Esclerosis lateral amiotrófica y miastenia gravis (síndrome overlap): presentación de 3 nuevos casos / Amyotrophic lateral sclerosis and myasthenia gravis overlap syndrome: 3 new cases

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Miositis necrosante autoinmune: a propósito de un caso / Autoimmune necrotising myopathy: A case report

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[Study of the diagnostic agreement on headaches between neurology and primary care]. / Estudio de concordancia diagnostica en cefalea entre neurologia y atencion primaria.

INTRODUCTION: Headache as a symptom is a very common disease and one of the main reasons for consultation in primary care. AIM: To analyze the characteristics of patients referred from primary care to general neurology whose chief complaint was headache and/or neuralgia and diagnostic agreement. PATIENTS AND METHODS: Cross-sectional study of all patients referred from primary care; demographic/clinical variables were collected and diagnostic hypothesis by primary care and general neurology were compared by determining their agreement. RESULTS: 2,514 were referred from primary care patients (588 of them on a preferential basis); in 378 cases the reason for consultation was headache and/or neuralgia (average 42.46 years; 77.8% female). In 139 patients it was established only a semiological diagnostic and other episodic migraine predominated (49.79%), chronic tension headache (18.41%) and trigeminal neuralgia (12.13%). Since general neurology, the most common diagnoses were, respectively, 33.86%, 24.05% and 18.67%. A compatible kappa coefficient of 0.543 (p < 0.05) with a moderate agreement when considering only those patients referred from primary care to a specific diagnosis was obtained. CONCLUSIONS: Headaches are a very common reason for consultation in primary care (15%). The diagnostic agreement is moderate in our health sector so it is necessary to design training programs to help outline the criteria for referral to specialists and improve care for our patients.

TITLE: Estudio de concordancia diagnostica en cefalea entre neurologia y atencion primaria.Introduccion. La cefalea como sintoma es una patologia frecuente y uno de los principales motivos de consulta por parte de atencion primaria. Objetivo. Analizar las caracteristicas de los pacientes derivados desde atencion primaria a la consulta de neurologia general con cefalea o neuralgia como motivo de consulta, y la concordancia diagnostica. Pacientes y metodos. Estudio descriptivo transversal de todos los pacientes remitidos desde atencion primaria; se recogieron variables demograficas/clinicas y se compararon las hipotesis diagnosticas de atencion primaria y neurologia, determinando su concordancia. Resultados. Se remitieron desde atencion primaria 2.514 pacientes (588 de ellos con caracter preferente); en 378 casos el motivo de la consulta fue cefalea o neuralgia (42,46 años de media; el 77,8%, mujeres). En 139 pacientes se establecio tan solo un diagnostico semiologico y en el resto predominaron la migraña episodica (49,79%), la cefalea tensional cronica (18,41%) y la neuralgia del trigemino (12,13%). Desde neurologia, los diagnosticos mas frecuentes fueron, respectivamente, 33,86%, 24,05% y 18,67%. Se obtuvo un coeficiente kappa de 0,543 (p < 0,05), compatible con una concordancia moderada al considerar solo los pacientes remitidos desde atencion primaria con un diagnostico concreto. Conclusiones. Las cefaleas constituyen un motivo de consulta desde atencion primaria muy frecuente (15%). La concordancia diagnostica es moderada en nuestro sector sanitario, por lo que es necesario diseñar programas de formacion que ayuden a perfilar los criterios de derivacion al especialista y mejorar la atencion a nuestros pacientes.

Levetiracetam y síndrome de piernas inquietas / No disponible

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[Characteristics of Guillain-Barré syndrome in the healthy area III of Aragon Country]. / Características del síndrome de Guillain-Barré en el área III de salud de la Comunidad Autónoma de Aragón.

INTRODUCTION: Guillain-Barré Syndrome is an acute immune-mediated inflammatory polyneuropathy characterized by flaccid paresia with arreflexia, changeable sensitive disorder and albuminocytologic dissociation in the cerebrospinal fluid (CSF). PATIENTS AND METHODS: We conducted a retrospective study of 30 GBS patients diagnosed in Hospital Clínico de Zaragoza between 1999 and 2005. Annual incidence, seasonal distribution, preceding acute infection; clinical, electrophysiological and electrocardiographic (ECG) data and evolution were all evaluated. RESULTS: The incidence was 1.56/100000 inhabitants/year. We observed an upward tendency during winter months. The rates of incidence were higher in men (66.7%) and increased with age in both sexes. Main clinical presentation form was paraparesia of lower limbs followed on cranial nerve palsy. Immunoglobulin therapy was received by 62.5% of patients. The rate of death was 10%. Thirteen per cent of patients had dysautonomia. Electrocardiographic abnormalities were present in 37% of patients. CONCLUSIONS: GBS incidence in Aragon Country is similar to that found in other studies. An increase with age and an upward tendency during the winter months was observed. High percentage of abnormalities in ECG but the majority of patients was asymptomatic.

