Neurohabilitation of Cognitive Functions in Pediatric Epilepsy Patients through LEGO®-Based Therapy.

In the pediatric population, epilepsy is one of the most common neurological disorders that often results in cognitive dysfunction. It affects patients' life quality by limiting academic performance and self-esteem and increasing social rejection. There are several interventions for the neurohabilitation of cognitive impairment, including LEGO®-based therapy (LEGO® B-T), which promotes neuronal connectivity and cortical plasticity through the use of assembly sets and robotic programming. Therefore, the aim of this study was to analyze the effect of LEGO® B-T on cognitive processes in pediatric patients with epilepsy. Eligible patients were identified; in the treatment group, an initial evaluation was performed with the NEUROPSI and BANFE-2 neuropsychological tests. Then, the interventions were performed once a week, and a final test was performed. In the control group, after the initial evaluation, the final evaluation was performed. An overall improvement was observed in the LEGO® B-T patients, with a significant increase in BANFE-2 scores in the orbitomedial, anterior prefrontal, and dorsolateral areas. In addition, in the gain score analysis, the orbitomedial and memory scores were significantly different from the control group. LEGO® B-T neurohabilitation is a remarkable option for epilepsy patients, who are motivated when they observe improvements.

Analysis of Adverse Drug Reactions in Pediatric Patients with Epilepsy: An Intensive Pharmacovigilance Study.

Epilepsy is a chronic neurological disease characterized by the presence of spontaneous seizures, with a higher incidence in the pediatric population. Anti-seizure medication (ASM) may produce adverse drug reactions (ADRs) with an elevated frequency and a high severity. Thus, the objective of the present study was to analyze, through intensive pharmacovigilance over 112 months, the ADRs produced by valproic acid (VPA), oxcarbazepine (OXC), phenytoin (PHT), and levetiracetam (LEV), among others, administered to monotherapy or polytherapy for Mexican hospitalized pediatric epilepsy patients. A total of 1034 patients were interviewed; 315 met the inclusion criteria, 211 patients presented ADRs, and 104 did not. A total of 548 ASM-ADRs were identified, and VPA, LEV, and PHT were the main culprit drugs. The most frequent ADRs were drowsiness, irritability, and thrombocytopenia, and the main systems affected were hematologic, nervous, and dermatologic. LEV and OXC caused more nonsevere ADRs, and PHT caused more severe ADRs. The risk analysis showed an association between belonging to the younger groups and polytherapy with ADR presence and between polytherapy and malnutrition with severe ADRs. In addition, most of the severe ADRs were preventable, and most of the nonsevere ADRs were nonpreventable.

Corrigendum: ALG1-CDG Caused by Non-Functional Alternative Splicing Involving a Novel Pathogenic Complex Allele.

[This corrects the article DOI: 10.3389/fgene.2021.744884.].

Anti-N-methyl-D-aspartate receptor encephalitis: An observational and comparative study in Mexican children and adults.

OBJECTIVE: To present a case series of encephalitis patients with anti-N-methyl-D-aspartate receptor antibodies, attending two neurological referral centers in a three-year period. METHODS: A retrospective, descriptive, comparative study included child and adult patients in two neurological populations, positive for antibodies against the NR1 and NR2 subunits of the glutamate (NMDA) receptor in serum and CSF, as determined during a three-year period. RESULTS: Sixty-six patients were included (40 children and 26 adults). Male patients were more affected (M: F ratio was 1:0.6). No differences in progression or hospitalization time were observed between groups. In children, 35% of patients showed herpetic infection before autoimmune encephalitis (P = 0.01). Among viral prodromal symptoms, upper respiratory tract infection (P = 0.02) and fever (P = 0.001) predominated in children, while infectious gastroenteritis was more frequent in adults (P = 0.03). Among neuropsychiatric signs, mental confusion (P = 0.0001) and orofacial dyskinesia/oromandibular dystonia (P = 0.0001) were frequent in children, while emotional lability (P = 0.03), catatonia (P = 0.0001), and headache (P = 0.005) predominated in adults. The score in the modified Rankin scale on admission was higher in children (4.3 ± 0.8 vs. 2.2 ± 1.3, P = 0.0001), but at one-year of clinical follow up no significant differences were found. CONCLUSIONS: Male patients were predominantly affected in our population. One-third of all patients developed prodromal infection. Neuropsychiatric clinical complaints were different in children and adults. However, post-hospitalization recovery was similar between groups.

