Estudio sobre el conocimiento de la población acerca del ictus y de los factores de riesgo vascular / Study into what the population knows about strokes and vascular risk factors

Introducción. La aplicación de tratamientos en fase aguda del ictus obliga a reducir al máximo el tiempo de llegada de los pacientes al hospital. Uno de los factores de demora en la atención es el desconocimiento de la enfermedad por la población. Sujetos y métodos. Nuestro objetivo es analizar el conocimiento que tiene la población del ictus y de los factores de riesgo vascular, así como la actitud ante éste. Para ello hemos realizado entrevistas a pacientes que acuden a consultas de neurología y a sus familiares. Resultados. Han sido 386 entrevistas, 158 mujeres y 228 hombres, con una edad media de 52 años. 230 sujetos (59%) no conocen el término ictus, aunque sí reconocen otros, como infarto o embolia. 175 (45%) no conocen ningún síntoma de infarto cerebral o responden incorrectamente. El síntoma más reconocido (86%) es la pérdida de fuerza en un hemicuerpo. Un 32% de los entrevistados no toma una actitud correcta ante los síntomas del ictus y, si se tratase de un ataque isquémico transitorio, un 25% no lo consideraría urgente. 198 (51%) no conocen ningún factor de riesgo vascular. De los que responden correctamente, el más nombrado es la hipertensión arterial. Los factores relacionados de forma independiente con un mejor conocimiento de la enfermedad son el nivel cultural alto y la edad joven. Conclusión. Existe un escaso conocimiento en la población del ictus y los factores de riesgo vascular. Son necesarias campañas informativas para mejorar la actitud de la población ante la enfermedad (AU)

Introuction. The application of treatments in the acute phase of a stroke makes it necessary to reduce as far as possible the time required for patients to reach hospital. One of the factors associated with delayed care is the population’s scant knowledge about the disease. Subjects and methods. Our aim is to analyse what the population knows about strokes and vascular risk factors, as well as their attitude towards it. To do so, we interviewed both patients who visited neurology departments and their relatives. Results. Altogether 386 interviews were carried out, with 158 females and 228 males and a mean age of 52 years. Two hundred and thirty subjects (59%) were unfamiliar with the Spanish term ictus, although they had heard of others, such as infarto or embolia. One hundred and seventy-five (45%) did not know any symptoms of a completed stroke, or answered incorrectly. The most widely recognised symptom (86%) is (partial) paralysis in one side of the body. Almost a third (32%) of the interviewees would not take a correct attitude if faced with symptoms of a stroke and if it were a transient ischaemic attack, 25% would not think it was serious. One hundred and ninety-eight (51%) did not know any vascular risk factors. Of those who answered correctly, the most frequently named factor is arterial hypertension. The factors related independently with a better knowledge of the disease are a high educational level and being young. Conclusions. The general population knows little about strokes and vascular risk factors. Information campaigns are needed to improve the population’s attitude towards this disease (AU)

[Study into what the population knows about strokes and vascular risk factors]. / Estudio sobre el conocimiento de la población acerca del ictus y de los factores de riesgo vascular.

INTRODUCTION: The application of treatments in the acute phase of a stroke makes it necessary to reduce as far as possible the time required for patients to reach hospital. One of the factors associated with delayed care is the population's scant knowledge about the disease. SUBJECTS AND METHODS: Our aim is to analyse what the population knows about strokes and vascular risk factors, as well as their attitude towards it. To do so, we interviewed both patients who visited neurology departments and their relatives. RESULTS: Altogether 386 interviews were carried out, with 158 females and 228 males and a mean age of 52 years. Two hundred and thirty subjects (59%) were unfamiliar with the Spanish term ictus, although they had heard of others, such as infarto or embolia. One hundred and seventy-five (45%) did not know any symptoms of a completed stroke, or answered incorrectly. The most widely recognised symptom (86%) is (partial) paralysis in one side of the body. Almost a third (32%) of the interviewees would not take a correct attitude if faced with symptoms of a stroke and if it were a transient ischaemic attack, 25% would not think it was serious. One hundred and ninety-eight (51%) did not know any vascular risk factors. Of those who answered correctly, the most frequently named factor is arterial hypertension. The factors related independently with a better knowledge of the disease are a high educational level and being young. CONCLUSIONS: The general population knows little about strokes and vascular risk factors. Information campaigns are needed to improve the population's attitude towards this disease.

