Neurocognitive effects and electrophysiological findings in ADHD and self-limiting centrotemporal spike wave epilepsy (SeLECTS) - A prospective tertiary care study.

OBJECTIVE: The study aimed to determine the prevalence of attention deficit hyperactivity disorder (ADHD) in patients with self-limiting epilepsy with centrotemporal spike wave (SeLECTS), as well as the electroclinical features associated with this comorbid condition and the neurocognitive effects using psychometric tests. Additionally, we analysed the electrophysiological findings and neurocognitive status of patients with ADHD to estimate the prevalence of epilepsy and neurocognitive effects in the ADHD population and evaluate their clinical features. METHOD: The study included patients diagnosed with SeLECT and ADHD who were matched for age and gender. Electrophysiological tests, psychometric tests, demographic and clinical characteristics of SeLECTS patients aged 7-13 years and ADHD patients of similar age were analysed. The study examined electrophysiological and psychometric tests, as well as demographic and clinical characteristics. Both groups underwent testing using the Wechsler Intelligence Scale for Children (WISC-R), Stroop Colour and Word Test (SCWT), and EEG (Electroencephalogram). The SeLECT group also underwent the Bender Visual-Motor Gestalt Test. RSULTS: No significant relationship was found between the SeLECT and ADHD groups in terms of age and gender. The rate of epileptiform discharge in EEG findings without a diagnosis of epilepsy was 5.6 % (n = 2) in the ADHD group. The rate of ADHD in the SeLECTS group was 28 % (n = 11). Although all subsections of the WISCR test were higher in the ADHD patient group than in the SeLECTS patient group, only verbal IQ and total IQ showed a significant difference. No significant differences were found between the completion times, error rates, and correction averages of the SCWT sections in both groups. There was no significant correlation found between the performance IQ, verbal IQ, and total intelligence scores in either the isolated SeLECTS patient group or the SeLECTS + ADHD patient group (p > 0.05). However, it is worth noting that verbal IQ was below normal in both groups and slightly lower in the SeLECT + ADHD group. Additionally, the mean SeWT completion time was significantly longer in the SeLECT + ADHD group than in the isolated SeLECT group. However, no significant difference was found in the Bender Gestalt Visual Motor Perception Test. In the psychometric analyses comparing the isolated SeLECTS, SeLECT + ADHD, and ADHD patient groups, the SCWT completion times were significantly longer in the SeLECT + ADHD group than in the other two groups. The verbal IQ score was significantly higher in the ADHD group than in the other two groups. CONCLUSION: In conclusion, although SeLECTS is commonly considered a benign form of epilepsy, our study found a high rate of comorbidity with ADHD. This condition has a negative impact on verbal intelligence and sustained attention, highlighting the importance of a complete neuropsychological evaluation at the stage of epilepsy diagnosis. It is crucial not to overlook the possibility of an ADHD diagnosis.

Two case reports and a literature review of typical GBS and rare GBS variants associated with COVID-19.

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) emerged in late 2019, and is the infectious agent that caused the coronavirus disease 2019 (COVID-19) pandemic. Although respiratory and gastrointestinal manifestations of SARS-CoV-2 are well defined, the spectrum of neurological involvement is less defined. The classic type of Guillain-Barré syndrome (GBS) progresses over days to weeks and has a monophasic course. Areflexia/hyporeflexia and ascending and symmetrical paralysis are observed clinically in patients. It is an autoimmune process that typically leads to the destruction of myelin after infection. There have been numerous reports of adult patients with the coexistence of GBS disease and active COVID-19 illness, but this number is lacking for children. In this study, we present a literature review of the etiological correlation between SARS-CoV-2 and GBS and describe the cases of two pediatric patients with acute monophasic Guillain-Barré syndrome (GBS) during active COVID-19 infection.

Psychometric Properties of the Turkish Version of the Epilepsy Module of the KINDL Quality of Life Scale.

Introduction: This study aims to present the psychometric properties of the Turkish version of the KINDLE quality of life scale, epilepsy module. Method: The psychometric properties of the KINDL Epilepsy module were evaluated from the aspect of internal consistency, reliability, and construct validity on an inpatient sample of 159 Turkish children (mean age 10.84±2.77 years) who had epilepsy. Results: The KINDL Epilepsy module (36 items) showed acceptable αcoefficients ranging from 0.80 (social well-being) to 0.55 (treatment) for each domain. EFA suggested three sub-dimensions that we named as Physical, Mental and Social Well-being sub-dimensions. Root Mean Square Error of Approximation was found as 0.053, and Comparative Fit Index was 0.95. Good known groups results supported the construct validity of the instrument. Correlations between the income perception, family support and Duration of Epilepsy and the domains of its Epilepsy module were significantly high, indicating a satisfactory convergent validity. Conclusion: The Turkish version of the KINDL Epilepsy module showed module is a promising tool in this study. However, further research on the versions of the module in other languages is needed for its global use.

