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1.
Epilepsy Behav ; : 107320, 2020 Aug 22.
Artículo en Inglés | MEDLINE | ID: mdl-32839145

RESUMEN

AIM: The aim of this study was to assess sleep architecture and sleep problems among three homogenous groups of children including children with drug-resistant focal epilepsy, children with newly diagnosed, drug-naïve focal epilepsy, and healthy children using overnight video-polysomnography (V-PSG) and a sleep questionnaire. METHODS: We compared sleep architecture among 44 children with drug-resistant focal epilepsy, 41 children with newly diagnosed, drug naïve focal epilepsy, and 36 healthy children. All children underwent an overnight V-PSG recording, and their parents completed the Children's Sleep Habits Questionnaire (CSHQ). Sleep recordings were scored according to the American Academy of Sleep Medicine criteria. RESULTS: Compared with children with newly diagnosed epilepsy and healthy controls, children with drug-resistant epilepsy receiving antiepileptic treatment showed disturbed sleep architecture, a significant reduction in time in bed, total sleep time, sleep efficiency, NREM3%, REM%, and a significant increase in awakenings, wake after sleep onset, and periodic leg movement. Children with drug-naïve, newly diagnosed focal epilepsy showed a statistically significant increase in sleep onset latency, rapid eye movement (REM) latency, N1%, awakenings, and a significant decrease in time in bed when compared with the controls. Children with drug-resistant epilepsy had the highest CSHQ total scores, while children with drug-naïve, newly diagnosed focal epilepsy had higher scores than healthy children. CONCLUSION: This is one of the few polysomnographic studies adding to the limited research on the sleep macrostructure of children with drug-resistant epilepsy compared with children with drug-naïve, newly diagnosed focal epilepsy and healthy children by obtaining objective measurements of sleep concurrently with a validated questionnaire. Children with drug-resistant epilepsy had a greater incidence of sleep disturbance on the basis of qualitative aspects and architecture of sleep than children with newly diagnosed epilepsy, suggesting the need for referral of children with drug-resistant epilepsy for overnight sleep evaluation in order to improve the clinical management and optimize therapeutic strategies.

2.
J Pediatr Endocrinol Metab ; 33(3): 437-441, 2020 Mar 26.
Artículo en Inglés | MEDLINE | ID: mdl-32069232

RESUMEN

Background Peroxisomal biogenesis disorders (PBDs) include a miscellaneous group of diseases which cause serious multisystem disease. Mutations of 13 different PEX genes lead to PBDs including Zellweger syndrome (ZS). Different types of mutations of PEX1 and PEX10 genes are correlated with broad-range phenotypes of PBDs. Case presentation Patient 1 is a 4-month-old boy who was affected by myoclonic seizures, poor oral feeding since birth. The patient was hypotonic and had hepatosplenomegaly. Patient 2 is a 2-month-old boy who presented with decreased movement, severe hypotonia and failure to thrive. The laboratory studies of the patients revealed increased plasma very-long-chain fatty acids (VLCFAs). The genetic analyses of patient 1 demonstrated the first homozygous missense mutation in the PEX10 gene. A novel homozygous missense mutation was found in the PEX1 gene in patient 2. Conclusions This report highlights that the detected homozygous missense mutations of PEX10 and PEX1 genes and the substitutions of specific amino acids lead to the severe form of PBDs.

3.
Eur J Paediatr Neurol ; 26: 82-88, 2020 May.
Artículo en Inglés | MEDLINE | ID: mdl-31818545

RESUMEN

Ictal pouting (Chapeau de gendarme sign) can be described as an inverted smile. It consists of a turned down mouth with the contraction of the chin, wrinkling of the lips and symmetrical lowering of the labial commissures. This shape resembles the gendarme's hat during Napoléon I's time. Chapeau de gendarme sign is frequently seen in frontal and temporal lobe seizures. Focal cortical dysplasias are intrinsically epileptogenic foci and are frequently seen in patients with ictal pouting in seizure semiology. In this report, we analyzed clinical data, video EEG recordings and brain imagings of three children presenting with ictal pouting semiology in whom patients' magnetic resonans images (MRIs) or positron emission tomographies (PETs) were positive or doubtful for FCD in all. In case 1 and 2 the epileptogenic zones were temporal or temporoinsular. In these patients, with involvement of temporal lobe, dystonia and automatisms were seen in the seizure semiology after chapeau de gendarme sign. In case 3 with frontal lobe origin, hypermotor movements were seen after ictal pouting. In the patients 1 and 2, the cortical dysplasias were in temporal lobe. In patient 3, PET demonstrated hypometabolism on left inferior frontal gyrus but we couldn't verify this finding with MRIs. Ictal pouting (Chapeau de gendarme sign) is a distinct seizure semiology that can often be overlooked and coexist with focal cortical thickening. We suggest that focal cortical dysplasias should be searched in patients with ictal pouting particularly in those with refractory focal seizures.


