Study of the possible link of 25-hydroxyvitamin D with Epstein-Barr virus and human herpesvirus 6 in patients with multiple sclerosis.

BACKGROUND AND PURPOSE: Although the causes of multiple sclerosis (MS) remain partially unknown, environmental and genetic factors are thought to play a role in its aetiopathogenesis. Hypovitaminosis D, Epstein-Barr virus (EBV) and human herpesvirus 6 (HHV-6) infections have been described as possible MS triggers. Our aim was to analyse the possible link between 25-hydroxyvitamin D [25(OH)D] and viruses in patients with MS. METHODS: We included 482 patients with MS in a 2-year study. Serum samples were collected to analyse 25(OH)D levels and, according to sample availability, antibody titres against EBV and HHV-6 by enzyme-linked immunosorbent assay. DNA was extracted from blood in order to analyse EBV and HHV-6 viral load by quantitative real-time polymerase chain reaction and to genotype MS-related single nucleotide polymorphisms (rs3135388, rs2248359 and rs12368653) when possible. RESULTS: The 25(OH)D levels were significantly higher in the first semester of the year than in the second. Carriers of the risk allele rs2248359-C showed lower 25(OH)D levels than non-carriers. For EBV, viral load was significantly higher when 25(OH)D levels were low, demonstrating an inverse correlation between 25(OH)D levels and EBV load. CONCLUSIONS: The 25(OH)D levels could be involved in the regulation of EBV replication/reactivation in patients with MS.

[Cryptogenic stroke in a young patient with heart disease and kidney failure]. / Ictus criptogenico en un paciente joven con cardiopatia y fallo renal.

INTRODUCTION: Fabry's disease is an infrequent metabolic pathology linked to the X chromosome which causes a wide variety of signs and symptoms. CASE REPORT: A 39-year-old male who was admitted to our stroke unit with right-side hemiparesis (1 + 0) and dysarthria (1). The score on the National Institute of Health Stroke Scale was 2. The patient presented angiokeratomas in both thighs. A computerised axial tomography scan of the head showed left thalamic acute infarction. The duplex scan of the supra-aortic trunks was normal, and the transcranial Doppler reflected a generalised increase in the pulsatility indices. Transthoracic echocardiography showed left ventricular hypertrophy and left atrial dilatation. He was discharged five days later, with antiaggregating medication but asymptomatic. The prolonged Holter-electrocardiogram recording showed paroxysmal atrial fibrillation. One notable value in the urine analysis was microalbuminuria of 281 mg/L. In view of the multi-organic involvement and the family history, a study for Fabry's disease was performed. Activity of the enzyme alpha-galactosidase A was diminished, and the presence of a mutation in the GLA gene was found. The patient's brother, who suffered from kidney failure and atrial fibrillation, was positive for this mutation. The patient is on treatment with agalsidase beta. CONCLUSIONS: Fabry's disease must be suspected in young males with heart disease, stroke or peripheral neuropathy, skin lesions, kidney failure and a history of cases in the family. Hormone replacement therapy must be established at an early stage, as it can improve the prognosis.

