Grado de cumplimiento terapéutico a los tres meses en pacientes con migraña / Degree of compliance with treatment at three months in migraine patients

Introducción: La adhesión al tratamiento preventivo oral (TPO) en la migraña se ve frecuentemente comprometida. El objetivo fue conocer el grado de adhesión al TPO en pacientes con migraña a los tres meses. Pacientes y métodos: Estudio multicéntrico observacional de pacientes diagnosticados de migraña episódica o crónica (criterios de la Clasificación Internacional de Cefaleas, tercera edición) en los que se iniciaba TPO. Se recogieron datos demográficos (edad, género, nivel estudios, estado civil) y de enfermedad (número de ataques, puntuación en las escalas Headache Impact Test-6 y Migraine Disability Assessment Scale). A los tres meses se pasó la escala de Morisky Green, que diferencia niveles de adhesión: excelente (0), moderada (1-2) y baja (3-4). Resultados: Participaron 100 pacientes, un 87% mujeres de 42 ± 13 años, el 14% con migraña crónica. El 53,2% comenzaba su primer TPO. Se iniciaron betabloqueantes en el 23,2%, antidepresivos tricíclicos en el 35,4%, flunaricina en el 21,2%, neuromoduladores en el 19,2% y antihipertensivos en el 1%. El 56% presentaba discapacidad grave y el 79,5%, impacto muy grave. La adhesión a los tres meses fue excelente en el 41,8%, moderada en el 28,6% y baja en el 29,6%. El motivo más frecuente de discontinuación fueron los efectos adversos (44%). Se encontró una relación significativa entre adhesión excelente y estado civil soltero (p = 0,046), y entre adhesión baja y efectos adversos (p = 0,009). No se encontraron diferencias significativas entre los TPO empleados y el grado de adhesión ni con el resto de variables estudiadas. Conclusiones: Aunque nuestros resultados son mejores que los publicados en la bibliografía, consideramos que la adhesión terapéutica en nuestro medio es baja y es prioritario educar a nuestros pacientes en este sentido.(AU)

Introduction: Adherence to oral preventive treatment (OPT) in migraine is often compromised. The aim was to determine the degree of adherence to OPT in migraine patients at three months. Patients and methods: We conducted a multicentre observational study of patients diagnosed with episodic or chronic migraine (criteria of the International Headache Classification, third edition) in whom OPT was initiated. Demographic data (age, gender, educational level, marital status) and disease data (number of attacks, Headache Impact Test-6 and Migraine Disability Assessment Scale scores) were collected. At three months, the Morisky-Green scale was administered, which differentiates levels of adherence: excellent (0), moderate (1-2) and low (3-4). Results: Altogether 100 patients participated in the study: 87% women aged 42 ± 13 years, 14% with chronic migraine; 53.2% of them were beginning their first OPT. Beta-blockers were initiated in 23.2%, tricyclic antidepressants in 35.4%, flunarizine in 21.2%, neuromodulators in 19.2% and antihypertensives in 1%. Severe disability was observed in 56%, and the impact was very severe in 79.5%. Adhesion at three months was excellent in 41.8%, moderate in 28.6% and low in 29.6%. The most frequent reason for discontinuing was the occurrence of adverse effects (44%). A significant relationship was found between excellent adherence and being single (p = 0.046), and between low adherence and adverse effects (p = 0.009). No significant differences were found between the OPT used and the degree of adherence or the other variables studied. Conclusions: Although our results are better than those published in the literature, we consider that therapeutic adherence in our setting is low and educating our patients in this regard is a priority.(AU)

[Degree of compliance with treatment at three months in migraine patients]. / Grado de cumplimiento terapéutico a los tres meses en pacientes con migraña.

