Optimized clinical management of Parkinson's disease with opicapone. Recommendations from Spanish experts. / Optimización del manejo clínico de opicapona en la enfermedad de Parkinson. Recomendaciones de expertos españoles.

Motor fluctuations are frequently seen in Parkinson disease patients on chronic treatment with levodopa. Management of motor fluctuation includes the addition of catechol-O-methyl transferase (COMT) inhibitors. Opicapone is a recent and selective third-generation COMT inhibitor which achieves marked increase in the bioavailability of levodopa. We present a consensus of a group of Spanish neurologists with extensive experience in the clinical management of motor fluctuations. The clinical experience of this group of experts is in line with clinical trials and confirms that opicapone is an effective drug in the control of motor fluctuations, regardless of the daily levodopa dose, or the use of other antiparkinsonian drugs. However, in the opinion of these experts, the ideal patient with Parkinson's disease to initiate treatment with opicapone is the one with mild motor fluctuations, since the ratio between clinical efficacy and adverse effects is more favorable. In general, it is an easy-to-use drug both in those first treated with a COMT inhibitor or those already on entacapone. In any case, the secondary side effects are easily managed.

TITLE: Optimización del manejo clínico de opicapona en la enfermedad de Parkinson. Recomendaciones de expertos españoles.Las fluctuaciones motoras constituyen una importante complicación en los pacientes con enfermedad de Parkinson tratados con levodopa. Entre las opciones terapéuticas para el manejo de las fluctuaciones motoras se cuenta con los inhibidores de la catecol-O-metil-transferasa (COMT), incluyendo la opicapona. La opicapona muestra una elevada afinidad por la COMT y consigue un aumento marcado de la biodisponibilidad de la levodopa. Se presenta el consenso de un grupo de expertos españoles en la enfermedad de Parkinson con experiencia en el tratamiento clínico de fluctuaciones motoras y el empleo de opicapona. La experiencia de este grupo de expertos, en consonancia con los ensayos clínicos, confirma que la opicapona es un fármaco eficaz en el control de las fluctuaciones motoras de la enfermedad de Parkinson, con independencia de la dosis de levodopa recibida o de la utilización de otros fármacos antiparkinsonianos. No obstante, a juicio de estos expertos, el paciente ideal para iniciar el tratamiento con opicapona es el que presenta fluctuaciones motoras leves, ya que muestra una mejor relación entre eficacia clínica y efectos adversos. En general, la opicapona es un fármaco de fácil manejo, tanto en pacientes que requieren opicapona como primer inhibidor de la COMT como en los previamente tratados con entacapona, o en los que están en tratamiento concomitante con otros fármacos antiparkinsonianos. En cualquier caso, los efectos secundarios son fácilmente corregibles.

[Laryngeal dystonia: novel forms of therapeutic administration of botulinum toxin by direct routes]. / Distonia laringea: nuevas formas de administracion terapeutica de toxina botulinica por via directa.

AIM: To describe our experience in the treatment of laryngeal dystonia (in abduction and adduction), with special emphasis given to the technical aspects (approach procedure, dosage and type of botulinum toxin type A used), as well as treatment response and possible side effects. PATIENTS AND METHODS: We conducted a cross-sectional descriptive study of a sample of patients with laryngeal dystonia treated by means of transoral administration of onabotulinumtoxinA or incobotulinumtoxinA over a period of 10 years (2007-2017). Data collected include demographic and clinical variables, treatment response (based on a self-rating scale), the duration of treatment and the appearance of side effects. SAMPLE SIZE: 15 patients (11 women; mean age: 44.06 years) with laryngeal dystonia (mean time since onset of 40 months; 12 patients with dystonia in adduction) and 174 administrations (92% incobotulinumtoxinA; average dosage of 5 U in each vocal cord). The procedure took an average of 11.7 minutes to perform. Response was good in 31% of the procedures and very good in 57.5%. Side effects were recorded in 14.4% of the procedures, although always mild and transitory, with a predominance of dysphagia and dysphonia. CONCLUSION: In our experience, transoral administration of botulinum toxin type A to treat laryngeal dystonia has proved to be a simple, quick, effective and safe technique.