TITLE: Ictus criptogenico en un paciente joven con cardiopatia y fallo renal.Introduccion. La enfermedad de Fabry es una patologia metabolica infrecuente ligada al cromosoma X, que provoca una amplia variedad de signos y sintomas. Caso clinico. Varon de 39 antilde;os que ingreso en nuestra unidad de ictus con hemiparesia derecha (1 + 0) y disartria (1). La puntuacion en la National Institute of Health Stroke Scale era de 2. Presentaba angioqueratomas en ambos muslos. La tomografia axial computarizada craneal mostraba un infarto agudo talamico izquierdo. El duplex de los troncos supraaorticos era normal, y el Doppler transcraneal reflejaba un aumento generalizado de los indices de pulsatilidad. El ecocardiograma transtoracico mostraba hipertrofia ventricular izquierda y dilatacion de la auricula izquierda. Recibio el alta cinco dias despues, asintomatico, con antiagregacion. El registro Holter-electrocardiografico prolongado mostraba fibrilacion auricular paroxistica. En la analitica de orina destacaba microalbuminuria de 281 mg/L. En vista de la afectacion multiorganica y la historia familiar, se curso estudio de enfermedad de Fabry. La actividad de la enzima alfa-galactosidasa-A se encontro disminuida, y se demostro la presencia de una mutacion en el gen GLA. Su hermano, que padecia insuficiencia renal y fibrilacion auricular, fue positivo para dicha mutacion. El paciente se encuentra en tratamiento con agalsidasa beta. Conclusiones. La enfermedad de Fabry debe sospecharse en varones jovenes con cardiopatia, ictus o neuropatia periferica, lesiones cutaneas, fallo renal e historia de familiares afectos. El tratamiento hormonal sustitutivo debe comenzarse precozmente, ya que puede mejorar el pronostico.

La formación en neurofisiología en el programa educativo de la especialidad de neurología en España / Neurophysiology training in the Neurology Specialist Education Program in Spain

Introducción: el periodo formativo en neurofisiología es una parte sustancial del programa de la especialidad de neurología en Espa˜na. La Comisión Nacional de Neurología (CNN), que es el órgano dependiente de los Ministerios de Sanidad y Educación, debe velar por el cumplimiento del programa. Material y métodos: durante el primer semestre de 2008 la CNN envió a cada una de las 69 unidades docentes acreditadas para la formación de neurología en Espa˜na un cuestionario para que los responsables docentes de cada unidad lo contestaran, donde se preguntaba sobre este periodo formativo. Resultados: de los 69 cuestionarios remitidos se recibieron 49 cumplimentados, lo que supone una tasa de respuesta del 71%. El periodo formativo de neurofisiología del programa de la especialidad de neurología se realiza en Espa˜na en el mismo hospital en 44 centros (90%); los 5 restantes envían sus NeF a 4 hospitales diferentes. La Unidad que realiza el periodo formativo de neurofisiología está integrada en el servicio de neurología en 19 (39%) hospitales, es independiente en 27 (55%) y la fórmula es mixta en 3 (6%). El 69% de los tutores docentes estaba satisfecho con la formación, pero en el 90% de los hospitales en los que la unidad estaba integrada en neurología y en el 65% de los que no existía esta relación. Los neurólogos en formación informan EEG en el 49% de las unidades docentes, realizan EMG/ENG en el 57% e informan potenciales evocados en el 35% tras su periodo formativo. Conclusiones: aunque el grado de satisfacción es alto, el nivel de responsabilidad que asumen los neurólogos en formación durante la rotación por neurofisiología parece que no cumple las exigencias previstas en el programa formativo, especialmente en aquellas unidades no integradas en servicios de neurología (AU)

Introduction: the training period in neurophysiology is a substantial part of the Neurology Specialist Program in Spain. The National Neurology Committee (La Comisión Nacional de Neurología (CNN), which is the body reporting to the Ministries of Health and Education, must ensure compliance to the Program. Material and methods: during the first trimester of 2008, the CNN sent a questionnaire, in which there was a question asking about this training period, to each of the managers of the 69 teaching units accredited for neurology training in Spain, for them to answer. Results: of the 69 questionnaires issued, 49 were received completed, which was a response rate of 71%. The neurophysiology training period of the neurology specialist program in Spain was carried out in the same hospital in 44 teaching unit (90%): the remaining 5 sent their neurology trainees to 4 different hospitals. The Unit that carried out the neurophysiology training period was incorporated into the Neurology Department in 27 (55%) cases, and the formula was mixed in 3 (6%). A total of 69% of tutors were satisfied with the training, but was 90% in the hospitals where the unit was integrated into Neurology, and was 65% where this relationship did not exist. The neurologists in training were informed about EEG in 49% of education units, performed EMG/ENG 57%, and informed about evoked potentials in 35% after their training period. Conclusions: although the level of satisfaction is high, the level of responsibility assumed by the neurologists in training during their rotation into neurophysiology does not appear to comply to the demands laid out in the training program, particularly in these units not integrated into Neurology Departments (AU)

Neurophysiology training in the Neurology Specialist Education Program in Spain.