INTRODUCTION: Adherence to oral preventive treatment (OPT) in migraine is often compromised. The aim was to determine the degree of adherence to OPT in migraine patients at three months. PATIENTS AND METHODS: We conducted a multicentre observational study of patients diagnosed with episodic or chronic migraine (criteria of the International Headache Classification, third edition) in whom OPT was initiated. Demographic data (age, gender, educational level, marital status) and disease data (number of attacks, Headache Impact Test-6 and Migraine Disability Assessment Scale scores) were collected. At three months, the Morisky-Green scale was administered, which differentiates levels of adherence: excellent (0), moderate (1-2) and low (3-4). RESULTS: Altogether 100 patients participated in the study: 87% women aged 42 ± 13 years, 14% with chronic migraine; 53.2% of them were beginning their first OPT. Beta-blockers were initiated in 23.2%, tricyclic antidepressants in 35.4%, flunarizine in 21.2%, neuromodulators in 19.2% and antihypertensives in 1%. Severe disability was observed in 56%, and the impact was very severe in 79.5%. Adhesion at three months was excellent in 41.8%, moderate in 28.6% and low in 29.6%. The most frequent reason for discontinuing was the occurrence of adverse effects (44%). A significant relationship was found between excellent adherence and being single (p = 0.046), and between low adherence and adverse effects (p = 0.009). No significant differences were found between the OPT used and the degree of adherence or the other variables studied. CONCLUSIONS: Although our results are better than those published in the literature, we consider that therapeutic adherence in our setting is low and educating our patients in this regard is a priority.

TITLE: Grado de cumplimiento terapéutico a los tres meses en pacientes con migraña.Introducción. La adhesión al tratamiento preventivo oral (TPO) en la migraña se ve frecuentemente comprometida. El objetivo fue conocer el grado de adhesión al TPO en pacientes con migraña a los tres meses. Pacientes y métodos. Estudio multicéntrico observacional de pacientes diagnosticados de migraña episódica o crónica (criterios de la Clasificación Internacional de Cefaleas, tercera edición) en los que se iniciaba TPO. Se recogieron datos demográficos (edad, género, nivel estudios, estado civil) y de enfermedad (número de ataques, puntuación en las escalas Headache Impact Test-6 y Migraine Disability Assessment Scale). A los tres meses se pasó la escala de Morisky Green, que diferencia niveles de adhesión: excelente (0), moderada (1-2) y baja (3-4). Resultados. Participaron 100 pacientes, un 87% mujeres de 42 ± 13 años, el 14% con migraña crónica. El 53,2% comenzaba su primer TPO. Se iniciaron betabloqueantes en el 23,2%, antidepresivos tricíclicos en el 35,4%, flunaricina en el 21,2%, neuromoduladores en el 19,2% y antihipertensivos en el 1%. El 56% presentaba discapacidad grave y el 79,5%, impacto muy grave. La adhesión a los tres meses fue excelente en el 41,8%, moderada en el 28,6% y baja en el 29,6%. El motivo más frecuente de discontinuación fueron los efectos adversos (44%). Se encontró una relación significativa entre adhesión excelente y estado civil soltero (p = 0,046), y entre adhesión baja y efectos adversos (p = 0,009). No se encontraron diferencias significativas entre los TPO empleados y el grado de adhesión ni con el resto de variables estudiadas. Conclusiones. Aunque nuestros resultados son mejores que los publicados en la bibliografía, consideramos que la adhesión terapéutica en nuestro medio es baja y es prioritario educar a nuestros pacientes en este sentido.

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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[Isolated hand palsy due to an ischaemic stroke]. / Paralisis aislada de la mano debido a un infarto isquemico.

TITLE: Paralisis aislada de la mano debido a un infarto isquemico.

Parálisis aislada de la mano debido a un infarto isquémico / Isolated hand palsy due to an ischaemic stroke

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[Lacosamide associated with high-degree block in a patient with trigeminal neuralgia]. / Lacosamida asociada a bloqueo de alto grado en un paciente con neuralgia del trigemino.

INTRODUCTION: Lacosamide is an antiepileptic drug whose exact mechanism of action remains unknown. It acts by increasing the slow inactivation of the voltage-dependent sodium channels of the cell membranes. It is indicated in the treatment of focal seizures with or without secondary generalisation and is occasionally used as adjunct treatment in neuropathic pain. Although the most frequent side effects are mild (dizziness, diplopia, blurred vision, headache, tremor, etc.), others such as supraventricular tachyarrhythmias, changes in repolarisation, atrioventricular blocks and even cardiac arrest or sudden death have been reported. CASE REPORT: A 74-year-old male, diagnosed with classic trigeminal neuralgia treated with 200 mg/12 h of carbamazepine, who visited due to a worsening of the pain in the trigeminal V1-V2 region. On the sixth day after admission, after adjusting the carbamazepine treatment to a descending regime, 400 mg/24 h of eslicarbazepine and 100 mg/12 h of intravenous lacosamide, he presented a complete atrioventricular block with extreme bradycardia that required the placement of a pacemaker. CONCLUSIONS: Voltage-dependent sodium channel blockade mainly affects non-sinusal cardiac tissue. An alteration in the atrioventricular or infrahisian node is more consistent with its mechanism of action. Other cases of atrioventricular block in this kind of polytherapy have been reported. Precaution is advised in the concomitant use of antiepileptic drugs, above all among those that prolong the PR interval, and they should be contraindicated in patients with a history of atrioventricular block, ischaemic heart disease or heart failure. Before starting, a baseline electrocardiogram and regular electrocardiographic monitoring are advised during the first few weeks.