Asunto(s)
Malformaciones del Desarrollo Cortical/complicaciones , Convulsiones/diagnóstico , Convulsiones/etiología , Adulto , Niño , Electroencefalografía/métodos , Músculos Faciales/fisiopatología , Femenino , Humanos , Masculino , Malformaciones del Desarrollo Cortical/fisiopatología , Espasmo/etiología
4.
J Pediatr Neurosci ; 13(2): 276-278, 2018.
Artículo en Inglés | MEDLINE | ID: mdl-30090157

RESUMEN

Citrullinemia type 1 (CTLN1) is a rare inherited urea cycle disorder, which resulted from the deficiency of argininosuccinate synthetase enzyme. We presented an infant who was hospitalized because of acute losses of tonus and cyanotic hypoventilation attacks lasting approximately 4-5 min. The physical and neurological examinations were normal. Ammonia level was in the normal range. Citrulline levels increased in both blood and urine. The blood sample was sent to mutation analysis, which showed one novel and one known mutation on ASS1 gene sequencing: a heterozygous novel mutation p.A94V (c.281C>T) and a heterozygous mutation p.W179R (c.535C>T). Urea cycle disorders should be considered in the differential diagnosis of unexplained brief apnea or hypoventilation attacks, even though those symptoms do not lead to hyperammonemia during infancy and childhood as seen in our patient. This is the first case in terms of atypical clinical presentation with a new mutation for CTLN1.

5.
Turk J Med Sci ; 48(4): 786-793, 2018 Aug 16.
Artículo en Inglés | MEDLINE | ID: mdl-30119154

RESUMEN

Background/aim: We evaluated the utility of electroencephalography (EEG) in children with neurological conditions and compared the results with those of our previous study on excessive uses of pediatric EEG, which was published in 2003. We also evaluated the possibility of subsequent EEGs and satisfactory duration of EEG recordings according to EEG type and admission status. We also evaluated the yield of varying durations of EEG recordings. Materials and methods: All consecutive pediatric EEG records performed at Gazi University EEG laboratory during a 1-year period were retrospectively reviewed. The indications of EEGs, the number of EEGs for each patient, condition and duration of EEG records, and activation techniques were evaluated in terms of detection of abnormalities by EEG. Results: We reviewed a total of 2045 EEGs in children aged 2 months­20 years. Of these, 776 were repeat EEGs (38%) and 485 recordings were ≥30 min (23.7%); 98% of EEG abnormalities appeared in the first 30 min. Overall, 90.5% of EEGs were ordered by a pediatric neurologist. There were similar requests for numbers of EEGs, but the rate of abnormal EEGs (43.6%) was significantly higher when compared to that of our previous study (36.2%). Conclusion: The results of this study show that the utility of EEG becomes more selective and interpretation of pediatric EEG improves depending on the increasing number of pediatric neurologists. A duration of 20­30 min of EEG recording is sufficient, on the condition of inclusion of nREM sleep records.


Asunto(s)
Electroencefalografía/tendencias , Enfermedades del Sistema Nervioso/diagnóstico , Pediatría/tendencias , Adolescente , Niño , Preescolar , Epilepsia/diagnóstico , Femenino , Humanos , Lactante , Masculino , Neurólogos , Pautas de la Práctica en Medicina/tendencias , Estudios Retrospectivos , Convulsiones/diagnóstico , Sueño , Turquia
6.
Childs Nerv Syst ; 34(9): 1799-1801, 2018 09.
Artículo en Inglés | MEDLINE | ID: mdl-29680919

RESUMEN

BACKGROUND: We describe the first child with drug-resistant epilepsy in whom vagus nerve stimulation aggravated seizures and emerged status epilepticus after the increase in vagal nerve stimulation current output. OBJECTIVE: A 13-year-old girl presented with refractory secondary generalized focal epilepsy. Vagal nerve stimulator was implanted because of drug-resistant epilepsy. After the increase of vagal nerve stimulator current output to a relatively high level, the patient experienced seizure aggravation and status epilepticus. CONCLUSION: We conclude that vagus nerve stimulation may induce paradoxical seizures and may lead to status epilepticus, similarly to some antiepileptic drugs.


