Has clinical activity in paediatric neurology changed in the past 11 years? / ¿Ha cambiado la actividad asistencial de la neurología pediátrica en 11 años?

INTRODUCTION: We believe that the demand for paediatric neurology (PN) care has increased over the past decade, and that reasons for requesting consultations have also changed. The objective of this study is to complete a registry study to profile the demand for PN care in 2013 and compare results to those from a study performed in 2002. METHODS: A prospective registry of PN healthcare activities was completed at Hospital Universitario de Getafe in 2013. Results were compared with those from a prospective registry study conducted in 2002. RESULTS: The number of visits increased from 1,300 in 2002 to 1,982 in 2013 (a 52.46% increase), and from 32.6 visits per 1,000 children to 57.48 (a 76.32% increase). Outpatient consultations accounted for 92.2% of all PN consultations in 2013. Currently, attention deficit-hyperactivity disorder (ADHD) is the most frequent diagnosis (27.6% in 2013 vs. 8.1% in 2002). Although the percentage of headache consultations has decreased (19% in 2013 vs. 22% in 2002), headache was still the most common reason for an initial visit in 2013 (32.1%), followed by ADHD (19.1%). Epilepsy remains the most frequent diagnosis in hospitalised patients (30.3% in 2013 vs. 36.7% in 2002). CONCLUSIONS: PN is fundamentally an outpatient activity that has increased considerably in recent years. This increase is mainly due to neurodevelopmental disorders, especially ADHD. We might state that the role of ADHD in PN is comparable to that of dementia in general neurology.

Estatus con alteraciones periictales en resonancia magnética cerebral versus encefalitis / Status with periictal changes in cerebral magnetic resonance versus encephalitis

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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.

[Treatment of hydrocephalus secondary to intraventricular haemorrhage in preterm infants. A review of the literature]. / Tratamiento de la hidrocefalia secundaria a hemorragia intraventricular en el prematuro. Revisión bibliográfica.

INTRODUCTION: The current incidence of intraventricular haemorrhage in low-weight preterm infants is 20%. The percentage of patients who are going to develop hydrocephalus secondary to this haemorrhage varies greatly. In the last 20 years different medical and surgical treatments have been put forward to prevent both the occurrence of haemorrhage and the development of hydrocephalus. DEVELOPMENT: In this study we review the literature on the different treatments used to control and treat hydrocephalus. From the medical point of view, treatment with pharmacological agents such as azetazolamide or furosemide offers more drawbacks than advantages as far as their capacity to prevent hydrocephalus is concerned. Treatments involving fibrinolytic agents offer a high risk of triggering new haemorrhages but in recent years their use has been taken up again in combination with ventricular drains. As regards surgical treatment of hydrocephalus due to haemorrhage, despite the risk of infection, it is more advisable to use ventricular drains than to introduce subgaleal reservoirs. At the present time, the best definitive treatment for hydrocephalus in preterm infants is still the ventriculoperitoneal shunt. The most suitable time is when the weight of the newborn infant exceeds 1500 g and the cerebrospinal fluid offers a protein count above 200 mg/dL. CONCLUSIONS: The long-term neurological development of these children depends mainly on the severity of the haemorrhage, but poorer prognoses are observed in cases in which complications arise from the control examinations of the valve shunt systems.

Tratamiento de la hidrocefalia secundaria a hemorragia intraventricular en el prematuro. Revisión bibliográfica / Treatment of hydrocephalus secondary to intraventricular haemorrhage in preterm infants. A review of the literature