[Ischaemic stroke in a patient with giant aneurysms in the internal carotid]. / Ictus isquémico en un paciente conaneurismas gigantes de carótida interna.

INTRODUCTION: Presentation of signs and symptoms of haemorrhage and/or ischaemia associated to an intracranial dissecting aneurysm is quite frequent. CASE REPORT: A 77-year-old male with a history of a stroke probably due to a cardioembolic causation, and consequently anticoagulation therapy was established. Ten years later, a tomography scan performed because of persistent headaches revealed the presence of fusiform mirror aneurysms in both supraclinoid carotids and early stages of development in the two middle cerebral arteries with predominance of the left-hand side. The anticoagulation therapy was withdrawn. Said aneurysmal alterations did not exist in the previous study, and so they are thought to have originated due to spontaneous dissection. A month later the patient suffered a stroke in the territory of the right middle cerebral artery, caused by partial occlusion of the aneurysm by a thrombus that gave rise to turbulent flow; distal micro-embolisms were also detected in the right middle cerebral artery. Our aetiological hypothesis, in view of the way events progressed, is an arterio-arterial embolism from the aneurysmal thrombus. Supported by data from the specialised literature available on the matter, we decided to implement surgical treatment, although this possibility was rejected by the family; the decision was thus taken to establish an antiaggregating treatment regimen and follow-up. CONCLUSIONS: Giant aneurysms are a potential source of haemorrhagic events, but we must not forget that secondary ischaemic events may also appear; more especially, the aneurysmal lumen can become partially occluded by thrombi and these then become the focus point for embolic events. A complete neuro-ultrasonographic study would be a very appropriate option with which to tailor the therapeutic decision to each patient.

Ictus isquémico en un paciente con aneurismas gigantes de carótida interna / Ischaemic stroke in a patient with giant aneurysms in the internal carotid

La presentación de cuadros hemorrágicos y/o isquémicos asociados a un aneurisma disecante intracranealno es un hecho infrecuente. Caso clínico. Varón de 77 años, con antecedente de accidente cerebrovascular de probable origen cardioembólico, por lo que fue anticoagulado. Diez años después, tras realizarle una tomografía por cefalea persistente,se objetivaron aneurismas fusiformes en espejo en ambas carótidas supraclinoideas e inicio en ambas arterias cerebrales medias con predominio de la izquierda, por lo que se suspendió el tratamiento anticoagulante. Dichas alteraciones aneurismáticas no existían en el estudio previo, por lo que se presupone que se originaron por disección espontánea. Al mes, el paciente sufre un ictus agudo en el territorio de la arteria cerebral media derecha, debido a la oclusión parcial del aneurismapor un trombo que provoca un flujo turbulento, y además se detectan microembolias distales en la arteria cerebral media derecha. Nuestra hipótesis etiológica, según el desarrollo de los acontecimientos, es un embolismo arterioarterial desde eltrombo aneurismático. Apoyados por la bibliografía disponible, decidimos plantear un tratamiento quirúrgico, si bien éste fue rechazado por la familia, por lo que se decidió pautar tratamiento antiagregante y seguimiento. Conclusiones. Los aneurismasgigantes constituyen una fuente potencial de eventos hemorrágicos, pero no debemos olvidar que también pueden aparecer eventos isquémicos secundarios, en especial si puede ocluirse parcialmente la luz aneurismática mediante trombos y ser éstos un foco de eventos embólicos. El estudio neuroecográfico completo sería una opción muy adecuada con la que individualizarla decisión terapéutica de cada paciente

