-
[show abstract]
[hide abstract]
ABSTRACT: Lacosamide is a US Food and Drug Administration (FDA)-approved antiepileptic drug for patients 17 years or older with partial epilepsy. There are sparse data on children. The objective of our study was to evaluate its efficacy/safety in children with refractory epilepsy. Forty children (mean age 14.3 years) were treated with lacosamide at our institution (adjunctive therapy in 36, monotherapy in 4). Fifteen patients had symptomatic focal epilepsy, 2 had cryptogenic focal epilepsy, 20 had symptomatic generalized epilepsy, and 3 had cryptogenic generalized epilepsy. Two had juvenile myoclonic epilepsy and 5 had Lennox-Gastaut syndrome. Forty-two percent had at least >50% reduction in seizure frequency, and 6 became seizure free. Average dose was 7 mg/kg/d and average follow-up was 9.2 months. Responders had a 76.5% mean decrease in seizures. Fifteen children experienced an adverse reaction and 7 discontinued lacosamide (4: Ineffective, I: insurance denial, 1: tremor, 1: behavior). Lacosamide is effective and well-tolerated in children with refractory epilepsy.
Journal of child neurology 11/2012; · 1.59 Impact Factor
-
Genetics in medicine: official journal of the American College of Medical Genetics 08/2012; 14(8):757-8. · 3.92 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: The technique of chromosomal microarray analysis identifies genetic imbalance. Evaluation of its diagnostic role in pediatrics is still underway. We describe our experience with chromosomal microarrays. We retrospectively reviewed the charts of children in the Sections of Neurology and Clinical Genetics at St. Christopher's Hospital for Children who had undergone microarray analysis between 2006 and 2009. Collected data included age, sex, and the presence of mental retardation, developmental delay, autism, learning disability, hypotonia, dysmorphic features, and epilepsy, and the use of microarray technique. Statistical analysis was performed using SPSS. There were 82 children (mean age ± S.D., 5.7 ± 5 years), including 45 (55%) boys and 37 (45%) girls. All patients exhibited a normal karyotype. Microarray analysis produced abnormal results in 20 (23.5%). Deletions comprised 74% of all abnormalities. Patients with ≥ 4 clinical variables demonstrated a 30.5% incidence of abnormal chromosomal microarray findings, compared with 8.7% of patients with ≤ 3 clinical variables (P = 0.039, χ(2) test). Logistic regression indicated that motor impairment (P = 0.039) and presence of epilepsy (P = 0.024) independently contributed to the model. The likelihood of an abnormal microarray result increased with the number of clinical abnormalities. Microarray analysis will likely become the diagnostic genetic test of choice in children with neurodevelopmental disorders or epilepsy.
Pediatric Neurology 12/2010; 43(6):391-4. · 1.52 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: About 5-10% of school-age children manifest migraine headaches. Treatment options for pediatric migraine are limited. Topiramate is approved for migraine prophylaxis in adults, but its use in children is limited. We retrospectively reviewed the records of 37 patients, i.e., 22 (60%) girls and 15 (40%) boys (mean age, 14 years; range, 7.3-20.5 years), diagnosed with migraine without aura in 30 (81%), with aura in four (11%), and abdominal, ophthalmoplegic, and catamenial in one each. The mean follow-up was 12 +/- 5 months standard deviation (S.D.). Clinical response was qualified as excellent, good, no change, or worse. Numbers of headaches per month were 15 +/- 7 S.D. prior to treatment and 3 +/- 3.4 S.D. (P < 0.001) after treatment. An excellent or good response (>50% migraine reduction) was attained in 28 patients (76%). Ten (27%) patients exhibited adverse effects. Patients taking >2 mg/kg/day were more likely to demonstrate side effects. The mean dose for patients without adverse effects was 1.27 +/- 0.7 mg/kg/day S.D. Those who reported adverse effects were taking a mean dose of 2.8 +/- 1.5 mg/kg/day S.D. This study demonstrated that topiramate is an effective, safe alternative for the prophylaxis of pediatric migraine. An acceptable risk/benefit maintenance dose was < or =2 mg/kg/day.
Pediatric Neurology 09/2009; 41(3):167-70. · 1.52 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: The diagnosis of epilepsy is basically clinical, but it frequently raises the differential diagnosis with non-epileptic events. The development of continuous EEG monitoring (CEEGM) in the past decades has allowed a better diagnosis of epileptic patients of all ages. In this paper we review the data available in the literature about the efficacy of the different modalities of CEEGM in the diagnosis of pediatric epilepsy, emphasizing our personal experience. In our studies the ambulatory CEEGM supplemented with video allowed to answer the question that prompted its request in 80% of patients diagnosed with epilepsy and in 83% of those with the suspected diagnosis of epilepsy. With ambulatory computer-assisted CEEGM those figures were 88% and 89%, respectively, and with inpatient video-CEEGM they were 82% and 51%, respectively. The latter is crucial in the evaluation of epilepsy patients who are candidates for surgical treatment. Inpatient video-CEEGM is also very important in the management of patients with acute encephalopathies admitted to the Intensive Care Units. Both, ambulatory or inpatient CEEGM, are very useful in the differential diagnosis of clinical epileptic versus non-epileptic events, as well as in the confirmation of the type of epilepsy or epileptic syndrome. The development of technological advances and new EEG modalities in the future will help to continue to consider electroencephalography as a very important technique in the study of brain function in patients with acute or chronic encephalopathies.
