Brain activity in well-controlled perinatally HIV-infected young adults: a fMRI pilot study. / Actividad cerebral en jóvenes infectados por el virus de la inmunodeficiencia humana por transmisión vertical: estudio piloto de resonancia magnética funcional.

INTRODUCTION AND AIM: Perinatal transmission of human immunodeficiency virus (PHIV) is considered a chronic disease that has highlighted several cognitive deficits. From birth to early adulthood, cognition is known to play a fundamental role. However, although neurocognitive processes associated with PHIV have been extensively described by psychometric testing, data is scarce on neural activity from functional magnetic resonance imaging (fMRI) which provides in vivo physiological information. SUBJECTS AND METHODS: We studied described impaired cognitive processes using fMRI on a group of PHIV adolescents with good immunovirological indications and healthy matched controls. Psychological status and neurocognitive functions were also assessed. RESULTS: There were no significant differences between HIV+ and HIV- groups, either on neurocognitive testing nor in fMRI activity for phonological fluency tasks. Prolonged duration of cART was positively associated with greater brain activity in left inferior frontal gyrus (LIFG) which could indicate functional compensation. CONCLUSIONS: These results suggest that neural activity through fMRI in PHIV adolescents with good daily functioning and good immunovirological control may be similar to their peers.

TITLE: Actividad cerebral en jóvenes infectados por el virus de la inmunodeficiencia humana por transmisión vertical: estudio piloto de resonancia magnética funcional.Introducción y objetivos. La infección por el virus de la inmunodeficiencia humana de transmisión vertical (VIH-TV) constituye una enfermedad crónica que puede asociar múltiples alteraciones cognitivas que pueden influenciar el desarrollo de estos pacientes desde la infancia a la vida adulta. Sin embargo, aunque las alteraciones neurocognitivas vinculadas al VIH-TV están ampliamente descritas y valoradas mediante pruebas psicométricas, no existen apenas estudios de actividad neuronal medida a través de la resonancia magnética funcional (RMf). Sujetos y métodos. Analizar la utilidad de la RMf a través de la realización de tareas motoras y de fluidez verbal en un grupo de adolescentes y jóvenes con VIH-TV con buen control inmunovirológico y compararlo con un grupo control negativo de características similares. Se evaluaron también alteraciones psicológicas y funciones neurocognitivas. Resultados. No se encontraron diferencias significativas entre el grupo VIH+ y el grupo control para las tareas ejecutadas durante la RMf ni en la evaluación neurocognitiva. Un mayor tiempo de terapia combinada antirretroviral se asoció de forma directa con una mayor actividad en el giro frontal inferior izquierdo, lo cual podría indicar una posible compensación funcional. Conclusiones. Estos resultados sugieren que la actividad neuronal medida a través de la RMf en adolescentes con VIH-TV y buen control inmunovirológico es similar a la de sus pares.

[Obstetric brachial plexus palsy: incidence, monitoring of progress and prognostic factors]. / Paralisis braquial obstetrica: incidencia, seguimiento evolutivo y factores pronosticos.

INTRODUCTION: Obstetric brachial plexus palsy is related with shoulder dystocia, and its main risk factor is macrosomia. Its incidence is estimated to be between 0.1 and 6.3 cases per 1,000 live newborn infants. Most cases are resolved but can give rise to permanent functional deficiency, which means that there is an interest to identify possible prognostic factors. PATIENTS AND METHODS: We conducted a descriptive study of newborn infants with obstetric brachial plexus palsy born in our hospital between the years 2011 and 2015. Maternal, perinatal and obstetric variables, as well as the type of lesion, were collected and were related with the possibility of recovery at six months. RESULTS: Altogether 32 cases were diagnosed, which represents an incidence of 1.44‰ of live newborn infants. 59% were males and 37.5% of them were macrosomic. The most frequent disorder was injury to the plexus at the proximal level (94%). 44% suffered from shoulder dystocia, and 47% still had sequelae at the sixth month. The antecedent of shoulder dystocia was related with a poor prognosis for recovery. CONCLUSIONS: The incidence of obstetric brachial plexus palsy has remained stable in recent years. The percentage of children who present sequelae at six months is significant. Prospective studies are needed to be able to establish the long-term prognostic factors of this pathology.

