Predictive Factors of the Use of Rituximab and Belimumab in Spanish Lupus Patients.

Objectives: To analyze the characteristics and the predictive factors of the use of rituximab and belimumab in daily practice in patients from the inception cohort Registro Español de Lupus (RELES). Material and methods: The study included 518 patients. We considered patients treated with biologics who received at least one dose of rituximab or belimumab, and possible indications of those manifestations registered at the same time or in the previous 2 months of the start of the therapy. Results: In our cohort, 37 (7%) patients received at least one biological treatment. Rituximab was prescribed in 26 patients and belimumab in 11. Rituximab was mainly prescribed for hemolytic anemia or thrombocytopenia (11 patients, 42%), lupus nephritis and neuropsychiatric lupus (5 patients each, 19%). Belimumab was mostly used for arthritis (8 patients, 73%). In the univariate analysis, the predictive factors at diagnosis for the use of biologic therapy were younger age (p = 0.022), a higher SLEDAI (p = 0.001) and the presence of psychosis (p = 0.011), organic mental syndrome (SOCA) (p = 0.006), hemolytic anemia (p = 0.001), or thrombocytopenia (p = 0.01). In the multivariant model, only younger age, psychosis, and hemolytic anemia were independent predictors of the use of biologics. Conclusions: Rituximab is usually given to patients with hematological, neuropsychiatric and renal involvement and belimumab for arthritis. Psychosis, hemolytic anemia and age at the diagnosis of lupus were independent predictive factors of the use of biological agents. Their global effects are beneficial, with a significant reduction in SLE activity and a low rate of side effects.

Pragmatic, open-label, single-center, randomized, phase II clinical trial to evaluate the efficacy and safety of methylprednisolone pulses and tacrolimus in patients with severe pneumonia secondary to COVID-19: The TACROVID trial protocol.

INTRODUCTION: Some COVID-19 patients evolve to severe lung injury and systemic hyperinflammatory syndrome triggered by both the coronavirus infection and the subsequent host-immune response. Accordingly, the use of immunomodulatory agents has been suggested but still remains controversial. Our working hypothesis is that methylprednisolone pulses and tacrolimus may be an effective and safety drug combination for treating severe COVID-19 patients. METHODS: and analysis: TACROVID is a randomized, open-label, single-center, phase II trial to evaluate the efficacy and safety of methylprednisolone pulses and tacrolimus plus standard of care (SoC) versus SoC alone, in patients at advanced stage of COVID-19 disease with lung injury and systemic hyperinflammatory response. Patients are randomly assigned (1:1) to one of two arms (42 patients in each group). The primary aim is to assess the time to clinical stability after initiating randomization. Clinical stability is defined as body temperature ≤37.5 °C, and PaO2/FiO2 > 400 and/or SatO2/FiO2 > 300, and respiratory rate ≤24 rpm; for 48 consecutive hours. DISCUSSION: Methylprednisolone and tacrolimus might be beneficial to treat those COVID-19 patients progressing into severe pulmonary failure and systemic hyperinflammatory syndrome. The rationale for its use is the fast effect of methylprednisolone pulses and the ability of tacrolimus to inhibit both the CoV-2 replication and the secondary cytokine storm. Interestingly, both drugs are low-cost and can be manufactured on a large scale; thus, if effective and safe, a large number of patients could be treated in developed and developing countries. TRIAL REGISTRATION NUMBER: NCT04341038 / EudraCT: 2020-001445-39.

[Sleep-related movement and behavioural disorders in adults]. / Trastornos del movimiento y de la conducta durante el sueño en el adulto.

Sleep-related movement and behaviour disorders may have an impact on sleep quality and lead to daytime symptoms. These groups of conditions include diseases such as restless legs syndrome, periodic leg movements, and REM and NREM parasomnias. The knowledge of their clinical features and management is of utmost importance for the neurologist and sleep specialist. Frequently, these patients are referred to such specialists and it is relevant to know that certain sleep disorders may be associated with other neurological conditions.

