Outbreak of Enterovirus Infection with Neurological Presentations in a Pediatric Population in Northern Spain: A Clinical Observational Study.

OBJECTIVE: The study aimed to describe the cases of neurological disease related to the outbreak of enterovirus (EV) in three regions in Northern Spain during 2016. MATERIALS AND METHODS: Multicenter retrospective observational study. Clinical, radiological, and microbiological data were analyzed from patients younger than 15 years with confirmed EV-associated neurological disease admitted to 10 hospitals of Asturias, Cantabria, and Castile and Leon between January 1 and December 31, 2016. RESULTS: Fifty-five patients were included. Median age was 24 months (interquartile range = 18.5 months). Fifteen patients were classified as aseptic meningitis (27.3%). In total, 37 cases presented brainstem encephalitis (67.3%), 25 of them due to EV-A71 with excellent prognosis (84.6% asymptomatic 2 months following the onset). Three cases of acute flaccid myelitis (5.5%) by EV-D68 were reported and presented persistent paresis 2 months following the onset. Microbiological diagnosis by reverse transcriptase polymerase chain reaction was performed in all cases, finding EV in cerebrospinal fluid in meningitis, but not in brainstem encephalitis and acute flaccid myelitis, where EV was found in respiratory or rectal samples. Step therapy was administrated with intravenous immunoglobulin (IVIG; 32.7%), methylprednisolone (10%), and plasmapheresis (3.6%). Four patients received fluoxetine (7.3%). Twenty patients needed to be admitted to pediatric intensive care unit (36.4%). CONCLUSION: Clinical, microbiological, and radiological diagnosis is essential in outbreaks of EV neurological disease, taking into account that it can be difficult to identify EV-A71 and EV-D68 in CSF, requiring throat or rectal samples. There is not specific treatment to these conditions and the efficacy and understanding of the mechanism of action of immune-modulatory treatment (IVIG, corticosteroids, and plasmapheresis) is limited.

[Para-infectious seizures: A retrospective multicentre study]. / Crisis parainfecciosas: estudio retrospectivo multicéntrico.

INTRODUCTION: Para-infectious seizures are afebrile seizures that are associated with mild infections, and occur in children with no pre-existing neurological illness. They are still little known in our environment. METHODS: A multicentre retrospective study was conducted that included patients with normal psychomotor development and had presented with one or more seizures in the context of a mild afebrile infection. RESULTS: A total of 38 patients (47% male, 53% female) were included in the study over a period of three years (2012-2015). The mean age was 2.1 years. A previous history of febrile seizures was found in 7.9% of them. Mean number of seizures per patient was 2.2, with 57.9% of them being tonic-clonic seizures. The mean duration of seizures was 3.2minutes. An EEG was performed during admission in 73.7% of cases. Lumbar punctures were performed in 34.2% of cases. All were normal. Neuroimaging tests were carried out in 36.9% of cases. Brain MRI was the imaging test performed in most cases (21.1%), with no any pathological findings. The most frequent infection found was acute gastroenteritis (68%), followed by upper respiratory tract infection (32%). Almost two-thirds (63.2%) of patients did not require anticonvulsant medication. Rectal diazepam was the most frequently used drug in emergencies. Intravenous medication was required by 28.9% of patients due to repeated seizures. The most frequently used drug in the non-emergency setting was valproic acid. Anticonvulsant treatment was continued after discharge in 16% of patients. Para-infectious seizures was the diagnosis in 76.3% of cases when discharged. CONCLUSIONS: Knowledge of para-infectious seizures, their clinical diagnosis and benign course is crucial, as this would avoid further testing and unnecessary treatments.

[Evaluation of coordination and balance in preterm children]. / Valoración de la coordinación y el equilibrio en niños prematuros.

INTRODUCTION: Recent studies show that many preterm children without apparent neurological sequelae present some difficulties in different areas, such as coordination or balance during their school years. The Movement Assessment Battery for Children-2 (MABC-2) has demonstrated to be a useful tool to validate the coordination, while the stabilometric platform was the reference standard test for validating the balance. PATIENTS AND METHOD: Case-control study carried out on preterm children from 7 to 10 years old and healthy term infant controls of the same ages. The same age band of MABC-2 was applied and the static balance by the stabilometric platform was analysed. RESULTS: A total of 89 subjects were included, 30 preterm children≤1,500g birthweight, 29 preterm children>1,500g birthweight, and 30 controls. Preterm children obtained the lowest scores on an overall basis in hand dexterity and balance tests in MABC-2, regardless of their birthweight. Lower gestational age was associated with poorer outcomes in hand dexterity and total scores in MABC-2. Balance results were similar using the stabilometric platform, regardless of prematurity. CONCLUSIONS: A little more than 10% preterm and term children could have coordination disorders or be at risk of developing them using the MABC-2. Despite the visual-motor coordination being similar, preterm children could face greater difficulties in hand dexterity while, in the absence of neurological comorbidity, preterm and term children balance could be comparable.