TITLE: Paralisis braquial obstetrica: incidencia, seguimiento evolutivo y factores pronosticos.Introduccion. La paralisis braquial obstetrica se relaciona con la distocia de hombros, y su principal factor de riesgo es la macrosomia. Su incidencia se estima entre 0,1 y 6,3 casos por 1.000 recien nacidos vivos. La mayoria de los casos se resuelve, pero puede provocar deficit funcional permanente, por lo que es de interes identificar posibles factores pronosticos. Pacientes y metodos. Estudio descriptivo de los recien nacidos con paralisis del plexo braquial obstetrica nacidos en el hospital entre los años 2011 y 2015. Se han recogido variables maternas, perinatales, obstetricas y del tipo de lesion, y se han relacionado con la posibilidad de la recuperacion a los seis meses. Resultados. Se diagnosticaron 32 casos, lo que supone una incidencia del 1,44‰ de recien nacidos vivos. El 59% fueron varones, y el 37,5%, macrosomicos. La afectacion mas frecuente fue la lesion del plexo a nivel proximal (94%). El 44% sufrio distocia de hombros, y el 47% permanecio con secuelas al sexto mes. El antecedente de distocia de hombros se relaciono con mal pronostico de recuperacion. Conclusiones. La incidencia de paralisis braquial obstetrica se mantiene estable en los ultimos años. El porcentaje de niños que presentan secuelas a los seis meses es relevante. Son necesarios estudios prospectivos para poder establecer los factores pronosticos a largo plazo de esta patologia.

[Language disorders in acute cerebellitis: beyond dysarthria]. / Alteraciones del lenguaje en la cerebelitis aguda: mas alla de la disartria.

INTRODUCTION: Acute cerebellitis is one of the main causes of cerebellar syndrome in infancy. Among the wide range of manifestations, headache and ataxia being the most predominant, we can find other less frequent, although nonetheless interesting, ones, such as language disorders, which go beyond the well-known cerebellar dysarthria. The different combinations in which the symptoms can appear, especially when not accompanied by ataxia, make the condition a real challenge for the clinician. CASE REPORTS: Two patients, aged 2 and 4 years, with clinical features, lab tests and neuroimaging results consistent with parainfectious acute cerebellitis. Both of them also presented a striking language disorder, one in the form of cerebellar mutism and the other in the form of hypofluency and agrammatism, the latter also developing in the absence of ataxia. Both cases progressed favourably, and mild speech alterations persisted in the follow-up visits. CONCLUSIONS: Cases such as these expand the range of clinical manifestations of acute cerebellitis. The involvement of the cerebellum in neurocognitive processes like language is becoming increasingly more important and, although many aspects are still only speculations, managing to define its true role will have important repercussions on the diagnosis, treatment and long-term prognosis of these patients.

TITLE: Alteraciones del lenguaje en la cerebelitis aguda: mas alla de la disartria.Introduccion. La cerebelitis aguda es una de las principales causas de sindrome cerebeloso en la infancia. Entre un amplio elenco de manifestaciones, en el que predominan la cefalea y la ataxia, podemos encontrar otras menos habituales, aunque interesantes, como las alteraciones del lenguaje, mas alla de la bien conocida disartria cerebelosa. Las diferentes combinaciones en que pueden aparecer los sintomas, especialmente cuando no se acompañan de ataxia, hacen de este cuadro un verdadero reto para el clinico. Casos clinicos. Se presentan dos pacientes, de 2 y 4 años, con clinica, pruebas de laboratorio y neuroimagen compatibles con cerebelitis aguda parainfecciosa, que asociaron una llamativa alteracion del lenguaje, uno en forma de mutismo cerebeloso y otro en forma de hipofluencia y agramatismo, y este ultimo cursaba ademas en ausencia de ataxia. La evolucion de ambos casos fue buena, y persistieron leves alteraciones del habla en el seguimiento posterior. Conclusiones. Casos como estos amplian el espectro de manifestaciones clinicas de la cerebelitis aguda. Cada vez cobra mayor importancia la participacion del cerebelo en procesos neurocognitivos como el lenguaje y, aunque muchos aspectos son aun especulativos, alcanzar a definir su verdadero papel tendra una repercusion en el diagnostico, el tratamiento y el pronostico a largo plazo de estos pacientes.