ALG1-CDG Caused by Non-functional Alternative Splicing Involving a Novel Pathogenic Complex Allele.

This study reports on a Mexican mestizo patient with a multi-systemic syndrome including neurological involvement and a type I serum transferrin profile. Clinical exome sequencing revealed complex alleles in ALG1, the encoding gene for the chitobiosyldiphosphodolichol beta-mannosyltransferase that participates in the formation of the dolichol-pyrophosphate-GlcNAc2Man5, a lipid-linked glycan intermediate during N-glycan synthesis. The identified complex alleles were NM_019109.5(ALG1): c.[208 + 16_208 + 19dup; 208 + 25G > T] and NM_019109.5(ALG1): c.[208 + 16_208 + 19dup; 1312C > T]. Although both alleles carried the benign variant c.208 + 16_208 + 19dup, one allele carried a known ALG1 pathogenic variant (c.1312C > T), while the other carried a new uncharacterized variant (c.208 + 25G > T) causing non-functional alternative splicing that, in conjunction with the benign variant, defines the pathogenic protein effect (p.N70S_S71ins9). The presence in the patient's serum of the pathognomonic N-linked mannose-deprived tetrasaccharide marker for ALG1-CDG (Neu5Acα2,6Galß1,4-GlcNAcß1,4GlcNAc) further supported this diagnosis. This is the first report of an ALG1-CDG patient from Latin America.

Neurological manifestations temporally associated with SARS-CoV-2 infection in pediatric patients in Mexico.

PURPOSE: To describe the temporal association of specific acute neurological symptoms in pediatric patients with confirmed SARS-CoV-2 infection between May and August 2020. METHODS: We performed a recollection of all the clinical and laboratory data of patients having acute neurological symptoms temporally associated with SARS-CoV-2 infection at a third-level referral hospital in Mexico City (Instituto Nacional de Pediatría). Patients in an age group of 0-17 years with acute neurological signs (including ascending weakness with areflexia, diminished visual acuity, encephalopathy, ataxia, stroke, or weakness with plasma creatinine kinase (CK) elevation) were evaluated. RESULTS: Out of 23 patients with neurological manifestations, 10 (43%) had a confirmed SARS-CoV-2 infection. Among the infected patients, 5 (50%) were males aged 2-16 years old (median age 11.8 years old). Four (40%) patients confirmed a close contact with a relative positive for SARS-CoV-2, while 6 (60%) cases had a history of SARS-CoV-2-related symptoms over the previous 2 weeks. The following diagnoses were established: 3 cases of GBS, 2 of ON, 2 of AIS, one of myositis with rhabdomyolysis, one ACA, and one of anti-NMDA-R encephalitis. CONCLUSIONS: Neurological manifestations temporally associated with SARS-CoV-2 infection were noticed in the pediatric population even without respiratory symptoms. In this study, 2 of 6 symptomatic patients had mild respiratory symptoms and 4 had unspecific symptoms. During this pandemic, SARS-CoV-2 infection should be considered as etiology in patients with acute neurological symptoms, with or without previous respiratory manifestations, particularly in teenagers.

Ketogenic Diet Provided During Three Months Increases KCC2 Expression but Not NKCC1 in the Rat Dentate Gyrus.

Ketogenic diet, a high fat and low carbohydrate diet, has been used as a non-pharmacological treatment in refractory epilepsy since 1920. In recent years, it has demonstrated to be effective in the treatment of numerous neurological and non-neurological diseases. Some neurological and neuropsychiatric disorders are known to be caused by gamma-aminobutyric acid (GABA)-mediated neurotransmission dysfunction. The strength and polarity of GABA-mediated neurotransmission are determined by the intracellular chloride concentration, which in turn is regulated by cation-chloride cotransporters NKCC1 and KCC2. Currently, it is unknown if the effect of ketogenic diet is due to the modulation of these cotransporters. Thus, we analyzed the effect of a ketogenic diet on the cation-chloride cotransporters expression in the dentate gyrus. We estimated the total number of NKCC1 immunoreactive (NKCC1-IR) neuronal and glial cells by stereology and determined KCC2 labeling intensity by densitometry in the molecular and granule layers as well as in the hilus of dentate gyrus of rats fed with normal or ketogenic diet for 3 months. The results indicated that ketogenic diet provided during 3 months increased KCC2 expression, but not NKCC1 in the dentate gyrus of the rat. The significant increase of KCC2 expression could explain, at least in part, the beneficial effect of ketogenic diet in the diseases where the GABAergic system is altered by increasing its inhibitory efficiency.