Medicina 02/2009; 69(1 Pt 1):92-100. · 0.47 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: In this paper we review the current information regarding the use of new antiepileptic drugs (AEDs) used as monotherapy in children. We specifically include the following AEDs: lamotrigine (Lamictal), topiramate (Topamax), zonisamide (Zonegran), levetiracetam (Keppra), and oxcarbazepine (Trileptal). All of these AEDs have a broad spectrum of action in the treatment of partial and generalized seizures, except Oxcarbazepine, which is effective only in partial seizures. It is unclear whether or not monotherapy with the new AEDs offers higher efficacy and/or lower side effects compared to classic AEDs (phenobarbital, phenytoin, carbamazepine, or valproate) thereby significantly improving the quality of life in children with epilepsy. More studies are needed to answer these questions.
Medicina 02/2009; 69(1 Pt 1):101-8. · 0.47 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: Lamotrigine (LTG) has shown to confer broad-spectrum, well-tolerated control of epilepsy. Monotherapy is preferable over polytherapy because of better compliance, fewer adverse events, less interactions, lower teratogenicity and lower cost. The aim of this study is to evaluate the efficacy and safety of LTG monotherapy on seizure control in a cohort of children and adolescents with epilepsy. We retrospectively reviewed the records of children and adolescents treated with LTG monotherapy at our institution between 2001 and 2006. Data collected included demographics, seizure type, etiology of seizures, age at onset of seizures and at initiation of LTG treatment, number of antiepileptic drugs (AEDs) prior to LTG, dose of LTG, length of follow-up, treatment response, and adverse events. Seventy-two children and adolescents were identified (mean age 12.1 years); 37.5% had mental retardation. Age at onset of epilepsy was 5.7 years (0-16). Twenty three percent had symptomatic focal epilepsy, 15.5% idiopathic focal epilepsy, 19.4% symptomatic generalized epilepsy and 41.6% idiopathic generalized epilepsy. LTG was used as first-line monotherapy in 26.4% of patients and as a second-line monotherapy in 73.6%. Age at initiation of LTG therapy was 10 years (2.8-19). Mean number of AEDs tried prior to LTG was 1.3 (0-6). Mean dose of LTG was 5.5mg/kg/day (1.1-13.7). Mean follow-up period was 33 months (3 weeks to 11.5 years). The degree of seizure reduction was as follows: seizure free in 42%, 75-90% reduction in 17.4%, 50-74% in 11.6%, 25-49% in 10%. Sixteen percent had no change in seizure control and 3% became worse. The most common adverse event was rash (6.9%). Six (8.3%) patients discontinued LTG because of the adverse events. No patient had Stevens-Johnson syndrome. In conclusion, LTG was effective and well-tolerated as monotherapy in children and adolescents for both focal and generalized epilepsies.
European journal of paediatric neurology: EJPN: official journal of the European Paediatric Neurology Society 07/2008; 13(2):141-5. · 2.01 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: The aim of this paper is to review the results of recent clinical studies of some therapies that have demonstrated a neuroprotective effect in perinatal hypoxic-ischemic encephalopathy (HIE) and to present the future perspectives of other clinical and basic research investigations. THERAPIES WITH DEMONSTRATED CLINICAL EFFICACY: ALLOPURINOL: It blocks the production of free radicals following hypoxia-ischemia. In a recent study, infants with hypoplastic left heart syndrome treated with allopurinol, but not those with other congenital cardiopathies, had significantly less number of complications than controls, including death, seizures, coma or cardiac events. OPIOIDS: In another recent study, newborns with HIE treated with morphine or phentanyl, had less severe brain damage on MRI and a better neurological outcome. HYPOTHERMIA: Both local (head cooling) or systemic (whole body) hypothermia have a neuroprotective effect in selected newborns with HIE. FUTURE PERSPECTIVES: ANTIEPILEPTIC DRUGS: They have multiple mechanisms of action that can block the biochemical cascade of neuronal damage in HIE. OTHER THERAPEUTIC MODALITIES: Among them the following should be emphasized: combined neuroprotective treatments, growth factors, genetic therapies, stem cell transplant, and neuroprotective immunization. In conclusion, a better knowledge of the molecular mechanisms of HIE pathogenesis and better clinical studies of neuroprotective therapies will open new possibilities aplicable to clinical practice. These advances will undoubtedly improve the prognosis of newborns with HIE.