Resonancia magnética sin sedación en recién nacidos / Magnetic resonance imaging without sedation in neonates

INTRODUCCIÓN: La posibilidad de llevar a cabo RM sin sedación en el período neonatal aumenta la seguridad del paciente, la disponibilidad y rentabilidad de la prueba. El objetivo fue describir la experiencia de 20 meses con el nuevo protocolo de RM sin sedación, en el que la preparación del paciente se realiza en la unidad neonatal. Pacientes y método: Estudio descriptivo prospectivo, de mayo del 2012 a diciembre del 2013. Los pacientes incluidos fueron neonatos con indicación de RM, estables y sin soporte ventilatorio. El procedimiento se fundamentó en la aplicación de cuidados centrados en el desarrollo y el uso de un colchón de vacío como sistema de inmovilización. La variable resultado principal fue el porcentaje de RM completadas con éxito. Desde octubre del 2012 se recogieron además la duración de la prueba y el número de interrupciones. RESULTADOS: Se llevaron a cabo 43 RM sin sedación, 41 cerebrales y 2 de columna vertebral. La tasa de éxito fue del 97,7% (42/43). La media de tiempo de RM fue 26,3min (IC del 95%, 23,3-29,3min; rango 16-50min). Se completó la prueba sin interrupciones en 20 de los 34 casos (58,8%) en los que se recogió este dato. La media de interrupciones fue 0,6 (IC del 95%, 0,3-0,8; rango 0-3) y la mediana 0. CONCLUSIONES: El protocolo tuvo una tasa de éxito superior al 90%. Por tanto, la RM sin sedación parece factible en nuestro medio, realizando gran parte de la preparación en la unidad neonatal para así disminuir la ocupación de la sala de RM

INTRODUCTION: The ability to perform magnetic resonance imaging (MRI) without sedation in the neonatal period increases patient safety, availability and profitability of the diagnostic tool. The aim in this study was to evaluate a new protocol of MRI without sedation during a 20-month period. In the protocol, the patients are prepared in the neonatal unit. PATIENTS AND METHOD: Prospective descriptive study, from May 2012 to December 2013. Patients included were neonates requiring MRI, clinically stable and not requiring ventilatory support. The method was based on the application of developmental centered care and the use of a vacuum matress to immobilize the baby. The principal outcome parameter of interest was the percentage of succesfully completed MRIs. The duration of the MRI and the number of interruptions, was also studied from October 2012. RESULTS: A total of 43 MRIs without sedation were carried out on 42 patients: 41 cerebral and 2 spinal. The success rate was 97.7% (42/43). The mean MRI time was 26.3minutes (95% CI 23.3-29.3 mins; range 16-50 mins). MRIs were completed without interruption in 20 of the 34 cases (58%) in which the duration was recorded. The number of interruptions per procedure varied from 0 to 3, with a mean of 0.6 (95% CI 0.3-0.8) and a median of 0. CONCLUSION: The protocol had a success rate of over 90%. Thus MRI without sedation seems applicable in Spanish hospitals, with most of the preparation being performed in the neonatal unit, in order to reduce the occupation of the MRI unit, as well as minimizing stress to the baby

Trombosis venosa cerebral en pacientes oncológicos en tratamiento con L-asparaginasa / Cerebral sinovenous thrombosis in children due to L-asparaginase

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[Magnetic resonance imaging without sedation in neonates]. / Resonancia magnética sin sedación en recién nacidos.