Presentation of signs and symptoms of haemorrhage and/or ischaemia associated to an intracranialdissecting aneurysm is quite frequent. Case report. A 77-year-old male with a history of a stroke probably due to a cardioembolic causation, and consequently anticoagulation therapy was established. Ten years later, a tomography scan performed because of persistent headaches revealed the presence of fusiform mirror aneurysms in both supraclinoid carotids and early stages of development in the two middle cerebral arteries with predominance of the left-hand side. The anticoagulation therapy was withdrawn. Said aneurysmal alterations did not exist in the previous study, and so they are thought to have originated due to spontaneous dissection. A month later the patient suffered a stroke in the territory of the right middle cerebral artery, caused by partial occlusion of the aneurysm by a thrombus that gave rise to turbulent flow; distal microembolisms were also detected in the right middle cerebral artery. Our aetiological hypothesis, in view of the way events progressed, is an arterio-arterial embolism from the aneurysmal thrombus. Supported by data from the specialised literature available on the matter, we decided to implement surgical treatment, although this possibility was rejected by the family; the decision was thus taken to establish an antiaggregating treatment regimen and follow-up. Conclusions. Giant aneurysms are a potential source of haemorrhagic events, but we must not forget that secondary ischaemic events may also appear; more especially, the aneurysmal lumen can become partially occluded by thrombi and these then become the focus point for embolic events. A complete neuro-ultrasonographic study would be a very appropriate option with which to tailor the therapeutic decision to each patient

[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

Alteraciones de la migración neuronal: una causa de epilepsia farmacorresistente curable / Neuronal migration disorders: a cause of curable medication resistant epilepsy

Introducción. La displasia cortical focal es una alteraciónde la migración neuronal que se manifiesta con crisis epilépticashabitualmente farmacorresistentes. La mejoría en las técnicasde neuroimagen durante los últimos años ha permitido el diagnósticoy tratamiento quirúrgico de algunos pacientes tratados en politerapiadurante muchos años con mal control de las crisis. Caso clínico.Varón de 22 años con crisis epilépticas desde los 18 meses,refractario a múltiples combinaciones de fármacos antiepilépticos(FAE) orales con electroencefalogramas que mostraban descargasfrontales, punta-onda rápida, de mayor expresión izquierda y técnicasde neuroimagen repetidamente normales. Tras su último ingresopor reagudización de las crisis se repitió el estudio de neuroimageny se observó displasia cortical focal frontal izquierda, por loque se inició valoración neuroquirúrgica. En la actualidad, seis mesesdespués de la intervención, el paciente no ha presentado nuevascrisis epilépticas y se ha iniciado la reducción del tratamiento conFAE orales. Conclusión. Resulta necesario repetir estudios de neuroimagenen pacientes estudiados previamente ante la posibilidadde rescatar de la refractariedad a un grupo de pacientes condenadosa mal control de sus crisis junto con los efectos secundarios demuchos de los FAE

Introduction. Focal cortical dysplasia is a neuronal migration disorder that appears with bouts of epileptic seizuresthat are usually medication resistant. The improvements introduced into neuroimaging techniques in recent years have madeit possible to diagnose and offer surgical treatment to certain patients who have been under polytherapy for many years withpoor control over their seizures. Case report. We report the case of a 22-year-old male with epileptic fits since the age of 18months, who was refractory to multiple combinations of oral antiepileptic drugs (AEDs) with electroencephalograms thatdisplayed frontal, fast spike-wave discharges, with greater expression on the left side and findings from neuroimaging techniquesthat were repeatedly normal. After being readmitted to hospital because his seizures had got worse, the neuroimaging studywas repeated and left frontal focal cortical dysplasia was observed, which led us to start considering neurosurgery. At present,six months after the intervention, the patient has had no further convulsive fits and therapy with oral AEDs is being reduced.Conclusions. Neuroimaging studies must be repeated in patients that have already been examined because in this way it maybe possible to save a group of patients (who would otherwise have to resign to poor control over their seizures as well as theside effects of many AEDs) from becoming medication resistant

[Neuronal migration disorders: a cause of curable medication resistant epilepsy]. / Alteraciones de la migración neuronal: una causa de epilepsia farmacorresistente curable.

