Degeneración hepatocerebral adquirida / Acquired hepatocerebral degeneration

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[A descriptive analysis of the demand for ambulatory neurological care in the health district of Huelva]. / Análisis descriptivo de la demanda de asistencia neurológica ambulatoria en un área sanitaria de Huelva.

INTRODUCTION: In recent years the demand for ambulatory neurological care has risen. Studying this situation can help to improve health care planning and the quality of the referrals from primary care. AIM: To analyse a number of variables involved in the first visits referred from primary care to neurology services. PATIENTS AND METHODS: We conducted a prospective, descriptive study in which data was consecutively collected about 500 new patients from primary care who had appointments for a visit to general neurology services in the health district of the province of Huelva. Demographic features, the reason for referral, initial diagnoses and the case resolution index were analysed. RESULTS: Mean age was 51 years old, and patients were predominantly female (63.4%). The most frequent diagnoses were headaches (42.8%) and cognitive impairment (12%). In 8.2% of cases the referred patients had no neurological pathology. Data showed that 40.2% were discharged after the first visit. CONCLUSIONS: The demographic characteristics and reasons for the visit were similar to those previously published on other regions in Spain. The high percentage of direct discharges translates into a poor selection of the patients referred from primary care. Improving the quality of referrals would make it possible to enhance the efficiency of ambulatory neurological care.

Análisis descriptivo de la demanda de asistencia neurológica ambulatoria en un área sanitaria de Huelva / A descriptive analysis of the demand for ambulatory neurological care in the health district of Huelva

Introducción. En los últimos años se ha incrementado la demanda de asistencia neurológica ambulatoria. Su estudio puede contribuir a mejorar la planificación sanitaria y la calidad de las derivaciones desde atención primaria. Objetivo. Analizar diversas variables de las primeras visitas remitidas desde atención primaria a las consultas de neurología. Pacientes y métodos. Estudio prospectivo descriptivo en el que se recogieron de forma consecutiva los datos de 500 pacientes nuevos procedentes de atención primaria citados en una consulta de neurología general en el área sanitaria de la provincia de Huelva. Se analizaron las características demográficas, el motivo de derivación, los diagnósticos iniciales y el índice de resolución de los casos. Resultados. La media de edad fue de 51 años, con un predominio femenino (63,4%). Los diagnósticos más frecuentes fueron las cefaleas (42,8%) y el deterioro cognitivo (12%). El 8,2% de los pacientes remitidos no tenía ninguna patología neurológica. El 40,2% fue dado de alta tras la primera visita. Conclusiones. Las características demográficas y los motivos de consulta son similares a los publicados previamente en otras comunidades españolas. El elevado porcentaje de altas directas traduce una escasa selección de los pacientes remitidos desde atención primaria. La mejora en la calidad de las derivaciones permitiría mejorar la eficiencia de la asistencia neurológica ambulatoria

Introduction. In recent years the demand for ambulatory neurological care has risen. Studying this situation can help to improve health care planning and the quality of the referrals from primary care. Aim.To analyse a number of variables involved in the first visits referred from primary care to neurology services. Patients and methods. We conducted a prospective, descriptive study in which data was consecutively collected about 500 new patients from primary care who had appointments for a visit to general neurology services in the health district of the province of Huelva. Demographic features, the reason for referral, initial diagnoses and the case resolution index were analysed. Results. Mean age was 51 years old, and patients were predominantly female (63.4%). The most frequent diagnoses were headaches (42.8%) and cognitive impairment (12%). In 8.2% of cases the referred patients had no neurological pathology. Data showed that 40.2% were discharged after the first visit. Conclusions. The demographic characteristics and reasons for the visit were similar to those previously published on other regions in Spain. The high percentage of direct discharges translates into a poor selection of the patients referred from primary care. Improving the quality of referrals would make it possible to enhance the efficiency of ambulatory neurological care

[Room tilt illusion: a rare symptom of migraine aura]. / Ilusión de inversión de la imagen visual: una manifestación excepcional de aura migrañosa.

INTRODUCTION: Room tilt illusion is a distorted perception of the spatial distribution of objects due to complex rotations in different planes. This distortion usually occurs in the vertical plane and is usually associated to vertebrobasilar ischemia. The etiology of the phenomenon varies greatly and only rarely corresponds to migraine aura. CASE REPORT: Since youth, a sixty-year-old woman had frequent episodes of room tilt illusion in the horizontal plane. These lasted from 30 minutes to 2 hours and were always followed by frontal headache fulfilling criteria for migraine (computerized tomography and magnetic resonance imaging) were normal. Visual symptoms and headache disappeared following treatment with flunarizine. DISCUSSION: Room tilt illusion might be a rare symptom of migraine aura. In this setting a functional depression of neurons from the posterior parietal cortex may occur, possibly mediated by chemical factors.