Asunto(s)
Epilepsia Refractaria/terapia , Convulsiones/etiología , Estado Epiléptico/etiología , Estimulación del Nervio Vago/efectos adversos , Niño , Epilepsia Refractaria/diagnóstico por imagen , Humanos , Convulsiones/diagnóstico por imagen , Estado Epiléptico/diagnóstico por imagen
7.
Epilepsy Behav ; 82: 150-154, 2018 05.
Artículo en Inglés | MEDLINE | ID: mdl-29625366

RESUMEN

OBJECTIVE: The aim of this study was to assess the electrographic criteria related to seizure recurrence and determine age-related seizure recurrence in children with rolandic epilepsy under long-term follow-up. METHODS: We retrospectively analyzed the data belonging to 109 patients with rolandic epilepsy with sufficient information regarding disease course and follow-up duration longer than 3years. Patients were divided into two categories: Group A (n: 75), comprised of "patients having fewer than four seizures", and Group B (n: 34), the "recurrence group comprised of patients having more than four seizures in the first three months". The number of spikes per minute during both wakefulness and sleep, the localization of spikes other than centrotemporal region, and the duration of spike-wave activity were evaluated longitudinally, with repeated electroencephalogram (EEG) recordings every 6months. RESULTS: The appearance of rolandic spikes in awake EEGs tended to be more prevalent in Group B than Group A. In Group B, spike rates significantly increased in the 12 and 18months after onset whereas spike rates increased significantly only 6months after onset in Group A. Seizure recurrence is mostly seen at 6-8years, and improvement becomes evident by age 12. The mean number of paroxysmal rolandic discharges during sleep was significantly higher in the younger age groups (3-5, 6-8), and the mean number of spikes per minute significantly decreased at ages 9-11 and over 12. CONCLUSION: Our study demonstrates that extended periods of high frequency of paroxysmal discharges, initial frontal EEG focus, and persistence of awake interictal abnormalities are highly effective in predicting seizure recurrence in patients with rolandic epilepsy (RE).


Asunto(s)
Epilepsia Rolándica/fisiopatología , Convulsiones/fisiopatología , Adolescente , Factores de Edad , Análisis de Varianza , Niño , Preescolar , Progresión de la Enfermedad , Electroencefalografía , Femenino , Estudios de Seguimiento , Lóbulo Frontal/fisiología , Humanos , Masculino , Recurrencia , Estudios Retrospectivos , Sueño/fisiología , Vigilia/fisiología
8.
Epilepsy Behav ; 81: 33-40, 2018 04.
Artículo en Inglés | MEDLINE | ID: mdl-29462779

RESUMEN

PURPOSE: The aim of this study was to evaluate the effects of vagus nerve stimulation (VNS) on heart rate variability (HRV) in children with epilepsy. METHODS: The subgroups of HRV, namely time domain (Standard deviation of NN interval (SDNN), SDNN index, Standard deviation of the averages of NN intervals (SDANN), Root mean square of successive differences (RMMSD), Adjacent NN intervals differing by more than 50 ms in the entire recording divided by the total number of all NN intervals (PNN50), triangular index) and frequency domain (Low-frequency (LF), High-frequency (HF), LF/HF), were investigated in 20 pediatric patients before and after 6 and 12months of VNS treatment during day and night by comparing their data with those of 20 control subjects. In addition, subgroups of age, epilepsy duration and localization, and antiepileptic drugs (AEDs) were also evaluated if they had further effects on basal HRV levels. RESULTS: Increased heart rates (HRs); decreased SDNN, SDANN, RMMSD, and PNN50; and increased LF/HF ratios were identified before VNS therapy (p<0.05). Even though remarkable improvement was seen after 6months of VNS treatment (p<0.05), no further changes were observed in 12-month compared with 6-month levels (p>0.05) in all parameters, still even significantly lower than those of controls (p<0.05). Longer duration of epilepsy and localization of epileptic focus, such as in the temporal lobe, were also found to further contribute to diminished basal HRV levels (p<0.05). CONCLUSION: The cardiovascular system is under deep sympathetic influence in children with epilepsy. Although VNS seems to provide a substantial improvement by achieving increased parasympathetic effects in short-term therapy, the levels were still lower than those of healthy children after either short- or long-term therapy. Therefore, impaired cardiovascular autonomic regulation may be associated with the epileptic process itself as well as with the contribution of some additional factors. Overall, different aspects such as age, epilepsy duration, epileptic focus, seizure frequency, and AEDs should also be considered for their further possible effects on HRV during VNS therapy.


Asunto(s)
Sistema Nervioso Autónomo/fisiopatología , Epilepsia/terapia , Frecuencia Cardíaca/fisiología , Estimulación del Nervio Vago , Nervio Vago/fisiología , Adolescente , Anticonvulsivantes/farmacología , Estudios de Casos y Controles , Niño , Preescolar , Epilepsias Parciales/fisiopatología , Epilepsia/fisiopatología , Femenino , Frecuencia Cardíaca/efectos de los fármacos , Humanos , Masculino , Convulsiones/fisiopatología , Factores de Tiempo
9.
Eur J Paediatr Neurol ; 21(5): 738-744, 2017 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-28623069