INTRODUCTION: The ability to perform magnetic resonance imaging (MRI) without sedation in the neonatal period increases patient safety, availability and profitability of the diagnostic tool. The aim in this study was to evaluate a new protocol of MRI without sedation during a 20-month period. In the protocol, the patients are prepared in the neonatal unit. PATIENTS AND METHOD: Prospective descriptive study, from May 2012 to December 2013. Patients included were neonates requiring MRI, clinically stable and not requiring ventilatory support. The method was based on the application of developmental centered care and the use of a vacuum matress to immobilize the baby. The principal outcome parameter of interest was the percentage of succesfully completed MRIs. The duration of the MRI and the number of interruptions, was also studied from October 2012. RESULTS: A total of 43 MRIs without sedation were carried out on 42 patients: 41 cerebral and 2 spinal. The success rate was 97.7% (42/43). The mean MRI time was 26.3 minutes (95% CI 23.3-29.3 mins; range 16-50 mins). MRIs were completed without interruption in 20 of the 34 cases (58%) in which the duration was recorded. The number of interruptions per procedure varied from 0 to 3, with a mean of 0.6 (95% CI 0.3-0.8) and a median of 0. CONCLUSION: The protocol had a success rate of over 90%. Thus MRI without sedation seems applicable in Spanish hospitals, with most of the preparation being performed in the neonatal unit, in order to reduce the occupation of the MRI unit, as well as minimizing stress to the baby.

Encefalopatía subaguda por metotrexato, características de la neuroimagen / Methotrexate-induced subacute encephalopathy, neuroimaging characteristics

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Respuesta clínica rápida y espectacular a plasmaféresis en un caso pediátrico de encefalitis anti-NMDA / Fast and spectacular clinical response to plasmapheresis in a paediatric case of anti-NMDA encephalitis

Introducción. La encefalitis autoinmune contra receptores N-metil-D-aspartato (NMDA) se diagnostica cada vez con mayor frecuencia en la edad pediátrica. Debe sospecharse en niños con sintomatología psiquiátrica, encefalopatía, movimientos anormales o crisis epilépticas. Los casos paraneoplásicos son menos frecuentes que en adultos. Caso clínico. Niño de 2,5 años con cuadro encefalopático subagudo que comenzó con crisis epilépticas seguidas de alteraciones del comportamiento, regresión neurológica, discinesias e insomnio. El estudio del líquido cefalorraquídeo fue normal, en la resonancia magnética craneal existía una lesión periventricular focal y captación leptomeníngea difusa y los electroencefalogramas seriados revelaron una actividad delta de gran amplitud intercalada con actividad epileptiforme intercrítica generalizada. Recibió tratamiento empírico con altas dosis de corticoides e inmunoglobulinas intravenosas sin respuesta. Tras demostrarse la positividad de anticuerpos contra el receptor NMDA se inició la plasmaféresis, con la que experimentó una mejoría rápida y espectacular. Tras más de 18 meses de seguimiento, sus secuelas se limitan a leves alteraciones conductuales y del lenguaje. No ha presentado recaídas ni ha precisado ningún tratamiento de mantenimiento. Conclusiones. La encefalitis anti-NMDA es un trastorno tratable y, ocasionalmente, el primer indicio de una neoplasia subyacente, por lo que su reconocimiento y tratamiento precoz es fundamental. El tratamiento de las formas no paraneoplásicas se basa en la inmunoterapia: glucocorticoides, inmunoglobulinas intravenosas, plasmaféresis e inmunosupresores. La plasmaféresis puede inducir una mejoría rápida y espectacular (AU)

