RESUMO
Spinal muscular atrophy (SMA) is a devastating neuromuscular disorder with limited treatment options. Nusinersen is the first disease-modifying therapy to treat children and adults with SMA. This study aimed to review the safety, tolerability, and efficacy data of a nusinersen treatment program in Polish children. A total of 298 patients aged from 0 to 18 years were included in the nusinersen treatment program in Poland between March 1 and September 20, 2019. All patients were prospectively followed for at least one year. The mean age at treatment onset was 6.9 years. SMA type 1 symptoms were reported in 127 patients (43.5%), SMA type 2 symptoms in 68 cases (23.3%), and SMA type 3 in 93 patients (31.8%). No patient met the inefficiency criteria defined in the program. One year after treatment initiation, all patients assessed by the CHOP-INTEND scale had improved or remained stable. The mean change in CHOP-INTEND score was an increase of 8.9 points between baseline and after one-year treatment (p < 0.001). Except for 2 fatal cases, not related to the treatment, no serious adverse events were reported. The results of our study indicate that treatment with nusinersen is beneficial for children with SMA regardless of their age, baseline functional status, or the number of SMN2 gene copies. Therapy with nusinersen was effective and well tolerated by patients.
Assuntos
Atrofia Muscular Espinal , Atrofias Musculares Espinais da Infância , Adulto , Criança , Humanos , Atrofia Muscular Espinal/tratamento farmacológico , Oligonucleotídeos/efeitos adversos , Polônia , Atrofias Musculares Espinais da Infância/tratamento farmacológicoRESUMO
BACKGROUND: Epidemiologic data on pediatric-onset multiple sclerosis (POMS) in Central and Eastern Europe are limited. The aim of this study was to determine the incidence, prevalence and the clinical features of POMS in Poland. METHODS: Registry-based retrospective study was conducted among Polish children population (age ≤ 18 years), between 1 January 2010 and 31 December 2019. A total of 329 pediatric or juvenile patients fulfilled the International Pediatric MS Study Group (IPMSSG) criteria for MS, reported to the Polish Multiple Sclerosis Registry, were considered for estimation of age- and sex-specific prevalence (per 100,000 persons), and incidence rates (per 100,000 person-years). The demographic data, clinical presentation and treatment strategies also were investigated. RESULTS: On December 31, 2019 in the database were collected data of 329 patients up to 18 years with POMS diagnosis (101 boys and 228 girls; mean age 15.3 ± 3.8 years). The age-adjusted prevalence standardized to the European Standard Population was 5.19/100,000 (95% confidence interval (CI), 4.64-5.78). A significantly higher prevalence was recorded in girls (7.41; 95% CI, 6.48-8.44) than in boys (3.08; 95% CI, 2.50-3.74; P<0.001). The mean annual standardized incidence in Poland between 2015 - 2019 was 0.77 (95%CI, 0.45-1.02) per 100,000 person-years. The highest overall standardized incidence 1.06 (95%CI, 0.82-1.34) was noted in 2018. Most of patients (95.7%) had relapsing-remitting disease with polysymptomatic onset in one-thirds of the cases, and 82.3% were treated with disease-modifying drugs. Family history of MS was reported in 26 cases (7.9%). CONCLUSION: In this first report of registry-based study from Poland an increasing prevalence and incidence of POMS was found during the last years. This temporal trend corroborate the findings of studies conducted elsewhere.