Analysis of the Pathogenic Variants of Genes Using a Gene Panel in Turkish Epilepsy Patients.

BACKGROUND: Epilepsy is a neurological disease that is mostly caused by genetic factors. The genetic diagnosis of patients in a pediatric epilepsy cohort was provided. METHODS: After phenotypic characterization, a 48-gene Next Generation Sequencing panel was performed in 110 Turkish children with epilepsy. The variants were called and annotated using the QIAGEN Ingenuity® Variant Analysis software. RESULTS: Of those carrying pathogenic mutations, two patients had mutations in the SCN1A gene and two patients in the TSC2 gene; other patients had mutations in the SCN1B, GRIN2B, KCNQ2, PCDH19, CHRNA2, and MECP2 genes. In total, nine out of 10 patients had pathogenic variants that were not previously reported. CONCLUSIONS: The genotype-phenotype correlations of these variants were discussed by comparing the clinical findings with the literature.

A transcranial magnetic stimulation study for the characterization of corticospinal pathway plasticity in children with neurological disorders.

We aimed to investigate cortical and radicular TMS-evoked motor evoked potentials (MEPs) in children with neurological disorders (n = 57, mean age: 5.45 years) and agematched healthy controls (n = 46). Four TMS parameters were analyzed: MEP amplitudes, the latencies of MEP, the latency jump (cortical MEP latency at rest - cortical active-MEP latency at with slightly contracted targeted muscle), and central motor conduction time. Children with neurological disorders were categorized according to the two major types of neuronal plasticity; excessive plasticity: 29 children with cerebral palsy and impaired plasticity: 28 children with neurodegenerative diseases, stroke, and central nervous system infections. The active-MEP abnormalities (absent and prolonged latencies) were correlated with the location of cortical involvement on MRI patterns. We obtained a significantly increased rate of abnormal cortical active-MEPs in children with impaired plasticity (21/28, 75%) compared with excessive plasticity (18/29, 62%). The rate of absent MEP response is three times more in children with impaired plasticity (43%) than in children with excessive plasticity (14%). A more reduced latency jump was measured in children with impaired plasticity compared to children with excessive plasticity. TMS-evoked active-MEPs and latency jumping are valuable parameters for characterizing neuronal plasticity in children with neurological disorders.

Nutritional interventions improve quality of life of caregivers of children with neurodevelopmental disorders.

Introduction: Children with neurodevelopmental disorders are at high risk for malnutrition. We aimed to investigate the impact of nutritional interventions to children with neurodevelopmental disorders and the quality of life of caregivers.Materials/Method: This is a prospective interventional study of 91 children with neurodevelopmental disorders. The children were separated into two groups: the intervention group and control group. The intervention group was selected from among children who had not been evaluated for nutrition and feeding problems by a pediatric gastroenterologist or dietician for the past one year. Children in the intervention group were called for follow-up visits and their nutritional intervention and anthropometric measurements were initiated by a pediatric gastroenterologist and dietician, at one month, three months, six months, and one year. The WHOQoL-BREF quality of life scale was completed by the caregivers of the children at baseline and at one year.Results: The intervention group had increased malnutrition (p < 0.001) and gastrointestinal system pathologies such as dysphagia (p < 0.001), constipation (p = 0.02), gastroesophageal reflux (p = 0.03) at baseline. After the nutritional intervention, 77.7% of the intervention group gained weight and 55.5% reached the target weight. The quality of life scale scores at baseline were lower among caregivers of the intervention group; however, they reached those of the control group after the nutritional intervention.Conclusion: Close multidisciplinary nutrition monitoring enables children with neurodevelopmental disorders to thrive appropriately and improves the quality of life of caregivers.

Coinheritance of novel mutations in NAGLU causing mucopolysaccharidosis type IIIB and in DDHD2 causing spastic paraplegia54 in a Turkish family.