Ilusión de inversión de la imagen visual: una manifestación excepcional de aura migrañosa / Room tilt illusion: a rare symptom of migraine aura

Introducción. La ilusión de inversión de la imagen visual consiste en una percepción alterada de la situación de los objetos que se produce por rotaciones completas en los distintos planos del espacio. Esta alteración es más frecuente en el plano vertical y se asocia sobre todo a isquemia vertebrobasilar. La etiología del fenómeno es muy diversa y sólo excepcionalmente corresponde a un aura migrañosa. Caso clínico. Mujer de 60 años que desde su juventud presenta episodios de inversión visual en el plano horizontal, de 30 min a 2 h de duración, que siempre se siguen de una cefalea bifrontal con criterios de migraña. Los estudios de neuroimagen (tomografía computarizada y resonancia magnética craneal) fueron normales. Tanto los síntomas visuales como la cefalea desaparecieron al instaurar tratamiento con flunarizina. Discusión. La ilusión de inversión de la imagen visual puede ser una manifestación infrecuente de aura migrañosa. El mecanismo fisiopatológico consiste en una depresión funcional neuronal transitoria de la corteza parietal posterior, posiblemente mediada por factores químicos

Introduction. Room tilt illusion is a distorted perception of the spatial distribution of objects due to complex rotations in different planes. This distortion usually occurs in the vertical plane and is usually associated to vertebrobasilar ischemia. The etiology of the phenomenon varies greatly and only rarely corresponds to migraine aura. Case report. Since youth, a sixty-year-old woman had frequent episodes of room tilt illusion in the horizontal plane. These lasted from 30 minutes to 2 hours and were always followed by frontal headache fulfilling criteria for migraine (computerized tomography and magnetic resonance imaging) were normal. Visual symptoms and headache disappeared following treatment with flunarizine. Discussion. Room tilt illusion might be a rare symptom of migraine aura. In this setting a functional depression of neurons from the posterior parietal cortex may occur, possibly mediated by chemical factors

[Cerebral calcifications: a clue for a diagnostic process in a nonspecific clinical case]. / Calcificaciones cerebrales típicas como orientación de cuadro clínico inespecífico.

Coeliac disease is a gluten sensitive enteropathy, autoimmune in origin, which has been traditionally regarded as a gastrointestinal disease. Years later it has been reported an extraintestinal affection. A huge number of neurological syndromes of unknown cause had been initially described in association with coeliac disease, with total or partial response to a gluten free-diet. A specific kind of occipital cerebral calcifications in relation to coeliac disease has been also described, and sometimes it means the existence of a syndrome called "Gobby's Syndrome". We show a patient with a mild unknown coeliac disease, a woman who had occipital cerebral calcifications in a TAC cerebral, which was made because of her intractable migraines and that it lead to the diagnosis. The migraine disappeared after a gluten free-diet, like similar cases reported by literature. The fact of existing neurological symptoms associated to coeliac diseases opens a therapeutic window of opportunity because they would respond to a gluten free-diet.

Calcificaciones cerebrales típicas como orientación de cuadro clínico inespecífico / Cerebral calcifications: a clue for a diagnostic process in a inespecific clinical case

La enfermedad celíaca es una enteropatía por sensibilidad al gluten de origen autoinmune, considerada clásicamente como enfermedad exclusivamente gastrointestinal. Posteriormente se han puesto de manifiesto manifestaciones extraintestinales. Un número importante de síndromes neurológicos catalogados de idiopáticos al inicio, han sido descritos en el contexto de enfermedad celiaca, presentando respuesta total o parcial tras dieta sin gluten. Se ha descrito asociado a enfermedad celiaca un tipo muy característico de calcificaciones cerebrales occipitales, que a veces forma parte de un síndrome específico llamado síndrome de Gobby. Presentamos una paciente con enfermedad celiaca oligosintomática, que presentaba las peculiares calcificaciones cerebrales que fueron halladas tras realización de una prueba de imagen al presentar migraña rebelde a tratamiento, y que orientaron hacia su diagnóstico. La migraña mejoró sustancialmente tras realizar dieta sin gluten, como en otros casos descritos en la literatura. El hecho de que existan manifestaciones neurológicas asociadas a enfermedad celiaca hace posible la reversibilidad de las mismas tras dieta exenta de gluten

Coeliac disease is a gluten sensitive enteropathy, autoimmune in origin, which has been traditionally regarded as a gastrointestinal disease. Years later it has been reported an extraintestinal affection. A huge number of neurological syndromes of unknown cause had been initially described in association with coeliac disease, with total or parcial response to a gluten free-diet. A specific kind of occipital cerebral calcifications in relation to coeliac disease has been also described, and sometimes it means the existence of a syndrom called “Gobby´s Syndrom”. We show a patient with a mild unknown coeliac disease, a woman who had occipital cerebral calcifications in a TAC cerebral, which was made because of her wild migraine and that it leaded the diagnosis. The migraine disappeared after a gluten free-diet, like similar cases reported by literature. The fact of existing neurological symtoms associated to coeliac diseases opens a therapeutc window of opportunity because they would repond to a gluten free-diet

Distonía aguda tras picadura de avispa / Acute dystonia following a wasp sting

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[Transcranial Doppler velocimetry in normotensive puerperal women]. / Velocimetría Doppler transcraneal en puérperas normotensas.