TITLE: Trastornos del movimiento y de la conducta durante el sueño en el adulto.Los trastornos del movimiento y de la conducta durante el sueño pueden tener un impacto en la calidad del sueño del paciente y dar lugar a síntomas diurnos. En estos grupos de enfermedades se incluyen entidades como el síndrome de piernas inquietas, los movimientos periódicos de las piernas y las parasomnias del sueño de movimientos oculares rápidos (REM) y no REM. El conocimiento de sus características clínicas y nociones sobre su manejo es de gran importancia para el neurólogo y especialista en sueño por su frecuencia e impacto en la calidad del sujeto. Con frecuencia, estos pacientes son referidos a dichos especialistas, y es relevante conocer que ciertos trastornos del sueño pueden asociarse a otras enfermedades neurológicas.

Infections in newly diagnosed Spanish patients with systemic lupus erythematosus: data from the RELES cohort.

OBJECTIVES: Using data of patients from the inception cohort Registro Español de Lupus Eritematoso Sistémico (RELES), we aimed to analyse the incidence of severe infection in the first two years of follow-up and how predictors of infection change during the course of systemic lupus erythematosus (SLE). MATERIAL AND METHODS: The study included 282 patients. Markers of lupus activity, prednisone doses and immunosuppressive therapy were compared between patients with and without infections in the first and second year of the disease. Drug therapy administered during the first month of follow-up has been considered as a potential predictor of infections during the first year and medications administered during the first year have been considered potential predictors of infections during the second. RESULTS: Nineteen patients (6.4%) had a documented episode of major infection during the first year of follow-up and 16 patients (5.67%) during the second. The following variables were associated with infections during the first year: hypocomplementaemia at diagnosis ( p < 0.01), nephritis at diagnosis ( p = 0.03), SLEDAI score ( p < 0.01), prednisone >30 mg/day ( p = 0.01), methylprednisolone pulses ( p = 0.05) and mycophenolate use ( p = 0.02). The independent variables in the final model were hypocomplementaemia (odds ratio (OR) 4.41, 95% confidence interval (CI) 0.96-20.20, p = 0.05) and a dose of prednisone >30 mg/day (OR 6.60, 95% CI 1.34-32.42, p = 0.02). The following variables were associated with infections during the second year: dose of prednisone > 7.5 mg/day ( p = 0.05), methylprednisolone pulses ( p = 0.07), duration of therapy with antimalarials ( p = 0.09), therapy with mycophenolate ( p = 0.01), therapy with cyclophosphamide ( p = 0.05). The independent variables in the final model were a dose of prednisone >7.5 mg/day (OR 4.52, 95% CI 0.99-21, p = 0.054) and duration of therapy with antimalarials as a protective factor (OR 0.99, 95% CI 0.99-1.00, p = 0.053). CONCLUSIONS: The low incidence of early infections in the RELES cohort is partially explained by the extended use of antimalarials and by the general avoidance of prolonged high doses of prednisone. Patients with high baseline activity are at a higher risk of infection during the first months but therapy with medium-high doses of prednisone is the main predictor of infectious events. Thus, every effort should be made to limit oral glucocorticoid use from the very beginning of the SLE course.

[FOXG1, a new gene responsible for the congenital form of Rett syndrome]. / FOXG1, un nuevo gen responsable de la forma congenita del sindrome de Rett.