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Esclerose Múltipla , Adolescente , Adulto , Criança , Feminino , Humanos , Incidência , Masculino , Esclerose Múltipla/epidemiologia , Polônia/epidemiologia , Sistema de Registros , Estudos Retrospectivos , Adulto JovemRESUMO
Background. Paediatric-onset MS (POMS) has a unique clinical profile compared to the more prevalent adult-onset MS. For this study, we aimed to determine the demographic and clinical characteristics of POMS in Poland as well as addressing some of its epidemiological aspects. Methods. A retrospective study was conducted based on the Polish Multiple Sclerosis Registry, considering a population of children and adolescents with MS (age ≤ 18 years). Data were collected by all 13 centres across Poland specializing in diagnosing and treating POMS. The actual course of the disease and its clinical properties were compared between child (≤12 years) and juvenile (>12 years) patients. MS onset and its prevalence were assessed at the end of 2019, stratified by age range. Results. A total of 329 paediatric or juvenile patients (228 girls, 101 boys) with a clinically definite diagnosis of MS, in conformity with the 2017 McDonald Criteria, were enrolled. For 71 children (21.6%), the first symptoms appeared before the age of 12. The female: male ratio increased with age, amounting to 1:1 in the ≤12 years group and to 2.9:1 in the >12 years group. In most cases, the disease had multi-symptomatic onset (31.3%), and its course was mostly of a relapsing−remitting character (95.7%). The initial Expanded Disability Status Score for both groups was 1.63 ± 1.1, whereas the annual relapse rate was 0.84 during the first 2 years. The time between the onset of symptoms and diagnosis was longer in the younger patients (8.2 ± 4.2 vs. 4.6 ± 3.6 months; p < 0.005). On 31 December 2019, the age-adjusted prevalence standardized to the European standard population was 5.19/100,000 (95% CI, 4.64−5.78). Significantly higher prevalence was noted in the 13−18 years group (7.12; 95% CI, 6.64−7.86) than in the 9−12 years group (3.41; 95% CI, 2.98−3.86) and the <9 years group (0.56; 95% CI, 0.46−0.64; p < 0.001). Conclusion. POMS commencing at the age of ≤12 years is rare, differing significantly from the juvenile-onset and adult MS in terms of clinical characteristics, course, and incidence, as stratified by gender.
RESUMO
BACKGROUND AND PURPOSE: Down syndrome (DS) is the most common genetic cause of mental retardation with deficits in language and memory. Mental retardation of varying degrees is the most consistent feature of DS. The objective of this study was to use high-resolution magnetic resonance imaging (MRI) techniques to investigate the volumes of the hippocampus, amygdala, and temporal and frontal lobes in children with DS compared with healthy children. MATERIAL AND METHODS: MRI of 49 patients was reviewed prospectively. The study included 23 children with DS (9 girls and 14 boys, mean age 6.7 ± 3.7 years) and 26 healthy children (11 girls and 15 boys, mean age 8.3 ± 2.4 years). Volumes of the right and left hippocampus, the right and left amygdala, temporal and frontal lobes and the total brain volume were measured by a radiologist who was unaware of the diagnosis. RESULTS: Total brain volume in children with DS was significantly lower compared with controls. It was associated with significantly lower volume of the frontal and temporal lobes. Children with DS had a significantly smaller right and left hippocampus volume and a significantly smaller right and left amygdala volume than did the control group. We also found a negative correlation between mental retardation and volume of the right hippocampus. CONCLUSIONS: The presence of these abnormalities from an early age contributes to the specific cognitive and developmental deficits seen in children with DS.
Assuntos
Tonsila do Cerebelo/patologia , Síndrome de Down/patologia , Lobo Frontal/patologia , Hipocampo/patologia , Lobo Temporal/patologia , Atrofia , Criança , Pré-Escolar , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Tamanho do Órgão/fisiologia , Estudos Prospectivos , Valores de ReferênciaRESUMO
BACKGROUND: The Interictal abnormalities of cerebral information processing in migraine were found by studying different modality-specific evoked and event related potentials, mostly visual and auditory. In this study we focused on short-latency somatosensory evoked potentials (SEP) in children and adolescents suffering from migraine with and without aura. MATERIAL AND METHODS: The study group consisted 111 of children and adolescents at the age of 7-18 years: 27 of them suffered from migraine with aura, 36 of them suffered from migraine without aura, 48 subjects have episodic tension-type headache. SEPs was performed interictally at least two days after the last headache attack. RESULTS: There were no significant differences in the latency averages of SEP components between all migraneurs and tension-type headache subjects. However, N9 and N13 latency averages were significantly shorter in migraine without aura group compared with migraine with aura and tensiom type headaches. We did not find any significant correlations for either headache type between evoked potentials parametrs and illness duration, unilateral localisation of pain, migraine in family and aura. CONCLUSIONS: In concert with similar studies in adult migraineurs, our findings showed no disturbances of somatosensory information processing in children with migraine with aura and without aura. The diagnosis of migraine in children actually remains predominantly based on medical history. However, electrophysiological techniques allow the study of some of the structures in vivo and enlarge our knowledge on controversial aspects of migraine pathophysiology.