Mucopolysaccharidosis type IIIB (MPSIIIB) is one of the lysosomal storage diseases, clinically related to developmental delay in the early phase and loss of skills in the late phases of the disease. The disease is caused by homozygous mutations in the NAGLU gene. Spastic paraplegia54 (SPG54) is a neurodegenerative disorder caused by homozygous mutations in the DDHD2 gene. Clinical features are progressive spasticity and weakness in the lower limbs and corpus callosum agenesis. We report on two siblings in a consanguineous family, presenting both the clinical and molecular diagnoses of MPSIIIB and SPG54 with novel mutations by using whole exome sequencing (WES). This interesting finding shows that we should be aware of the importance of using WES for diagnosing rare diseases in consanguineous families.

A transcranial magnetic stimulation study for the investigation of corticospinal motor pathways in children with cerebral palsy.

The aim of this study is to perform transcranial magnetic stimulation (TMS)-based investigation of corticospinal motor pathways in children with cerebral palsy (CP) secondary to hypoxic-ischemic encephalopathy (HIE). TMS parameters including motor evoked potentials (MEPs) and central motor conduction time (CMCT) were recorded in 38 children with CP and 46 age-matched healthy controls. The z-score of MEPs were analyzed with respect to the types of MRI patterns of cortical involvement in children with CP. MEP latency values were correlated with the weight and height of children and to reflect the maturation of the corticospinal pathway. TMS evoked MEPs with prolonged onset latencies in 64% of children with CP while 10% of the CP group failed to elicit MEPs. Related with the MRI pattern, multicystic encephalomalacia (89%) was associated with the highest rates of abnormal cortical MEPs, as followed by periventricular leukomalacia (80%), basal ganglia involvement (66%) and focal cortical involvement (60%) patterns. Children with CP as compared with healthy controls had similar CMCT values on the upper and lower extremities in children with all cortical MR patterns. MEP abnormalities with TMS were consistent with the extent of motor cortex lesions on MRI patterns in CP children with HIE.

Levels of Melatonin in Continuous Spikes and Waves During Sleep.

Levels of melatonin have been reported before in children with epilepsy, but such has not been reported to date in those with continuous spikes and waves during sleep. The aim of the present study was to assess serum melatonin levels and melatonin circadian rhythm in patients with continuous spikes and waves during sleep and epilepsy. Serum melatonin was measured in 39 children stratified into 3 groups. Group 1 included 15 patients with continuous spikes and waves during sleep, group 2 included 12 epilepsy patients, and group 3 included 12 controls, respectively. Blood samples were taken from all participants at 1:00 am and 9:00 am and melatonin levels were measured using a quantitative enzyme-linked immunosorbent assay test. The 9:00 am melatonin levels of group 1 were significantly decreased and pair groups were compared. The Pa value (representing a comparison between groups 1 and 2) was .002, the Pb value (representing a comparison between groups 1 and 3) was .001, and the Pc value (representing a comparison between groups 2 and 3) was .86. These findings suggest that the 9:00 am melatonin levels were significantly decreased in the comparison of groups 2 and 3. Further detailed research is necessary to determine the factors leading to the rapid decline of morning melatonin levels of children with continuous spikes and waves during sleep.

Childhood onset limb-girdle muscular dystrophies in the Aegean part of Turkey.

The aim of this study is to analyze the epidemiology of the clinical and genetic features of childhood-onset limb-girdle muscular dystrophies (LGMD) in the Aegean part of Turkey. In total fifty-six pediatric cases with LGMD followed in four different pediatric neurology departments in the Aegean region of Turkey were evaluated. Among them, LGMD2C was the most common followed by LGMD2A, LGMD2D, and LGMD2F with equal frequencies. In twenty-eight patients (50%) the diagnosis could be confirmed by genetic analysis, where SGCG proved to be disease-causing in most of the cases. About half of the patients were diagnosed with whole exome or targeted gene sequencing. A positive correlation between muscle biopsy and genetic findings were observed in 11% of the patients. We report one novel frameshifting mutation in TTN. Knowledge on frequencies of childhood-onset limb-girdle muscular dystrophies and related genes in Turkey will lead to a prompt diagnosis of these neuromuscular disorders.

Evaluation of clinical course and neurocognition in children with self-limited infantile epilepsy in a Turkish cohort study.