Medicina 02/2007; 67(6 Pt 1):543-55. · 0.47 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: The clinical features of seizures occurring in the pediatric intensive care unit (PICU) setting are not well characterized. Adult ICU studies reveal an incidence of seizures ranging from 0.8% to 3.3%, with vascular, metabolic abnormalities, and drug withdrawal being the most common etiologies. The objective of this study is to investigate the clinical characteristics of seizures in children admitted to the PICU at our institution.
We performed a retrospective review of all patients with diagnoses of seizures or epilepsy, admitted to our PICU from 2002 to 2004. Of 6,820 admissions, 32 patients, aged one month to 19 years had seizures in the PICU.
The incidence of seizures was 0.5%. Developmental delay or mental retardation was present in 37% of patients. Seizures were generalized in 26 (81%), and focal in 6 (19%); 34% had status epilepticus. The etiology of seizures was epilepsy in 11 (34%). Seizures that do not meet the diagnosis of epilepsy were diagnosed in 21 (66%) including post-craniotomy in five (23%), febrile seizures in three (14%), encephalitis in three (14%), and hydrocephalus in three (14%). Thirty-one patients (97%) were initially treated with either lorazepam or fosphenytoin.
Seizures in PICU have different clinical characteristics from those in adults. Recognizing the common seizure etiologies in PICU is likely to lead to a more prompt and effective treatment. Antiepileptic drug prophylaxis may be useful in post-craniotomy patients. A neurological consultation and EEG evaluation are of the utmost importance to help rule in or out epileptic disorders in the PICU.
Epileptic disorders: international epilepsy journal with videotape 01/2007; 8(4):277-84. · 1.50 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: We hypothesized that neonatal seizures lead to increased Ca(2+) influx (nCa(2+)I) in neuronal nuclei of newborn rats and that such increase is nitric-oxide mediated. Neuronal nuclear (45)Ca(2+) influx (nCa(2+)I) was measured in neuronal nuclei of 25 10-day-old male rat-pups newborn brains. They were divided into five groups (n = 5/group). (I) control; (II) hypoxia without seizures; (III) hypoxia with seizures; (IV) kainate, 2 mg/kg intraperitoneal (i.p.)-induced seizures and (V) 7-nitroindazole (7-NINA), 1 mg/kg i.p. pretreated, kainate-induced seizures. nCa(2+)I was significantly (P < 0.05) increased following hypoxia or seizures (hypoxic- or kainate-induced). Post-hypoxic seizures further enhanced nCa(2+)I increase induced by hypoxia (P < 0.05). 7-NINA abated the nCa(2+)I increase induced by kainate. We conclude that (1) kainate or hypoxia-induced seizures in newborn rats modify the neuronal nuclear membrane function, resulting in increased nCa(2+)I, (2) seizures exacerbate the hypoxia-induced increased nCa(2+)I incurred after hypoxia and (3) intranuclear calcium surges during kainate-induced neonatal seizures are nitric oxide-mediated.
Neurochemical Research 10/2006; 31(10):1231-7. · 2.24 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: We report a new case of encephalocraniocutaneous lipomatosis, a rare neurocutaneous syndrome of unknown etiology with involvement of tissues arising from the mesoderm and ectoderm: skin, eye, adipose tissue, and brain. We also review the neurologic manifestations of the syndrome, the most frequent of which include seizures, ventricular enlargement, calcifications, mental retardation, and cerebellopontine angle tumor. Our patient had an extensive extradural spinal cord lipomatous lesion, emphasizing the importance of screening for spinal abnormalities in asymptomatic patients with this condition.
Journal of Child Neurology 11/2003; 18(10):725-9. · 1.75 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: A case of intracranial meningioma is reported in a 5-month-old infant. To date, 25 cases have been reported in the world literature in children less than 1 year of age. Macrocephaly was the most prominent clinical finding. Skull radiological studies, head CT scans, and cerebral angiography were definitive tools in making the diagnosis. Pathological analysis was conclusive. Complete surgical extirpation was the treatment of choice; the tumor weighed 600 g. The child remains stable 24 months later.
Child s Nervous System 03/1988; 4(2):112-115. · 1.54 Impact Factor
-
[show abstract]
[hide abstract]
ABSTRACT: La Insuficiencia Renal Crónica constituye un problema de salud cada vez más importante debido a la incidencia y prevalencia crecientes en los últimos años. Numerosas son las causas y factores que influyen en el desarrollo y progresión de la enfermedad. El diagnóstico y tratamiento precoz son fundamentales para el pronóstico, y de ellos dependerá la futura necesidad de tratamiento sustitutivo renal en estos pacientes. En esta revisión se tratan los aspectos más importantes en cuanto a epidemiología y tratamiento conservador (no dialítico) de la insuficiencia renal crónica.
Archivos de medicina, Vol. 1, Nº. 3, 2005.