Introducción. La hemorragia intraventricular en los prematuros de poco peso tiene una incidencia actual de un 20%. El porcentaje de pacientes que van a desarrollar hidrocefalia secundaria a esta hemorragia es muy variable. En los últimos 20 años se han descrito diferentes tratamientos médicos y quirúrgicos para evitar que se produzca la hemorragia y que se desarrolle una hidrocefalia. Desarrollo. En este trabajo se ha realizado una revisión bibliográfica de los diferentes tratamientos para controlar y tratar la hidrocefalia. Desde el punto de vista médico se ha comprobado que los tratamientos con fármacos como la acetazolamida o furosemida presentan más desventajas que beneficios en cuanto a la capacidad para prevenir la hidrocefalia. Los tratamientos con fibrinolíticos tienen un alto riesgo de provocar nuevas hemorragias, pero en los últimos años se ha reiniciado su utilización combinada con drenajes ventriculares. En cuanto al tratamiento quirúrgico de la hidrocefalia por la hemorragia, a pesar del riesgo de infección, es más aconsejable el drenaje ventricular que la colocación de reservorios subgaleales. En el momento actual, el mejor tratamiento definitivo para la hidrocefalia del prematuro continua siendo la derivación ventriculoperitoneal. El momento más oportuno es cuando el neonato presente un peso superior a 1.500 g y el líquido cefalorraquídeo tenga una cifra de proteínas inferior a 200 mg/dL. Conclusión. El desarrollo neurológico a largo plazo de estos niños depende principalmente de la gravedad de la hemorragia, pero se observa un peor pronóstico en aquellos casos en los que existen complicaciones derivadas de las revisiones de los sistemas derivativos valvulares

Introduction. The current incidence of intraventricular haemorrhage in low-weight preterm infants is 20%. The percentage of patients who are going to develop hydrocephalus secondary to this haemorrhage varies greatly. In the last 20 years different medical and surgical treatments have been put forward to prevent both the occurrence of haemorrhage and the development of hydrocephalus. Development. In this study we review the literature on the different treatments used to control and treat hydrocephalus. From the medical point of view, treatment with pharmacological agents such as azetazolamide or furosemide offers more drawbacks than advantages as far as their capacity to prevent hydrocephalus is concerned. Treatments involving fibrinolytic agents offer a high risk of triggering new haemorrhages but in recent years their use has been taken up again in combination with ventricular drains. As regards surgical treatment of hydrocephalus due to haemorrhage, despite the risk of infection, it is more advisable to use ventricular drains than to introduce subgaleal reservoirs. At the present time, the best definitive treatment for hydrocephalus in preterm infants is still the ventriculoperitoneal shunt. The most suitable time is when the weight of the newborn infant exceeds 1500 g and the cerebrospinal fluid offers a protein count above 200 mg/dL. Conclusions. The long-term neurological development of these children depends mainly on the severity of the haemorrhage, but poorer prognoses are observed in cases in which complications arise from the control examinations of the valve shunt systems

[Variability in the treatment of headaches in visits to the Neuropaediatrics service at six Spanish hospitals]. / Variabilidad en el tratamiento de las cefaleas en las consultas de Neuropediatría de seis hospitales españoles.

INTRODUCTION: Headache is one of the chief reasons for visiting Neuropaediatric units. Variability in clinical practice has been defined as variations in the rates a clinical procedure is employed. The aim of this work is to study the variability in the treatment of children's headaches in six Spanish hospitals. PATIENTS AND METHODS: We conducted a retrospective study involving patients who first visited because of a headache some time in the year 1999. Data were collected in December 2003. The following variables were analysed: type of headache, neuroimaging scans, beginning of prophylaxis, follow-up visits to clinic and number of patients who dropped out of the follow-up. RESULTS: In all, 372 children were attended. 63.4% of the headaches were considered as being migraine-type; 43% of the patients were submitted to neuroimaging and prophylaxis was established in 36.8%. Mean follow-up time in the clinic was 10.6 months and the mean number of visits was 3. 30% had dropped out of the follow-up. In the comparative analysis, significant differences were found in all the variables studied. CONCLUSIONS: Overall results are similar to those found in other series. The differences found between centres can be accounted for by differences in population, differences in health professionals' styles of medical practice or by the nonexistence of clear guidelines regarding a particular procedure.