TITLE: Distonia laringea: nuevas formas de administracion terapeutica de toxina botulinica por via directa.Objetivo. Describir nuestra experiencia en el tratamiento de la distonia laringea (en abduccion y aduccion), destacando los aspectos tecnicos (procedimiento de abordaje, dosis y tipo de toxina botulinica de tipo A utilizada), asi como la respuesta al tratamiento y los posibles efectos adversos. Pacientes y metodos. Estudio descriptivo transversal de una muestra de pacientes con distonia laringea tratados mediante administracion transoral de onabotulinumtoxina o incobotulinumtoxina A durante un periodo de 10 años (2007-2017). Se recogen las variables demograficas y clinicas, la respuesta al tratamiento (a partir de una escala de autoevaluacion), la duracion de este y la aparicion de efectos adversos. Resultados. Tamaño muestral: 15 pacientes (11 mujeres; edad media: 44,06 años) con distonia laringea (tiempo medio de evolucion de 40 meses; 12 pacientes con distonia en aduccion) y 174 administraciones (92% incobotulinumtoxina A; dosis media de 5 U en cada cuerda vocal). La duracion media del procedimiento fue de 11,7 minutos. La respuesta fue notable en el 31% de los procedimientos y alta en el 57,5%. Se registraron efectos adversos en el 14,4% de los procedimientos, siempre de caracter leve y transitorio, con predominio de la disfagia y la disfonia. Conclusion. En nuestra experiencia, la administracion transoral de toxina botulinica de tipo A como tratamiento de la distonia laringea ha demostrado ser una tecnica sencilla, rapida, eficaz y segura.

[The hypothalamus in Huntington's disease]. / El hipotalamo en la enfermedad de Huntington.

INTRODUCTION: Disorders affecting sleep and the circadian rhythm, autonomic clinical signs and symptoms, and neuroendocrine alterations are frequent characteristics in Huntington's disease, some of which present in early stages of the disease. It is reasonable to think that some of these features could result from a hypothalamic dysfunction affecting the centre regulating sleep, metabolism and the autonomic nervous system. AIM: The study presents the evidence available to date that suggests the involvement of a hypothalamic disorder in Huntington's disease. DEVELOPMENT: Histopathological, hormonal and neuroimaging research relates this area of the brain to Huntington's disease. The experimental findings and those obtained with animal models or in studies conducted with patients are summarised. Likewise, the clinical repercussions (sleep and circadian rhythm disorders, psychiatric and cognitive pathologies, and the clinical signs and symptoms linked to autonomic dysfunction) secondary to possible involvement of the hypothalamus in this disease are also described. CONCLUSIONS: The hypothalamus acts as a centre that integrates the neuroendocrine and autonomic functions, and plays a significant role in cognitive and behavioural signs and symptoms. Disorders of this type have been highlighted in Huntington's disease. Further studies are needed to elucidate the role and scope of this region of the brain in this disease.

TITLE: El hipotalamo en la enfermedad de Huntington.Introduccion. Los trastornos del sueño y del ritmo circadiano, la sintomatologia autonomica y las alteraciones neuroendocrinas son caracteristicas frecuentes de la enfermedad de Huntington, y algunos de ellos se presentan en estadios tempranos de ella. Es plausible pensar que algunos de estos rasgos podrian ser el resultado de una disfuncion hipotalamica del centro regulador del sueño, el metabolismo y el sistema nervioso autonomo. Objetivo. Se exponen las evidencias disponibles hasta el momento actual que sugieren una afectacion hipotalamica en la enfermedad de Huntington. Desarrollo. Investigaciones histopatologicas, hormonales y de neuroimagen relacionan esta region cerebral con la enfermedad de Huntington. Se resumen los hallazgos, tanto experimentales como en modelos animales, o en estudios realizados con pacientes. Asimismo, se describen las repercusiones clinicas (alteraciones del sueño y del ritmo circadiano, la patologia psiquiatrica y cognitiva, y la clinica vinculada con disfuncion autonomica) secundarias a una posible afectacion hipotalamica en esta enfermedad. Conclusiones. El hipotalamo se comporta como un centro integrador de las funciones neuroendocrinas y autonomicas, y presenta un papel no desdeñable sobre la sintomatologia cognitiva y conductual. Alteraciones al respecto se han destacado en la enfermedad de Huntington. Son necesarios posteriores estudios para aclarar el papel y el alcance de esta region cerebral en esta enfermedad.