TITLE: Lacosamida asociada a bloqueo de alto grado en un paciente con neuralgia del trigemino.Introduccion. La lacosamida es un farmaco antiepileptico cuyo mecanismo de accion exacto se desconoce. Actua aumentando la inactivacion lenta de los canales de sodio dependientes del voltaje de las membranas celulares. Indicado en el tratamiento de crisis focales con o sin generalizacion secundaria, ocasionalmente se emplea como tratamiento coadyuvante en el dolor neuropatico. Aunque los efectos adversos mas frecuentes son leves (mareo, diplopia, vision borrosa, cefalea, temblor…), se han descrito taquiarritmias supraventriculares, cambios en la repolarizacion, bloqueos auriculoventriculares e incluso parada cardiaca o muerte subita. Caso clinico. Varon de 74 años, diagnosticado de neuralgia del trigemino clasica en tratamiento con 200 mg/12 h de carbamacepina, que acude por reagudizacion del dolor en el territorio trigeminal V1-V2. El sexto dia de ingreso, tras ajustar el tratamiento con carbamacepina en pauta descendente, 400 mg/24 h de eslicarbacepina y 100 mg/12 h de lacosamida intravenosa, presenta bloqueo auriculoventricular completo con bradicardia extrema que precisa la implantacion de un marcapasos definitivo. Conclusiones. El bloqueo de canales de sodio dependientes del voltaje afecta predominantemente al tejido cardiaco no sinusal. Una alteracion en el nodo auriculoventricular o infrahisiano es mas congruente con su mecanismo de accion. Existen mas casos comunicados de bloqueo auriculoventricular en este tipo de politerapia. Se recomienda precaucion en el uso concomitante de farmacos antiepilepticos, sobre todo entre los que prolonguen el intervalo PR, asi como su contraindicacion en pacientes con antecedentes de bloqueo auriculoventricular, cardiopatia isquemica o insuficiencia cardiaca. Antes de su inicio, se aconseja realizar un electrocardiograma basal y monitorizacion electrocardiografica regular durante las primeras semanas.

La exploración de la fluidez verbal semántica con tareas alternativas a la categoría «animales» / Exploration of the semantic verbal fluency with alternatives to the «animal» category tasks

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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

[Amnesic stroke caused by hippocampal infarction]. / Ictus amnésico por infarto hipocámpo.

INTRODUCTION: The term amnesic stroke is used to describe a condition in which the dominant symptom is a relatively persistent acute amnesia of a vascular origin. It may appear in cases of lesions in the posterior cerebral artery (PCA), thalamic arteries and anterior choroidal artery, and clinical reports describing this condition are scarce. CASE REPORT: We describe the case of a 77-year-old male with vascular risk factors who presented sudden onset mnemonic deficit that affected basic activities of daily living, and apathy. Examination of the patient revealed an anterograde episodic amnesia with an inability to retain new information and short-term memory was also clearly affected, although access to old memories remained relatively intact. There was no other sensory-motor focus. A CAT scan showed a right-side sylvian infarction and hypodense areas in both hippocampuses. The neuropsychological evaluation revealed global amnesia: CME (in Spanish, MEC) 17/30; working memory with CME 7/27; SVF (animals): 7; the 7-minute test (visual/verbal memory: free recall 2/16, facilitated 9/16); clock test: 3/9. Later progression was good, with recovery of short-term memory and the capacity to retain concepts, and the patient was again able to perform the activities he previously carried out. CONCLUSIONS: There are three amnesic stroke syndromes with different semiological characteristics, depending on the vascular territory, that is, the PCA, thalamic arteries and anterior choroidal artery. In this case, the most striking points are the more intense disorders affecting short-term memory and retention, with relative conservation of long-term memory, and the location of the stroke in the hippocampus. Its good outcome allows it to be distinguished from vascular dementia.