Características del síndrome de Guillain-Barré en el área III de salud de la Comunidad Autónoma de Aragón / Characteristics of Guillain-Barré syndrome in the healthy area III of Aragon Country

Introducción: el síndrome de Guillain-Barré (SGB) es una polineuropatía inflamatoria autoinmune caracterizada por la presencia de parálisis fláccida con arreflexia, trastorno sensorial variable y disociación albúmino-citológica en el LCR.Pacientes y métodos: estudio retrospectivo de 30 pacientes diagnosticados de SGB entre 1999 y 2005 en el Hospital Clínico de Zaragoza. Se evaluó la incidencia anual, distribución estacional y regional, antecedentes de infección, características clínicas, neurofisiológicas y electrocardiográficas (ECG). Resultados: la incidencia fue de 1,56/100000 habitantes/año. Mayor frecuencia de eventos en invierno sin alcanzar significación estadística. Mayor frecuencia en sexo masculino (66,7%) e incremento de la incidencia con la edad en ambos sexos. La forma de presentación más frecuente fue la paraparesia de extremidades inferiores seguida de la afectación de pares craneales. El 62,5% recibió tratamiento inmunomodulador. La mortalidad fue del 10%. El 13% presentó alteraciones autonómicas. Alteraciones electrocardiográficas en el 37% de los pacientes. Conclusiones: la incidencia del SGB en Aragón es similar a la que se encontró en otras series. Observamos un aumento de la incidencia con la edad y predilección por los meses de invierno. Elevado porcentaje de pacientes con anomalías ECG mayoritariamente asintomáticas

Introduction: Guillain-Barré Syndrome is an acute immune-mediated inflammatory polyneuropathy characterized by flaccid paresia with a rreflexia, change able sensitive disorder and albumino cytologic dissociation in the cerebrospinal fluid (CSF). Patients and methods: We conducted a retrospective study of 30 GBS patients diagnosed in Hospital Clínico de Zaragoza between 1999 and 2005. Annual incidence, seasonal distribution, preceding acute infection; clinical, electrophysiological and electrocardiographic (ECG) data and evolution were all evaluated. Results: The incidence was 1.56/100000 inhabitants/year. We observed an upward tendency during winter months. The rates of incidence were higher in men (66.7%) and increased with age in both sexes. Main clinical presentation form was paraparesia of lower limbs followed on cranial nerve palsy. Immunoglobulin therapy was received by 62.5%of patients. The rate of death was 10%. Thirteen per cent of patients haddys autonomia. Electrocardiographic abnormalities were present in 37% of patients. Conclusions: GBS incidence in Aragon Country is similar to that found in other studies. An increase with age and an upward tendency during the winter months was observed. High percentage of abnormalities in ECG but the majority of patients was asymptomatic

Factores pronósticos y supervivencia en la enfermedad de neurona motora / Pronostic factors and survival in motor neuron disease

Introducción. La esclerosis lateral amiotrófica (ELA), la más común de las enfermedades de neurona motora (ENM) del adulto, se presenta combinando signos de afectación de neurona motora superior (NMS) e inferior (NMI), con una elevada mortalidad. Otras ENM menos frecuentes son la atrofia muscular progresiva (AMP) y la esclerosis lateral primaria (ELP).Objetivos. Describir datos demográficos, formas de presentación clínica y evolución de pacientes con ENM buscando factores pronósticos.Métodos. Estudio retrospectivo de una serie de pacientes con ENM, registrando datos demográficos, variables clínicas y mortalidad-supervivencia, analizados mediante paquete estadístico SPSS 6.1.2.Resultados. Son 54 pacientes con ENM (50 ELA, 3 AMP y 1 ELP), 30 (55,6%) hombres y 24 (44,4%) mujeres. Formas de inicio bulbar en 13 casos (24,1%) y espinal en 41 (75,9%). Edad media al inicio: 60 +- 14 (29-82 años); más tardía en las mujeres (p< 0,05), y en los pacientes con forma bulbar (p<0,05), y en los pacientes con forma bulbar (p<0,05), con claro predominio femenino (p<0,03). Hay 31 pacientes muertos (57,4%) y 23 vivos (42,6%), con supervivencia media desde el inicio de los síntomas de 46,51 +- 40,9 meses (11-209 meses), observando relación inversamente proporcional con la edad de inicio de la enfermedad (p<0,02). Se asocian con una mayor probabilidad de morir las formas de inicio bulbar (p<0,01) y la extensión de los signos de afectación de MNI, especialmente a nivel bulbar y cervical (p<0,005), mientras que la existencia de signos de afectación de NMS en neuroimagen tiene una mayor probabilidad de sobrevivir (p<0,05).Conclusiones. La edad avanzada y las formas de inicio bulbar son factores de mal pronóstico, y especialmente el predominio de afectación NMI sobre NMS