RESUMEN

PURPOSE: We examined the various types of epilepsy in children with neonatal hypoglycemia in order to define electroclinical and prognostic features of these patients. METHOD: We retrospectively reviewed the medical records of patients with a history of symptomatic neonatal hypoglycaemia who have been followed at Gazi University Hospital Pediatric Neurology Department between 2006 and 2015. Patients with perinatal asphyxia were excluded. Details of each patient's perinatal history, neurological outcome, epilepsy details, seizure outcome and EEG and brain MRI findings were reviewed. RESULTS: Fourty five patients (range 6 mo-15 y) with a history of symptomatic neonatal hypoglycaemia were included the study. Epilepsy developed in 36 patients and 23 of them had intractable epilepsy. All patients had occipital brain injury. CONCLUSION: We observed that most of the patients, either manifesting focal or generalized seizures, further develop intractable epilepsy. This finding establishes neonatal hypoglycemia as a possible cause to be considered in any case of intractable epilepsy.


Asunto(s)
Epilepsia/diagnóstico , Epilepsia/etiología , Hipoglucemia/complicaciones , Convulsiones/diagnóstico , Convulsiones/etiología , Adolescente , Niño , Preescolar , Electroencefalografía , Femenino , Humanos , Lactante , Recién Nacido , Imagen por Resonancia Magnética , Masculino , Pronóstico , Estudios Retrospectivos
10.
Childs Nerv Syst ; 33(9): 1501-1507, 2017 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-28534259

RESUMEN

INTRODUCTION: The goal of this study was to evaluate the utility of orbital ultrasonography and magnetic resonance imaging in the diagnosis of idiopathic intracranial hypertension (IIH). METHOD: We reviewed the medical records of patients referred to our department for suspected IIH. RESULTS: Seven children were diagnosed with IIH. Nine children revealed pseudopapilledema by optic coherence tomography and/or orbital ultrasonography. When the axial sequences were reexamined, patients with papilledema had optic nerve sheath (ONS) enlargement (6.62 ± 0.70 mm); patients with pseudopapilledema had ONS diameter as 4.62 ± 0.64 mm. There was a significant correlation between the CSF opening pressure and ONS diameter (p < 0.005, r = 0.661). In the papilledema group, the presence of proposed subtle markers as increased tortuosity in the optic nerve was found in six patients. Five of seven patients had a target sign, intraocular protrusion of the optic nerve, and posterior globe flattening. DISCUSSION: Ophthalmological review is important to avoid unnecessary procedures for detection of true papilledema. ONS diameter is a reliable neuroimaging marker as other subtle markers.


Asunto(s)
Enfermedades Hereditarias del Ojo/diagnóstico por imagen , Hipertensión Intracraneal/diagnóstico por imagen , Enfermedades del Nervio Óptico/diagnóstico por imagen , Papiledema/diagnóstico por imagen , Adolescente , Niño , Preescolar , Enfermedades Hereditarias del Ojo/complicaciones , Femenino , Humanos , Hipertensión Intracraneal/etiología , Presión Intracraneal , Imagen por Resonancia Magnética , Masculino , Nervio Óptico/diagnóstico por imagen , Enfermedades del Nervio Óptico/complicaciones , Papiledema/complicaciones , Estudios Retrospectivos , Ultrasonografía
11.
Epilepsy Res ; 129: 41-50, 2017 01.
Artículo en Inglés | MEDLINE | ID: mdl-27894011

RESUMEN

OBJECTIVE: The aim of this study was to compare both ILAE 2010 and semiological seizure classification (SSC) in terms of their applicability and utility and to predict epileptogenic zone in children with epilepsy. METHODS: Both ILAE 2010 classification and SSC which is a part of five dimensional classification were applied for a total 138 children by dividing into younger (≤6y/o) and older (>6y/o) age groups over the last two years as retrospectively. After the assessment of the seizures types, epilepsy syndromes, and etiologies; the data were also compared to evaluate if having correlation between epileptogenic zone and seizure subtypes in both ILAE 2010 and SSC. RESULTS: ILAE 2010 indicated that 66.7% of the patients had focal seizures, 15.9% had generalized seizures, and 14.4% had seizures of unknown origin. The SSC revealed that the most frequent seizure type was simple seizures (56.5%), second frequent one was complex motor seizures (46.4%) and dialeptic seizures (39.9%). To predict epileptogenic zone, SSC was found to be more specific than ILAE in terms of the more subgroups of SSC were related to the more subgroups of epileptogenic zone (p<0.05). Furthermore, there was a clear correlation between focal foci and specific seizure types in older ages, while many foci caused to same seizure types and tend to no clear focal foci with generalized onset in younger ages. On the other hand, the relationship between epileptogenic zone and etiology was more remarkable in the five dimensional classification. SIGNIFICANCE: Preference of seizure classification system is unique for each patient and depends on requirements. Therefore, one dimension cannot be sufficient for evaluation the nature of the seizures in some patients. Furthermore, age related evolution of the seizure types should not be ignored due to ongoing maturation state of the brain. ILAE 2010 and SSC have weak and strong points compared to each other. Semiological seizure classification is more informative in terms of identifying the epileptogenic zone which may be important in specific occasions like pre-surgical work up, while ILAE is simple and easier method which can be applied for seizure description and their characteristics in daily practice.