INTRODUCTION: The training period in neurophysiology is a substantial part of the Neurology Specialist Program in Spain. The National Neurology Committee (La Comisión Nacional de Neurología (CNN), which is the body reporting to the Ministries of Health and Education, must ensure compliance to the Program. MATERIAL AND METHODS: During the first trimester of 2008, the CNN sent a questionnaire, in which there was a question asking about this training period, to each of the managers of the 69 teaching units accredited for neurology training in Spain, for them to answer. RESULTS: Of the 69 questionnaires issued, 49 were received completed, which was a response rate of 71%. The neurophysiology training period of the neurology specialist program in Spain was carried out in the same hospital in 44 teaching unit (90%): the remaining 5 sent their neurology trainees to 4 different hospitals. The Unit that carried out the neurophysiology training period was incorporated into the Neurology Department in 27 (55%) cases, and the formula was mixed in 3 (6%). A total of 69% of tutors were satisfied with the training, but was 90% in the hospitals where the unit was integrated into Neurology, and was 65% where this relationship did not exist. The neurologists in training were informed about EEG in 49% of education units, performed EMG/ENG 57%, and informed about evoked potentials in 35% after their training period. CONCLUSIONS: Although the level of satisfaction is high, the level of responsibility assumed by the neurologists in training during their rotation into neurophysiology does not appear to comply to the demands laid out in the training program, particularly in these units not integrated into Neurology Departments.

Ciliary ganglioplegic migraine: migraine-related prolonged mydriasis.

BACKGROUND: Transient, unilateral mydriasis has been reported in migraine patients, and this has been regarded as a possible co-morbidity between migraine and Adie's tonic pupil. Mydriasis that outlasts the duration of migraine attacks is rare. METHODS: Through an eight-year period we have studied all patients referred to our neurological clinic because of migraine attack with mydriasis. All the patients underwent thorough neurologic and ophthalmologic examinations including MRI of the brain and testing of the pupil response to ocular instillation of dilute pilocarpine (0.125%). RESULTS: Seven women and two men, with a mean age of 33.8 ± 12.3 years (range: 19-52) were included. The patients presented during one hemicranial migraine attack with an ipsilateral mydriasis that persisted for a mean of three months, while migraine headaches remained with their typical episodic course. In all the patients a cholinergic supersensitivity in the symptomatic pupil was demonstrated, thus pointing to a dysfunction of the ipsilateral ganglionic parasympathetic fibers. CONCLUSIONS: Synchronous co-localization of the features suggests a pathogenic link between the pupil dysfunction and migraine, rather than a simultaneous coexistence of two independent disorders. Likely explanations include a latent Adie's pupil that could have been triggered during a particular migraine attack; a ciliar ganglionic lesion/dysfunction produced by the migrainous process; an ophthalmoplegic migraine with selective parasympathycoparesis; or an episodic ciliar ganglionitis with migrainous features. Ciliary ganglioplegic migraine is proposed as a nominal term pointing to the possible anatomic source of the migrainous-related pupil dysfunction; the pathogenesis remains unknown.