Introduction: Amyotrophic lateral sclerosis (ALS) is the most common adult motor neuron disease (MND), presenting with a combination of upper (UMN) and lower (lmn) motor neuron signs, with high mortality. other less frequent mnd are progressive muscular atrophy (pma) and primary lateral sclerosis (pls). OBJECTIVES: To describe demographic data, clinical forms of presentation and evolution of patients with MND, looking for prognostic indicators factors. METHODS: Retrospective study of a series of patients with MND, registering demographic data, clinical variables and mortality-survival, analyzed by means of statistical package SPSS 6.1.2. RESULTS: They are 54 patients with MND (50 ALS, 3 PMA and 1 PLS), 30 (55.6%) men and 24 (44.4%) women, bulbar beginning forms in 13 cases (24.1%) and spinal in 41 (75.9%). Average age at beginning 60+/-14 (29-82 years), more delayed in women (p<0.05), and in patients with bulbar form (p<0.05) with clear predominance of female sex (p<0.03). There are 31 dead patients (57.4%) and 23 alive ones (42.6%), with average survival from the beginning symptoms of 46.51+/-40.9 months. Nine months (11-209), observing inversely proportional relation with the age of beginning of the disease (p<0.02). Bulbar beginning forms (p<0.01) and LMN affectation signs, specially bulbar and cervical levels (p<0,005), are associated with a greater probability of dying while the existence of UMN affectation signs on imaging techniques 362 ques has a greater probability of survival (p<0,05). CONCLUSIONS: The advanced age and the bulbar beginning forms are factors of bad prognosis, specially the predominant affectation of LMN over UMN

[Pronostic factors and survival in motor neuron disease]. / Factores pronósticos y supervivencia en la enfermedad de neurona motora.

INTRODUCTION: Amyotrophic lateral sclerosis (ALS) is the most common adult motor neuron disease (MND), presenting with a combination of upper (UMN) and lower (lmn) motor neuron signs, with high mortality. other less frequent mnd are progressive muscular atrophy (pma) and primary lateral sclerosis (pls). OBJECTIVES: To describe demographic data, clinical forms of presentation and evolution of patients with MND, looking for prognostic indicators factors. METHODS: Retrospective study of a series of patients with MND, registering demographic data, clinical variables and mortality-survival, analyzed by means of statistical package SPSS 6.1.2. RESULTS: They are 54 patients with MND (50 ALS, 3 PMA and 1 PLS), 30 (55.6%) men and 24 (44.4%) women, bulbar beginning forms in 13 cases (24.1%) and spinal in 41 (75.9%). Average age at beginning 60+/-14 (29-82 years), more delayed in women (p<0.05), and in patients with bulbar form (p<0.05) with clear predominance of female sex (p<0.03). There are 31 dead patients (57.4%) and 23 alive ones (42.6%), with average survival from the beginning symptoms of 46.51+/-40.9 months. Nine months (11-209), observing inversely proportional relation with the age of beginning of the disease (p<0.02). Bulbar beginning forms (p<0.01) and LMN affectation signs, specially bulbar and cervical levels (p<0,005), are associated with a greater probability of dying while the existence of UMN affectation signs on imaging techniques 362 ques has a greater probability of survival (p<0,05). CONCLUSIONS: The advanced age and the bulbar beginning forms are factors of bad prognosis, specially the predominant affectation of LMN over UMN.

[Model of analysis in two halves for semantic fluency tasks]. / Modelo de análisis en dos mitades para tareas de fluidez semántica.