Association between REM sleep behaviour disorder and impulse control disorder in patients with Parkinson's disease. / Relación entre el trastorno de conducta del sueño REM y el trastorno de control de impulsos en pacientes con enfermedad de Parkinson.

INTRODUCTION: The relationship between impulse control disorder (ICD) and REM sleep behaviour disorder (RBD) has not yet been clarified, and the literature reports contradictory results. Our purpose is to analyse the association between these 2 disorders and their presence in patients under dopaminergic treatment. METHODS: A total of 73 patients diagnosed with Parkinson's disease and treated with a single dopamine agonist were included in the study after undergoing clinical assessment and completing the single-question screen for REM sleep behaviour disorder and the short version of the questionnaire for impulsive-compulsive behaviours in Parkinson's disease. RESULTS: Mean age was 68.88 ± 7.758 years. Twenty-six patients (35.6%) were classified as probable-RBD. This group showed a significant association with ICD (P=.001) and had a higher prevalence of non-tremor akinetic rigid syndrome and longer duration of treatment with levodopa and dopamine agonists than the group without probable-RBD. We found a significant correlation between the use of oral dopamine agonists and ICD. Likewise, patients treated with oral dopamine agonists demonstrated a greater tendency toward presenting probable-RBD than patients taking dopamine agonists by other routes; the difference was non-significant. CONCLUSIONS: The present study confirms the association between RBD and a higher risk of developing symptoms of ICD in Parkinson's disease.

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

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

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

[Mnemonic complaints and chronic migraine]. / Quejas mnesicas y migraña cronica.

INTRODUCTION. Patients with chronic migraine often report lower cognitive performance, which affects their quality of life. AIMS. To analyse whether the mnemonic capacity of patients with chronic migraine is altered or not. SUBJECTS AND METHODS. A cross-sectional study was conducted in patients with chronic migraine evaluated consecutively in our unit, and paired by age (18-60 years) and gender with a control group consisting of cognitively healthy volunteers. The following cognitive instruments were administered: Folstein Minimental State Examination (MMSE), Memory Alteration Test (M@T), Montreal Cognitive Assessment (MoCA) and working memory. RESULTS. A total of 30 patients with chronic migraine were included (mean age: 49.33 ± 10.05 years) paired with a control group of 30 healthy volunteers (mean age: 44.83 ± 10.91 years). The mean elapsed time since onset of the patients with chronic migraine was 4.47 ± 2.74 years. On performing a comparative analysis between the two groups, significant differences were found with overall lower scores in the group of patients with chronic migraine in the MoCA (24.16 versus 29), M@T (43.76 versus 48.8) and working memory tests (17.5 versus 24.26). Performance in the MMSE was similar in both groups. CONCLUSIONS. Patients with chronic migraine can have lower cognitive performance regardless of distracting elements, such as pharmacological factors or psychiatric comorbidity, since chronic migraine can be understood as yet another element within the spectrum of chronic pain.

