and 2-month follow-up. Measures of dysglycemia (hypoglycemia, hyperglycemia, and glycemic lability) and glucose variability were computed for the first 48 h of life. We evaluated the ability of EEG and glucose measures to predict neurodevelopmental outcomes at ≥ 18 months, using logistic regressions (with area under the receiver operating characteristic [AUROC] curves). RESULTS: The post-rewarming global delta power (average all electrodes), hyperglycemia and glycemic lability predicted moderate/severe neurodevelopmental outcome separately (AUROC = 0.8, 95%CI [0.7,0.9], p < .001) and even more so when combined (AUROC = 0.9, 95%CI [0.8,0.9], p < .001). After adjusting for NE severity and magnetic resonance imaging (MRI) brain injury, only global delta power remained significantly associated with moderate/severe neurodevelopmental outcome (odds ratio [OR] = 0.9, 95%CI [0.8,1.0], p = .04), gross motor delay (OR = 0.9, 95%CI [0.8,1.0], p = .04), global developmental delay (OR = 0.9, 95%CI [0.8,1.0], p = .04), and auditory deficits (OR = 0.9, 95%CI [0.8,1.0], p = .03). CONCLUSIONS: In NE, global delta power post-rewarming was predictive of outcomes at ≥ 18 months. SIGNIFICANCE: EEG markers post-rewarming can aid prediction of neurodevelopmental outcomes following NE.
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Eletroencefalografia , Hipotermia Induzida , Humanos , Masculino , Feminino , Recém-Nascido , Eletroencefalografia/métodos , Estudos Retrospectivos , Transtornos do Neurodesenvolvimento/fisiopatologia , Transtornos do Neurodesenvolvimento/etiologia , Transtornos do Neurodesenvolvimento/diagnóstico , Hiperglicemia/fisiopatologia , Hiperglicemia/complicações , Hipoglicemia/fisiopatologia , Hipoglicemia/complicações , Encefalopatias/fisiopatologia , Glicemia/metabolismo , LactenteRESUMO
BACKGROUND: A growing body of literature demonstrates that survivors of congenital heart defects (CHD) are at increased risk of neurodevelopmental delay, which frequently manifests as motor delay during the first year of life. OBJECTIVE: The aim of this study was to determine prenatal predictors of an early atypical neurodevelopment. This information could help assist decision-making during prenatal counseling. STUDY DESIGN: In this retrospective cohort study, we evaluated the records of 75 children with CHD followed at the Clinique d'Investigation Neuro-Cardiaque (CINC) of the CHU Ste-Justine born between 2013 and 2016. The neurodevelopmental outcome was determined using the Alberta Infant Motor Scale (AIMS) at 4 months. Associations between prenatal factors and atypical neurodevelopment (AIMS < 10th percentile) were assessed using bivariate and multivariate analyses. RESULTS: Forty-four infants (58.7%) had atypical neurodevelopment. When there was no extra cardiac anomaly seen on prenatal ultrasound, a head to abdominal ratio (HC/AC) below 1.1 was associated with a four-fold increased risk of atypical neurodevelopment (OR = 4.54; 95% CI = 1.24-16.64 p = .023). There was no difference in identified genetic anomaly in both groups. However, there was a trend toward more extra cardiac anomalies in infants with atypical neurodevelopment (27.3%) compared to 9.7% in those with typical neurodevelopment (p = .061). CONCLUSION: Our study shows that early atypical neurodevelopment affects the majority of children with CHD and highlights the importance of post-natal monitoring by a specialized team. A thorough prenatal ultrasound is important to screen for those at higher risk i.e. those with extra cardiac anomaly and HC/AC below 1.1. A larger cohort is needed to validate those results.