INTRODUCTION: Focal cortical dysplasia is a neuronal migration disorder that appears with bouts of epileptic seizures that are usually medication resistant. The improvements introduced into neuroimaging techniques in recent years have made it possible to diagnose and offer surgical treatment to certain patients who have been under polytherapy for many years with poor control over their seizures. CASE REPORT: We report the case of a 22-year-old male with epileptic fits since the age of 18 months, who was refractory to multiple combinations of oral antiepileptic drugs (AEDs) with electroencephalograms that displayed frontal, fast spike-wave discharges, with greater expression on the left side and findings from neuroimaging techniques that were repeatedly normal. After being readmitted to hospital because his seizures had got worse, the neuroimaging study was repeated and left frontal focal cortical dysplasia was observed, which led us to start considering neurosurgery. At present, six months after the intervention, the patient has had no further convulsive fits and therapy with oral AEDs is being reduced. CONCLUSIONS: Neuroimaging studies must be repeated in patients that have already been examined because in this way it may be possible to save a group of patients (who would otherwise have to resign to poor control over their seizures as well as the side effects of many AEDs) from becoming medication resistant.

[Language and cognitive impairment: a semiological study into visual naming]. / Lenguaje y deterioro cognitivo: un estudio semiológico en denominación visual.

INTRODUCTION: The presence of anomia and/or paraphasias in patients with cognitive impairment suggests an associated deterioration of their language. Visual naming makes it possible to examine these signs in a controlled manner as the target word is already known. AIM. To conduct a semiological study of naming in normal aging, cognitive impairment and language impairment conditions. SUBJECTS AND METHODS: The study consisted in an analysis of nine types of signs (correct response, increased latencies, circumlocutions, absence of response, semantic verbal paraphasias, verbal paraphasias with a similar form, unrelated verbal paraphasias, phonemic paraphasias and neologisms) in a visual naming task (6 items), in two groups with normal language--controls > 70 years and patients with Alzheimer's disease (AD) with onset of amnesia--and two groups with language impairment--vascular aphasia and anomic AD-. RESULTS: Patients failed to perform naming correctly in 4.2% of the responses in controls, 10% in patients with amnesic AD, 30% in patients with vascular aphasia and 50% in patients with anomic AD. Semantic paraphasias were observed in the two groups with normal language abilities (controls and amnesic AD), although frequencies were low. Signs that suggest difficulties in accessing/retrieving lexical items were more frequent in patients with AD and ran parallel to the degree of anomia. One notable finding was the absence of signs of phonological dysfunction in the two groups with AD, regardless of the degree of anomia. CONCLUSIONS: Semiological quantification makes it possible to distinguish differences in the degree of anomia and in the pattern of errors both in controls and in patients with amnesic onset AD and among patients with vascular aphasia and neurodegenerative anomia.

Lenguaje y deterioro cognitivo: un estudio semiológico en denominación visual / Language and cognitive impairment: a semiological study into visual naming