Ictus amnésico por infarto hipocámpico / Amnesic stroke caused by hippocampal infarction

Introducción. El término ictus amnésico describe uncuadro de amnesia aguda relativamente persistente como síntomadominante y de origen vascular. Puede aparecer en las lesiones dela arteria cerebral posterior (ACP), las arterias talámicas y laarteria coroidea anterior. Existen pocas descripciones clínicas.Caso clínico. Varón de 77 años, con factores de riesgo vascular,que presentó déficit de memoria de instauración brusca con repercusiónen las actividades básicas de la vida diaria y apatía. En laexploración destacaba una amnesia episódica anterógrada conimposibilidad de fijar nueva información y una alteración clara dela memoria a corto plazo, con preservación relativa del acceso alos recuerdos antiguos. No existía otra focalidad sensitivomotora.En la TC se objetivó un infarto evolucionado silviano derecho yáreas hipodensas en ambos hipocampos. La evaluación neuropsicológicaobjetivó una amnesia global: MEC, 17/30; memoria detrabajo con MEC, 7/27; FVS (animales), 7; test de los siete minutosen memoria visuoverbal, recuerdo libre, 2/16, y facilitado, 9/16;test del reloj, 3/9. La evolución posterior fue favorable, con recuperaciónde la memoria y la capacidad de fijación a corto plazo eincorporación a sus actividades previas. Conclusiones. Existen tressíndromes de ictus amnésico con características semiológicas diferentesen función del territorio vascular: ACP, arterias talámicas y arteria coroidea anterior. En este caso, destaca la afectación másintensa de la memoria a corto plazo y de la fijación, con preservaciónrelativa de la memoria a largo plazo, junto a la localizaciónhipocámpica del ictus. La buena evolución permite diferenciarlo dela demencia vascular

Introduction. The term amnesic stroke is used to describe a condition in which the dominant symptom is a relativelypersistent acute amnesia of a vascular origin. It may appear in cases of lesions in the posterior cerebral artery (PCA),thalamic arteries and anterior choroidal artery, and clinical reports describing this condition are scarce. Case report. Wedescribe the case of a 77-year-old male with vascular risk factors who presented sudden onset mnemonic deficit that affectedbasic activities of daily living, and apathy. Examination of the patient revealed an anterograde episodic amnesia with aninability to retain new information and short-term memory was also clearly affected, although access to old memoriesremained relatively intact. There was no other sensory-motor focus. A CAT scan showed a right-side sylvian infarction andhypodense areas in both hippocampuses. The neuropsychological evaluation revealed global amnesia: CME (in Spanish,MEC) 17/30; working memory with CME 7/27; SVF (animals): 7; the 7-minute test (visual/verbal memory: free recall 2/16,facilitated 9/16); clock test: 3/9. Later progression was good, with recovery of short-term memory and the capacity to retainconcepts, and the patient was again able to perform the activities he previously carried out. Conclusions. There are threeamnesic stroke syndromes with different semiological characteristics, depending on the vascular territory, that is, the PCA,thalamic arteries and anterior choroidal artery. In this case, the most striking points are the more intense disorders affectingshort-term memory and retention, with relative conservation of long-term memory, and the location of the stroke in thehippocampus. Its good outcome allows it to be distinguished from vascular dementia

[The supermarket test: preliminary normative data in our milieu]. / Test del supermercado: datos normativos preliminares en nuestro medio.

INTRODUCTION: Semantic verbal fluency (SVF) tests are often used in basic neuropsychological evaluation. They are not time consuming and are easy to apply, but the normative data have been validated mainly for the Anglo-Saxon population, which can lead us to make mistakes in classifying normality. AIMS: To evaluate the category 'things you can buy at a supermarket' as a task for exploring SVF applied to a Castilian-speaking population of Spaniards with the aim of conducting a pilot normative test in our milieu. SUBJECTS AND METHODS: The 'things you can buy at a supermarket' task was applied to a sample of 139 healthy subjects without cognitive impairment, whose mother tongue is Spanish. The subjects were subclassified according to their level of schooling in years, age groups and sex. RESULTS: Total mean production (1 minute) = 20.1 +/- 8. No differences were seen in the comparative analysis according to sexes. By age: < 50 years = 33 +/- 6; 50-59 = 24.6 +/- 6; 60-69 = 16.5 +/- 5; 70-79 = 15.5 +/- 6; > 79 years = 13.5 +/- 6. By years of schooling: < 10 years = 19 +/- 6; > 10 years = 29 +/- 5. CONCLUSIONS: Mean output of words is 20 in one minute, with a percentile distribution where the deficit criterion (p10) would be in an output below 10 words. Overall, greater output is observed in the first half minute. There are a number of socio-demographic factors, such as age and mean number of years of schooling, that have been proved statistically to exert an influence on semantic capacity in this test. No differences were observed according to sex. We present the mean results, as well as the overall percentile distribution and results according to age and schooling, because we think they can serve as preliminary normative data in our milieu.