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Cardiopatias Congênitas , Lactente , Gravidez , Criança , Feminino , Humanos , Estudos Retrospectivos , Cardiopatias Congênitas/complicações , Estudos de Coortes , AlbertaRESUMO
BACKGROUND: Therapeutic hypothermia (TH) without sedation may lead to discomfort, which may be associated with adverse consequences in neonates with hypoxic-ischemic encephalopathy (HIE). The aim of this study was to assess the association between level of exposure to opioids and temperature, with electroencephalography (EEG) background activity post-TH and magnetic resonance imaging (MRI) brain injury in neonates with HIE. METHODS: Thirty-one neonates with mild-to-moderate HIE who underwent TH were identified. MRIs were reviewed for presence of brain injury. Quantitative EEG background features including EEG discontinuity index and spectral power densities were calculated during rewarming and post-rewarming periods. Dose of opioids administered during TH and temperatures were collected from the medical charts. Multivariable linear and logistic regression analyses were conducted to assess the associations between cumulative dose of opioids and temperature with EEG background and MRI while adjusting for markers of HIE severity. RESULTS: Higher opioid doses (ß = -0.21, p = 0.02) and reduced skin temperature (ß = 0.14, p < 0.01) were associated with lower EEG discontinuity index recorded post-TH. Higher opioid doses (ß = 0.75, p = 0.01) and reduced skin temperature (ß = -0.39, p = 0.02) were also associated with higher EEG Delta power post-TH. MRI brain injury was observed in 14 patients (45%). In adjusted regression analyses, higher opioid doses (OR = 0.00; 95%CI: 0-0.19; p = 0.01), reduced skin temperature (OR = 41.19; 95%CI: 2.27-747.86; p = 0.01) and reduced cooling device output temperature (OR = 1.91; 95%CI: 1.05-3.48; p = 0.04) showed an association with lower odds of brain injury. CONCLUSIONS: Higher level of exposure to opioids and reduced skin temperature during TH in mild-to-moderate HIE were associated with improved EEG background activity post-TH. Moreover, higher exposure to opioids, reduced skin temperature and reduced device output temperature were associated with lower odds of brain injury on MRI.
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Analgesia , Lesões Encefálicas , Hipotermia Induzida , Hipóxia-Isquemia Encefálica , Analgésicos Opioides/uso terapêutico , Lesões Encefálicas/complicações , Eletroencefalografia/métodos , Humanos , Hipotermia Induzida/métodos , Hipóxia-Isquemia Encefálica/complicações , Hipóxia-Isquemia Encefálica/diagnóstico por imagem , Hipóxia-Isquemia Encefálica/terapia , Recém-Nascido , Imageamento por Ressonância Magnética/métodos , TemperaturaRESUMO
OBJECTIVES: To determine the etiologies and evolution of rhabdomyolysis in children. STUDY DESIGN: We performed a retrospective study of patients with rhabdomyolysis who were seen in our tertiary care university-affiliated pediatric hospital. Patients in outpatient clinics, seen in the emergency department, or admitted from 2001 to 2002 were selected. With a standardized case report form, we collected predetermined data from each patient's chart. RESULTS: A total of 130 patients with rhabdomyolysis were included in the study (male, 56%; mean age, 7.5 ± 5.9 years). The median elevation of creatine phosphokinase was 2207 IU/L (range, 1003 to 811 428 IU/L). The most frequent diagnoses were viral myositis (29, 22.3%), trauma (24, 18.4%), surgery (24, 18.4%), hypoxia (12 , 9.2%), and drug reaction (8, 6.2%). Metabolic myopathy was found only in one patient (0.8%). In 17 patients (13.1%), no definite diagnosis could be made. CONCLUSIONS: Etiologies of rhabdomyolysis in children are varied and differ from those reported in adults. In most patients, rhabdomyolysis is benign and without recurrence. In our series, rhabdomyolysis was the initial symptom of a metabolic myopathy in only one patient.