TITLE: Quejas mnesicas y migraña cronica.Introduccion. Los pacientes con migraña cronica refieren frecuentemente un menor rendimiento cognitivo, lo que interfiere en su calidad de vida. Objetivo. Analizar si existe alteracion en la capacidad mnesica de los pacientes con migraña cronica. Sujetos y metodos. Estudio transversal en pacientes con migraña cronica valorados de forma consecutiva en nuestra unidad, pareados en edad (18-60 años) y sexo con un grupo control constituido por voluntarios cognitivamente sanos. Se administraron los siguientes instrumentos cognitivos: test minimental de Folstein (MMSE), test de alteracion de memoria (T@M), evaluacion cognitiva de Montreal (MoCA) y memoria de trabajo. Resultados. Se incluyo un total de 30 pacientes con migraña cronica (edad media: 49,33 ± 10,05 años) pareados con un grupo control de 30 voluntarios sanos (edad media: 44,83 ± 10,91 años). El tiempo medio de evolucion de los pacientes con migraña cronica fue de 4,47 ± 2,74 años. Al realizar el analisis comparativo entre ambos grupos, se encontraron diferencias significativas con puntuaciones mas bajas de forma global en el grupo de pacientes con migraña cronica en el test de MoCA (24,16 frente a 29), T@M (43,76 frente a 48,8) y memoria de trabajo (17,5 frente a 24,26). El rendimiento en el MMSE fue similar en ambos grupos. Conclusiones. Los pacientes con migraña cronica pueden tener un menor rendimiento cognitivo independientemente de elementos distractores, como el farmacologico o la comorbilidad psiquiatrica, dado que la migraña cronica puede entenderse como un elemento mas dentro del espectro del dolor cronico.

Presentación: IV Reunión Nacional de Enfermedad de Parkinson y Trastornos del Movimiento / Presentation

No disponible

[Therapeutic use of tetrabenazine]. / Utilizacion terapeutica de la tetrabenacina.

INTRODUCTION: Tetrabenazine is a benzoquinolizine with a high antidopaminergic potential due to a monoamine depletion effect that acts equally on the three main neurotransmitters (dopamine, noradrenalin and serotonin). This potential explains why this group of pharmaceutical agents has been used for years to treat different types of hyperkinetic syndromes. In the lines that follow, we examine both the pharmacokinetic and the pharmacodynamic characteristics of tetrabenazine. DEVELOPMENT: A thorough review is performed of the literature on the main indications established over the years for the therapeutic utilisation of tetrabenazine, the most important hyperkinetic syndromes of which include: tardive dyskinesias, athetosis, ballism, dystonias (primary, tardive, etc.), tics or Tourette syndrome, and finally the semiological group consisting of choreas (Huntington's disease, Sydenham's chorea and other paediatric choreas). CONCLUSIONS: Tetrabenazine appears to be an excellent pharmacological agent for use in a number of pathologies that are accompanied by hyperkinesias; it is well tolerated and has few complications or side effects deriving from its administration.

Utilización terapéutica de la tetrabenacina / Therapeutic use of tetrabenazine

Introducción. La tetrabenacina es una benzoquinolicina con gran potencial antidopaminérgico por un efecto deplecionador de monoaminas que afecta por igual a los tres neurotransmisores mayoritarios (dopamina, noradrenalina y serotonina); es ese potencial el que ha hecho que todo este grupo de fármacos se haya venido utilizando desde hace años para tratar diferentes tipos de síndromes hipercinéticos. En las líneas siguientes revisamos las características de la tetrabenacina, tanto farmacocinéticas como farmacodinámicas. Desarrollo. Se realiza una extensa revisión bibliográfica de las principales indicaciones establecidas a lo largo de los años para la utilización terapéutica de la tetrabenacina, entre las que destacan los siguientes síndromes hipercinéticos: discinesias tardías, atetosis, balismos, distonías (primarias, tardías...), tics o síndrome de Tourette, y finalmente el grupo semiológico de las coreas (enfermedad de Huntington, corea de Sydenham y otras coreas pediátricas...). Conclusión. La tetrabenacina parece ser un excelente fármaco, bien tolerado y con escasas complicaciones o efectos secundarios derivados de su administración, en múltiples patologías que cursan con hipercinesia (AU)