Characterization and Outcomes of Epileptic Seizures in Mexican Pediatric Patients With Anti-N-Methyl-D-Aspartate Receptor Encephalitis.

Introduction Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is one of the most common autoimmune encephalitides. The frequency of anti-NMDAR encephalitis is known to exceed the frequency of any individual viral encephalitis in young subjects. Epileptic seizures are a cardinal symptom in anti-NMDAR encephalitis; a significant amount of pediatric patients exhibit seizures as the first symptom of the disease, and most of them will develop them during the acute phase. The use of antiepileptic drugs (AEDs) is a cornerstone of the treatment of these patients, but the choice of agent and duration of treatment is currently unknown. Materials and methods This was a single-center retrospective review case series of all pediatric patients with a confirmed diagnosis of anti-NMDAR encephalitis and epileptic seizures admitted to the National Institute of Pediatrics in Mexico City from January 2012 to July 2019. Results We included a total of 31 patients (males 64.5%, median age: 10 years). No patient showed evidence of teratoma; only 38% of cases had a viral prodrome. Most patients initially exhibited psychiatric symptoms (51%), but the leading cause in soliciting medical assistance was the presence of epileptic seizures (71%). About 85% of patients presented epileptic seizures during the course of the illness, predominantly focal onset seizures (42% focal to bilateral tonic-clonic seizures, 32% focal seizures with impaired awareness). Electroencephalogram (EEG) was abnormal in 97% of patients; the characteristic extreme delta brush pattern was found in 9% of patients. Two AEDs on average were required to control seizures during the acute stage. In six (19%) patients, human herpesvirus (HHV) was detected in cerebrospinal fluid (CSF); all of them had epileptic seizures, which were more resistant to pharmacological treatment during the acute phase, requiring a higher number of AED (median 2.5 vs. 2). The development of epilepsy after acute encephalitis was uncommon; at 24 months, only one patient continued to have epileptic seizures. One of the factors most closely related to the persistence of epileptic seizures was the inadequate response to immunotherapy after four weeks. The functional prognosis was generally good; at a two-year follow-up, only two (10%) patients had a significant disability [modified Rankin Scale (mRS) score: 3-5]; both patients had seizures at a one-year follow-up. Conclusions Sustained use of AEDs after the acute phase of anti-NMDAR encephalitis is controversial. We found that the continuation of AEDs after the acute phase could be considered in the following scenarios: status epilepticus (SE), inadequate response to immunotherapy at four weeks, and a high mRS score at discharge and during follow-up. In other cases, discontinuation of AED may be warranted. More studies are needed in our country to replicate these results.

Predominance of Dystrophinopathy Genotypes in Mexican Male Patients Presenting as Muscular Dystrophy with A Normal Multiplex Polymerase Chain Reaction DMD Gene Result: A Study Including Targeted Next-Generation Sequencing.

The complete mutational spectrum of dystrophinopathies and limb-girdle muscular dystrophy (LGMD) remains unknown in Mexican population. Seventy-two unrelated Mexican male patients (73% of pediatric age) with clinical suspicion of muscular dystrophy and no evidence of DMD gene deletion on multiplex polymerase chain reaction (mPCR) analysis were analyzed by multiplex ligation-dependent probe amplification (MLPA). Those with a normal result were subjected to Sanger sequencing or to next-generation sequencing for DMD plus 10 selected LGMD-related genes. We achieved a diagnostic genotype in 80.5% (n = 58/72) of patients with predominance of dystrophinopathy-linked genotypes (68%, n = 49/72), followed by autosomal recessive LGMD-related genotypes (types 2A-R1, 2C-R5, 2E-R4, 2D-R3 and 2I-R9; 12.5%, n = 9/72). MLPA showed 4.2% of false-negatives for DMD deletions assessed by mPCR. Among the small DMD variants, 96.5% (n = 28/29) corresponded to null-alleles, most of which (72%) were inherited through a carrier mother. The FKRP p.[Leu276Ile]; [Asn463Asp] genotype is reported for the first time in Mexican patients as being associated with dilated cardiomyopathy. Absence of dysferlinopathies could be related to the small sample size and/or the predominantly pediatric age of patients. The employed strategy seems to be an affordable diagnosis approach for Mexican muscular dystrophy male patients and their families.