OBJECTIVES: To know transcranial Doppler (TCD) normal values in postpartum normotensive women. To evaluate the effect of different variables on cerebral blood flow velocity. PATIENTS AND METHODS: TCD examination was carried out in 100 postpartum normotensive women. Six intracranial arteries were evaluated. Different variables such as arterial pressure, hematocrit and clinical data related to pregnancy and delivery were recorded. A descriptive analysis of qualitative variables was made and the mean and standard deviation of each quantitative variable was calculated. We compared mean velocities (MV) by each side, and assessed the correlation between cerebral blood flow velocity and arterial pressure, hematocrit and the rest of variables. RESULTS: Mean and standard deviations of MV were: right MCA 73.10 14.33; left MCA 75.68 15.84; right ACA 57.61 12.92; left ACA 60.14 14.35; right PCA 42.77 9.28; left PCA 45.54 9.58. Statically significant higher values of MV in the left hemispheric arteries were found. The other analysed variables did not modify the MV values. CONCLUSIONS: In postpartum normotensive women MV values were higher in the left hemisphere. The knowledge of normal puerperium values for TCD is useful to identify cerebral hemodynamic pathological states such those in postpartum eclampsia and postpartum cerebral angiopathy.

Velocimetría Doppler transcraneal en puérperas normotensas / Transcranial Doppler velocimetry in normotensive puerperal women

Objetivos. Conocer los valores normales de Doppler transcraneal (DTC) en puérperas normotensas (PNT) en nuestro medio.Evaluar la influencia de diferentes variables sobre el flujo sanguíneo cerebral. Pacientes y métodos. Se realizaron 100 estudios DTC en PNT, y se sonorizaron seis arterias intracraneales. Se recogieron diversas variables como presión arterial, hematocrito y datos clínicos referidos al embarazo y parto. Se realizó un análisis descriptivo en el caso de las variables cualitativas, y las variables cuantitativas se expresaron como media más desviación estándar (DE). Se compararon las velocidades medias (VM) de cada arteria según la lateralidad hemisférica, y su relación con las cifras tensionales, el hematocrito y el resto de las variables. Resultados. Las VM expresadas como media y DE fueron: ACM derecha 73,10ñ14,33; ACM izquierda 75,68ñ15,84; ACA derecha 57,61ñ12,92; ACA izquierda 60,14ñ14,35; ACP derecha 42,77ñ 9,28; ACP izquierda 45,54ñ9,58. Se comprobó un aumento de VM en las arterias de lateralidad izquierda con respecto a las de lateralidad derecha, estadísticamente significativo. Ninguna de las variables del estudio influyó en los valores de flujo sanguíneo cerebral. Conclusión.Los valores de VM fueron más altos en el hemisferio izquierdo. Resulta útil conocer valores de referencia puerperales en mujeres asintomáticas para poder identificar alteraciones hemodinámicas cerebrales propias de estados patológicos, como eclampsia tardía y angiopatía cerebral posparto (AU)

Objectives. To know transcranial Doppler (TCD) normal values in postpartum normotensive women. To evaluate the effect of different variables on cerebral blood flow velocity. Patients and methods. TCD examination was carried out in 100 postpartum normotensive women. Six intracranial arteries were evaluated. Different variables such as arterial pressure, hematocrit and clinical data related to pregnancy and delivery were recorded. A descriptive analysis of qualitative variables was made and the mean and standard deviation of each quantitative variable was calculated. We compared mean velocities (MV) by each side, and assessed the correlation between cerebral blood flow velocity and arterial pressure, hematocrit and the rest of variables. Results. Mean and standard deviations of MV were: right MCA 73.10±14.33; left MCA 75.68±15.84; right ACA 57.61±12.92; left ACA 60.14±14.35; right PCA 42.77±9.28; left PCA 45.54±9.58. Statically significant higher values of MV in the left hemispheric arteries were found. The other analysed variables did not modify the MV values. Conclusions. In postpartum normotensive women MV values were higher in the left hemisphere. The knowledge of normal puerperium values for TCD is useful to identify cerebral hemodynamic pathological states such those in postpartum eclampsia and postpartum cerebral angiopathy (AU)

Necrosis estriatal bilateraltras picadura de avispa / Bilateral striatal necrosis folowing a wasp sting