Introducción. La presencia de anomia y/o parafasias enpacientes con deterioro cognitivo sugiere un deterioro asociado dellenguaje. La denominación visual permite explorar estos signos deforma controlada al conocerse la palabra diana. Objetivo. Estudiosemiológico de la denominación en el envejecimiento normal, deteriorocognitivo y deterioro del lenguaje. Sujetos y métodos. Análisisde nueve tipos de signos (respuesta correcta, incremento de latencias,circunloquios, ausencia de respuesta, parafasias verbales semánticas,parafasias verbales con similitud formal, parafasias verbalesno relacionadas, parafasias fonémicas y neologismos), en unatarea de denominación visual (6 ítems), en dos grupos con lenguajenormal –controles > 70 años y pacientes con enfermedad de Alzheimer(EA) de inicio amnésico– y dos grupos con deterioro del lenguaje–afasia vascular y EA anómico–. Resultados. La denominaciónfalló en el 4,2% de las respuestas en controles, el 10% en pacientescon EA amnésico, el 30% en pacientes con afasia vascular y el 50%en pacientes con EA anómico. En los dos grupos con lenguaje normal(controles y EA amnésico) se observaron parafasias semánticas,con frecuencias bajas. Los signos que sugieren dificultad en el acceso/recuperación léxica fueron más frecuentes en los pacientes conEA, de forma paralela al grado de anomia. Destaca la ausencia designos de disfunción fonológica en los dos grupos con EA, de formaindependiente del grado de anomia. Conclusión. La cuantificaciónsemiológica permite objetivar diferencias en el grado de anomia yen el perfil de errores, tanto entre controles y pacientes con EA deinicio amnésico, como entre los pacientes con afasia vascular y anomianeurodegenerativa

Introduction. The presence of anomia and/or paraphasias in patients with cognitive impairment suggests an associateddeterioration of their language. Visual naming makes it possible to examine these signs in a controlled manner as the targetword is already known. Aim. To conduct a semiological study of naming in normal aging, cognitive impairment and languageimpairment conditions. Subjects and methods. The study consisted in an analysis of nine types of signs (correct response,increased latencies, circumlocutions, absence of response, semantic verbal paraphasias, verbal paraphasias with a similarform, unrelated verbal paraphasias, phonemic paraphasias and neologisms) in a visual naming task (6 items), in two groupswith normal language –controls > 70 years and patients with Alzheimer’s disease (AD) with onset of amnesia– and two groupswith language impairment –vascular aphasia and anomic AD–. Results. Patients failed to perform naming correctly in 4.2% ofthe responses in controls, 10% in patients with amnesic AD, 30% in patients with vascular aphasia and 50% in patients withanomic AD. Semantic paraphasias were observed in the two groups with normal language abilities (controls and amnesic AD),although frequencies were low. Signs that suggest difficulties in accessing/retrieving lexical items were more frequent in patientswith AD and ran parallel to the degree of anomia. One notable finding was the absence of signs of phonological dysfunction inthe two groups with AD, regardless of the degree of anomia. Conclusions. Semiological quantification makes it possible todistinguish differences in the degree of anomia and in the pattern of errors both in controls and in patients with amnesic onsetAD and among patients with vascular aphasia and neurodegenerative anomia

Afasia como única manifestación de estado epiléptico parcial / Aphasia as the sole symptom of partial status epilepticus

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[Progressive myoclonic cerebellar ataxia as a manifestation of Creutzfeldt-Jakob disease]. / Ataxia cerebelosa mioclónica progresiva como manifestación de la enfermedad de Creutzfeldt-Jakob.

INTRODUCTION: Progressive myoclonic cerebellar ataxia is a clinical entity with an important spectrum of possible diagnoses that requires a complex and exhaustive differential diagnosis. CASE REPORT: A 53-year-old male patient with no relevant medical history who was admitted to hospital because of an unstable gait, together with mild bilateral dysymmetry and the progressive and insidious widening of the base of support. The patient's symptoms then became more pronounced and included myoclonus and a deterioration of the higher functions. The patient died four months after the onset of the symptoms. The explorations that were conducted included a pathological study of the brain, which confirmed the diagnosis of classical spongiform encephalopathy (Creutzfeldt-Jakob disease). CONCLUSIONS: Creutzfeldt-Jakob disease must be included in the differential diagnosis of progressive cerebellar ataxias.