Neurofibromatosis type 1 associated with moyamoya syndrome in children.

BACKGROUND: Vascular abnormalities in neurofibromatosis type 1 may arise anywhere in the cardiovascular system, and cerebrovascular involvement is the predominant feature of moyamoya syndrome. Because neurofibromatosis type 1 is a neurocutaneous disorder and routine follow-up with cranial MRI is not standard practice in asymptomatic children, accurate epidemiologic data are lacking. On follow-up, clinical and radiologic progression is often found in patients with moyamoya syndrome. METHODS: We performed a retrospective analysis on children with neurofibromatosis type 1 who had been diagnosed with moyamoya syndrome on cranial MRI. RESULTS: Of the 197 children diagnosed with neurofibromatosis type 1, 168 had undergone a cranial MRI, and four (2.3%) of them had moyamoya syndrome. At diagnosis, one child had headache and vomiting related to a right frontal hematoma and the other three children were asymptomatic, including one child with a previous history of renal arteriopathy. In two children moyamoya syndrome was unilateral. CONCLUSIONS: The association between moyamoya syndrome and neurofibromatosis type 1 is rare, but it poses a potential risk of clinicoradiologic progression. Targeted monitoring of children with neurofibromatosis type 1 ensures an early diagnosis of moyamoya syndrome.

[Analysis of a series of cases with an initial diagnosis of acute disseminated encephalomyelitis over the period 2000-2010]. / Análisis de una serie de casos con diagnostico inicial de encefalomielitis aguda diseminada en el periodo 2000-2010.

INTRODUCTION: Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease that essentially affects the white matter of the central nervous system. The diagnosis is based on clinical-imaging and developmental findings. Magnetic resonance imaging of the brain is the most useful diagnostic tool. The disease course is usually monophasic and the preferred initial treatment is with corticoids. PATIENTS AND METHODS: We conducted a retrospective study of 18 patients with a presumptive diagnosis of ADEM. Symptoms, imaging findings, progress and treatment were analysed. The definitive diagnosis was established in 12 patients, excluding one patient with positive polymerase chain reaction for herpes simplex virus in cerebrospinal fluid, one with a clinical picture that was consistent but normal magnetic resonance imaging of the brain, and four with an onset that was similar to ADEM whose definitive diagnoses were: Rassmusen's syndrome, haemophagocytic syndrome, brain tumour, and MELAS (mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes). RESULTS: The median age was 31 months with no predominance of either sex. Infection of the upper respiratory tract was the most frequent cause in children over 2 years of age and of the gastrointestinal tract in those under the age of 2. All of them presented altered levels of consciousness and multifocal neurological deficits. The most frequent imaging finding was multifocal alteration of the white matter in both hemispheres. Corticoids were the preferred treatment in most cases. Progression was favourable in nearly all patients except for two, who were left with important sequelae. CONCLUSIONS: ADEM may present at any age, including in infants. There are a number of conditions that can mimic ADEM in the early stages.

TITLE: Analisis de una serie de casos con diagnostico inicial de encefalomielitis aguda diseminada en el periodo 2000-2010.Introduccion. La encefalomielitis aguda diseminada (EMAD) es una enfermedad desmielinizante que afecta fundamentalmente a la sustancia blanca del sistema nervioso central. El diagnostico se basa en hallazgos clinicorradiologicos y evolutivos. La resonancia magnetica cerebral es la herramienta diagnostica mas util. El curso suele ser monofasico y el tratamiento inicial de eleccion, los corticoides. Pacientes y metodos. Estudio retrospectivo de 18 pacientes con diagnostico de sospecha inicial de EMAD. Se analizo la sintomatologia, los hallazgos radiologicos, la evolucion y el tratamiento. El diagnostico definitivo se establecio en 12 pacientes, excluyendo un paciente con reaccion en cadena de la polimerasa positiva para el virus herpes simple en el liquido cefalorraquideo, uno con clinica compatible pero resonancia magnetica cerebral normal, y cuatro con inicio similar a EMAD cuyos diagnosticos definitivos fueron: sindrome de Rassmusen, sindrome hemofagocitico, tumor cerebral y MELAS (encefalomiopatia mitocondrial con acidosis lactica y accidentes cerebrovasculares). Resultados. La mediana de edad fue de 31 meses, sin predominio de sexo. La infeccion de la via respiratoria superior fue la causa mas frecuente en niños mayores y la gastrointestinal, en menores de 2 años. Todos presentaron alteracion en el nivel de conciencia y deficits neurologicos multifocales. El hallazgo radiologico mas frecuente fue la alteracion multifocal bihemisferica de la sustancia blanca. Los corticoides fueron el tratamiento de eleccion en la mayoria. La evolucion fue favorable en casi todos los pacientes excepto en dos, que tuvieron secuelas importantes. Conclusiones. La EMAD puede presentarse a cualquier edad, incluyendo lactantes. Hay multiples entidades que pueden simular una EMAD en un inicio.