Test del supermercado: datos normativos preliminares en nuestro medio / The supermarket test: preliminary normative data in our milieu

Introducción. Los tests de fluidez verbal semántica (FVS) se utilizan frecuentemente en la evaluación neuropsicológica básica. Requieren poco tiempo y se aplican fácilmente, pero los datos normativos se han validado, principalmente, para una población anglosajona, lo que nos puede conducir a clasificaciones erróneas de la normalidad. Objetivo. Evaluar la categoría 'cosas que se pueden comprar en un supermercado' como tarea en la exploración de la FVS, aplicada a población española castellanoparlante, con la finalidad de realizar un estudio normativo piloto en nuestro medio. Sujetos y métodos. Sobre una muestra de 139 sujetos sanos, con el castellano como lengua materna, sin deterioro cognitivo, se aplicó la tarea 'cosas que se pueden comprar en un supermercado'. Los sujetos se subclasificaron según el nivel de escolarización en años, por grupos de edad y por sexo. Resultados. Producción media total (1 minuto) = 20,1 ñ 8. El análisis comparativo por sexo no demuestra diferencias. Por edad: 79 años = 13,5 ñ 6. Por escolaridad en años: 10 años = 29 ñ 5. Conclusiones. La producción media de palabras es de 20 en un minuto, con una distribución por percentil donde el criterio de déficit (p10) se situaría en una producción inferior a 10 palabras. De manera global, se observa una mayor producción en la primera mitad del minuto. Existen factores sociodemográficos que han demostrado estadísticamente su influencia en la capacidad semántica de este test, como la edad y la escolaridad medida en años. No se han observado diferencias según el sexo. Aportamos los resultados medios, la distribución por percentil global y según la edad y la escolaridad, pues consideramos que pueden servir como datos normativos preliminares en nuestro medio (AU)

Introduction. Semantic verbal fluency (SVF) tests are often used in basic neuropsychological evaluation. They are not time consuming and are easy to apply, but the normative data have been validated mainly for the Anglo-Saxon population, which can lead us to make mistakes in classifying normality. Aims. To evaluate the category ‘things you can buy at a supermarket’ as a task for exploring SVF applied to a Castilian-speaking population of Spaniards with the aim of conducting a pilot normative test in our milieu. Subjects and methods. The ‘things you can buy at a supermarket’ task was applied to a sample of 139 healthy subjects without cognitive impairment, whose mother tongue is Spanish. The subjects were subclassified according to their level of schooling in years, age groups and sex. Results. Total mean production (1 minute) = 20.1 ± 8. No differences were seen in the comparative analysis according to sexes. By age: < 50 years = 33 ± 6; 50-59 = 24.6 ± 6; 60-69 = 16.5 ± 5; 70-79 = 15.5 ± 6; > 79 years = 13.5 ± 6. By years of schooling: < 10 years = 19 ± 6; > 10 years = 29 ± 5. Conclusions. Mean output of words is 20 in one minute, with a percentile distribution where the deficit criterion (p10) would be in an output below 10 words. Overall, greater output is observed in the first half minute. There are a number of sociodemographic factors, such as age and mean number of years of schooling, that have been proved statistically to exert an influence on semantic capacity in this test. No differences were observed according to sex. We present the mean results, as well as the overall percentile distribution and results according to age and schooling, because we think they can serve as preliminary normative data in our milieu (AU)

[Paraproteinemic neuropathies: foundations for a specific therapy related to patients' quality of life]. / Neuropatias paraproteinemicas: bases para un tratamiento especifico con relacion a la calidad de vida de los pacientes.