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Potenciais Somatossensoriais Evocados , Enxaqueca com Aura/fisiopatologia , Enxaqueca sem Aura/fisiopatologia , Adolescente , Criança , Feminino , Humanos , Masculino , Tempo de Reação , Cefaleia do Tipo Tensional/fisiopatologiaRESUMO
The oxidant-antioxidant balance disorders underlie a number of acute and chronic diseases of the central nervous system (CNS). It is believed that oxidative stress plays a role in the pathogenesis of migraine. The study objective was to assess the processes of lipid peroxidation with malondialdehyde (MDA) as its major indicator and to determine the activities of antioxidant enzymes: superoxide dismutase (SOD), glutathione peroxidase (GSH-Px) and glutathione reductase (GSSG-R) in the serum and erythrocytes of patients at developmental age with migraine with and without aura. The study group consisted of 34 patients at the age of 10-18 years (mean +/- standard deviation: 14.04 +/- 2.29 years), suffering from migraine. The control group included 38 patients, aged 4-17 years (mean age 12.11 +/- 3.46). MDA concentration and activities of SOD, GSH-Px and GSSG-R were determined in serum and erythrocytes of all the patients. In the migraine group, the MDA levels in serum and erythrocytes were statistically significantly lower than in control subjects (p < 0.001). In the migraine group, serum GSH-Px activity was significantly higher (p < 0.05). The GSSG-R activity in the erythrocytes of migraine children was significantly higher compared to controls (p < 0.001). SOD activity was decreased and GSH-Px was increased (non-significantly) in erythrocytes of migraineurs. Our results confirm the disturbances of lipid peroxidation processes in migraine and suggest the activation of antioxidant mechanisms. Its important indicator seems to be the increase in the GSSG-R activity in the erythrocytes and the GSH-Px activity in serum between migraine attacks. Further studies are necessary.
Assuntos
Glutationa Peroxidase/sangue , Glutationa Redutase/sangue , Peroxidação de Lipídeos , Malondialdeído/sangue , Transtornos de Enxaqueca/sangue , Transtornos de Enxaqueca/enzimologia , Superóxido Dismutase/sangue , Adolescente , Criança , Pré-Escolar , Feminino , Glutationa Peroxidase/metabolismo , Humanos , Masculino , Transtornos de Enxaqueca/classificação , Superóxido Dismutase/metabolismoRESUMO
A prospective study was undertaken of 129 children with spastic cerebral palsy to clarify the relationship between magnetic resonance imaging (MRI) findings and clinical features of cerebral palsy. Low birth weight, asphyxia, prematurity, seizures, mental development, Gross Motor Function Classification System, and MRI findings were analyzed. Significant abnormalities relevant to the cerebral palsy were evident on imaging in 123 (95.3%). A similar percentage of MRI abnormalities were detected in the groups, 45 (100%) in patients with tetraplegic cerebral palsy, 37 (92.5%) in children with diplegic cerebral palsy, and 42 (95.4%) with hemiplegic cerebral palsy. Periventricular leukomalacia was detected more frequently in the children with spastic diplegia than in the patients with tetraplegia or hemiplegia. Cerebral atrophy was found more often in the tetraplegic group compared to the diplegic patients. Porencephalic cysts were detected more frequently in children with spastic hemiplegia. Congenital brain anomalies were found in a higher proportion in tetraplegic children. Significant correlations between the MRI findings and Gross Motor Function Classification System in the diplegic and tetraplegic patients were found. No correlations between the MRI results and risk factors for cerebral palsy in the tetraplegic patients were noted. Early detection of brain abnormalities in children with cerebral palsy may help in the prognosis and in the initiation of appropriate therapy
Assuntos
Paralisia Cerebral/patologia , Imageamento por Ressonância Magnética/métodos , Adulto , Paralisia Cerebral/classificação , Paralisia Cerebral/fisiopatologia , Cognição/fisiologia , Feminino , Humanos , Modelos Lineares , Masculino , Transtornos Mentais/etiologia , Transtornos Mentais/patologia , Atividade Motora/fisiologia , Estudos Prospectivos , Estudos Retrospectivos , Fatores de Risco , Estatísticas não ParamétricasRESUMO
The aim of this study was to evaluate the influence of antiepileptic therapy on antioxidant enzyme activity and lipid peroxidation in the erythrocytes of children with epilepsy. For this purpose, the activity of superoxide dismutase, glutathione peroxidase, and glutathione reductase and the malondialdehyde concentration in 61 healthy children and 90 children with epilepsy were measured. The activities of all of these enzymes were insignificantly higher, whereas the malondialdehyde concentration was significantly lower in the patients treated with carbamazepine monotherapy. In patients treated with valproate monotherapy, the activities of all enzymes were insignificantly lower, whereas the malondialdehyde concentration was insignificantly higher. In patients treated with polytherapy, the activity of superoxide dismutase was insignificantly lower, whereas the activity of glutathione peroxidase and glutathione reductase was insignificantly higher and the malondialdehyde concentration was lower. There were differences in glutathione reductase activity between the valproate monotherapy group and both the carbamazepine monotherapy and polytherapy groups and in malondialdehyde concentrations between the carbamazepine and valproate groups. The results indicate that the oxidant-antioxidant balance in children with epilepsy is modified by antiepileptic therapy.