Introduction. Tetrabenazine is a benzoquinolizine with a high antidopaminergic potential due to a monoamine depletion effect that acts equally on the three main neurotransmitters (dopamine, noradrenalin and serotonin). This potential explains why this group of pharmaceutical agents has been used for years to treat different types of hyperkinetic syndromes. In the lines that follow, we examine both the pharmacokinetic and the pharmacodynamic characteristics of tetrabenazine. Development. A thorough review is performed of the literature on the main indications established over the years for thetherapeutic utilisation of tetrabenazine, the most important hyperkinetic syndromes of which include: tardive dyskinesias, athetosis, ballism, dystonias (primary, tardive, etc.), tics or Tourette syndrome, and finally the semiological group consisting of choreas (Huntington’s disease, Sydenham’s chorea and other paediatric choreas). Conclusions. Tetrabenazine appears to be an excellent pharmacological agent for use in a number of pathologies that are accompanied by hyperkinesias; it is well tolerated and has few complications or side effects deriving from its administration (AU)

Anorgasmia por topiramato / No disponible

No disponible

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

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

[Clinical, functional and quality of life differences in patients of different gender with essential tremor]. / Diferencias clínicas, funcionales y de calidad de vida en pacientes con temblor esencial de ambos sexos.

INTRODUCTION: Essential tremor (ET) is a chronic disease with important variability in its clinical manifestation, discapacity and quality of life (QoL) affectation. As previous reports have described differences in clinical manifestations of ET according to gender, this study proposes to check its existence in the Spanish population and to detect the presence of discapacity or alterations in QoL between different gender. METHODS: Patients with ET attended in the neurology clinic of a sanitary area were evaluated in a prospective consecutive way. Their clinical characteristics were recorded and discapacity and QoL were assessed through the Fahn and Tolosa scale for ET and the SF-36 health questionnaire. Comparisons between groups of both genders have been established. RESULTS: 104 patients with ET were evaluated (46 males and 58 females). Clinically there were differences concerning the localization and type of tremor. Although the intensity and the presence of discapacity were similar in both genders, women showed a lower QoL compared to men in the "physical role" dimension. CONCLUSIONS: There are differences in the clinical expression and in the affectation of QoL in patients with ET of different gender. Women had a lower score in the "physical role" dimension of QoL in comparison to their reference population, meaning lesser satisfaction with their performance capacity of their usual activities. QoL, thus, does not appear in this study as something only related with the degree of discapacity, but rather as having other determinant factors.

Diferencias clínicas, funcionales y de calidad de vida en pacientes con temblor esencial de ambos sexos / Clinical, functional and quality of life differences in patients of different gender with essential tremor

Introducción. El temblor esencial (TE) es una enfermedad crónica con importante variabilidad respecto a sus manifestaciones clínicas, discapacidad y afectación de calidad de vida (CdV). Dado que estudios previos describen diferencias en las manifestaciones clínicas del TE según el sexo, se plantea este estudio para comprobar su existencia en población española y detectar la presencia de discapacidad o alteraciones en CdV en los diferentes sexos. Métodos. Se han evaluado de forma prospectiva consecutiva pacientes con TE atendidos en las consultas de neurología de un área sanitaria, registrándose sus características clínicas y valorando discapacidad y CdV mediante la escala de TE de Fahn y Tolosa y el cuestionario de salud SF-36. Se han establecido comparaciones entre ambos sexos. Resultados. Participaron 104 pacientes con TE (46 varones y 58 mujeres). Clínicamente hubo diferencias en cuanto a localización y tipo de temblor. Aunque la intensidad del temblor y la presencia de discapacidad fue similar en ambos sexos, las mujeres manifestaron menor CdV respecto a los hombres en la dimensión de «rol físico». Conclusiones. Existen diferencias en la expresión clínica y en la afectación de CdV de pacientes con TE de diferentes sexos. Las mujeres presentaron menos puntuación en la dimensión «rol físico» de CdV respecto a su población de referencia, indicando un menor grado de satisfacción con la capacidad de realización de sus actividades habituales. La CdV, por tanto, no aparece en este estudio como un aspecto relacionado solamente con el grado de discapacidad, sino que puede tener otros factores determinantes