INTRODUCTION: Monoclonal gammopathy (MG) is reported in 1% of subjects above 50 years of age and in 3% of those above the age of 70. Of all patients with MG, 3% present polyneuropathy (PNP). The abbreviation MGUS (monoclonal gammopathy of undetermined significance) is used to describe a benign proliferation of M component (monoclonal) according to Kyle's criteria, with a potential to be malignant that is indicated by clinical, biochemical and haematological parameters. Aims. The aim of this study was to evaluate the effect of the clinical exacerbation of MGUS associated neuropathy, by referring to the existing literature and to our own experience in order to set out a number of proposals for treatment based on the deterioration of the patient's quality of life. CASE REPORTS: Case 1: a 53 year old female with predominantly sensory sensory motor demyelinating polyradiculoneuropathy, associated to IgG lambda MGUS. Case 2: a 65 year old female with demyelinating sensory motor polyradiculoneuropathy associated to IgM kappa MGUS. In both cases there was a rapidly progressing neurological deterioration and no other data exist to suggest malign lymphoproliferation. CONCLUSIONS: In addition to the treatments established for MG linked PNP, which are based on the modulation of the immune response, it has also been proved that when malign proliferation of plasmacytes exists specific treatment of the gammopathy can significantly stabilise or improve the neuropathic symptoms. We propose gammopathy specific haematological treatment in patients who still meet Kyle's criteria for MGUS with a torpid neurological course and scarce response to therapy with immunomodulators, prior to an important deterioration in the quality of life. We also suggest establishing this rapidly progressing clinical course as a criterion that indicates the process of becoming malign as put forward by Eurelings et al.

[Stroke as the first manifestation of a concealed pancreatic neoplasia]. / Ictus como primera manifestación de una neoplasia pancreática oculta.

INTRODUCTION: Stroke is very seldom seen as the first manifestation of a neoplasia. The mechanisms that are most frequently involved in its pathogenesis are Trousseau's syndrome, haematological disorders such as disseminated intravascular coagulation or thrombocytosis, and the release of mucin by the tumour. Secondarily, non bacterial thrombotic endocarditis, septic or neoplastic cell embolism, venous sinus thrombosis and atherosclerosis secondary to radiotherapy have been reported. CASE REPORTS: Case 1: a 27-year-old male who presented a stroke in the right thalamus with a torpid progression and digestive complications; neuroimaging studies revealed unknown neoplasia of the head of the pancreas. Case 2: a 53-year-old male with repeated stroke (left sylvian and right occipitotemporal) and progressive neurological deterioration secondary to disseminated neoplastic disease, which had its origins in the body and tail of the pancreas. In both cases all the diagnostic explorations carried out in an attempt to determine the aetiopathogenesis of the stroke were negative. The post mortem study in the second patient revealed the existence of a non bacterial thrombotic endocarditis, which was taken as being one of the mechanisms involved. CONCLUSIONS: The pathogenic mechanisms of cerebral ischemia associated to neoplasia are discussed and the primary and secondary forms are differentiated. We highlight the idea that when dealing with a stroke with an unknown aetiology and a torpid progression, the neurologist must consider the existence of a concealed neoplasia with stroke as its first manifestation.

[Migraine with pleocytosis: a case of atypical progression]. / Migraña con pleocitosis: un caso de evolucion atipica.

AIMS: Migraine with cerebrospinal fluid pleocytosis is a clinical pattern consisting in intermittent headaches accompanied by episodes of transient neurological deficit and lymphocytic pleocytosis, which last for varying lengths of time and have a spontaneous resolution. We describe the case of migraine with pleocytosis (MP) with atypical progression that responded well to corticoids. CASE REPORT: A 27 year old male with no familial or personal history of migraine who began with bouts of intense headaches that were preceded by neurological deficit (dysphasia and hemiparesis of the right hand side) lasting varying amounts of time (including hours). The serial study of cerebrospinal fluid (CSF) revealed a notable degree of lymphocytosis (400/mm3) with increased intracranial pressure (ICP) (> 30 mmHg). The other explorations that were performed (microbiological study of CSF and blood, humoral and cellular immunity, MR angiography and thyroid hormones, among others) were all normal. The neurological exploration carried out in the following days revealed a mild paresis of the right lateral rectus muscle, which was attributed to the intracranial hypertension and which clearly disabled the patient. As a consequence of the recurrence of the seizures and the long lasting increase in ICP, therapy was begun with 1 mg/kg/day of prednisone, at a descending rate; clinical remission and normalisation of the CSF was achieved within a period of two months. DISCUSSION: We describe this case of MP because of how atypical the progression was (paresis of the right abducent nerve secondary to the increase in ICP) and the possibility of associating corticoids in cases in which the benignity of the entity is in doubt.