Ataxia cerebelosa mioclónica progresiva como manifestación de la enfermedad de Creutzfeldt-Jakob / Progressive myoclonic cerebellar ataxia as a manifestation of Creutzfeldt-Jakob disease

Introducción. La ataxia cerebelosa mioclónica progresiva es una entidad clínica con un importante espectro de posibilidades diagnósticas que obligan a un diagnóstico diferencial complejo y exhaustivo. Caso clínico. Paciente varón de 53 años de edad, sin antecedentes de interés, que ingresa por presentar inestabilidad en la marcha, con una leve dismetría bilateral y ampliación de la base de sustentación de instauración insidiosa y progresiva. Posteriormente, la sintomatología del paciente se acentuó y se asoció a mioclonías y a un deterioro de las funciones superiores. Cuatro meses después del inicio de los síntomas, el paciente falleció. Las exploraciones que se realizaron incluyeron el estudio anatomopatológico cerebeloso, que confirmó el diagnóstico de encefalopatía espongiforme (enfermedad de Creutzfeldt-Jakob) en su forma clásica. Conclusión. La enfermedad de Creutzfeldt-Jakob debe incluirse en el diagnóstico diferencial de las ataxias cerebelosas progresivas (AU)

Introduction. Progressive myoclonic cerebellar ataxia is a clinical entity with an important spectrum of possible diagnoses that requires a complex and exhaustive differential diagnosis. Case report. A 53-year-old male patient with no relevant medical history who was admitted to hospital because of an unstable gait, together with mild bilateral dysmetry and the progressive and insidious widening of the base of support. The patient’s symptoms then became more pronounced and included myoclonus and a deterioration of the higher functions. The patient died four months after the onset of the symptoms. The explorations that were conducted included a pathological study of the brain, which confirmed the diagnosis of classical spongiform encephalopathy (Creutzfeldt-Jakob disease). Conclusions. Creutzfeldt-Jakob disease must be included in the differential diagnosis of progressive cerebellar ataxias (AU)

[Giant aneurysms of the basilar artery. Six cases]. / Aneurismas gigantes del tronco basilar. A propósito de 6 casos.

The authors describe 6 cases of giant aneurysm of the basilar artery diagnosed in the Neurology Department of the Hospital Clínico Universitario of Zaragoza over the last 8 years by cerebral CT, arteriography and NMR with the clinical, diagnostic, and evolutive features of the same being reported. In 2 cases the clinical manifestations initiated as subarachnoid hemorrhage, in another two as AIT, in the fifth case as cerebral infarction and in the sixth case with pseudotumoral clinical manifestations with a necropsy study being available in the latter case. Three of the 6 patients died and of the remaining three only one was, successfully operated on. Anatomopathologic, clinical, therapeutic and diagnostic aspects of giant aneurysms of the basilar trunk are also reviewed with NMR being recommended as the technique of choice in diagnosis.

[Magnetic resonance in Hallevorden-Spatz disease]. / Resonancia magnética en la enfermedad de Hallevorden-Spatz.

The Hallevorden-Spatz disease should be suspected in children or young people with motor, pyramidal or extrapyramidal symptomatology and deterioration of superior functions specially upon the existence of family history. Pathognomonic diagnosis may only be obtained by necropsy but magnetic resonance images, although not pathognomic, are sufficiently characteristics to strongly support the diagnosis. Four cases of Hallevorden-Spatz disease are presented with special emphasis on the MR images characteristic of this disease.

[Contribution of the set-test to the diagnosis of Alzheimer's disease]. / Aportaciones del set-test al diagnóstico de la enfermedad de Alzheimer.

The categoric verbal fluency (colors, animals, fruits and cities) was prospectively evaluated with the set-test in a group of 71 patients with dementia (Alzheimer's disease 38, vascular dementia 19, mixed dementia 2, other dementias 12). In all types of dementia, greater decreases in the categoric verbal fluency were found with greater degrees of severity. However, for equal degree of deterioration, patients with Alzheimer's disease had the smallest scores in the set-test as compared with other types of dementia. 90% of the percentile distribution of patients with Alzheimer's disease had set-test scores below 25. The hypothesis that a set-test score lower than 25 is diagnostic for Alzheimer's disease has a 87% sensitivity and a 67% specificity. It is concluded that in patients with criteria of probable Alzheimer's disease a set-test score lower than 25 supports the diagnosis.