Asunto(s)
Epilepsia/clasificación , Epilepsia/fisiopatología , Convulsiones/clasificación , Convulsiones/fisiopatología , Factores de Edad , Niño , Preescolar , Epilepsia/tratamiento farmacológico , Epilepsia/etiología , Femenino , Humanos , Masculino , Estudios Retrospectivos , Convulsiones/tratamiento farmacológico , Convulsiones/etiología
12.
Epilepsy Behav ; 63: 67-72, 2016 10.
Artículo en Inglés | MEDLINE | ID: mdl-27566969

RESUMEN

OBJECTIVE: This study aimed to investigate the efficacy of resective surgery in children with focal lesional epilepsy by evaluating the predictive value of pre- and postsurgical factors in terms of seizure freedom. METHODS: This study included 61 children aged between 2 and 18years who were admitted to the pediatric video-EEG unit for presurgical workup. Each patient was evaluated with a detailed history, video-EEG, neuroimaging, and postsurgical outcomes according to Engel classification to predict postsurgical seizure freedom. All the possible factors including history, etiology, presurgical evaluation, surgical procedures, and postsurgical results were analyzed for their predictive value for postoperative seizure freedom. RESULTS: Of the 61 patients, 75% were diagnosed as having temporal lobe epilepsy (TLE), and 25% were diagnosed with extra-TLE. Two years after the surgery, 78.6% were seizure-free, of which 89% had TLE, and 50% had extra-TLE (p<0.05). Patients were more likely to have a favorable outcome for seizure freedom if they had rare seizure frequency, focal EEG findings, and focal seizures; had a temporal epileptogenic zone; or had TLE and hippocampal sclerosis. On the other hand, patients were more likely to have unfavorable results for seizure freedom if they had younger age of seizure onset, frequent seizures before the surgery, a frontal or multilobar epileptogenic zone, secondarily generalized seizures, extra-TLE with frontal lobe surgery, or focal cortical dysplasia. SIGNIFICANCE: Resective surgery is one of the most effective treatment methods in children with intractable epilepsy. A history of young age of seizure onset, frequent seizures before surgery, secondarily generalized seizures, a multilobar epileptogenic zone, frontal lobe surgery, and focal cortical dysplasia (FCD) are the most important predictive factors indicating that a patient would continue having seizures after surgery. On the other hand, focal seizure semiologies, temporal lobe localization, and hippocampal sclerosis indicate that a patient would have better results in terms of seizure freedom.


Asunto(s)
Epilepsias Parciales/cirugía , Epilepsia del Lóbulo Temporal/cirugía , Malformaciones del Desarrollo Cortical/cirugía , Lóbulo Temporal/cirugía , Adolescente , Niño , Preescolar , Electroencefalografía , Epilepsias Parciales/complicaciones , Epilepsias Parciales/etiología , Epilepsia del Lóbulo Temporal/etiología , Femenino , Humanos , Masculino , Malformaciones del Desarrollo Cortical/complicaciones , Neuroimagen , Resultado del Tratamiento
13.
Eur J Paediatr Neurol ; 20(4): 549-54, 2016 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-27140809

RESUMEN

BACKGROUND: Epilepsy is one of the most common chronic neurologic disorders. Daily periodicity of epileptic seizures has been known for over a century. The diurnal patterns of epileptic seizures have also been observed in studies. AIM: To investigate the sleep/wake cycle, day/night, and 24-h periodicity of various seizure subtypes and seizure onset localizations in children. METHODS: We analyzed the clinical seizures of 170 consecutive epilepsy patients who underwent video-electroencephalography (EEG) monitoring over the last 5 years. Semiology of the seizures was classified according to the semiological seizure classification. Origin of the seizures was defined by the onset of ictal activity on EEG. Seizures were evaluated in terms of occurrence during the day (06:00-18:00 h) or night (18:00-06:00 h), in wakefulness or in sleep, and within a 3-h time interval throughout 24 h. RESULTS: A total of 909 seizures were analyzed. Auras, dialeptic, myoclonic, hypomotor, atonic seizures, and epileptic spasms occurred more frequently in wakefulness; tonic, clonic, and hypermotor seizures occurred more frequently in sleep. Auras, dialeptic, and atonic seizures and epileptic spasms occurred more often during daytime; hypermotor seizures occurred more often at night. Generalized seizures were seen most frequently in wakefulness (between 12:00 and 18:00 h); frontal lobe seizures were seen at night and in sleep (between 24:00 and 03:00 h); temporal lobe seizures were seen in wakefulness (between 06:00 and 09:00 h and between 12:00 and 15:00 h); occipital seizures were seen during daytime and in wakefulness (between 09:00 and 12:00 h and between 15:00 and 18:00 h, respectively); parietal seizures were seen mostly during daytime. CONCLUSIONS: Seizures in children occur in specific circadian patterns and in specific sleep/wake distributions depending on seizure onset location and semiology.