INTRODUCTION: Rett syndrome (RS) is a neurodevelopmental disorder that affects girls almost exclusively. The identification of mutations in the MECP2 and CDKL5 genes offers genetic confirmation of the clinical diagnosis. The FOXG1 gene appears to be a novel cause of the congenital variant of RS. CASE REPORT: We describe the first Spanish patient with the atypical (congenital) variant of RS with mutation of the FOXG1 gene and the case is compared with 12 patients previously reported in the literature; clinical criteria that suggest alterations in FOXG1 are proposed. The patient was referred at the age of 6 months due to overall retardation, axial hypotonia, microcephaly and a peculiar phenotype. Magnetic resonance imaging of the brain revealed hypoplasia of the corpus callosum, frontal atrophy and ventriculomegaly. The appearance of hand-to-mouth stereotypic movements at 12 months pointed the clinical diagnosis towards an atypical variant of RS, the congenital form; there was progressive improvement of visual contact and interest in her surroundings. Frequent respiratory infections and obstructive sleep apnoea syndrome. At the age of 5 years there was partial control over the axial tone, grasping with the hands, good contact and babbling, without epilepsy or behavioural disorders. The MECP2 and subtelomeric deletion study did not reveal any alterations; two polymorphisms were identified in the CDKL5 gene and a pathogenic mutation was found in FOXG1 (c.624C>G p.Tyr203X). CONCLUSIONS: It has been shown that 92% of patients with mutations in the FOXG1 gene present the congenital form of RS with severe generalised hypotonia, early acquired microcephaly (-3 to -6 standard deviations) and peculiar phenotype. When faced with a diagnosis of RS with no alterations in the MECP2 and CDKL5 genes, especially in the case of the congenital variant, the FOXG1 gene must be investigated. The molecular diagnosis confirms the clinical diagnosis and provides the family with genetic counselling.

Lupus pernio. Presentación de una serie de 8 pacientes / Lupus pernio. A report of a series of 8 patients

Objetivos. Aunque el lupus pernio (LP) es la lesión cutánea más característica de la sarcoidosis crónica, en nuestro país se han comunicado muy pocos casos. El objetivo del estudio fue revisar la frecuencia y características clínicas de los pacientes con LP en una serie amplia de pacientes con sarcoidosis. Métodos. Revisión retrospectiva de la frecuencia y características de los pacientes diagnosticados de LP de la serie de sarcoidosis de nuestro centro durante un periodo de 35 años. Resultados. De 507 pacientes con sarcoidosis, 8 (1,6%) presentaron LP. La edad media fue de 42 años. En 6 casos el LP fue la forma de presentación de la sarcoidosis. Cinco pacientes mostraron afectación de la piel nasal y un caso presentó afectación severa de la mucosa nasal. Todos los pacientes fueron tratados con antimaláricos, 4 con corticoides, 2 con láser o con combinaciones con otros fármacos. Resultados. Ningún paciente con afectación cutánea nasal presentó remisión del LP. Conclusiones. El LP es poco frecuente en las formas clínicas de la sarcoidosis de nuestro país. La afectación cutánea nasal no responde al tratamiento. La reciente introducción del infliximab puede representar un avance en el tratamiento del LP(AU)

Objectives. Although lupus pernio (LP) is the most characteristic cutaneous lesion of chronic sarcoidosis, only a few cases have been reported in our country. The aim of this study was to review the frequency and clinical characteristics of patients with LP in a large series of patients with sarcoidosis. Methods. A retrospective review of the frequency and characteristics of patients diagnosed as having LP from the series of sarcoidosis of our institution for a period of 35 years was performed. Results. Eight (1.6%) out of 507 patients with sarcoidosis were diagnosed of LP. Mean age was 42 years. In 6 patients, LP was the presentation form of sarcoidosis. Five patients had involvement of the nasal skin and one patient severe involvement of the nasal mucosa. All the patients were treated with antimalarial drugs, 4 with oral corticosteroids, 2 with laser therapy, or with combinations with other drugs. None of the patient having nasal skin involvement showed remission of LP. Conclusions. LP is a rare clinical form of sarcoidosis in our country. No treatment is effective for nasal skin involvement. The recent introduction of infliximab may represent an advance in the treatment of LP(AU)

[Lupus pernio. A report of a series of 8 patients]. / Lupus pernio. Presentación de una serie de 8 pacientes.