The outcome of children with self-limited infantile epilepsy was reported to be normal psychosocial and cognitive development as a characteristic criterion. We aimed to investigate the clinical course and neurocognitive outcome in children with self-limited infantile epilepsy in a Turkish cohort. The clinical course, electroencephalographic (EEG) characteristics, neuroimaging, treatment, and outcome of children with self-limited infantile epilepsy were retrospectively analyzed. All infants were reevaluated with the Denver Developmental Screening Test in addition to neurologic examination. Of 44 patients, self-limited familial infantile epilepsy was diagnosed in 8 infants (18.2%) and self-limited nonfamilial infantile epilepsy in 28 (63.6%). Interictal EEGs and neurologic examinations were normal in all cases. Fine motor and gross motor skills, language, adaptive personal/social skills were near-normal in all patients with self-limited familial infantile epilepsy. Delay in language parameters was observed in 2 infants with self-limited nonfamilial infantile epilepsy. Language skills should be thoroughly evaluated with detailed neurocognitive screening tests in patients with self-limited infantile epilepsy.

Histopathological and genetic features of patients with limb girdle muscular dystrophy type 2C.

OBJECTIVE: In this study, it was aimed to describe the clinical, histopathological and genetic features of 20 patients with gamma sarcoglycanopathy confirmed by muscle biopsies and genetic analysis. MATERIAL AND METHOD: We retrospectively reviewed 20 patients from whom muscle biopsy specimens were obtained between 2007 and 2012. All patients were clinically diagnosed as muscular dystrophy and biopsy materials were collected from five different centers of neurological disorders. All DNAs were extracted from muscle tissues or blood samples of patients and genetic tests (mutation analyses for gamma sarcoglycan gene and deletion-duplication analyses for all 4 sarcoglycan genes) were performed. RESULTS: The mean age of the patients was 7.6 years (2 -21 years). Only one case (5%) was older than 14 years. The mean CPK level was 10311 U/L (1311 - 35000 U∕L). There were 4 siblings in these series. Expression defects of gamma sarcoglycan staining were determined in (15 males, and 5 females) all patients with muscle biopsy specimens. But only in 9 of them, disease-causing defects could be determined with genetic analyses. CONCLUSION: The present study has demonstrated that both examination of muscle biopsy specimens and DNA analysis remain important methods in the differential diagnosis of muscular dystrophies. Because dystrophinopathies and sarcoglycanopathies have similar clinical manifestation.

Ambulatory blood pressure parameters in office normotensive obese and non-obese children: relationship with insulin resistance and atherosclerotic markers.

OBJECTIVES: To determine differences in ambulatory blood pressure (ABP) parameters between office normotensive obese and non-obese children and to evaluate correlations of ABP parameters with insulin resistance and the lipid profile. SUBJECTS AND METHODS: Thirty-eight obese [body mass index (BMI) above the 95th percentile] and 38 non-obese children aged 9-17 years were recruited. All subjects who were normotensive during office visits and who underwent 24-hour ABP monitoring were evaluated. Insulin resistance and the lipid profile were also evaluated. RESULTS: The mean daytime, night-time and 24-hour systolic blood pressure (SBP) and the daytime and 24-hour diastolic blood pressure (DBP) in normotensive obese children were significantly higher compared to the values in non-obese children (p < 0.05). There was no difference in the frequency of nocturnal non-dippers and nocturnal hypertension (night-time SBP at or above the 95th percentile) between the two groups (p > 0.05). Children with night-time SBP at or above the 95th percentile and non-dippers had higher atherosclerotic markers than children with night-time SBP below the 95th percentile and dippers (p < 0.05). In logistic regression analysis, the low-density lipoprotein cholesterol (LDL-C):high-density lipoprotein cholesterol (HDL-C) ratio and night-time SBP had significantly positive associations with being obese in adolescents (OR 6.54, 95% CI 1.15-37.07, p = 0.03, and OR 1.1, 95% CI 1.01-1.19, p = 0.02, respectively). CONCLUSION: Normotensive obese children had higher ABP parameters. A high LDL-C:HDL-C ratio and night-time SBP were associated with an increased risk of being obese. High LDL-C:HDL-C ratios and total cholesterol:HDL-C levels in children and adolescents may be risk factors for night-time hypertension.

Changing views of cerebral palsy over 35 years: the experience of a center.