Asunto(s)
Ritmo Circadiano , Epilepsia Generalizada/fisiopatología , Epilepsia/fisiopatología , Sueño/fisiología , Vigilia/fisiología , Adolescente , Niño , Preescolar , Electroencefalografía , Epilepsia del Lóbulo Temporal/fisiopatología , Femenino , Humanos , Masculino , Monitoreo Fisiológico
14.
Childs Nerv Syst ; 32(4): 641-6, 2016 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-26767841

RESUMEN

PURPOSE: Over the past two decades, vagus nerve stimulation (VNS) has become an accepted and viable treatment modality for intractable epilepsy both in children and adults. Earlier studies have demonstrated short-term seizure outcomes, usually for up to 5 years; so far, none have reported an extended outcome in children. We aimed to assess long term seizure outcome in children with intractable epilepsy for more than 5 years. METHODS: We identified patients who had VNS implantation for treatment of intractable epilepsy from March 2000 to March 2015 at our Epilepsy Center and collected data including demographic, age at epilepsy onset and VNS implantation, duration of epilepsy, seizure type, number of antiepilepsy drugs (AEDs), and monthly seizure frequency before VNS implantation and at the last clinic visit. Phone surveys were conducted with patients without recent clinic follow-up. RESULTS: Fifty-six patients (aged 4-17 at the time of implant) are the subjects of the study. Seizure reduction of >50 % was achieved in 9.8 % (6th month), 24 % (2nd year), 46.4 % (3rd year), and 54 %(5th year), and overall 35 (62.5 %) of the 56 subjects had a greater than 50 % reduction in seizure frequency at the last follow-up. Eleven patients became seizure free. The results, once obtained, were maintained steadily or even improved over time without any loss of efficacy during the follow-up. The only parameter, significantly related with clinical response, was age at seizure onset. The most frequent adverse events were hoarseness, cough, sore throat, and anorexia, experienced by 13 patients. Two patients had local wound infections and lead to the removal of the stimulator. An improvement in alertness, attention, and psychomotor activity, independent of the efficacy of vagal nerve stimulation, was observed in 8 patients. CONCLUSION: To our knowledge, this is the first pediatric study evaluating seizure outcome over more than 5 years of follow-up, and demonstrates a favorable seizure outcome of >50 % seizure frequency in 62.5 % of patients and seizure freedom in 11 patients. It is well tolerated over an extended period of time.


Asunto(s)
Epilepsia Refractaria/terapia , Resultado del Tratamiento , Estimulación del Nervio Vago/métodos , Adolescente , Niño , Preescolar , Epilepsia Refractaria/diagnóstico por imagen , Femenino , Humanos , Estudios Longitudinales , Imagen por Resonancia Magnética , Masculino , Estudios Retrospectivos
15.
Pediatr Neurol ; 53(2): 151-6, 2015 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-26101095

RESUMEN

BACKGROUND: Increased intracranial hypertension is defined as elevated intracranial pressure with absence of hydrocephalus, vascular or structural abnormalities, and normal cerebrospinal fluid content. Magnetic resonance imaging abnormalities of the optic nerve and sheath that have been described in adults include increased nerve tortuosity, flattening in posterior aspect of globe, intraocular protrusion of the optic nerve, and enlarged optic nerve sheath. PURPOSE: We evaluated accuracy of those proposed markers on magnetic resonance imaging in children with increased intracranial hypertension that are described in adults. MATERIALS AND METHODS: Eleven patients between 3 and 15 years of age with intracranial hypertension were selected for re-evaluation of magnetic resonance imaging that had been previously described as normal to determine the presence of: (1) increased tortuosity and elongation of the optic nerve; (2) enlargement of the optic nerve sheath on axial and coronal T2 so called by us "target sign" and postcontrast T1 sequences; (3) flattening in posterior aspect of the globe; and (4) intraocular protrusion of the optic nerve head. RESULTS: Of the 11 patients, tortuosity of the optic nerve (10/11, 90.9%) and enlarged optic nerve sheath--target sign (7/11, 63.6%)--were the most common findings. Flattening in the posterior aspect of globe (5/11, 45.5%) and intraocular protrusion (3/11, 27.3%) were also detected as a novel magnetic resonance imaging findings. CONCLUSION: Magnetic resonance imaging findings of the optic nerve and sheath include valuable signs of intracranial hypertension not only in adults but also in children. This is the first detailed analysis of the magnetic resonance imaging findings in children with increased intracranial hypertension.