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Anticonvulsivantes/uso terapêutico , Epilepsia/sangue , Epilepsia/enzimologia , Glutationa Peroxidase/sangue , Glutationa Redutase/sangue , Superóxido Dismutase/sangue , Adolescente , Adulto , Estudos de Casos e Controles , Criança , Pré-Escolar , Epilepsia/tratamento farmacológico , Eritrócitos/metabolismo , Feminino , Humanos , Lactente , Peroxidação de Lipídeos/fisiologia , Masculino , Malondialdeído/sangueRESUMO
Carbamazepine (CBZ) is a drug of choice for the treatment of simple or complex partial seizures and secondary generalized seizures in adults and children. Vigabatrin (VGB) is a relatively new second line antiepileptic drug and was first registered for use in Poland more than ten years ago. Few reports have been published on the comparison of efficacy of VGB in children with epilepsy. The objective of this study is to evaluate the safety, efficacy and EEG effects of initial VGB monotherapy compared with initial CBZ monotherapy in children with newly diagnosed epilepsy. We present results of a prospective, outpatient and open study carried out in the University Hospital Center in Bialystok. Twenty-six children with partial epilepsy treated with VGB and 28 patients treated with CBZ were studied. The evaluation of the efficacy of the two drugs did not reveal any significant differences. Very good (reduction > 75%) seizure control was achieved in 22 out of 26 patients (84.6%) in the VGB group. One patient had a 50-75% decrease of seizures (good effect), similarly one child had a 25-50% reduction of seizures (mild effect). In two patients, we observed increased seizures (myoclonic jerks). Very good seizure control was achieved in 17 out of 28 patients (60.7%) in the CBZ group. Good seizure control was achieved in 5 out of 28 patients (17.8%) and mild control was seen in two children. No improvement was observed in 4 (14%) of the patients. The EEG background activity was improved in VGB-treated patients. No effect on the EEG background activity was observed in CBZ-treated children. VGB seems to be a safe and effective antiepileptic drug as primary monotherapy for epilepsy in children with similar proportion of side effects as CBZ.