Migraña con pleocitosis: un caso de evolución atípica / Migraine with pleocytosis: a case of atypical progression

Introducción. La migraña con pleocitosis (MP) de llíquido cefalorraquídeo (LCR) es un cuadro clínico consistente en cefaleas intermitentes acompañadas de episodios deficitarios neurológicos transitorios y pleocitosis linfocitaria, que se suceden durante un período variable y con resolución espontánea. Presentamos un caso de MP de evolución atípica con buena respuesta a corticoides. Caso clínico. Se trata de un varón de 27 años, sin antecedentes familiares ni personales de migraña, que inicia con episodios de cefalea intensa, precedidos de déficit neurológico (disfasia y hemiparesia derecha) de duración variable (incluso horas). El estudio seriado del LCR reveló una marcada linfocitosis (400/mm3) con incremento de la presión intracraneal (PIC) (más de 30 mmHg). El resto de las exploraciones practicadas (estudio microbiológico de LCR y sangre, inmunidad humoral y celular, angiorresonancia magnética y hormonas tiroideas, entre otras) fueron normales. La exploración neurológica en los días posteriores reveló una leve paresia del recto externo derecho, que se atribuyó a su hipertensión intracraneal y que claramente incapacitó al paciente. Dada la recurrencia de las crisis y la persistente elevación de la PIC, se inicia un tratamiento con prednisona (1 mg/kg/día, en pauta descendente) y se consigue la remisión clínica y la normalización del LCR en el plazo de dos meses. Discusión. Aportamos la descripción de este paciente afectado de MP por la atipicidad de su evolución (paresia del VI par derecho secundaria al aumento de PIC) y la posibilidad de asociar corticoides en aquellos casos en que se cuestione la benignidad de dicha entidad (AU)

Aims. Migraine with cerebrospinal fluid pleocytosis is a clinical pattern consisting in intermittent headaches accompanied by episodes of transient neurological deficit and lymphocytic pleocytosis, which last for varying lengths of time and have a spontaneous resolution. We describe the case of migraine with pleocytosis (MP) with atypical progression that responded well to corticoids. Case report. A 27-year-old male with no familial or personal history of migraine who began with bouts of intense headaches that were preceded by neurological deficit (dysphasia and hemiparesis of the right-hand side) lasting varying amounts of time (including hours). The serial study of cerebrospinal fluid (CSF) revealed a notable degree of lymphocytosis (400/mm3 ) with increased intracranial pressure (ICP) (> 30 mmHg). The other explorations that were performed (microbiological study of CSF and blood, humoral and cellular immunity, MR angiography and thyroid hormones, among others) were all normal. The neurological exploration carried out in the following days revealed a mild paresis of the right lateral rectus muscle, which was attributed to the intracranial hypertension and which clearly disabled the patient. As a consequence of the recurrence of the seizures and the long-lasting increase in ICP, therapy was begun with 1 mg/kg/day of prednisone, at a descending rate; clinical remission and normalisation of the CSF was achieved within a period of two months. Discussion. We describe this case of MP because of how atypical the progression was (paresis of the right abducent nerve secondary to the increase in ICP) and the possibility of associating corticoids in cases in which the benignity of the entity is in doubt (AU)

. Aims. Migraine with cerebrospinal fluid pleocytosis is a clinical pattern consisting in intermittent headaches accompanied by episodes of transient neurological deficit and lymphocytic pleocytosis, which last for varying lengths of time and have a spontaneous resolution. We describe the case of migraine with pleocytosis (MP) with atypical progression that responded well to corticoids. Case report. A 27-year-old male with no familial or personal history of migraine who began with bouts of intense headaches that were preceded by neurological deficit (dysphasia and hemiparesis of the right-hand side) lasting varying amounts of time (including hours). The serial study of cerebrospinal fluid (CSF) revealed a notable degree of lymphocytosis (400/mm3 ) with increased intracranial pressure (ICP) (> 30 mmHg). The other explorations that were performed (microbiological study of CSF and blood, humoral and cellular immunity, MR angiography and thyroid hormones, among others) were all normal. The neurological exploration carried out in the following days revealed a mild paresis of the right lateral rectus muscle, which was attributed to the intracranial hypertension and which clearly disabled the patient. As a consequence of the recurrence of the seizures and the long-lasting increase in ICP, therapy was begun with 1 mg/kg/day of prednisone, at a descending rate; clinical remission and normalisation of the CSF was achieved within a period of two months. Discussion. We describe this case of MP because of how atypical the progression was (paresis of the right abducent nerve secondary to the increase in ICP) and the possibility of associating corticoids in cases in which the benignity of the entity is in doubt (AU)