INTRODUCTION: Semantic verbal fluency (SVF) tasks involve the activation of language, semantic memory, working memory and the executive functions. The second half of the test increases the demands on working memory and search for lexical items, which can make it more difficult than the first half. AIM: To conduct a pilot study in order to standardise the two halves of the SVF test ('animals' category) in adults over 50 years of age. SUBJECTS AND METHODS: Two verbal fluency tasks (semantic and phonological) and the Spanish version of the Mini-Mental State Examination with working memory were applied in 122 controls (range: 50-89 years), who were distributed according to age and schooling. RESULTS: There is a significant difference between the two halves of the test adjusted for age and schooling (p < 0.0001). Scoring in the second half showed a higher correlation with age, schooling and working memory than the first half. Performance in the two halves was standardised for three age groups (50-59, 60-79 and 80-89 years) and two levels of schooling (high and low). CONCLUSIONS: The number of names in the second half of the SVF test was lower than in the first half in all the groups, which supports the hypothesis of a greater cognitive demand, probably on working memory and the search for lexical items in semantic memory. Having normative values available for the two halves of the SVF test allows simultaneous and independent interpretation of the performance at two levels of intra-task difficulty. This model of analysis complements the traditional assessment and can easily be applied in day-to-day clinical practice.

Modelo de análisis en dos mitades para tareas de fluidez semántica / Model of analysis in two halves for semantic fluency tasks

Introducción. Las tareas de fluidez verbal semántica (FVS) implican la activación del lenguaje, la memoria semántica, la memoria de trabajo y las funciones ejecutivas. En la segunda mitad del test aumentan las demandas sobre la memoria de trabajo y la búsqueda lexical, lo que puede aumentar su dificultad relativa respecto de la primera mitad. Objetivo. Estudio piloto de estandarización de las dos mitades del test de FVS, categoría ‘animales’, en adultos mayores de 50 años. Sujetos y métodos. Se aplicaron dos tareas de fluidez verbal (semántica y fonológica) y el miniexamen cognitivo con memoria de trabajo en 122 controles (rango: 50-89 años), distribuidos por edad y escolarización. Resultados. Existe una diferencia significativa entre las dos mitades del test ajustado por edad y escolarización (p < 0,0001). La puntuación en la segunda mitad mostró una correlación más alta que la primera mitad con la edad, la escolarización y la memoria de trabajo. El rendimiento en ambas mitades se estandarizó para tres grupos por edad (50-59, 60-79 y 80-89 años) y dos niveles de escolarización (alta y baja). Conclusiones. El número de nombres en la segunda mitad del test de FVS fue inferior a la primera mitad en todos los grupos, lo que apoya la hipótesis de una mayor demanda cognitiva, probablemente en memoria de trabajo y búsqueda lexical en la memoria semántica. La disponibilidad de valores normativos para las dos mitades del test de FVS permite la interpretación simultánea e independiente del rendimiento en dos niveles de dificultad intra-task. Este modelo de análisis complementa la valoración tradicional y se puede aplicar de forma sencilla en la clínica diaria

Introduction. Semantic verbal fluency (SVF) tasks involve the activation of language, semantic memory, working memory and the executive functions. The second half of the test increases the demands on working memory and search for lexical items, which can make it more difficult than the first half. Aim. To conduct a pilot study in order to standardise the two halves of the SVF test (‘animals’ category) in adults over 50 years of age. Subjects and methods. Two verbal fluency tasks (semantic and phonological) and the Spanish version of the Mini-Mental State Examination with working memory were applied in 122 controls (range: 50-89 years), who were distributed according to age and schooling. Results. There is a significant difference between the two halves of the test adjusted for age and schooling (p < 0.0001). Scoring in the second half showed a higher correlation with age, schooling and working memory than the first half. Performance in the two halves was standardised for three age groups (50-59, 60-79 and 80-89 years) and two levels of schooling (high and low). Conclusions. The number of names in the second half of the SVF test was lower than in the first half in all the groups, which supports the hypothesis of a greater cognitive demand, probably on working memory and the search for lexical items in semantic memory. Having normative values available for the two halves of the SVF test allows simultaneous and independent interpretation of the performance at two levels of intra-task difficulty. This model of analysis complements the traditional assessment and can easily be applied in day-to-day clinical practice

[Facial diplejia: a regional variant of Guillain-Barré syndrome]. / Diplejia facial: variante regional del síndrome de Guillain-Barré.

Facial palsy is a uncommon clinical manifestation that it can be caused by different etiologies. We show a patient with a chronic periodontal disease who presented a sudden facial palsy, initially in left-face but it becomes bilateral quickly. In few days he associated paresthesias in his right arm and hyporreflexia. These clinical findings with albumin-cytological dissociation, they had allowed to establish the diagnosis of regional variant of Guillain-Barré Syndrome (SGB). Facial diplejia is an idiopathic manifestation in 25% of patients, and this is the most common cause. However, facial diplejia can be secondary to many etiologies as SGB. The affectation of facial nerve associated to other motor symptoms in SGB is frequent, but it is not frequent the presentation as facial diplejia alone. In conclusion, it is necessary a high clinical suspicion to do a lumbar punction (PL) and MRI to reject neoplasic pathologies and to obtain a diagnosis and an adequate treatment.