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Anticonvulsivantes/uso terapêutico , Carbamazepina/uso terapêutico , Epilepsias Parciais/tratamento farmacológico , Vigabatrina/uso terapêutico , Adolescente , Anticonvulsivantes/efeitos adversos , Carbamazepina/efeitos adversos , Criança , Pré-Escolar , Eletroencefalografia/efeitos dos fármacos , Feminino , Humanos , Masculino , Estudos Prospectivos , Convulsões/epidemiologia , Convulsões/prevenção & controle , Vigabatrina/efeitos adversosRESUMO
BACKGROUND AND PURPOSE: Cognitive Event-Related Potentials (CERP) reflect sensory information processing: cognitive function and early memory. Studies of CERP in adult migraneurs yielded contradictory results. The aim of our study was to evaluate CERP in children and adolescents with migraine with and without aura during the interictal period. MATERIAL AND METHODS: The study was carried out on 111 children aged 7-18 years (mean 12.92 (2.78) with idiopathic attack headaches. In this group migraine with aura (MA) was diagnosed in 27 patients, migraine without aura (MO) in 36 children and episodic tension-type (TH) headaches in 48 patients. RESULTS: The latencies N2 and P3 were significantly longer (p < 0.05 and p < 0.01, respectively) in the group of all migraneurs (MA + MO, n = 63) as compared with the TH group. In the MO group not only N2 and P3 latency, but also P2 latency (p < 0.05) were longer, if particular types of migraine were compared with tension-type headaches. There were no statistically significant differences between mean latencies in MA and TH groups. Analyzing CERP amplitudes, the N1/P2 amplitude was significantly higher in MO patients than in the TH group only. We found longer P2, N2 and P3 latencies and higher N1/P2 amplitude in MO patients in comparison with MA patients. We found correlation between P3 latency and the age of patients with migraine. There were no statistically significant correlations for either headache type between CERP parameters and illness duration, sex of patients and unilateral localisation of headache. CONCLUSIONS: The CERP parameter changes in children with migraine point out the disturbances of cognitive functions also for auditory modalities. It suggests generalized dysfunction of cortical information processing in the interictal period of migraine.
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Cognição/fisiologia , Potenciais Evocados Auditivos/fisiologia , Transtornos de Enxaqueca/diagnóstico , Transtornos de Enxaqueca/fisiopatologia , Adolescente , Criança , Feminino , Humanos , Masculino , Cefaleia do Tipo Tensional/diagnóstico , Cefaleia do Tipo Tensional/fisiopatologiaRESUMO
Multiple sclerosis (MS) is a rare disorder of the central nervous system in children. In spite of the investigations of the last decade its etiology has not been established. The authors of the paper presented data on the specific nature of children MS using the current literature. They also demonstrated the role of interferon (INF) and clinical trials of MS treatment in children. The authors described their own experiences in the treatment of a boy with the youth MS using INF. The patient has suffered from MS since he was 15; INF therapy was introduced after two relapses of the disease. The results of the 18-month therapy with INF suggest beneficial immunomodulatory effects of interferon on MS course in children. The presented patient is continuing the therapy while his clinical status is being observed.
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Adjuvantes Imunológicos/uso terapêutico , Encéfalo/patologia , Interferon-alfa/uso terapêutico , Interferon beta/uso terapêutico , Esclerose Múltipla/tratamento farmacológico , Esclerose Múltipla/patologia , Criança , Potenciais Somatossensoriais Evocados/fisiologia , Humanos , Esclerose Múltipla/fisiopatologiaRESUMO
BACKGROUND: The aim of this study was to examine the putative protective effect of calcium channel blockers on hippocampal neurons in the experimental model of excitotoxic damage. METHODS: Seven-day old primary dissociated cultures of rat hippocampal neural cells containing one of the following calcium channel blockers: cinnarizine, flunarizine or nimodipine were exposed to glutamate-induced injury. Quantitative assessments of neuronal injury were accomplished by measuring lactate dehydrogenase (LDH) activity in the media 24 h after exposure to glutamate and by counting and establishing the apoptotic and necrotic cells in flow cytometry with Annexin V-FITC/PI staining. RESULTS: In our experiment, glutamate induced a 339% elevation of apoptotic cells and a 289% increase of necrotic cells in hippocampal neurons as compared to control cultures without drugs. In cultures containing flunarizine, glutamate-induced cell apoptosis was suppressed by 62% while necrosis showed no significant alternation. Cinnarizine exerted no anti-apoptotic effects on glutamate-injured cultured hippocampal neurons, while nimodipine intensified the apoptotic pathway of cell death and promoted an increase in the number of apoptotic neurons by 26%. When cinnarizine or nimodipine were used, the percentage of necrotic cells was significantly lower when compared with glutamate-injured cultures and it amounted to 44% and 24% for cinnarizine and nimodipine, respectively. CONCLUSIONS: The obtained results suggest the beneficial anti-apoptotic potential of flunarizine and the anti-necrotic potential of cinnarizine against glutamate-induced death of cultured hippocampal neurons. Nimodipine can protect neurons against necrosis, but has an intensified adverse pro-apoptotic effect on cultured neurons in the experimental model of excitotoxic injury.