Variabilidad en el tratamiento de las cefaleas en las consultas de Neuropediatría de seis hospitales españoles / Variability in the treatment of headaches in visits to the neuropaediatrics service at six spanish hospitals

Introducción. La cefalea es una de las principales causasde consulta en las unidades de Neuropediatría. La variabilidad en lapráctica médica se ha definido como la variaciónes en las tasas deun procedimiento clínico. El objetivo de este trabajo es estudiar lavariabilidad en el manejo de las cefaleas infantiles entre seis hospitalesespañoles. Pacientes y métodos. Se trata de un trabajo retrospectivoen el que se incluyen aquellos pacientes que acudieron a laconsulta debido a una cefalea por primera vez durante el año 1999.La recogida de datos se realizó en diciembre de 2003. Se analizaronlas siguientes variables: tipo de cefalea, realización de neuroimagen,inicio de profilaxis, tiempo de seguimiento en consultas y número depacientes que abandonaron el seguimiento. Resultados. Se atendieron372 niños. Un 63,4% de las cefaleas se consideró migrañosa; aun 43% de los pacientes se les practicó neuroimagen y se inició profilaxisa un 36,8%. La media de seguimiento en consultas fue de 10,6meses, y el número medio de visitas fue de tres. Un 30% había abandonadoel seguimiento. En el análisis comparativo, se han encontradodiferencias significativas en todas las variables estudiadas. Conclusiones.Los resultados globales son similares a los encontrados enotras series. Las diferencias encontradas entre los centros puedeninterpretarse como debidas a las diferencias poblacionales, por losdiferentes estilos de práctica médica de los profesionales, o bien porla inexistencia de pautas claras con respecto a un determinado procedimiento

Introduction. Headache is one of the chief reasons for visiting Neuropaediatric units. Variability in clinical practicehas been defined as variations in the rates a clinical procedure is employed. The aim of this work is to study the variability inthe treatment of children’s headaches in six Spanish hospitals. Patients and methods. We conducted a retrospective studyinvolving patients who first visited because of a headache some time in the year 1999. Data were collected in December 2003.The following variables were analysed: type of headache, neuroimaging scans, beginning of prophylaxis, follow-up visits toclinic and number of patients who dropped out of the follow-up. Results. In all, 372 children were attended. 63.4% of theheadaches were considered as being migraine-type; 43% of the patients were submitted to neuroimaging and prophylaxis wasestablished in 36.8%. Mean follow-up time in the clinic was 10.6 months and the mean number of visits was 3. 30% haddropped out of the follow-up. In the comparative analysis, significant differences were found in all the variables studied.Conclusions. Overall results are similar to those found in other series. The differences found between centres can beaccounted for by differences in population, differences in health professionals’ styles of medical practice or by the inexistenceof clear guidelines regarding a particular procedure

[Paediatric neurology health care activity in a tertiary hospital. A study comparing it with adult neurology and non-neurological paediatrics]. / Actividad asistencial de la neurología pediátrica en un hospital de nivel 3. Estudio comparativo con la neurología de adultos y la pediatría no neurológica.

INTRODUCTION: Paediatric neurology (PN) remains 'hidden' somewhere between paediatrics and neurology in almost all health care centres and this makes it difficult to allocate resources in a proper manner. AIMS: Our objective in this study was to analyse PN health care activity and compare it with adult neurology (AN) and with non-neurological paediatrics (NNP). MATERIAL AND METHODS: PN health care activity registry for the year 2002. AN and NNP data were collected from the hospital's Computer Service. RESULTS: In all, 1300 PN visits were made, 428 of which were new and 872 successive, which represent 16.2% of paediatric visits (30% new, 13.3% successive) and 10.3% of neurological visits (12.4% new, 10.2% successive). The rates of new and successive visits in PN are: 32.6, 10.7 and 21.9 per 1000 children, and in AN: 44.2, 12.5 and 31.7 per 1000 adults. A total number of 94 hospitalised children were attended, 3.3% of all paediatric admissions and 9.2% of the total neurological attention in inpatients. The rates are 2.36 per 1000 children in PN and 3.9 per 1000 adults in AN. There is greater demand among smaller children. The most frequent pathologies are headaches in clinical visits and epilepsy in hospitalised patients. CONCLUSIONS: PN is essentially a service used by outpatients. It accounts for a high percentage of hospital paediatric visits. The activity rates per 1000 children and per 1000 adults are similar. The increased birth rate raises PN activity to a level that is higher than would normally be desirable, due to the growth in the population.

[The present state of paediatric neurology in the Autonomous Community of Madrid: variability in the distribution of resources]. / Situacion de la neurologia pediatrica en la Comunidad Autonoma de Madrid: variabilidad en la distribucion de los recursos.