Diplejia facial: variante regional del síndrome de Guillian-Barré / Facial diplejia: a regional variant of Guillian-Barre syndrome

Diplejia facial es una manifestación clínica poco frecuente que puede presentarse como consecuencia de múltiples etiologías. Presentamos un paciente con una infección periodontal crónica que sufrió de forma brusca una parálisis facial inicialmente izquierda, que rápidamente se hizo bilateral. La aparición posterior de parestesias distales en extremidad superior derecha e hiporreflexia, junto con la disociación albúmino-citológica en el LCR, permitieron establecer el diagnóstico de variante regional del Síndrome de Guillian-Barré (SGB). La diplejia facial es idiopática en el 25% de casos, siendo la causa más frecuente. Sin embargo, existe un elevado porcentaje de casos secundarios a múltiples etiologías como el SGB donde es frecuente la afectación del nervio facial habitualmente asociado a otros trastornos motores siendo infrecuente la diplejia facial aislada. Concluimos la necesidad de sospecha clínica para realización de punción lumbar y RNM para descartar procesos neoplásicos y poder determinar la etiología responsable para establecer un adecuado abordaje terapéutico

Facial palsy is a uncommon clinical manifestation that it can be caused by different ethiologies. We show a patient with a chronic periodontal disease who presented a sudden facial palsy, initially in left-face but it becomes bilateral quickly. In few days he associated paresthesias in his right arm and hiporreflexia. These clinical findings with albumin-citological dissociation, they had allowed to establish the diagnosis of regional variant of Guillain-Barré Syndrome (SGB). Facial diplejia is an idiopathic manifestation in 25% of patients, and this is the most common cause. However, facial diplejia can be secondary to many ethiologies as SGB. The affectation of facial nerve associated to other motor symptoms in SGB is frequent, but it is not frequent the presentation as facial diplejia alone. In conclusion, it is necessary a high clinical suspicion to do a lumbar punction (PL) and MRI to reject neoplasic pathologies and to obtain a diagnosis and an adequatted treatment

Cefalea sexual asociada a quiste aracnoideo / Sexual headaches associated to an arachnoid cyst

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[Clinical, electrophysiological and immunological evaluation of the response to treatment with intravenous immunoglobulins in several immune-mediated neuropathies]. / Valoración clínica, electrofisiológica e inmunológica de la respuesta al tratamiento con inmunoglobulinas intravenosas en distinta neuropatías inmunomediadas.

INTRODUCTION AND AIMS: Chronic immune-mediated neuropathies are characterised by their being predominantly demyelinating, by being associated to specific antibodies and by their response to immunotherapy. We evaluated the clinical, electrophysiological and immunological responses following treatment with intravenous immunoglobulins (IVIg) in different clinical forms. CASE REPORTS: We report on three patients with: 1. Multifocal motor neuropathy, 2. Multifocal sensory-motor neuropathy, and 3. Hypertrophic brachial plexopathy, who were evaluated before and 14 days after treatment with IVIg by means of clinical scales (MRC, Rankin), electrophysiological studies (ENG-EMG), and antibody (antiganglioside) determination. The three patients showed clinical improvement (> 20% MRC, > 1 Rankin) between the 4th and 7th day after the infusion, and this was maintained for 4-16 weeks. The ENG in cases 1 and 2 revealed conduction block (CB) in some nerves, lowered amplitude in others, and signs of demyelination. Following treatment, the CB disappeared in some nerves, while in others there was an improvement in the distal amplitude (distal block), but with no correlative improvement in the proximal amplitude, revealing new CBs that had previously gone undetected. The sum of post-treatment amplitudes improved in cases 1 and 2. In case 3, we were unable to demonstrate the existence of CBs, although we believe that they did exist proximally, and we found indirect signs of CB in EMG. The three cases had increased antiganglioside IgM antibodies levels, which did not undergo any significant variations. CONCLUSIONS: A 'consistent' clinical improvement was observed following treatment with IVIg. There was no correlation between the electrophysiological response (although apparent) and the degree of clinical response. CBs exist at different levels in the same nerve, which can be revealed after treatment. Following therapy, antiganglioside antibody levels remain high.