Assuntos
Bloqueadores dos Canais de Cálcio/farmacologia , Ácido Glutâmico/efeitos adversos , Hipocampo/efeitos dos fármacos , Doenças do Sistema Nervoso/tratamento farmacológico , Neurônios/efeitos dos fármacos , Animais , Apoptose/efeitos dos fármacos , Morte Celular/efeitos dos fármacos , Células Cultivadas , Ácido Glutâmico/metabolismo , Hipocampo/metabolismo , L-Lactato Desidrogenase/metabolismo , Necrose/tratamento farmacológico , Necrose/metabolismo , Doenças do Sistema Nervoso/metabolismo , Neurônios/metabolismo , Ratos , Ratos Sprague-DawleyRESUMO
Many experimental studies indicate that some antiepileptic drugs possess neuroprotective properties in varied models of neuronal injury. Levetiracetam is a second-generation antiepileptic drug with a novel mechanism of action. In the present study, we evaluated the putative neuroprotective effect of levetiracetam on primary hippocampal cultures at seven day in vitro. Cell death was induced by incubation of neural cultures in hypoxic conditions over 24 hours. Neuronal injury was assessed by morphometric investigation of death/total ratio of neurons in light microscopy using Trypan blue staining and by evaluation of lactate dehydrogenase (LDH) release in the culture medium. Our results indicate that pre-conditioning of hippocampal cultures with high concentrations of levetiracetam (100 µM and 300 µM) protects neurons against hypoxia-induced death. Two-fold higher number of neurons remained viable as compared to control cultures without drug. Lack of neuroprotective action of the drug on hippocampal neural cultures was observed, when a low concentration (10 µM) of levetiracetam was used.
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Hipocampo/citologia , Hipocampo/efeitos dos fármacos , Neurônios/efeitos dos fármacos , Neurônios/patologia , Fármacos Neuroprotetores/farmacologia , Piracetam/análogos & derivados , Animais , Morte Celular/efeitos dos fármacos , Hipóxia Celular/efeitos dos fármacos , Sobrevivência Celular/efeitos dos fármacos , Células Cultivadas , Relação Dose-Resposta a Droga , Hipocampo/patologia , L-Lactato Desidrogenase/antagonistas & inibidores , L-Lactato Desidrogenase/metabolismo , Levetiracetam , Neurônios/citologia , Neurônios/enzimologia , Piracetam/farmacologia , Ratos , Ratos Sprague-Dawley , Relação Estrutura-AtividadeRESUMO
Down syndrome (DS), or trisomy 21, is one of the most common autosomal mutations. The overexpression of the ß-amyloid precursor protein gene, located on chromosome 21, causes an increased production of the specific amyloid. The current study is a continuation of our earlier investigations relating to the profile of metabolic changes in the frontal lobes of DS patients as assessed by proton magnetic resonance spectroscopy ((1)H MRS). The aims of the study were the morphological assessment of the brain using magnetic resonance imaging (MRI) and the evaluation of metabolic disorders of the temporal lobes using (1)H MRS in DS children. The study group included 20 children with DS aged 3-15 years and treated in the Department of Pediatric Neurology and Rehabilitation, Medical University of Bialystok. The control group included healthy children (n = 20). MRI scans of the heads of DS children were performed using a 1.5 T MR scanner under standard conditions. (1)H MRS investigations were also carried out to assess metabolic changes in the temporal lobes. Metabolites, such as N-acetylaspartate (NAA), glutamate-glutamine complex (Glx), choline (Cho), myoinositol (mI) and γ-aminobutyric acid (GABA), were determined in both temporal lobes with reference to the internal marker creatine (Cr). Results were compared with the control group.We found a statistically significant decrease in NAA/Cr, Cho/Cr, mI/Cr and GABA/Cr ratios. The Glx/Cr ratio in both temporal lobes of DS patients did not differ from the control group. Our results indicate metabolic neurotransmitter disorders in the central nervous system in children with DS.