Asunto(s)
Hipertensión Intracraneal/diagnóstico , Imagen por Resonancia Magnética , Adolescente , Niño , Preescolar , Femenino , Humanos , Angiografía por Resonancia Magnética , Masculino , Nervio Óptico/patología
16.
Seizure ; 29: 63-8, 2015 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-26076845

RESUMEN

PURPOSE: The purpose of this study was to determine the long-term surgical outcomes of magnetic resonance imaging (MRI)-negative, fluorodeoxyglucose positron emission tomography (FDG-PET)-positive patients with temporal lobe epilepsy (TLE) and compare them with those of patients with mesial temporal sclerosis (MTS). METHODS: One hundred forty-one patients with TLE who underwent anterior temporal lobectomy were included in the study. The surgical outcomes of 24 patients with unilateral temporal hypometabolism on FDG-PET without an epileptogenic lesion on MRI were compared with that of patients with unilateral temporal hypometabolism on FDG-PET with MTS on MRI (n=117). The outcomes were compared using Engel's classification at 2 years after surgery. Clinical characteristics, unilateral interictal epileptiform discharges (IEDs), histopathological data and operation side were considered as probable prognostic factors. RESULTS: Class I surgical outcomes were similar in MRI-negative patients and the patients with MTS on MRI (seizure-free rate at postoperative 2 years was 79.2% and 82% in the MRI-negative and MTS groups, respectively). In univariate analysis, history of febrile convulsions, presence of unilateral IEDs and left temporal localization were found to be significantly associated with seizure free outcome. Multivariate analysis revealed that independent predictors of a good outcome were history of febrile convulsions and presence of unilateral IEDs. CONCLUSION: Our results suggest that epilepsy surgery outcomes of MRI-negative, PET positive patients are similar to those of patients with MTS. This finding may aid in the selection of best candidates for epilepsy surgery.


Asunto(s)
Encéfalo/cirugía , Epilepsia del Lóbulo Temporal/cirugía , Adulto , Encéfalo/diagnóstico por imagen , Encéfalo/patología , Encéfalo/fisiopatología , Electroencefalografía , Epilepsia del Lóbulo Temporal/diagnóstico por imagen , Epilepsia del Lóbulo Temporal/patología , Epilepsia del Lóbulo Temporal/fisiopatología , Femenino , Fluorodesoxiglucosa F18 , Estudios de Seguimiento , Lateralidad Funcional , Humanos , Imagen por Resonancia Magnética , Masculino , Tomografía de Emisión de Positrones , Pronóstico , Radiofármacos , Estudios Retrospectivos , Convulsiones/diagnóstico por imagen , Convulsiones/patología , Convulsiones/fisiopatología , Convulsiones/cirugía , Resultado del Tratamiento , Esclerosis Tuberosa/diagnóstico por imagen , Esclerosis Tuberosa/patología , Esclerosis Tuberosa/fisiopatología , Esclerosis Tuberosa/cirugía
17.
Seizure ; 26: 86-93, 2015 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-25799908

RESUMEN

PURPOSE: To evaluate the electroclinical features, treatment effectiveness, and outcome of 59 patients with epileptic encephalopathy with electrical status epilepticus during sleep. METHODS: Medical-files of 59 patients with electrical status epilepticus during sleep were retrospectively evaluated for data concerning: history, physical and neurological examinations, sleep and awake EEG's, psychometric tests and brain MRI. RESULTS: A total of 31 boys and 28 girls were identified. Patients were evaluated in two groups: symptomatic/structural and idiopathic group. There was no significant difference between the etiological groups in term of mean age at ESES onset, mean interval between the first seizure and the onset of ESES. The mean age at seizure onset was earlier in the symptomatic/structural group than the idiopathic ones. The mean follow-up time after the ESES onset was 4.5 years for all patients. The most effective antiepileptic drugs in our series were clobazam and levetiracetam. In refractory patients, steroid treatment was found effective during the early course of the disease. In the idiopathic group, cognitive decline has improved. However in the symptomatic group, patients did not respond to the treatment and cognitive deterioration did not improve in one third of the group. CONCLUSION: The long-term outcome of ESES is highly variable and usually depends on etiology and the duration of ESES. The most efficious antiepileptic drugs in our study are clobazam and levetiracetam.