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Creatina Quinase/sangue , Rabdomiólise/sangue , Rabdomiólise/etiologia , Adolescente , Criança , Pré-Escolar , Estado Terminal/epidemiologia , Hipersensibilidade a Drogas/epidemiologia , Feminino , Humanos , Hipóxia/epidemiologia , Lactente , Recém-Nascido , Masculino , Doenças Musculares/epidemiologia , Doenças Musculares/metabolismo , Miosite/epidemiologia , Miosite/virologia , Complicações Pós-Operatórias/epidemiologia , Recidiva , Estudos Retrospectivos , Rabdomiólise/mortalidade , Rabdomiólise/terapia , Sepse/epidemiologia , Ferimentos e Lesões/epidemiologiaRESUMO
Vigabatrin (VGB) is approved as monotherapy for pediatric patients with Infantile Spasms (IS). Duration of VGB use should be limited because of the risk of retinal and neurotoxicity, but the optimal length of treatment is unknown. Our study aimed to determine the risk of spasms relapse after 6 months of VGB as first-line therapy in IS patients deemed VGB good responders. The participants were 44 infants with IS who demonstrated both absence of clinical spasms and hypsarrhythmia four weeks after starting VGB, obtained from two cohorts: 29 patients from a multicenter prospective cohort and 15 patients from a retrospective single-center cohort. We divided them post hoc into two groups according to the duration of VGB treatment: 6-month group (n=34) and >6-month group (n=10) and compared outcome between the two groups. No patient in either group had a relapse of spasms. For patients with non-identified etiology (NIE) in the 6 months treatment group, no other seizure types were observed. Late epilepsy, in the form of focal seizures, emerged in only 5/37 patients (3/30 in the 6-month treatment group; 2/7 in the extended treatment group); all within the first 6-9 months after VGB initiation. Our study provides substantial evidence that a shortened VGB course of 6 months could be sufficient to treat and prevent relapse of spasms in children with IS, particularly those with NIE.
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Espasmos Infantis , Vigabatrina , Anticonvulsivantes/efeitos adversos , Criança , Humanos , Lactente , Estudos Prospectivos , Estudos Retrospectivos , Espasmo/tratamento farmacológico , Espasmos Infantis/tratamento farmacológico , Resultado do Tratamento , Vigabatrina/efeitos adversosRESUMO
Purpose: This retrospective study aims to describe the gross motor development of children aged 4 to 24 months with congenital heart disease (CHD) enrolled in a systematic developmental follow-up program and to describe the frequency of physical therapy sessions they received between 4 and 8 months of age. Methods: Twenty-nine infants with CHD underwent motor evaluations using the AIMS at 4 months, and the Bayley-III at 12 and 24 months. Results: Based on AIMS, 79% of 4-month-old infants had a gross motor delay and required physical therapy. Among these, 56.5% received one to two physical therapy sessions, and 43.5% received three to six sessions. Infants who benefited from regular interventions tended to show a better improvement in motor scores from 12 to 24 months. Conclusion: This study highlights the importance of early motor screening in infants with CHD and suggests a potential benefit of early physical therapy in at-risk children. Abbreviations: CHD: Congenital heart disease; AIMS: Alberta Infant Motor Scales; Bayley-III: Bayley Scales of Infant and Toddler Development, Third edition; Bayley-III/GM: Gross Motor section of the Bayley Scales of Infant and Toddler Development, Third edition.
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Desenvolvimento Infantil , Deficiências do Desenvolvimento/epidemiologia , Intervenção Médica Precoce/métodos , Cardiopatias Congênitas/terapia , Movimento , Modalidades de Fisioterapia , Pré-Escolar , Feminino , Cardiopatias Congênitas/fisiopatologia , Humanos , Lactente , Recém-Nascido , MasculinoRESUMO
This longitudinal study aims to describe the trajectory of language development in children with CHD aged 12-24 months assessed through an early monitoring and individualized intervention program. We also sought to determine whether early language performances, at 12 months of age, predict 24-month language abilities. We conducted developmental assessments of 49 children with CHD using the Bayley Scales of Infant and Toddler Developmental, third edition (Bayley-III) at 12 and 24 months, and the MacArthur-Bates Communicative Development Inventories (MBCDI) at 12, 18 and 24 months. Compared to normative populations, CHD patients showed significantly lower mean scores in both receptive and expressive language scales of the Bayley-III and the MBCDI at 12 months, whereas at 18 and 24 months only expressive language scores were reduced. No differences were found in the cognitive scale. Communicative gestures at 12 months were significantly predictive of language skills at 24 months of age. Our findings indicate specific vulnerability of language outcome, especially in expressive skills, rather than a global cognitive impairment in our patients with CHD. We recommend using communicative gestures as an early marker of language development to improve our ability to detect language delays in this population.