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Aminoácidos/metabolismo , Síndrome de Down/metabolismo , Espectroscopia de Ressonância Magnética/métodos , Neurotransmissores/metabolismo , Lobo Temporal/metabolismo , Adolescente , Ácido Aspártico/análogos & derivados , Ácido Aspártico/metabolismo , Criança , Pré-Escolar , Colina/metabolismo , Creatina/metabolismo , Síndrome de Down/patologia , Feminino , Lobo Frontal/metabolismo , Ácido Glutâmico/metabolismo , Glutamina/metabolismo , Humanos , Inositol/metabolismo , Masculino , Prótons , Lobo Temporal/patologia , Ácido gama-Aminobutírico/metabolismoRESUMO
Cytokines participate in many physiological processes including the regulation of immune and inflammatory responses. Production of some important cytokines in children with Down syndrome (DS) is depressed or increased. In this study we analysed the selected anti- inflammatory cytokines: interleukin-4 (IL-4), interleukin-10 (IL-10), interleukin-13 (IL-13) in plasma of children and adolescents with DS. The study group consisted of 20 patients with Down syndrome and 33 healthy subjects at the age of 5-17 years. Levels of: IL-4, IL-10 and IL-13 in plasma samples were determined by specific enzyme- linked immunosorbent assay (ELISA) techniques according to manufacturer's instructions. IL-4 was detectable in 25% subjects with Down syndrome and in 28.6% healthy subjects. IL-13 was detectable in 15% patients with Down syndrome and in 15.2% healthy subjects, respectively. IL-10 was detectable in 1 of 20 patients with Down syndrome and in 2 of 33 healthy subjects only. No significant correlations between measurable cytokine levels and age and gender were found. No significant increased concentration of selected anti- inflammatory cytokines were detected.
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Citocinas/sangue , Síndrome de Down/imunologia , Adolescente , Criança , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , Interleucina-10/sangue , Interleucina-13/sangue , Interleucina-4/sangue , MasculinoRESUMO
Studies have shown fluctuations of cytokine levels in patients with migraine headaches; however, further studies are needed to verify these results. Our previous studies suggest increased levels of pro-inflammatory cytokines, such as IL-1alpha, sTNF-RI and TNF-alpha, in children with migraine headaches. In this study, we analyzed anti-inflammatory cytokines interleukin-4 (IL-4), interleukin-10 (IL-10) and interleukin-13 (IL-13) in plasma from children and adolescents with migraine and tension-type headaches during the interictal period. The study group consisted of 35 children and adolescents between 8-18 years old, suffering from migraine headaches with or without aura. The control group consisted of 33 patients suffering from episodic tension-type headaches. IL-4 was detected in 17.1% of patients with migraine headaches and in 28.6% of patients with tension-type headaches. IL-13 was detected in 17.1% of patients with migraine headaches and in 15.2% of patients with tension-type headaches. IL-10 was only detected in 3 of 68 (4.4%) patients. Any significant correlations between measurable cytokine levels and age, gender, aura, duration of disease, frequency and severity of headaches were determined. Any significant fluctuations of selected anti-inflammatory cytokines during the headache-free period in children with migraine and tension-type headaches have been found, immune dysfunction in migraineurs could not be excluded.
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Citocinas/sangue , Transtornos de Enxaqueca/imunologia , Adolescente , Criança , Feminino , Humanos , Interleucina-10/sangue , Interleucina-13/sangue , Interleucina-4/sangue , Interleucina-5/sangue , MasculinoRESUMO
Toward understanding the role of cytokines in migraine, this study focused on selected proinflammatory cytokines. The study group consisted of 21 children who had migraine with and without aura; the control group was 24 children with episodic tension-type headache. Plasma interleukin-1 alpha was undetectable in 19 control subjects with tension-type headache, but was detectable in 16 patients with migraine, which suggests that interleukin-1 alpha level might be higher in migraine. Soluble tumor necrosis factor receptor 1 in the migraine group was significantly higher than in the control group (P < 0.0005). Migraine patients tended to have increased tumor necrosis factor alpha level, compared with the control group. The interleukin-1 alpha level was significantly higher in migraine with aura than in migraine without aura (P < 0.05). Tumor necrosis factor alpha and soluble tumor necrosis factor receptor 1 levels tended to be increased in the migraine with aura subgroup. The results suggest that proinflammatory cytokines may be involved in the pathogenesis of migraine attacks, although fluctuations in cytokine levels could be different in children than in adults. Such difference could be due to long medical history of migraine in adult patients and frequent intake of analgesic drugs or prophylactic treatment.