In this study, it was aimed to evaluate the demographic and clinical characteristics of cerebral palsy (CP) cases over a 35-year period. Findings of 442 patients with CP followed from 1995 to 2006 (Group 2) were compared with 208 patients with CP followed between 1972 and 1994 (Group 1) in the same pediatric neurology division. Ratios of both prematurity (38% vs. 17.7%) and very low birth weight (VLBW) infants (13.8% vs. 1.5%) significantly increased in Group 2. There was also a four-fold increase in cesarean delivery in Group 2 (42.3% vs. 9.6%). A significant increase in the rate of early diagnosis during the first year was also found in this group (56.9% vs. 39.4%). The rate of spastic diparesis cases has significantly increased (33.7% vs. 7.7%), while the rate of spastic tetraparesis cases has significantly decreased (63.5% vs. 37.3%). It was seen that preventable risk factors continue today.

Gastric emptying of solids and its relationship with microalbuminuria in children and adolescents with type 1 diabetes mellitus.

BACKGROUND: The aims of the present study were to determine the frequency of delayed gastric emptying in children and adolescents with type 1 diabetes mellitus (T1DM) and to investigate the relationship between gastric emptying rate and other contributing factors (e.g. serum HbA1c, duration of diabetes and microalbuminuria) in these patients. METHODS: This was a clinical trial evaluating the rate of gastric emptying of solid meals in 33 children and adolescents with T1DM and in 26 healthy peers using a radionuclide method. Three consecutive overnight urine collections were used to calculate the albumin excretion rate. RESULTS: There was no significant difference in the gastric half-emptying time (GE t½ ) between patients and controls (151.7 ± 154.5 vs 109.8 ± 60.5 min, respectively; P=0.885) or the frequency of delayed gastric emptying (36.4% vs 30.8%, respectively; P=0.433). There was a moderately positive correlation between GE t½ and the duration of diabetes (r=0.380; P=0.029). There was no correlation between GE t½ and microalbumin levels in T1DM patients. In these patients, the body mass index standard deviation scores were significantly lower than in patients with normal gastric emptying (-0.13 ± 0.87 vs 0.7 ± 1.23, respectively; P=0.044). CONCLUSION: Progression of delayed gastric emptying is more likely to be related to a longer duration of diabetes than glycemic control in children and adolescents with T1DM. Patients with delayed gastric emptying are thinner compared with patients with a normal rate of gastric emptying; they may also be asymptomatic.

Prediction of neurodevelopmental outcome in term neonates with hypoxic-ischemic encephalopathy.

BACKGROUND: Hypoxic ischemic encephalopathy may result in many neurological deficits. It is crucial to make early diagnosis and assess the prognosis correctly. AIMS: We aimed to determine the factors to evaluate the prognosis of hypoxic ischemic encephalopathy. METHODS: Electroencephalography, neuroimaging, periodic neurological exams and a developmental test at 44-48 months after discharge from the hospital were performed on twenty five term newborn infants with clinical evidence of hypoxic ischemic encephalopathy. RESULTS: Normal/mildly abnormal neonatal electroencephalography correlated with favorable outcome, particularly if neuroimaging was normal. The cranial MRI sensitivity was 83.3%, while the specificity was 57.9%, the positive predictive value was 38.5%, and the negative predictive value was 91.6%. Moderate/severely abnormal electroencephalography and multifocal/diffuse cortical or deep gray matter lesions correlated with poor outcome. CONCLUSIONS: Newborn infants with hypoxic ischemic encephalopathy should be treated in neonatal intensive care units, assessed with periodic neurological examination, electroencephalogram and brain imaging. This would help to initiate early intervention and improve the outcome of patients.

Electrophysiologic assessment of spasticity in children using H-reflex.

We investigated a possible correlation between Hoffmann's reflex (H-reflex) and the Modified Ashworth Scale (MAS) in children with spasticity. H-reflex latencies, amplitudes (H amplitude), Hmax/Mmax amplitude, and MAS were simultaneously measured in 30 children who had bilateral spasticity on the lower extremities. Children with MAS scores of 1 and +1 composed Group I (n=11), and children with MAS scores of 2 and 3 composed Group II (n=26) and Group III (n=23), respectively. The H-reflex latencies were significantly shorter and Hmax/Mmax ratios were significantly higher in patients with cerebral palsy than controls irrespective of the degree of the MAS. The H-reflex latencies in patients with MAS of 1 or +1 were significantly longer than in patients with MAS of 2. Other than between these two groups for H-reflex latencies, no significant differences were revealed among the three different MAS groups for either H-reflex latencies or Hmax/Mmax ratios. There is a positive correlation between spasticity assessed by MAS and H-reflex. We concluded that the H-reflex is a reliable electrophysiologic test for assessment of spasticity in children.