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Cardiopatias Congênitas/complicações , Transtornos do Desenvolvimento da Linguagem/epidemiologia , Transtornos do Desenvolvimento da Linguagem/etiologia , Desenvolvimento da Linguagem , Pré-Escolar , Feminino , Humanos , Lactente , Estudos Longitudinais , MasculinoRESUMO
PURPOSE: Children with complex congenital heart disease (CHD) experience high incidence of perioperative seizures. Population-based studies also report high epilepsy co-morbidity in CHD. Given the increasing survival of patients with CHD and the interference of seizures and epilepsy with the long-term outcomes, characterizing them in this population is of high relevance. This study investigated the incidence and risk factors of perioperative clinical seizures (CS) and epilepsy in a prospective cohort of children with complex CHD who underwent cardiac surgery. METHODS: We included 128 consecutive children with CHD, followed for at least two years at the neurocardiac clinic of Montreal's Sainte-Justine University Hospital Center. We collected perinatal, surgical, critical care and clinical follow-up information and performed logistic regression to reveal risk factors of CS and epilepsy. RESULTS: Ten patients (7.8%) experienced perioperative CS. Four of them (40%) developed epilepsy. The incidence of epilepsy was therefore 3.1%. Higher surgical complexity scores, delayed sternal closure, extracorporeal membrane oxygenation (ECMO) use, longer intensive care and hospital stay were associated with CS. ECMO use and hospital stay were also associated with epilepsy. Nine (90%) patients with CS had brain injuries: five strokes, one white matter and three hypoxic-ischemic injury (HII). All patients with HII developed epilepsy, which became intractable in one of them. CONCLUSION: Our study reports high incidence, surgical risk factors and brain injury patterns underlying CS and epilepsy in CHD. Further studies are needed to investigate how epilepsy interferes with neurodevelopment and quality of life in CHD.
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Procedimentos Cirúrgicos Cardíacos/estatística & dados numéricos , Ponte Cardiopulmonar/estatística & dados numéricos , Epilepsia/epidemiologia , Cardiopatias Congênitas/epidemiologia , Avaliação de Resultados em Cuidados de Saúde/estatística & dados numéricos , Período Perioperatório , Complicações Pós-Operatórias/epidemiologia , Convulsões/epidemiologia , Comorbidade , Feminino , Cardiopatias Congênitas/cirurgia , Humanos , Incidência , Lactente , Recém-Nascido , Masculino , Estudos Prospectivos , Fatores de RiscoRESUMO
Gamma band electroencephalography (EEG) abnormalities have been reported in patients with epilepsy. We aimed to investigate whether patients with febrile seizures (FS) show abnormalities of the gamma frequency steady-state visual evoked potential (SSVEP) components evoked by intermittent photic stimulation (IPS). We analyzed the magnitude and phase alignment of the 50-100 Hz SSVEP components elicited by IPS from 12 FS patients, 5 siblings of FS patients, and 15 control children between 6 and 36 months of age. Patients with FS showed significantly higher SSVEP magnitude and phase alignment values when compared to both the siblings and control groups. Detected abnormalities could either represent the direct consequence of seizures or indicate a preexisting tendency to hypersynchrony in FS patients. Future prospective studies could assess whether SSVEP abnormalities are associated with complex rather than simple FS, or have a prognostic value for the development of epilepsy following FS.
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Convulsões Febris/diagnóstico , Criança , Eletroencefalografia , Potenciais Evocados Visuais/fisiologia , Feminino , Humanos , Masculino , Estimulação Luminosa , Fatores de Risco , Índice de Gravidade de DoençaRESUMO
Infants with congenital heart disease are at risk of impaired neurodevelopment, which frequently manifests as motor delay during their first years of life. This delay is multifactorial in origin and environmental factors, such as a limited experience in prone, may play a role. In this study, we evaluated the motor development of a prospective cohort of 71 infants (37 males) with congenital heart disease at 4 months of age using the Alberta Infant Motor Scales (AIMS). We used regression analyses to determine whether the 4-month AIMS scores predict the ability to walk by 18 months. The influence of demographic and clinical variables was also assessed. Fifty-one infants (71.8%) were able to maintain the prone prop position (AIMS score of ≥3 in prone) at 4 months. Of those, 47 (92.2%) were able to walk by 18 months compared to only 12/20 (60%) of those who did not maintain the position. Higher AIMS scores were predictive of a greater likelihood of walking by 18 months ( P < .001), with the scores in prone having a higher predictive ability compared to those in other positions (Exp(B) 15.2 vs 4.0). Shorter hospital stays and female gender were also associated with an earlier onset of walking. In conclusion, our study demonstrates that early ventral performance in infants with congenital heart disease impacts the age of acquisition of walking and could be used to guide referral to rehabilitation.