OBJECTIVES: Although lupus pernio (LP) is the most characteristic cutaneous lesion of chronic sarcoidosis, only a few cases have been reported in our country. The aim of this study was to review the frequency and clinical characteristics of patients with LP in a large series of patients with sarcoidosis. METHODS: A retrospective review of the frequency and characteristics of patients diagnosed as having LP from the series of sarcoidosis of our institution for a period of 35 years was performed. RESULTS: Eight (1.6%) out of 507 patients with sarcoidosis were diagnosed of LP. Mean age was 42 years. In 6 patients, LP was the presentation form of sarcoidosis. Five patients had involvement of the nasal skin and one patient severe involvement of the nasal mucosa. All the patients were treated with antimalarial drugs, 4 with oral corticosteroids, 2 with laser therapy, or with combinations with other drugs. None of the patient having nasal skin involvement showed remission of LP. CONCLUSIONS: LP is a rare clinical form of sarcoidosis in our country. No treatment is effective for nasal skin involvement. The recent introduction of infliximab may represent an advance in the treatment of LP.

Myeloid-related protein 8/14 levels in children with obstructive sleep apnoea.

Obstructive sleep apnoea (OSA) is common in children and leads to multiple end-organ morbidities. Myeloid-related protein (MRP) 8/14 plays an important pathophysiological role in atherosclerosis, and plasma levels correlate with endothelial cell dysfunction. We hypothesised that MRP8/14 levels would be altered in children with OSA. 255 children (aged 7.6+/-1.5 yrs) were included after a sleep study and a morning blood sample. MRP8/14 and interleukin-6 plasma levels were assayed using ELISA and C-reactive protein by immunoturbidometry. Endothelial function was assessed as the hyperaemic response after occlusion of the brachial artery. Plasma log MRP8/14 levels showed apnoea/hypopnoea index (AHI) dose-dependent increases regardless of obesity. Moreover, log MRP8/14 levels correlated with log AHI (r = 0.340, p<0.001) after controlling for age and body mass index Z-score, and with endothelial function. Children with the highest MRP levels (>1.34 ug x mL(-1)) had 2.4- and 5.4-fold increased odds of mild OSA and moderate-to-severe OSA, respectively, after adjusting for confounding variables. Plasma MRP8/14 levels are associated with paediatric OSA and may reflect increased risk for cardiovascular morbidity. The short- and long-term consequences of elevated MRP8/14 on cardiovascular function in the context of paediatric OSA remain to be defined.

[Neurobiological consequences of sleep apnea syndrome in children]. / Consecuencias neurobiológicas del síndrome de apnea del sueño infantil.

INTRODUCTION AND DEVELOPMENT: Sleep disorders in general, and more specifically those related to obstructive sleep apnea syndrome (OSAS) in children, are associated with cognitive and behavioural dysfunctions. Both restriction and fragmentation of sleep as well as intermittent hypoxia are involved in the pathophysiological alterations triggered by this neurobiological comorbidity. The mechanisms that eventually give rise to these neurobehavioural disorders appear to involve a number of biological pathways, particularly oxidative stress and systemic inflammation. CONCLUSIONS: The role played by inter-individual susceptibility, together with the environmental conditions and lifestyle, may account for the larger part of the variance in the phenotype. Moreover, the usual clinical prototype of the patient referred to a children's sleep unit due to snoring has evolved a lot in the past 15 years. We have gone from the patient who presents adenotonsillar hypertrophy with no associated obesity (as was the case in the early nineties) to the prototype of a patient who visits our sleep unit with a slight or moderate adenotonsillar hypertrophy, and with an obese biotype that is very similar to that of the adult patient with OSAS. For this reason we therefore propose the use of the terms type I and type II OSAS in children, and their different manifestations and clinical course are discussed.