INTRODUCTION: Paediatric neurology (PN) lies halfway between neurology and paediatrics, with no official acknowledgment. It is therefore difficult to determine exactly how resources are shared out. AIMS: The aim of this study is to analyse the public resources devoted to PN in the different health care areas within the Autonomous Community of Madrid (ACM). MATERIALS AND METHODS: In May 2002 we carried out a survey among child neurologists (CN) from the 9 independent health care districts within the ACM. RESULTS: A total of 28 CN work for the public health service in the ACM, eight of whom are employed on a part time basis. Only 53% are owners. The ratio of CN per 100000 inhabitants (inhab.) is 0.55 (0.45 after correction for part time work). The figure varies from one area to another from 0.07 to 1 CN/100000 inhab. (1/12000 1/220000 children). Part time neurologists work in the outer districts of the ACM. In most areas it is difficult to carry out diagnostic tests and these become even complicated when the child is small and requires some anaesthetic procedure. Neuropsychological assessment is one of the basic evaluations performed in PN and this is not easily performed in most centres. CONCLUSIONS: The CN/100000 inhab. ratio in the ACM is lower than that commonly recommended. The distribution of resources varies greatly and does not match demographic criteria. Temporary and part time jobs are commonplace in the peripheral areas. It is difficult for most centres to perform diagnostic tests.

[Assessing quality of life in Parkinson's disease using the PDQ-39. A pilot study]. / Valoración de la calidad de vida en la enfermedad de Parkinson mediante el PDQ-39. Estudio piloto.

The aim of this pilot study was to analyze an adaptation of the PDQ-39, a Parkinson's disease (PD) quality of life questionnaire, for use in Spanish. Fifty patients were enrolled. Patient characteristics and medical histories were recorded, including scores on the following scales: Hoehn and Yahr (HY), PD unified (UPDRS), Schwab and England (SE), Intermediate Scale for Assessment of PD (ISAPD). Barthel Index (BI), Pfeiffer's SPMSQ, Global Deterioration Scale (GDS), Geriatric Depression Scale (GDS). Hospital Depression and Anxiety Scale (HDA) and the PDQ-39. Descríptive statistics were recorded, as well as Spearman's r and ANOVA results. The dimensions that correlated well with PD scale scores were mobility, daily life activities and cognitive deterioration. The dimension stigma correlated with complications on the UPDRS and the ISAPD. Emotional well-being correlated with subscale I of the UPDRS, the GDS and the HDA (r = 0.39-0.79, p < 0.01-0.001). Physical discomfort correlated only with depression and anxiety. Stage of disease and level of depression influenced most dimensions assessed by the PDQ-39. Some psychosocial factors that are important components of well-being are scarcely reflected by the clinical scales usually applied.

Intermediate scale for assessment of Parkinson's disease. Characteristics and structure.

The present study is devoted to the verification of the basic metrical characteristics of the ISAPD. One hundred and sixty-seven Parkinson's disease (PD) patients were included. Group A (n = 40) was simultaneously assessed by five raters who applied the ISAPD and other PD rating scales (PDRS). A set of timed tests, the MiniMental State Examination (MMSE) and the Hamilton Scale for Depression (HSD) were administered by an independent examiner. Group B (n = 127) was individually assessed through the UPDRS and the other PDRSs by separate neurologists in four different hospitals. The ISAPD was administered in 7.0 +/- 3.7 min. The internal consistency of this scale was high (Cronbach's alpha = 0.97). The inter-rater reliability of their items was very satisfactory (for all items, kappa > 0.70). There was a high correlation with the Hoehn and Yahr classification (r(s) = 0.71; p < 0.001) and some timed tests. The convergent validity with the other PDRS (UPDRS and Schwab and England Scale) was also very high (r(s) = 0.83-0.92; p < 0.001). The ISAPD also correlated with the MMSE and the HSD. Factor analysis identified three factors (activities of daily living; gait and mobility; speech and eating) that explained 76% of the variance. The ISAPD is an easy to apply, reliable, and valid scale that fulfills the aim for which it was designed.