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Deficiências do Desenvolvimento/etiologia , Cardiopatias Congênitas/complicações , Transtornos das Habilidades Motoras/etiologia , Caminhada/fisiologia , Estudos de Coortes , Feminino , Humanos , Lactente , Recém-Nascido , Modelos Logísticos , Masculino , Atividade MotoraRESUMO
PURPOSE: Seizures are common in critically ill neonates. Both seizures and antiepileptic treatments may lead to short term complications and worsen the outcomes. Predicting the risks of seizure reoccurrence could enable individual treatment regimens and better outcomes. We aimed to identify EEG signatures of seizure reoccurrence by investigating periictal electrographic features and spectral power characteristics in hypothermic neonates with hypoxic-ischemic encephalopathy (HIE) with or without reoccurrence of seizures on rewarming. METHODS: We recruited five consecutive HIE neonates, submitted to continuous EEG monitoring, with high seizure burden (>20% per hour) while undergoing therapeutic hypothermia. Two of them had reoccurrence of seizures on rewarming. We performed quantitative analysis of fifteen artifact-free consecutive seizures to appreciate spectral power changes between the interictal, preictal and ictal periods, separately for each patient. Visual analysis allowed description of electrographic features associated with ictal events. RESULTS: Every patient demonstrated a significant increase in overall spectral power from the interictal to preictal and ictal periods (p<0.01). Alpha power increase was more pronounced in the two patients with reoccurrence of seizures on rewarming and significant when comparing both interictal-to-preictal and interictal-to-ictal periods. This alpha activity increase could be also appreciated using visual analysis and distinguished neonates with and without seizure reoccurrence. CONCLUSION: This distinct alpha activity preceding ictal onset could represent a biomarker of propensity for seizure reoccurrence in neonates. Future studies should be performed to confirm whether quantitative periictal characteristics and electrographic features allow predicting the risks of seizure reoccurrence in HIE neonates and other critically ill patients.
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Encéfalo/fisiopatologia , Hipotermia Induzida , Hipóxia-Isquemia Encefálica/fisiopatologia , Hipóxia-Isquemia Encefálica/terapia , Convulsões/fisiopatologia , Convulsões/terapia , Eletroencefalografia , Feminino , Humanos , Hipóxia-Isquemia Encefálica/complicações , Hipóxia-Isquemia Encefálica/diagnóstico , Recém-Nascido , Masculino , Prognóstico , Estudos Retrospectivos , Convulsões/complicações , Convulsões/diagnósticoAssuntos
Encéfalo , Convulsões , Eletroencefalografia , Humanos , Recém-Nascido , Monitorização Fisiológica , Convulsões/terapiaRESUMO
OBJECTIVE: To investigate how rewarming impacts the evolution of EEG background in neonates with hypoxic-ischemic encephalopathy (HIE) undergoing therapeutic hypothermia (TH). METHODS: We recruited a retrospective cohort of 15 consecutive newborns with moderate (9) and severe (6) HIE monitored with a continuous EEG during TH and at least 12h after its end. EEG background was analyzed using conventional visual and quantitative EEG analysis methods including EEG discontinuity, absolute and relative spectral magnitudes. One patient with seizures on rewarming was excluded from analyses. RESULTS: Visual and quantitative analyses demonstrated significant changes in EEG background from pre- to post-rewarming, characterized by an increased EEG discontinuity, more pronounced in newborns with severe compared to moderate HIE. Neonates with moderate HIE also had an increase in the relative magnitude of slower delta and a decrease in higher frequency theta and alpha waves with rewarming. CONCLUSIONS: Rewarming affects EEG background in HIE newborns undergoing TH, which may represent a transient adaptive response or reflect an evolving brain injury. SIGNIFICANCE: EEG background impairment induced by rewarming may represent a biomarker of evolving encephalopathy in HIE newborns undergoing TH and underscores the importance of continuously monitoring the brain health in critically ill neonates.