Ictus como primera manifestación de una neoplasia pancreática oculta / Stroke as the first manifestation of a concealed pancreatic neoplasia

Introducción. El ictus como primera manifestación de una neoplasia es excepcional. Los mecanismos patogénicos más frecuentemente implicados son el síndrome de Trousseau, trastornos hematológicos, como la coagulación intravascular diseminada o la trombocitosis, y la liberación de mucina por parte del tumor. Secundariamente, se han apuntado, entre otros, la endocarditis trombótica no bacteriana, el embolismo séptico o de células neoplásicas, las trombosis de senos venosos y la aterosclerosis secundaria a radioterapia. Casos clínicos. Caso 1: varón de 27 años de edad que presenta ictus talámico derecho de evolución tórpida, con complicaciones digestivas; en los estudios de neuroimagen se halla eoplasia de la cabeza del páncreas no conocida. Caso 2: varón de 53 años con ictus de repetición (silviano izquierdo y temporooccipital derecho), con deterioro neurológico progresivo secundario a una enfermedad neoplásica diseminada con origen en el cuerpo y la cola del páncreas. En ambos casos, todas las exploraciones diagnósticas realizadas dirigidas a conocer la etiopatogenia del ictus fueron negativas. El estudio necrópsico en el segundo paciente reveló la existencia de una endocarditis trombótica no bacteriana, que se apuntó como uno de los mecanismos implicados. Conclusiones. Se discuten los mecanismos patogénicos de isquemia cerebral asociados a neoplasia, y se distingue entre primarios y secundarios. Se destaca la idea de que, ante un ictus de etiología no filiada y evolución tórpida, el neurólogo debe plantearse la existencia de una neoplasia oculta con un ictus como primera manifestación (AU)

Introduction. Stroke is very seldom seen as the first manifestation of a neoplasia. The mechanisms that are most frequently involved in its pathogenesis are Trousseau’s syndrome, haematological disorders such as disseminated intravascular coagulation or thrombocytosis, and the release of mucin by the tumour. Secondarily, non-bacterial thrombotic endocarditis, septic or neoplastic cell embolism, venous sinus thrombosis and atherosclerosis secondary to radiotherapy have been reported. Case reports. Case 1: a 27-year-old male who presented a stroke in the right thalamus with a torpid progression and digestive complications; neuroimaging studies revealed unknown neoplasia of the head of the pancreas. Case 2: a 53-year-old male with repeated stroke (left sylvian and right occipitotemporal) and progressive neurological deterioration secondary to disseminated neoplastic disease, which had its origins in the body and tail of the pancreas. In both cases all the diagnostic explorations carried out in an attempt to determine the aetiopathogenesis of the stroke were negative. The postmortem study in the second patient revealed the existence of a non-bacterial thrombotic endocarditis, which was taken as being one of the mechanisms involved. Conclusions. The pathogenic mechanisms of cerebral ischemia associated to neoplasia are discussed and the primary and secondary forms are differentiated. We highlight the idea that when dealing with a stroke with an unknown aetiology and a torpid progression, the neurologist must consider the existence of a concealed neoplasia with stroke as its first manifestation (AU)

[Visual neglect secondary to left frontal haemorrhage]. / Negligencia visual secundaria a hemorragia frontal izquierda.

INTRODUCTION: The term visual neglect refers to a lack of attention to visual stimuli coming from the contralateral hemifield. The patient does not seek objects in the abnormal field and often only sees half of a paragraph when reading. Although the right inferior parietal lobe is the most frequently damaged region, the same symptomatology can be due to injury to the frontal lobe, the basal ganglia and the thalamus. CASE REPORT: Female aged 71 with a mitral prosthesis and atrial fibrillation in treatment with dicumarols, who presented a left frontal parenchymatous haemorrhage. The exploration revealed right visual hemineglect without ipsilateral homonymous hemianopsia, associated to a right hemiparesis. DISCUSSION: Even when visual neglect has been described in lesions affecting the parietal lobe, especially on the right hand side, the same symptomatology can be due to injury to the basal ganglia, the thalamus and the frontal lobe. In this last case, it seems that the basis for the pathogenesis lies in the damage done to the underlying white matter, which would disconnect the posterior parietal cortex from the prefrontal cortex.