Mutational spectrum of PTS gene and in silico pathological assessment of a novel variant in Mexico.

BACKGROUND: Tetrahydrobiopterin (BH4) is the cofactor for 6-pyruvoyl-tetrahydropterin synthase (PTPS); it is involved in BH4 biosynthesis and is encoded by PTS gene. Its deficiency (PTPSD) is characterized by hyperphenylalaninemia (HPA) and deficit in central monoamine neurotransmitters. We describe the clinical and mutational spectrum of five patients with PTPSD, from four unrelated Mexican families. All patients had symptomatic diagnosis and presented severe early neurological manifestations and HPA. METHODS: Clinical and biochemical data from studied patients were recorded. Responsible PTPSD genotypes was determined by direct and bidirectional Sanger DNA sequencing of the six PTS coding exons and their exon-intron borders, and these were directly searched in the available relatives. The novel PTS missense variant [NM_3000317.2:331G > T, p.(Ala111Ser)] was subjected to in silico, to predict a possible deleterious effect. RESULTS: Diminished fetal movements were perceived as a uniform characteristic in the studied group. DNA sequencing showed two known p.(Arg25∗) and p.(Val132TyrFs∗19) and the novel missense p.(Ala111Ser) PTS variants, the latter representing potentially a frequent PTPSD-responsible allele (50%, 4/8) in Mexican patients. In silico protein modeling analysis of the p.(Ala111Ser) variant revealed loss of hydrophobic interactions between the alanine and neighboring valines, suggesting that these changes in polarity may be detrimental for enzyme function, structure and/or stability. CONCLUSIONS: This work contributes to the knowledge of PTPS molecular spectrum. The delayed diagnosis of these patients emphasizes the importance of considering BH4 metabolism defects in the differential diagnosis of HPA, especially for countries that are beginning their HPA newborn screening programs.

Anticonvulsant and antioxidant effects of Tilia americana var. mexicana and flavonoids constituents in the pentylenetetrazole-induced seizures.

Tilia genus is commonly used around the world for its central nervous system properties; it is prepared as tea and used as tranquilizing, anticonvulsant, and analgesic. In this study, anticonvulsant activity of the Tilia americana var. mexicana inflorescences and leaves was investigated by evaluating organic and aqueous extracts (100, 300, and 600 mg/kg, i.p.) and some flavonoids in the pentylenetetrazole-induced seizures in mice. Moreover, antioxidant effect of these extracts and flavonoids was examined in an in vitro study by using spectrophotometric technique. Significant activity was observed in the methanol extract from inflorescences. An HPLC analysis of the methanol extract from inflorescences and leaves of Tilia allowed demonstrating the respective presence of some partial responsible flavonoid constituents: quercetin (20.09 ± 1.20 µg/mg and 3.39 ± 0.10 µg/mg), rutin (3.52 ± 0.21 µg/mg and 8.94 ± 0.45 µg/mg), and isoquercitrin (1.74 ± 0.01 µg/mg and 1.24 ± 0.13 µg/mg). In addition, significant but different antioxidant properties were obtained among the flavonoids and the extracts investigated. Our results provide evidence of the anticonvulsant activity of Tilia reinforcing its utility for central nervous system diseases whose mechanism of action might involve partial antioxidant effects due to the presence of flavonoids.

Effect of treatment and additional disease on pharmacokinetic of valproic acid in children with epilepsy.