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Eletroencefalografia/métodos , Hipotermia Induzida/métodos , Hipóxia-Isquemia Encefálica/diagnóstico , Hipóxia-Isquemia Encefálica/terapia , Reaquecimento/métodos , Nascimento a Termo/fisiologia , Estudos de Coortes , Feminino , Humanos , Hipóxia-Isquemia Encefálica/fisiopatologia , Recém-Nascido , Masculino , Estudos RetrospectivosRESUMO
OBJECTIVE: To evaluate whether structural and microstructural brain abnormalities in neonates with congenital heart disease (CHD) correlate with neuronal network dysfunction measured by analysis of EEG connectivity. METHODS: We studied a prospective cohort of 20 neonates with CHD who underwent continuous EEG monitoring before surgery to assess functional brain maturation and network connectivity, structural magnetic resonance imaging (MRI) to determine the presence of brain injury and structural brain development, and diffusion tensor MRI to assess brain microstructural development. RESULTS: Neonates with MRI brain injury and delayed structural and microstructural brain development demonstrated significantly stronger high-frequency (beta and gamma frequency band) connectivity. Furthermore, neonates with delayed microstructural brain development demonstrated significantly weaker low-frequency (delta, theta, alpha frequency band) connectivity. Neonates with brain injury also displayed delayed functional maturation of EEG background activity, characterized by greater background discontinuity. INTERPRETATION: These data provide new evidence that early structural and microstructural developmental brain abnormalities can have immediate functional consequences that manifest as characteristic alterations of neuronal network connectivity. Such early perturbations of developing neuronal networks, if sustained, may be responsible for the persistent neurocognitive impairment prevalent in adolescent survivors of CHD. These foundational insights into the complex interplay between evolving brain structure and function may have relevance for a wide spectrum of neurological disorders manifesting early developmental brain injury.
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PURPOSE: To assess the influence of the family history (FH) of epilepsy or febrile seizures (FSs) on the clinical presentation of FSs and on their outcome. METHODS: We reviewed the charts of 482 children admitted to the Ste-Justine Hospital with FSs between 3 months and 6 years of age and followed for at least 5 years. RESULTS: Children with a positive FH of epilepsy (n=67) showed significantly more focal and recurrent FSs than those without such a FH. The risk of developing partial epilepsy (n=17) or generalized epilepsy (n=19) was significantly greater in children with focal or recurrent FSs, respectively. In children with focal FSs, only two out of 30 (6.7%) children with a negative FH of epilepsy developed partial epilepsy compared with four out of nine (44.4%) children with a positive FH. In children with recurrent FSs, as much as seven out of 34 (20.6%) children with a positive FH of epilepsy developed generalized epilepsy compared to only eight out of 161 (0.05%) of those with a negative FH. Nevertheless, when not taking into account the clinical presentation of FSs, the positive FH of epilepsy constituted a risk factor for developing generalized but not partial epilepsy. Finally, children with a positive FH of FSs (n=120) exhibited significantly more recurrent FSs than those without such a FH, but this did not modify the risk of epilepsy. CONCLUSION: The FH of FSs and/or epilepsy should be taken into account when evaluating the risk of FSs recurrence and of epilepsy.