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Tartamudeo como única manifestación de un infarto cerebral / Stuttering as the only manifestation of brain infarct

Un varón de 53 años adquirió de forma aguda un tartamudeo. En la exploración existían bloqueos y repeticiones de forma exclusiva en las primeras sílabas de las palabras durante la conversación, mejorando en tareas automáticas. No existía afasia ni otros déficit neurológicos. Una RM cerebral reveló un pequeño infarto cortical en la circunvolución precentral izquierda. La arteriografía cerebral detectó una estenosis aterosclerótica en el segmento intracavernoso de la arteria carótica interna izquierda. Un embolismo arterioarterial fue el mecanismo propuesto para este ictus y el paciente fue tratado con anticoagulantes orales durante los siguientes 6 meses. El tartamudeo mejoró progresivamente y el paciente estaba asintomático un mes tras el ictus. Un tartamudeo adquirido ictal sintomático de un infarto cerebral sin afasia ni otros déficit es excepcional. Un pequeño infarto en el área motora como causa de tartamudeo aislado adquirido añade nueva información sobre los circuitos neurológicos implicados en este fenómeno (AU)

[Late symptomatic initiation of unilateral cerebellar hypoplasia]. / Inicio sintomático tardío de una hipoplasia cerebelosa unilateral.

A 65-year-old woman developed a progressive action tremor on her left hand for the last two years. On examination an intentional left hand tremor with dysmetria and tendency to deviate to left on tandem were identified. There was no mental deterioration. CT scan and MR imaging disclosed a marked unilateral, left, cerebellar hypoplasia. MR also showed multiple lacunae. Neurological family disease and exposition to drugs or alcohol were not present. In some cases cerebellar hypoplasia could become symptomatic at advanced age as result of concurrent cerebral disease affecting the compensating structures for congenital defect. In this patient a vascular mechanism is suggested as possible late trigger.

Inicio sintomático tardío de una hipoplasia cerebelosa unilateral / Late symptomatic initiation of a unilateral cerebellous hypoplasia

Una mujer de 65 años presentaba desde hacía 2 años un temblor de acción progresivo en la mano izquierda. La exploración objetivó un temblor intencional con componente de dismetría en la mano izquierda y una lateropulsión a la izquierda en el tándem. Las funciones superiores eran normales. La TAC y RM pusieron de manifiesto una marcada hipoplasia cerebelosa unilateral izquierda. La RM demostró, además, infartos lacunares múltiples. No existía historia familiar de enfermedad neurológica, ni exposición a fármacos o alcohol. En algunos pacientes, una hipoplasia cerebelosa unilateral tendría su inicio clínico a una edad avanzada, al concurrir otras lesiones cerebrales que afectarían a estructuras compensadoras del defecto congénito. En esta paciente el posible desencadenante tardío podría ser un mecanismo vascular (AU)

[Stuttering as the only manifestation of a cerebral infarct]. / Tartamudeo como única manifestación de un infarto cerebral.

A 53-year-old right-handed man acutely developed stuttering. On his examination there was blocks and repetitions exclusively on first syllables of words in conversational speech, improving in automatic tasks. There was neither aphasia, nor other neurological deficits. An MR imaging of the brain showed a circumscribed cortical infarct on the left precentral circunvolution. Cerebral angiography was consistent with atherosclerotic narrowing of the intracavernous segment of left internal carotid artery. Artery-to-artery embolism was the suggested mechanism for this stroke and the patient was treated with oral anticoagulants for six months. Stuttering improved progressively and the patient became asymptomatic a month after the stroke. Ictal acquired stuttering symptomatic of ischemic stroke without aphasia or other neurological deficit is exceptional. An small infarct on the left motor area as cause of isolated acquired stuttering adds new information about neural circuits involved in this phenomenon.

[Lumbosacral plexopathy as a form of presentation of an aneurysm of the iliac artery]. / Plexopatía lumbosacra como forma de presentación de un aneurisma de la arteria ilíaca.

INTRODUCTION: The etiology of lumbosacral plexopathy is often due to compression. One of the less common causes of this is aneurysm of the iliac artery. However, 13% of the patients with this disorder initially have symptoms of plexus irritation or deficit. CLINICAL CASE: We describe the case of a 42 year-old-man, with no previous medical history, who complained of right-sided sciatica for the previous three months. On examination there were clinical signs of a lesion of the right lumbosacral plexus. On CT of the pelvis and MR of the lumbar spine there were images compatible with an aneurysm of the right iliac artery. This diagnosis was confirmed on arteriography. Treatment was surgical (aneurysmography and right iliofemoral by-pass). The pain disappeared and the motor deficit improved considerably. CONCLUSION: When a patient presents with lumbosacral plexopathy, an iliac aneurysm should be considered as a possible etiological factor. In such cases early diagnosis is essential, since surgical treatment will usually resolve the clinical problem.