Consecuencias neurobiológicas del síndrome de apnea del sueño infantil / Neurobiological consequences of sleep apenea syndrome in children

Introducción y desarrollo. Los trastornos de sueño en general, y más específicamente los relacionados con el síndrome de apnea obstructiva del sueño (SAOS) en niños, se presentan asociados a disfunciones cognitivas y conductuales. Tanto la restricción como la fragmentación del sueño y la hipoxia intermitente están involucradas en la fisiopatología que provoca esta comorbilidad neurobiológica. Los mecanismos que acaban por provocar estas alteraciones neuroconductuales parecenimplicar múltiples vías biológicas, especialmente el estrés oxidativo y la inflamación sistémica. Conclusiones. El papel de la susceptibilidad interindividual, sumado a las condiciones medioambientales y de estilo de vida, puede explicar de manera sustancial la varianza en el fenotipo. Además, el prototipo clínico del paciente remitido a una unidad de sueño infantil por ronquido habitual ha evolucionado en los últimos 15 años. Se está pasando del paciente que presenta una hipertrofia adenoamigdalar sin obesidad asociada (tal como ocurría a principio de los años noventa) al prototipo de paciente que acude anuestra unidad de sueño con una ligera o moderada hipertrofia adenoamigdalar, y de biotipo obeso muy similar a la del paciente adulto con SAOS. Por ello, proponemos la nomenclatura de SAOS infantil en tipo I y tipo II, y discutimos sus diferentesmanifestaciones y curso clínico

Introduction and development. Sleep disorders in general, and more specifically those related to obstructive sleep apnea syndrome (OSAS) in children, are associated with cognitive and behavioural dysfunctions. Both restriction and fragmentation of sleep as well as intermittent hypoxia are involved in the pathophysiological alterations triggered by thisneurobiological comorbidity. The mechanisms that eventually give rise to these neurobehavioural disorders appear to involve a number of biological pathways, particularly oxidative stress and systemic inflammation. Conclusions. The role played by inter-individual susceptibility, together with the environmental conditions and lifestyle, may account for the larger part of the variance in the phenotype. Moreover, the usual clinical prototype of the patient referred to a children’s sleep unit due to snoring has evolved a lot in the past 15 years. We have gone from the patient who presents adenotonsillar hypertrophy with noassociated obesity (as was the case in the early nineties) to the prototype of a patient who visits our sleep unit with a slight or moderate adenotonsillar hypertrophy, and with an obese biotype that is very similar to that of the adult patient with OSAS. Forthis reason we therefore propose the use of the terms type I and type II OSAS in children, and their different manifestations and clinical course are discussed

Unidad de Cuidados Intermedios de Medicina Interna / Intermediate Care Unit of Internal Medicine

No disponible

Association between ACE gene I/D polymorphism and clinical presentation and prognosis of sarcoidosis.

Serum angiotensin converting enzyme (SACE) concentration is considered a marker of sarcoidosis activity. This concentration is influenced by an insertion/deletion (I/D) polymorphism of the ACE gene, such that SACE levels follow the pattern DD>ID>II. The aim of our work was to study the relationship between I/D polymorphism and susceptibility to sarcoidosis, as well as the relation between this polymorphism and the clinical presentation and evolution of the disease in 177 sarcoidosis patients. A group of 104 individuals without sarcoidosis was included as control. Genotyping was done by a polymerase chain reaction (PCR) method, and SACE concentration at diagnosis was determined by a kinetic method. No differences were observed in genotype or allele distributions between patients and controls, nor between patients considering the type of presentation (Löfgren versus non-Löfgren) and evolution of the disease (acute versus chronic). As reported for healthy populations, SACE concentrations followed the pattern DD>ID>II in sarcoidosis patients, but significant differences between genotypes existed only in the Löfgren group (p = 0.003) and in acute patients (p = 0.02). SACE concentrations at diagnosis were lower in acute patients (p = 0.05) and in Löfgren's syndrome (p = 0.04), but this seemed to occur only in ID individuals (p = 0.02 and p = 0.01, respectively). No relation was thus found between I/D polymorphism and susceptibility to sarcoidosis, but ACE I/D genotyping may improve the assessment of disease activity, both at diagnosis and during the follow-up of treated and untreated patients.