Análisis de las unidades docentes de neurología en España y cumplimientode los criterios de acreditación / Analysis of the neurology teaching unit in Spain and compliance of accreditation criteria

Introducción. Ante la promulgación de la Ley de Ordenación dela Profesiones Sanitarias y la publicación del nuevo programa de laespecialidad de neurología, la Comisión Nacional de Neurología considerónecesario conocer la situación actual de las unidades docentes(UD) acreditadas para la especialidad en lo referente a los criterios deacreditación vigentes. El objetivo de este trabajo es conocer dichasituación.Métodos. Estudio transversal mediante una encuesta de cumplimentaciónvoluntaria (cuestionario ad hoc) remitida por correoen el año 2005 a todas UD acreditadas en ese momento. Incluyópreguntas específicas respecto a los requerimientos estructurales,humanos y de organización contemplados en dichos criterios deacreditación.Resultados. Se remitieron 64 cuestionarios (porcentaje de respuesta:76,6%). El 87 % de las UD cumplían los requisitos estructuralespara la asistencia neurológica, aunque el 21 % tenían menos de4 despachos para consulta y menos de 15 camas neurológicas. El 25%de las UD no cumplían los requisitos en lo referente a los facultativos deplantilla con dedicación a tiempo completo. Aunque prácticamente el100% cumplían los requisitos organizativos cuantitativos asistenciales,sólo el 69 % refirieron tener guardias de neurología, aunque porel diseño del cuestionario no es posible conocer su grado de tutorización.Prácticamente el 100% cumplen los requisitos de docencia einvestigación.Conclusiones. Aunque el grado de cumplimiento de los actualesCA es alto, existen deficiencias preocupantes, fundamentalmente enrelación al número de facultativos de plantilla contratados a tiempocompleto y a la disponibilidad de guardias de neurología debidamentetutorizadas (AU)

Introduction. Faced with the promulgation of the HealthCare Professionals Ordinance Law and the publication of the newprogram of the Neurology training, the National Committee ofneurology has considered it necessary to know the current situationof the Teaching Units (TU) accredited for the neurology trainingin regards to the existing accreditation criteria (AC). Thepurpose of this study is to know said situation.Methods. A cross-sectional study performed by a voluntarilyfilled-out survey (ad hoc questionnaire) sent by regular mailin the year 2005 to all the TU accredited at that time. It includedspecific questions regarding the structural, human andorganizational requirements contemplated in those accreditationcriteria.Results. A total of 64 questionnaires were sent (response percentage:76.6%). Eighty seven percent of the TU met the structuralrequirements for neurological care, although 21 % had fewerthan 4 offices for visits and fewer than 15 neurology beds. A totalof 25 % of the TU did not met the requirements in regards to fulltimestaff members. Although almost 100% met the quantitativecare organizational requirements, only 69 % reported that therewere neurology duties. However, the grade of the tutorial systemcould not be known due to the survey design. Almost 100% metthe teaching and research requirements.Conclusions. Although the compliance grade of the currentAC is high, there are important deficiencies, basically related tothe number of full-time staff professionals and the availability ofduly tutorized neurology duties (AU)

[Analysis of the neurology teaching unit in Spain and compliance of accreditation criteria]. / Análisis de las unidades docentes de neurología en España y cumplimiento de los criterios de acreditación.

INTRODUCTION: Faced with the promulgation of the Health Care Professionals Ordinance Law and the publication of the new program of the Neurology training, the National Committee of neurology has considered it necessary to know the current situation of the Teaching Units (TU) accredited for the neurology training in regards to the existing accreditation criteria (AC). The purpose of this study is to know said situation. METHODS: A cross-sectional study performed by a voluntarily filled-out survey (ad hoc questionnaire) sent by regular mail in the year 2005 to all the TU accredited at that time. It included specific questions regarding the structural, human and organizational requirements contemplated in those accreditation criteria. RESULTS: A total of 64 questionnaires were sent (response percentage: 76.6%). Eighty seven percent of the TU met the structural requirements for neurological care, although 21 % had fewer than 4 offices for visits and fewer than 15 neurology beds. A total of 25 % of the TU did not met the requirements in regards to full-time staff members. Although almost 100% met the quantitative care organizational requirements, only 69 % reported that there were neurology duties. However, the grade of the tutorial system could not be known due to the survey design. Almost 100% met the teaching and research requirements. CONCLUSIONS: Although the compliance grade of the current AC is high, there are important deficiencies, basically related to the number of full-time staff professionals and the availability of duly tutorized neurology duties.