Introduction. Autoimmune encephalitis against N-methyl-D-aspartate (NMDA) receptors is being diagnosed more and more frequently in the paediatric age. It should be suspected in children with psychiatric symptoms, encephalopathy, abnormal movements or epileptic seizures. Paraneoplastic cases are less frequent than in adults. Case report. We report the case of a boy, 2.5 years of age, with subacute encephalopathic signs and symptoms and epileptic seizures followed by behaviour disorders, neurological regression, dyskinesias and insomnia. Results of a cerebrospinal fluid study were normal, the magnetic resonance scan of the head revealed a focal periventricular lesion and diffuse leptomeningeal uptake; moreover, the serial electroencephalograms showed high-amplitude delta activity interspersed with generalised intercritical epileptiform activity. The patient was given empirical treatment with high doses of corticoids and intravenous immunoglobulins with no response. After showing up positive for antibodies against the NMDA receptor, plasmapheresis was begun, which led to his swift and spectacular recovery. After more than 18 months’ follow-up, his sequelae are limited to mild behavioural and language alterations. He has had no relapses and has not needed any kind of maintenance treatment. Conclusions. Anti-NMDA encephalitis is a treatable disorder and, sometimes, the first evidence of an underlying neoplasia, which makes its early recognition and treatment essential. Treatment of the non-paraneoplastic forms are based on immunotherapy: glucocorticoids, intravenous immunoglobulins, plasmapheresis and immunosuppressants. Plasmapheresis can bring about a fast, spectacular improvement (AU)

[Fast and spectacular clinical response to plasmapheresis in a paediatric case of anti-NMDA encephalitis]. / Respuesta clinica rapida y espectacular a plasmaferesis en un caso pediatrico de encefalitis anti-NMDA.

INTRODUCTION: Autoimmune encephalitis against N-methyl-D-aspartate (NMDA) receptors is being diagnosed more and more frequently in the paediatric age. It should be suspected in children with psychiatric symptoms, encephalopathy, abnormal movements or epileptic seizures. Paraneoplastic cases are less frequent than in adults. CASE REPORT: We report the case of a boy, 2.5 years of age, with subacute encephalopathic signs and symptoms and epileptic seizures followed by behaviour disorders, neurological regression, dyskinesias and insomnia. Results of a cerebrospinal fluid study were normal, the magnetic resonance scan of the head revealed a focal periventricular lesion and diffuse leptomeningeal uptake; moreover, the serial electroencephalograms showed high-amplitude delta activity interspersed with generalised intercritical epileptiform activity. The patient was given empirical treatment with high doses of corticoids and intravenous immunoglobulins with no response. After showing up positive for antibodies against the NMDA receptor, plasmapheresis was begun, which led to his swift and spectacular recovery. After more than 18 months' follow-up, his sequelae are limited to mild behavioural and language alterations. He has had no relapses and has not needed any kind of maintenance treatment. CONCLUSIONS: Anti-NMDA encephalitis is a treatable disorder and, sometimes, the first evidence of an underlying neoplasia, which makes its early recognition and treatment essential. Treatment of the non-paraneoplastic forms are based on immunotherapy: glucocorticoids, intravenous immunoglobulins, plasmapheresis and immunosuppressants. Plasmapheresis can bring about a fast, spectacular improvement.

[Parkinsonism and Camptocormia with focal spinal myopathy: case report and responsiveness to treatment]. / Parkinsonismo y camptocormia con miopatia focal espinal: descripcion de un caso y respuesta al tratamiento.

INTRODUCTION: Camptocormia is characterised by extreme flexion of the thoracolumbar spine. It suffered an increase during walking and it is relieved in supine position. Camptocormia has been described in psychogenic disorders, but in other diseases, including Parkinson's disease as well. It has been recently described several cases with focal spinal myopathy, and we present a patient with this clinical association. CASE REPORT: This 82-year-old man had a 6-year history of parkinsonian symptoms, mostly of rigid-akinetic type. He was in stage 4 on Hoehn & Yahr scale, and he had reached 62 points on Unified Parkinson Disease Rating Scale. Over the past 6-8 months, he developed progressive forward flexion of the trunk with clinical features of camptocormia. He suffered flogotic symptoms and signs on her lower back, and there were no dystonic posture or clinical features. Lumbar computerised tomography showed fat replacement of the paravertebral L3 muscles. A surgical paravertebral muscle examination and biopsy were performed, showing diffuse fat replacement and only a marginal myopathic focus. It was made several therapeutic approaches, with levodopa dose increase, reduction, fractioning, and addition of dopa-agonists. All of these strategies failed. It was determined to try a steroid course, but there were no improvement, so physiotherapy and rehabilitation measures did. He finally was confined to wheelchair. CONCLUSIONS: Physiopathologic and therapeutic aspects of camptocormia in Parkinson's disease are unclear. Their relationship could be casual or causal. Patients with clear inflammatory myopathy could benefit from steroid therapy, but patients with end-stage myopathy probably do not so.