BACKGROUND: Most of bayesian pharmacokinetic studies and the influence of clinical variables have been carried out in adults. PURPOSE: The aim was to estimate population-based pharmacokinetic of valproic acid (VPA) and to determine the effect of treatment and additional disease on its performance in children with epilepsy. MATERIAL AND METHODS: For the study steady-state serum concentrations of VPA were determined from 108 epileptic patients (44 females and 64 males) who were receiving the anticonvulsant as main drug of treatment with age range since 1 to 16 years (median 4y, 6m) and weight since 5.2 to 50 kg (median 17.5 kg). All patients had their renal, hepatic and nutritional functions normal. One compartment model using interactive two-stage Bayesian approach was employed in the analysis. RESULTS; Population estimates of CL/F and V/F for VPA were 0.022 +/- 0.013 L/h and 0.217 +/- 0.134 L/kg, respectively. These estimates were significantly affected by weight, age, carbamazepine (CBZ) and gastroesophageal reflux (GER). The final regression models were: CL/F (L/h) = 0.0696 + 0.0031 (Age) + 0.0075 (Weight); and V/F (L) = 0.674 + 0.0308 (Age) + 0.0756 (Weight). Prediction of VPA serum concentration in other validation group revealed an important improvement in the predictive performance of VPA concentrations in comparison with the basic model that did not include any co-variables. CONCLUSIONS: Based on a population model of children with epilepsy, the pharmacokinetic of VPA could be altered by weight, age and the administration of CBZ and additional GER to epilepsy.

Neurocysticercosis: clinical, radiologic, and inflammatory differences between children and adults.

BACKGROUND: Human neurocysticercosis (NC) is caused by Taenia solium larvae lodged in the central nervous system. NC is clinically heterogeneous, ranging from asymptomatic infection to severely incapacitating and even fatal presentations. Although NC affects adults and children, age-related factors have not been thoroughly studied. METHODS: We describe and compare the clinical, radiologic, and inflammatory features of pediatric and adult Mexican NC cases. Two hundred six NC cases (92 pediatric and 114 adult) diagnosed by computed tomography or magnetic resonance imaging were included. RESULTS: Seizures were more frequent in children (80.4% versus 56.1%), and intracranial hypertension and headaches were more frequent in adults (27.2% versus 15.2% and 35.1% versus 21.7%, respectively). Different causes underlie the different distribution of seizures and intracranial hypertension in the 2 patient groups. In pediatric NC patients, single colloidal parenchymal cysts were the most common radiologic findings compared with adults in whom multiple viable parasites in the basal subarachnoidal cisterns or in the ventricles were seen. Cerebrospinal fluid inflammation was greater in adults than in children (P = 0.02). CONCLUSIONS: This study documents significant age-related radiologic, clinical, and inflammatory differences in Mexican NC patients. Possible causes and relevance of these age-associated findings are discussed.

Estudio multicéntrico, abierto, para evaluar eficacia y seguridad de metilfenidato de liberación controlada en niños con trastorno por déficit de atención con hiperactividad (TDAH) / Multicentric study, to assess efficacy and security of controlled release OROS methylphenidate in children with attention deficit hyperactivity disorders

Introducción. Objetivo: evaluar la eficacia y seguridad de clorhidrato de metilfenidato de liberación controlada en sistema OROS (MPH OROS, Concerta®), en niños con trastorno por déficit de atención con hiperactividad (TDAH) previamente tratados con metilfenidato de liberación inmediata (MLI). Material y métodos. Se incluyeron 97 niños entre 6 y 16 años, con diagnóstico de TDAH de cualquier subtipo, según los criterios del DSM-IV, con tratamiento previo por lo menos 4 semanas antes con MLI en dosis de 10 a 60 mg por día, y que presentaban buena respuesta clínica. Se excluyeron niños con otras enfermedades psiquiátricas o metabólicas. La dosis de MPH OROS se administró una vez al día entre 18 a 54 mg. Se utilizaron escalas de impresión global clínica (CGI), Escala Iowa Conners para padres y maestros, Escala de Interacción de Pares, efectos sobre sueño y apetito, escala Yale de tics, somatometría y registro de eventos adversos en cada visita. Resultados. De los 97 niños, abandonaron 26 [4 (4%) por respuesta insuficiente y 2 (2%) por hiporexia y calambres]. Se analizaron 71 pacientes, 64 (90%) masculinos, 7 (10%) femeninos, con edad promedio 9 ± 2 años, con peso inicial promedio 33.8 + 9.7 kg y peso final 34.7 + 9.9 kg. La dosis de MPH OROS fue de 18 mg en 64% de los pacientes. Ningún paciente desarrolló tics de novo. La mejoría clínica de los pacientes acorde a las escalas de Iowa Conners y de pares fue significativa (P =0.001, t de Student). Los eventos adversos más comunes fueron cefalea (7%) e hiporexia (6%) leves y transitorios. Conclusiones. Estos resultados preliminares permiten indicar que MPH OROS es una buena alternativa para el manejo de los niños con TDAH y el cambio por MLI en algunos pacientes mostró una mejoría superior sin impacto negativo en sueño y apetito.