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Interleucina-1alfa/sangue , Enxaqueca com Aura/sangue , Enxaqueca sem Aura/sangue , Receptores Tipo I de Fatores de Necrose Tumoral/sangue , Cefaleia do Tipo Tensional/sangue , Fator de Necrose Tumoral alfa/sangue , Adolescente , Criança , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , MasculinoRESUMO
Epilepsy is rarely considered as a major component of Down syndrome. We evaluated the prevalence of epileptic seizures in 252 (97 girls and 155 boys) children and adolescents with Down syndrome evaluated at Department of Pediatric Neurology between 1994 and 2007. Results showed that 15 (6%) patients had epileptic seizures: 8 partial seizures; 1 infantile spasms, 1 Lennox-Gastaut syndrome, and 5 generalized tonic-clonic seizures. Electroencephalography was performed on all patients with Down syndrome. Focal changes, spikes, generalized slowing, and hypsarrhythmia were recorded. The electroencephalography was found to be abnormal in Down syndrome with epilepsy in 100%. Almost 60% of patients with Down syndrome and epilepsy had seizures, but 40% of the patients were seizures-free. Quantitative electroencephalography analysis revealed significant differences between children with Down syndrome and the control groups in the alpha, delta, and beta rhythms. Our findings are in accordance with other reports.
Assuntos
Córtex Cerebral/fisiopatologia , Síndrome de Down/epidemiologia , Síndrome de Down/fisiopatologia , Eletroencefalografia/métodos , Epilepsia/epidemiologia , Epilepsia/fisiopatologia , Adolescente , Ritmo alfa , Ritmo beta , Criança , Pré-Escolar , Estudos de Coortes , Comorbidade , Ritmo Delta , Epilepsias Parciais/diagnóstico , Epilepsias Parciais/epidemiologia , Epilepsias Parciais/fisiopatologia , Epilepsia/diagnóstico , Epilepsia Tônico-Clônica/diagnóstico , Epilepsia Tônico-Clônica/epidemiologia , Epilepsia Tônico-Clônica/fisiopatologia , Potenciais Evocados , Feminino , Humanos , Lactente , Masculino , Valor Preditivo dos Testes , Prevalência , Adulto JovemRESUMO
PURPOSE: To determine if there is any association between the findings of visual evoked potentials (VEPs), somatosensory evoked potentials (SEPs), and magnetic resonance imaging (MRI) findings with the neurodevelopment and severity in children with cerebral palsy (CP). METHODS: The present study included 15 children with spastic diplegic CP and five children with spastic hemiplegic CP and 42 healthy children as controls. The number of the controls was two-times greater than the study group to increase statistical power of this study. VEPs and SEPs were recorded in the CP children and compared with healthy controls. All MR scans were obtained using a 1.5 T MR scanner. RESULTS: A significant difference was found in the latencies P100 (VEP) between the CP and controls. No correlations between increased P100 latencies and asphyxia, prematurity, the CP severity, MRI findings and mental retardation were noted. A significant difference in N13-N20 conductions (SEPs) between the subjects with CP and the control group was found. SEPs were positively correlated with mental retardation in CP children. The brain lesions in MRI showed a significant correlation with the CP severity scores and mental retardation. CONCLUSION: The differences in VEPs and SEPs were determined between CP children and healthy children. The MRI findings were positively correlated with the CP severity and mental retardation.
Assuntos
Encéfalo/fisiopatologia , Paralisia Cerebral/fisiopatologia , Potenciais Somatossensoriais Evocados , Potenciais Evocados Visuais , Adolescente , Paralisia Cerebral/diagnóstico , Criança , Pré-Escolar , Feminino , Humanos , Imageamento por Ressonância Magnética , MasculinoRESUMO
Patients with cerebral palsy (CP) may have some problems other than this motor impairment: mental retardation, epilepsy and sensory disturbance. Healthy children and children with CP have an enhanced capacity for learning and memory compared to adults. There are few tools for brain plasticity investigations. The utility of the neurophysiologic and MRI techniques in the determination of brain reorganization and repair in patients with cerebral palsy is described. The authors discuss their results of quantitative EEG and spectroscopy MRI studies in children with CP. Quantitative EEG and spectroscopy MRI can be useful tools in the determination of these processes in children with CP.