Introduction. Essential tremor (ET) is a chronic disease with important variability in its clinical manifestation, discapacity and quality of life (QoL) affectation. As previous reports have described differences in clinical manifestations of ET according to gender, this study proposes to check its existence in the Spanish population and to detect the presence of discapacity or alterations in QoL between different gender. Methods. Patients with ET attended in the neurology clinic of a sanitary area were evaluated in a prospective consecutive way. Their clinical characteristics were recorded and discapacity and QoL were assessed through the Fahn and Tolosa scale for ET and the SF-36 health questionnaire. Comparisons between groups of both genders have been established. Results. 104 patients with ET were evaluated (46 males and 58 females). Clinically there were differences concerning the localization and type of tremor. Although the intensity and the presence of discapacity were similar in both genders, women showed a lower QoL compared to men in the «physical role» dimension. Conclusions. There are differences in the clinical expression and in the affectation of QoL in patients with ET of different gender. Women had a lower score in the «physical role» dimension of QoL in comparison to their reference population, meaning lesser satisfaction with their performance capacity of their usual activities. QoL, thus, does not appear in this study as something only related with the degree of discapacity, but rather as having other determinant factors

[Differential diagnosis of intracranial calcifications]. / Diagnostico diferencial de las calcificaciones intracraneales.

INTRODUCTION: Intracranial calcifications are a frequent chance finding in many neuroimaging tests. We report two clinical cases with intracranial calcifications as a common feature, but which had very different aetiologies and presenting symptoms. CASE REPORTS: The first case involved a 51-year-old male with a single tonic-clonic convulsive seizure associated to hypocalcemia secondary to hypoparathyroidism who presented extensive brain calcifications affecting the dentate nuclei, pons, periependymal and basal ganglia, and whose convulsive attacks remitted once normal Ca2+ values were restored. The second case was a 25-year-old female patient whose convulsive seizures were yet another symptom of Fahr's syndrome, which was associated to spastic paralysis, athetosis, mental retardation and occasionally hypoplasia of the optic nerve; the calcifications were located in the grey nuclei of the cerebrum and cerebellum. CONCLUSIONS: A correct history and physical and neurological examination must be carried out, and a detailed study of Ca2+/ P+ and hormonal metabolism is also required. A CAT scan is the preferred neuroimaging technique to achieve a proper differential diagnosis and to decide on the therapeutic approach that is best suited to the pathophysiological mechanisms that lead to the formation of these calcium deposits.

[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.

[Quetiapine and ziprasidone in the treatment of the psychotic disorders in Parkinson's disease]. / Quetiapina y ziprasidona en el tratamiento de los trastornos psicóticos de la enfermedad de Parkinson.

INTRODUCTION: As neurologists we often find it necessary to use antipsychotics in our clinical practice. They are not only used in the treatment of the behavioural disorders accompanying dementias, but also in the psychotic symptoms that frequently appear or complicate the clinical progress of our patients suffering from Parkinson. PATIENTS AND METHODS: Taking into account the numerous side effects of the typical antipsychotic drugs, we began a prospective study on the use of a new antipsychotic agent, quetiapine, in the treatment of 134 patients with Parkinson's disease who presented signs or symptoms of parkinsonian psychosis at some time during the course of their clinical progression; results were analysed at three and six months. At the same time, we review the efficacy of another antipsychotic agent, ziprasidone, which from the chemical point of view fulfils a profile that suits the needs of our patients. At the time of writing this paper we have recorded 43 cases of patients with Parkinson's disease and concomitant parkinsonian psychoses, which responded adequately to treatment without any kind of side effects. RESULTS: The administration of quetiapine in doses of 25-50 mg/day brought about an important improvement in the control over the signs of parkinsonian psychoses in our patients. The same occurred with the administration of 20-40 mg/day of ziprasidone. Both drugs were found to be extremely well tolerated and of great clinical value in the treatment of this clinical entity. CONCLUSIONS: Both quetiapine and ziprasidone are drugs that can be of great value in the treatment of parkinsonian psychoses. In addition to their high degree of clinical effectiveness, they also have a broad safety profile and no side effects.

[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.

[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.

[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.