Introduction. Objective: to assess the efficacy of controlled release OROS MPH (Concerta®) in children with attention deficit/hyperactivity disorder (ADHD) previously treated with immediate release methylphenidate (MPH IR). Material and methods. Children with ADHD, all subtypes, ages 6 to 12 years, with good response to MPH, were switched from IR MPH to OROS MPH once a day (qd in the morning) at 18 to 54 mg/day in a 1 year follow-up trial.The primary end-points for analysis were the last available patient visit using last observation carried forward.The scales used were CGI,Yale's for tics, somatometry, appetite and sleep evaluation from parents, and adverse events record. Results.We included 97 patients, 26 drop-outs [4 (4%) for treatment failure and 2 (2%) hyporexia and cramps]. Of the 71 patients, 64 (90%) male and 7 (10%) female, mean age 9 + 2 years, initial mean weigth 33.8 + 9.7 kg and final 34.7 + 9.9 kg (normal growth). Children with OROS MPH showed significantly greater reductions in core ADHD symptoms. On the basis of mean teacher and parents Iowa Conners and Peers interactions ratings were a significant improvement (P =0.001, Student t). CGI ratings were improvement as well.The most common adverse events were headache (7%) and hyporexia (6%) mild and transient. Conclusion. For the treatment of core ADHD symptoms, OROS MPH dosed qd were well tolerated and efficacy treatment and there were no negative impact on sleep and appetite.

Trastorno por déficit de atención con hiperactividad: Actualidades diagnósticas y terapéuticas / Attention deficit hyperactivity disorder: Diagnostic and therapeutics currents

El trastorno por déficit de atención con hiperactividad es la entidad más frecuente en la consulta pediátrica neuropsiquiátrica. Numerosos estudios sustentan el origen biológico de esta enfermedad. Es un padecimiento crónico que se caracteriza por una tríada de síntomas consistente en inatención, hiperactividad e impulsividad, que afectan el funcionamiento académico, social y laboral de quien lo padece. El diagnóstico se establece de forma clínica; existe una elevada comorbilidad con otras psicopatologías. El abordaje terapéutico debe contemplar tanto la intervención farmacológica como la estrategia principal, siendo los estimulantes la primera línea de elección. Las intervenciones psicosociales son complementarias.

Attention deficit hyperactivity disorder it is the most frequent causes of pediatric neuro psychiatric consultation. Numerous studies sustain the biological origin of this illness. It is a chronic suffering that is characterized by a consistent triad of symptoms in attention deficit, hyperactivity and impulsiveness that affect the academic, social and physical activities of those who suffer it. The diagnosis is clinical and there is strong comorbidity with other psychiatric disorders. The main therapeutic approach is with the stimulants the first line drugs. The psychosocial intervention is complementary.

Short report: neonatal screening pilot study of Toxoplasma gondii congenital infection in Mexico.

Congenital toxoplasmosis is an obstetric problem in Mexico, but its actual frequency is unknown. Using a network for screening of non-infectious disorders, we performed a pilot study to determine the frequency of IgM antibodies to Toxoplasma gondii in 1,003 infants (53.1% male, mean +/- SD age = 18.3 +/- 13.0 days, birth weight = 3.116 +/- 0.453 kg) in Mexico City from March to April 2003. Blood samples embedded in filter paper were assayed for IgM antibodies using a capture enzyme-linked immunosorbent assay and results were confirmed by Western blot. Two asymptomatic newborns, one of them premature, had IgM and IgG antibodies in a serum sample taken from both the infant and the mother and were clinically followed. Our data suggest a frequency of approximately two cases of congenital T. gondii infection per 1,000 newborns in Mexico City.