Parkinsonismo y camptocormia con miopatía focal espinal: descripción de un caso y respuesta al tratamiento / Parkinsonism and camptocormia with focal spinal myopathy: case report and responsiveness to treatment

Introducción. La camptocormia es un trastorno caracterizadopor una postura anormal en flexión pronunciada del tronco,que desaparece al tumbarse y empeora al caminar; es de etiologíadiversa. Desde las consideraciones iniciales como trastorno psicogénico,se reconoce ya como un trastorno postural característicode la enfermedad de Parkinson, y se han descrito algunos casos enrelación con miopatías focales lumbares. Presentamos un pacientecon esta asociación clínica. Caso clínico. Varón de 82 años conparkinsonismo rígido-acinético de seis años de evolución, en estadio4 de la escala de Hoehn y Yahr, y 62 puntos en la Unified ParkinsonDisease Rating Scale. En 6-8 meses, desarrolló progresivamenteuna camptocormia dolorosa, sin datos de distonía asociados,y con signos flogóticos dorsolumbares. La tomografía axialcomputarizada lumbar mostró atrofia de los músculos espinales ysustitución grasa. La biopsia de músculos paraespinales mostródegeneración grasa y algún foco miopático marginal. Tras modificacionesinfructuosas de su medicación dopaminérgica (incremento,disminución, fraccionamiento, agonistas dopaminérgicos), serealizó ciclo de tratamiento esteroideo por vía oral, sin observarsemejoría alguna. Tampoco mejoró con rehabilitación o fisioterapia,y quedó confinado a una silla de ruedas. Conclusiones. Los aspectosfisiopatológicos y terapéuticos en la camptocormia con miopatíafocal asociada a la enfermedad de Parkinson son poco claros, ypueden relacionarse de modo causal o casual. Los pacientes conclara miopatía inflamatoria podrían beneficiarse de terapia esteroideaprecozmente, pero no aquellos con estadios finales de miopatíafocal espinal

Introduction. Camptocormia is characterised by extreme flexion of the thoracolumbar spine. It suffered an increaseduring walking and it is relieved in supine position. Camptocormia has been described in psychogenic disorders, but in otherdiseases, including Parkinson’s disease as well. It has been recently described several cases with focal spinal myopathy, andwe present a patient with this clinical association. Case report. This 82-year-old man had a 6-year history of parkinsoniansymptoms, mostly of rigid-akinetic type. He was in stage 4 on Hoehn & Yahr scale, and he had reached 62 points on UnifiedParkinson Disease Rating Scale. Over the past 6-8 months, he developed progressive forward flexion of the trunk with clinicalfeatures of camptocormia. He suffered flogotic symptoms and signs on her lower back, and there were no dystonic posture orclinical features. Lumbar computerised tomography showed fat replacement of the paravertebral L3 muscles. A surgicalparavertebral muscle examination and biopsy were performed, showing diffuse fat replacement and only a marginalmyopathic focus. It was made several therapeutic approaches, with levodopa dose increase, reduction, fractioning, and additionof dopa-agonists. All of these strategies failed. It was determined to try a steroid course, but there were no improvement, sophysiotherapy and rehabilitation measures did. He finally was confined to wheelchair. Conclusions. Physiopathologic andtherapeutic aspects of camptocormia in Parkinson’s disease are unclear. Their relationship could be casual or causal. Patientswith clear inflammatory myopathy could benefit from steroid therapy, but patients with end-stage myopathy probably do not so