[Do our elderly have an adequate nutritional status?]. / ¿Tienen nuestros ancianos un adecuado estado nutricional? ¿Influye su institucionalización?

UNLABELLED: Determine the prevalence of malnutrition in valid adults older than 75 years old in Xinzo (Spain), and study institutionalization as a possible risk factor of malnutrition. METHODS: This is a cross-sectional study of prevalence. SAMPLE: 311 people over 75 years. VARIABLES STUDIED: Age, gender, marital status, education level, institutionalization or not, nutritional status (MNA and anthropometric parameters), social support (Duke- Unc scale), Quality of life (Euro- Quol scale); associated diseases, swallowing problems, type of diet and medications. RESULTS: The median age was 82.55 years (SD: 4.83), 51.8% were women. The 52.7% were married, regarding the educational level, 76.8% had completed the primary education. The 17.4% were institutionalized. The median of pathologies was 3 (SD:1.42 and the number of drugs for individual was 4 (SD:2.44). The 54.70% lived with another family or partner. The median of quality of life was 6.84. According to the results of the MNA did not find any case of malnutrition, but a 20.3% of patients present values of risk. Multivariate analysis found relationship between the presence or not of malnutrition and the institutionalization OR = 0.403 (95% CI: 0.186-0.872), the number of pathologies OR = 1.301 (95% CI: 1.032-1.641), quality of life OR = 1.401 (95% CI: 1.145-1.716). CONCLUSION: Our valid elders are well nourished. The age, a good quality of life and a good support are protective factors. The risk of malnutrition is associated to a higher age, to institutionalization and to higher number of pathologies.

Determinar el estado nutricional de los ancianos de un área de salud rural y ver si la institucionalización es un factor de riesgo. Diseño del estudio: Estudio observacional descriptivo en SAP de Xinzo de Limia 3. Sujetos: El tamaño muestral fue de 311 pacientes mayores de 75 años, seleccionados por muestreo aleatorio simple. Mediciones: Edad, sexo, estado civil, nivel de estudios, institucionalización o no, estado nutricional: valorado mediante el cuestionario MNA y parámetros antropométricos; apoyo social: medido mediante la escala de Duke- Unc; Calidad de vida: con la escala Euro-Quol; patologías asociadas; trastornos de la deglución; tratamiento habitual: tipo de dieta, fármacos. Resultados principales: La mediana de edad era de 82,55 años (DT 4,83 años) y el 51,8% eran mujeres, el 52,7% estaba casado y el 76,8% referían estudios primarios. La mediana de patologías por individuo era del 3 (DT: 1,42) y del número de fármacos usados era de 4 (DT 2,44). El 54,70% vivía acompañado por su pareja u otro familiar. Estaban institucionalizados el 17,4%. La mediana de calidad de vida era de 6,84. Según los resultados del MNA no encontramos ningún caso de desnutrición, pero un 20.3% de los pacientes presentan valores de riesgo. En el análisis multivariante encontramos relación entre la presencia o no de desnutrición y la institucionalización OR = 0,40 (IC 95%, 0,18- 0,87), con el nº de patologías OR = 1,30 (IC 95%, 1,03-1,64), calidad de vida OR = 1,40 (IC 95%, 1,14-1,71). Conclusiones: Los pacientes ancianos validos estudiados presentan un buen estado nutricional. Los pacientes con riesgo de presentar desnutrición son un 20,3%, siendo la institucionalización, los mayores de 85 años con mayor número de patologías los que presentan mayor riesgo de desnutrición. La peor calidad de vida y el menor apoyo social influyen negativamente.