Clinical-etiological and therapeutic profile of 719 Mexican epileptic children.

RATIONALE: We performed this study with the intention of describing the clinical-etiological characteristics and therapeutic responses in a group of epileptic children seen at the Instituto Nacional de Pediatría (INP), a tertiary facility in Mexico City. METHODS: All patients who attended the Epileptic Clinic between March and June 1998 and fulfilled the selection criteria were enrolled in the study. Clinical and therapeutic response data were recorded. Three groups were formed by etiology. Statistical tests were two-tailed, with alpha=0.05. RESULTS: In all, 719 patients were studied. The distribution by etiology was as follows: group I, idiopathic (123 patients); group II, cryptogenic (132); and group III, symptomatic (464). In group I, 56% of the patients were female. Mean age at onset was 5 years 2 months (SD: 3 years 8 months) in group I; 1 year 11 months (SD: 2 years 5 months) in group II; and 2 years 10 months (SD: 3 years 1 month) in group III. The mean evolution time was 5 years 4 months (SD: 4 years) in all groups. The most frequent variety of epilepsy in the three groups was generalized epilepsy, followed by partial epilepsy. The following epileptic syndromes were identified: in group I, 28 patients had epilepsy with generalized tonic-clonic seizures, 15, absence epilepsy, and 6, benign rolandic seizures; in group II, all 32 patients had focal cryptogenic epilepsy; in group III, 235 had generalized symptomatic and 192, focal symptomatic epilepsy. The main etiologies were hypoxic ischemic encephalopathy (24%) and neural infections (22%). Appropriate seizure control was achieved in 108 (87%) patients in group I; 84 (64%) in group II; and 315 (68%) in group III. In group I, no patient needed more than two antiepileptic drugs and 90% had normal psychomotor development. CONCLUSION: When the three groups were compared in terms of appropriate epileptic control and normal psychomotor development, group I differed from the other groups and the difference was statistically significant.

Tratamiento actual de la epilepsia / Current treatment of epilepsy

Se revisan los elementos clínicos para establecer el diagnóstico de epilepsia y sus variedades según la clasificación de la Liga Internacional contra la Epilepsia emitida en 1989. Se proponen los lineamientos básicos para su tratamiento adecuado con un régimen de monoterapia, previa revisión de las principales características e indicaciones de los fármacos convencionales y de los de nueva generación

Neurocisticercosis en la edad pediátrica: Perfil social y clínico en 122 pacientes / Neurocysticercosis in children. Social and clinical profile in 122 Mexican patients

La neurocisticercosis (NC) es un problema de salud pública en México. Existe poca información sobre esta enfermedad en la edad pediátrica. El objetivo del presente trabajo es conocer las características clínicas y sociales en pacientes con NC tratados en el servicio de Neurología del I.N.P. en los últimos 10 años. Fueron 122 pacientes, 58 por ciento del sexo femenino, la edad promedio fue ocho años. El 50 por ciento de los pacientes vive en áreas hurbanas; 77 familias cuentan sólo con escolaridad básica. Las principales manifestaciones clínicas son epilepsia en 78 por ciento, hipertensión intracraneana en 20 por ciento y cefalea en 18 por ciento. En todos los pacientes se hizo TAC cerebral; en 20 se realizó IRM. Se encontró reacción positiva para cisticercosis en el LCR en 76 por ciento. La evolución de los pacientes fue inadecuada en 26 por ciento y la mortalidad de 1.2 por ciento

Infusión continua de diazepam para el manejo de estado epiléptico refractario / Continuous infusion of diazepam in the management of refractory status epilepticus

El estado epiléptico es una urgencia neurológica con elevada morbimortalidad, más aún cuando es refractario al tratamiento habitual. Se presenta la experiencia con 20 pacientes con estado epiléptico, tratados con infusión continua de diazepam. La fluctuó entre dos meses y 13 años. Hubo cinco niñas y quince niños. Quince tenían estado epiléptico generalizado y cinco con estado epiléptico parcial. Dieciocho tenían diagnóstico de epilepsia sintomátic y dos, criptogénica. La respuesta a la infusión fue favorable en 17 casos; en ninguno se requirió ventilación asistida o uso de aminas presoras en forma simultánea. Este tratamiento no causó morbilidad asociada