Aspectos psicoeducativos en el abordaje del trastorno obsesivo-compulsivo en niños y adolescentes: información para padres y maestros / Psycho-educative aspects in child obsesive-compulsive disorder treatment: Parents and teachers information

La terapia cognitivo-conductual ha sido considerada, durante los últimos años, como una de las estrategias terapéuticas, empíricamente comprobadas, para el tratamiento de los trastornos obsesivo compulsivos, tanto en población adulta como infantil. En combinación con este tipo de intervención, debido a las importantes implicaciones psicosociales que presenta este trastorno, se consideran de vital importancia las intervenciones psicoeducativas en el entorno familiar y escolar. En todo este proceso será importante la formación e implicación tanto de los padres como de los maestros, en relación a las características del trastorno y de la terapéutica cognitivo-conductual. En las páginas ulteriores se presentan los dosieres de información que han sido redactados como parte del protocolo de intervención de los trastornos obsesivo-compulsivos en niños y adolescentes (AU)

Estrategias terapéuticas en el tratamiento del TOC infanto-juvenil: aspectos psicosociales / Therapeutic strategies for the child obsessive-compulsive disorder treatment: psycho-social aspects

Entre los tratamientos de primera elección para el trastorno obsesivo-compulsivo, una de las estrategias empíricamente comprobadas tanto en población adulta como infantil es la terapia cognitiva. El tratamiento de este tipo de trastornos requiere una intervención de tipo multidisciplinar, donde en combinación con la terapia cognitivo-conductual, deberemos incorporar actuaciones de tipo psicoeducativo en el entorno familiar y escolar. En este proceso será importante la formación y el trabajo combinado con padres y maestros. En las siguientes páginas se presenta la información que ha sido estructurada, en la Unidad de Paidopsiquiatría del Hospital Materno-Infantil de la Vall d'Hebron en respuesta a las necesidades que presentaba nuestro protocolo de intervención para los trastornos obsesivo-compulsivo en niños y adolescentes (AU)

[Face recognition in autism]. / Reconocimiento de caras en autismo.

INTRODUCTION: We conducted a survey of the literature on face recognition (FR), an activity that is essential for social relations and their dynamics. Unlike the recognition of non facial objects, this type of recognition is a special process since it is based on the detection of individual features. The most characteristic clinical parameter of autistic subjects is their inability to relate socially, possibly due to the difficulty they have in processing faces, although they are more skilled at recognising objects. DEVELOPMENT: We describe the two mechanisms involved in FR, one based on features and the other referring to the whole. The latter can be further divided into overall processing that allows a whole image to be compared with another previously assimilated image, and the processing of the arrangement of a face that is recognised as a whole. These may correspond to two different neuronal pathways. During the first days of life, the newborn baby has a predilection for faces in their feature and overall aspects, and processing of the arrangement is slower. Visual development in autistic children is erratic, similar to the level of a newborn infant, and their lack of interest for human faces is apparent during the first year of life, as they look at everything as if they were objects, that is, by features. CONCLUSIONS: The analysis of the literature enabled us to determine how FR mechanisms develop in the earliest days of the infant s life. It also highlighted the importance of the integrity of the pathway that facilitates stimulation for the recognition of facial arrangement, which is altered in autistic children perhaps from the peripheral area to the cortex. Further work on peripheral pathways and the fundamental cortical connections that are affected in autistic subjects will help us to understand the inefficiency of their facial arrangement recognition system.

Bacteremic pneumococcal cellulitis compared with bacteremic cellulitis caused by Staphylococcus aureus and Streptococcus pyogenes.