Asunto(s)
Anticonvulsivantes/uso terapéutico , Estado Epiléptico/complicaciones , Estado Epiléptico/tratamiento farmacológico , Encéfalo/efectos de los fármacos , Encéfalo/fisiopatología , Niño , Preescolar , Electroencefalografía , Femenino , Humanos , Lactante , Estudios Longitudinales , Imagen por Resonancia Magnética , Masculino , Examen Neurológico , Sueño , Resultado del Tratamiento
18.
Case Rep Pediatr ; 2015: 783936, 2015.
Artículo en Inglés | MEDLINE | ID: mdl-25802788

RESUMEN

Subacute sclerosing panencephalitis (SSPE) is a devastating disease of the central nervous system (CNS) caused by persistent mutant measles virus infection. The diagnosis of SSPE is based on characteristic clinical and EEG findings and demonstration of elevated antibody titres against measles in cerebrospinal fluid. Subacute sclerosing panencephalitis can have atypical clinical features at the onset. Herein, we report an unusual case of subacute sclerosing panencephalitis in a child with recurrent febrile seizures. The disease progressed with an appearance of myoclonic jerks, periodic high amplitude generalized complexes on EEG, and elevated titers of measles antibodies in cerebrospinal fluid leading to the final diagnosis of subacute sclerosing panencephalitis.

19.
Epilepsy Behav ; 27(1): 124-9, 2013 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-23416282

RESUMEN

We aimed to determine the types and clinical characteristics of paroxysmal nonepileptic events (PNEs) in children. During a 13-year period, 765 patients underwent long-term video-EEG monitoring, and 95 (12.4%) of them were identified to have PNEs. The most common diagnoses were conversion disorder, parasomnias, staring spells, movement disorders, and hypnic jerks. Paroxysmal nonepileptic events originated from physiologic or organic (43.2%) and psychogenic (56.8%) causes. Mean delay in diagnosis was 3.1 years. Mean ages at diagnosis were 8.8 and 13.8 years in physiologic or organic and psychogenic groups, respectively. A marked female predominance was seen in the psychogenic group, whereas males slightly predominated in the physiologic or organic group. In the physiologic or organic group, events were less frequent, longer in duration, and commonly manifested as subtle motor activity, whereas subtle and prominent motor activities were encountered equally in both groups. Concomitant epilepsy was present in 10.5% of the patients. Differences in clinical characteristics may be helpful in differentiating physiologic or organic PNEs in children from psychogenic PNEs.


Asunto(s)
Trastornos de Conversión/diagnóstico , Convulsiones/diagnóstico , Convulsiones/psicología , Adolescente , Factores de Edad , Edad de Inicio , Niño , Preescolar , Trastornos de Conversión/psicología , Femenino , Humanos , Estudios Longitudinales , Masculino , Trastornos del Movimiento/complicaciones , Estudios Retrospectivos , Convulsiones/complicaciones , Grabación de Cinta de Video
20.
Endocr Res ; 37(4): 163-74, 2012.
Artículo en Inglés | MEDLINE | ID: mdl-22630163

RESUMEN

AIM: This study aimed to evaluate the effects of monotherapy with valproate or oxcarbazepine on the linear growth of children with idiopathic epilepsy. METHODS: Antiepileptic treatment with valproate or oxcarbazepine was initiated in 76 patients. These were evaluated at baseline and at 6 and 18 months after commencement of therapy to determine height standard deviations (height z-scores). Serum ghrelin, insulin-like growth factor-1, and insulin-like growth factor-binding protein-3 levels were measured. RESULTS: In prepubertal patients receiving oxcarbazepine, height z-scores were elevated after 6 and 18 months of therapy (p = 0.008 and p = 0.001, respectively); in pubertal patients, a significant increase was noted at the 18th month of therapy (p = 0.004). In prepubertal patients receiving oxcarbazepine, serum standardized insulin-like growth factor-1 and insulin-like growth factor-binding protein-3 levels were significantly higher at the 18th month of therapy compared with baseline (p = 0.005 and p = 0.004, respectively). In puber-tal patients receiving valproate, serum ghrelin levels were significantly decreased at the 18th month of therapy compared with baseline (p = 0.006). CONCLUSION: Exposure to oxcarbazepine stimulated linear growth in epileptic patients through mechanisms involving the release of insulin-like growth factor-1 and insulin-like growth factor-binding protein-3. In contrast, expo-sure to valproate did not affect linear growth, but did lead to a decrease in serum ghrelin levels.


Asunto(s)
Anticonvulsivantes/uso terapéutico , Estatura/efectos de los fármacos , Carbamazepina/análogos & derivados , Epilepsia/tratamiento farmacológico , Pubertad/efectos de los fármacos , Ácido Valproico/uso terapéutico , Adolescente , Carbamazepina/uso terapéutico , Niño , Preescolar , Femenino , Ghrelina/sangre , Humanos , Proteína 3 de Unión a Factor de Crecimiento Similar a la Insulina/sangre , Factor I del Crecimiento Similar a la Insulina/análisis , Masculino , Oxcarbazepina , Pubertad/sangre
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