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Epilepsia/complicações , Epilepsia/genética , Convulsões Febris/complicações , Convulsões Febris/genética , Criança , Pré-Escolar , Epilepsias Parciais/etiologia , Epilepsias Parciais/genética , Epilepsia Generalizada/etiologia , Epilepsia Generalizada/genética , Feminino , Predisposição Genética para Doença , Humanos , Masculino , Prontuários Médicos , Recidiva , Estudos Retrospectivos , Fatores de RiscoRESUMO
AIM: Lacosamide is an antiepileptic drug approved for the treatment of focal epilepsy in adult patients. The aim of this observational study was to review our centre's experience with lacosamide and to characterize its effectiveness and tolerability as an adjunctive antiepileptic drug in a retrospective cohort of children with refractory focal epilepsy. METHODS: We retrospectively reviewed the medical records of 22 patients who received lacosamide from November 2009 to April 2014 at the CHU Ste-Justine, University of Montreal. Treatment responders were defined as children with a ≥50% reduction in seizure frequency compared to baseline, and this was determined three months after the initiation of treatment and at the last follow-up visit. RESULTS: We included 14 boys and eight girls with a mean age of 12.9 years (SD: 5.2; range: 5.2-20.7 years) at the initiation of treatment. The average length of follow-up was 11.9 months. Patients had previously received an average of 7.5 antiepileptic drugs. The mean number of concomitant antiepileptic drugs was 2.3. The mean initial and maintenance doses were 2.9 and 8.4 mg/kg/d, respectively. Thirteen (59%) and ten (45%) patients were responders after three months of treatment and at the last follow-up visit, respectively. One became seizure-free. Adverse effects were reported in 11 patients and none were severe. Responders and non-responders were identical with respect to all studied parameters except gender, with the proportion of responders being greater in girls than in boys (75% vs 29%; p=0.035). CONCLUSION: Our study adds evidence that lacosamide appears to be a safe and effective adjunctive therapy for children with refractory focal epilepsy.
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Acetamidas/uso terapêutico , Anticonvulsivantes/uso terapêutico , Epilepsia Resistente a Medicamentos/terapia , Epilepsias Parciais/terapia , Acetamidas/efeitos adversos , Adolescente , Anticonvulsivantes/efeitos adversos , Criança , Pré-Escolar , Terapia Combinada , Dieta Cetogênica , Epilepsia Resistente a Medicamentos/dietoterapia , Epilepsia Resistente a Medicamentos/tratamento farmacológico , Epilepsias Parciais/dietoterapia , Epilepsias Parciais/tratamento farmacológico , Feminino , Humanos , Lacosamida , Masculino , Estudos Retrospectivos , Resultado do Tratamento , Estimulação do Nervo Vago , Adulto JovemRESUMO
Infantile spasms constitute a severe epileptic encephalopathy of infancy with poor long-term developmental outcome. Many diverse etiologies have been associated with infantile spasms, but the pathophysiological process is still not fully understood. We describe 2 cases of previously healthy 1- and 3-month-old infants who suffered a nonaccidental head injury with extensive cerebral lesions. Both presented with acute focal seizures rapidly controlled with phenobarbital. Nevertheless, they developed infantile spasms after a latency period of 3-4 months. Spasms were rapidly controlled with vigabatrin. Both children manifested with developmental delay, either exacerbated (case 1) or elicited (case 2) by infantile spasms. Our report highlights nonaccidental head injury as a risk factor for developing infantile spasms following a seizure-free latency period. A better understanding of the pathophysiology linking accidental brain trauma with infantile spasms could lead to more effective neuroprotective strategies. In the meantime, increased awareness and follow-up are warranted.
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Traumatismos Craniocerebrais/complicações , Espasmos Infantis/complicações , Humanos , Lactente , Recém-Nascido , Imageamento por Ressonância Magnética , MasculinoRESUMO
Tuberous sclerosis complex (TSC) is associated with the potential development of benign hamartomas, including subependymal giant cell astrocytomas (SEGAs). Intracranial hypertension can be caused by SEGAs due to their propensity to block the foramen of Monro. The traditional management approach is to monitor SEGAs with periodic neuroimaging and to resect those that exhibit serial growth and/or cause clinical signs of intracranial hypertension. Recent observations suggest that rapamycin therapy may induce partial regression of SEGAs, therefore providing a potential alternative to resection. The authors present the case of an 8-year-old girl with bilateral SEGAs that led to progressive hydrocephaly and incipient signs of papilledema. Three months after initiating rapamycin therapy, the SEGAs exhibited significant reduction in size (82.6% on the left and 46.7% on the right), and the lesions remained stable 5 months later. Compared with previous case reports, similar or even greater antitumor efficacy was achieved with much lower trough levels of rapamycin (10–15 compared with 3.3–4.5 ng/ml, respectively). The authors discuss various aspects of rapamycin therapy and address unresolved issues that highlight the need for further prospective clinical trials.