In order to better characterize bacteremic cellulitis caused by Streptococcus pneumoniae, a review was conducted of 10 cases of bacteremic pneumococcal cellulitis, which represented 0.9% of all cases of pneumococcal bacteremia (n=1,076) and 3.2% of all cases of community-acquired bacteremic cellulitis (n=312) that occurred in the Hospital de Bellvitge, Barcelona, from 1984 to 2001. In addition to these 10 cases, 28 cases of bacteremic pneumococcal cellulitis from the literature (Medline 1975-2001) were reviewed. Pneumococcal cellulitis of the face, neck, and trunk was observed more frequently in patients with systemic lupus erythematosus and hematologic disorders, while pneumococcal cellulitis of the limbs was more common in patients with diabetes, alcoholism, and parenteral drug use. In the Hospital de Bellvitge group, bacteremic cellulitis due to Streptococcus pneumoniae was more frequently associated with severe underlying diseases than that due to Staphylococcus aureus or Streptococcus pyogenes (100%, 57%, and 72%, respectively;P=0.01). A concomitant extracutaneous focus of infection (e.g., respiratory tract infection) suggesting hematogenous spread with metastatic cellulitis was more frequent in patients with pneumococcal cellulitis, while a local cutaneous entry of microorganisms was feasible in most patients with Staphylococcus aureus or Streptococcus pyogenes cellulitis. The 30-day mortality was 10% in patients with pneumococcal cellulitis, 13% in patients with Staphylococcus aureus cellulitis, and 23% in patients with Streptococcus pyogenes cellulitis (P=0.3). Thus, bacteremic pneumococcal cellulitis is an unusual manifestation of pneumococcal disease and occurs mainly in patients with severe underlying diseases. In most cases, pneumococcal cellulitis has a different pathophysiologic mechanism than cellulitis caused by Staphylococcus aureus or Streptococcus pyogenes.

Reconocimiento de caras en el autismo / Face recognition in autism

Introducción. Se realiza una revisión bibliográfica sobre el reconocimiento de caras (RDC), actividad fundamental para las relaciones sociales y su dinámica. Este tipo de reconocimiento es un proceso especial, en contraste con el reconocimiento de objetos no faciales, pues se basa en la detección de características individuales. El parámetro clínico más característico de los autistas es su imposibilidad para la relación social, posiblemente por su dificultad para el procesamiento de caras, aunque poseen una mayor habilidad en el reconocimiento de objetos. Desarrollo. Se plantean los dos mecanismos en el RDC, el de los rasgos y el de conjunto. El segundo comprende el procesamiento global, que permite comparar la totalidad de la imagen con una imagen previamente asimilada, y el procesamiento de la configuración de la cara reconocida como un todo; ambos pueden corresponder a dos redes neuronales distintas. En los primeros días de vida, el recién nacido tiene preferencia por las caras en sus aspectos de rasgos y global, y es más lento el proceso de la configuración. El desarrollo visual en los niños autistas es errático, como si fuese el nivel de un recién nacido, y su desinterés por las caras humanas es evidente en el primer año de vida, al mirar todo como objetos, por rasgos. Conclusiones. El análisis de la bibliografía ha permitido plantear cómo se desarrollan los mecanismos de lRDC desde los primeros días de vida, y la importancia que desempeña la integridad de la vía que facilita la estimulación para el reconocimiento de la configuración facial, alterada en los niños autistas, posiblemente desde la periferia al córtex. Nuevos trabajos sobre las vías periféricas y las conexiones corticales fundamentales afectadas en autistas ayudarán a comprender la ineficiencia de su sistema de reconocimiento de la configuración facial (AU)

[Cognitive evoked potentials in autistic children]. / Potenciales evocados cognitivos en niños autistas.

AIMS: The aim of this study was to examine the latency, amplitude and distribution of N400 potential in order to evaluate the semantic processing capacity in autistic children and in children suffering from Asperger s syndrome (AS), and to compare them with a control group. PATIENTS AND METHODS: 24 autistic children, six boys with AS and 25 controls, aged between 6 and 14 years old. The cases were examined using the DSM IV diagnostic criteria. Auditory stimulation was performed with pairs of congruent and incongruent words: two lists of 20 pairs of semantically related words (congruent) and 20 pairs of words with no semantic relationship whatsoever (incongruent). RESULTS: The most striking parameter is the increase in latency in N400 for the group of autistic children, which did not occur in the group of children with AS. Maximum N400 negativity for the children with autism was found in the left frontocentral region. No significant differences were observed for the amplitude of N400 between the three groups that were studied. CONCLUSION: Neurophysiologically, the autistic children and those affected by AS perhaps use different neuronal networks in semantic processing. The N400 wave can be a valid test for monitoring verbal processing in these children.