Assessment of sleep-related breathing disorders in patients with duchenne muscular dystrophy.

BACKGROUND: Respiratory failure is a significant cause of morbidity and mortality in neuromuscular diseases. Although clinical findings and respiratory function tests aid in diagnosing sleep-related breathing disorders, polysomnography is the gold standard for the diagnosis of these disorders. We aimed to investigate the role of sleep-wake symptoms and clinical findings of patients with Duchenne muscular dystrophy (DMD) in predicting sleep-related breathing disorders through the comparison of polysomnography findings. In addition, we evaluated the sleep architecture of our patients. METHODS: A total of 35 children (12 patients with DMD and 23 controls) were included in this cross-sectional study. Activity status and clinical severity of the patients were determined by history and clinical findings such as scoliosis, obesity. All subjects were hospitalized for one night in the Sleep Unit and their polysomnography examinations were performed. Sleep, breathing, arousals and limb movements were scored manually according to the American Sleep Disorders Association criteria. RESULTS: Nocturnal and daytime symptoms were present in 50% of patients with DMD, 40.8% were wheelchair-bound and 58% had scoliosis. Obstructive sleep apnea was noted in 16.6% of patients with DMD. The apnea-hypopnea index, leg movement index were significantly higher in the DMD group as compared to the control group (P < 0.05). The number of desaturations, total arousal index and the percentage of total superficial sleep were significantly higher in patients with wheelchair, scoliosis, sleep-wake symptoms. CONCLUSIONS: Being wheelchair-bound or having scoliosis do not predict sleep-related breathing disorders, so patients with DMD should be followed-up via polysomnography. Sleep-wake symptoms should be carefully questioned in these patients and symptomatic patients should be referred to pediatric respiratory units.

Neurocognitive evaluation in children with occipital lobe epilepsy.

PURPOSE: This study aimed to explore cognitive functions in patients with childhood epilepsy with occipital paroxysms (CEOP) and to compare the performance of these patients with that of patients with symptomatic occipital epilepsy (SOE) and healthy control subjects. METHOD: Twenty-eight patients with epilepsy (17 CEOP, 11 SOE) were enrolled. The control group had similar demographical characteristics. Cognitive functions evaluated with Wechsler Intelligence Scale for Children-revised edition (WISC-R), The Visual Aural Digit Span (VADS) and Bender Visual Motor Gestalt Test (BVMG). RESULTS: The WISC-R showed lower performance IQ with WISC-R in patients with occipital epilepsy than in healthy controls. The VADS test only showed lower scores in children with symptomatic occipital epilepsy. Mean BVMG test scores were significantly abnormal in both subgroups of childhood epilepsy with occipital paroxysms (early-onset CEOP/late-onset CEOP) and the group with SOE. CONCLUSION: Patients with CEOP, especially the late-onset form, have significant problems in the domains of visuomotor coordination, memory and attention. The academic performance of these patients should be monitored carefully in follow-up and appropriate educational support should be given as necessary.

Convulsive status epilepticus in children: etiology, treatment protocol and outcome.

This study aimed to determine the etiology, treatment protocol and outcome of convulsive status epilepticus (SE) in children. An institutional treatment protocol using benzodiazepines (diazepam and midazolam) was assessed in a retrospective case study. The treatment protocol (Ege Pediatric Status Epilepticus Protocol or EPSEP) was developed based on an operational definition of pediatric SE according to the duration of seizure activity. Pediatric SE is divided into three categories: initial SE (20-30 min), established SE (30-60 min) and refractory SE (>60 min). Eight (30%) of the studied episodes were initial SE, 10 (37%) were established SE, and 9 (33%) were refractory SE. With respect to the etiological spectrum of SE, 11 (40%) children had meningitis or encephalitis. Febrile SE was identified in 7 (26%) patients. Only 2 episodes of initial SE (7.5%) were controlled with first step of the protocol (two concomitant-doses of rectal diazepam). Midazolam bolus and infusions (up to 1.2 µg/kg/min) were used to treat 22 episodes of SE (9 refractory SE, 10 established SE and 3 initial SE). Complete arrest of convulsive SE was achieved in 21 of 22 (95%) episodes with midazolam infusion. We concluded that the combined use of benzodiazepines (diazepam+midazolam) was safe and effective in the treatment of convulsive SE in children.