[What do medical students think about functional neurological disorders?] / ¿Qué piensan los estudiantes de Medicina sobre los trastornos neurológicos funcionales?

INTRODUCTION: Functional neurological disorders (FND) are a frequent reason for visits in neurology. However, specific training on these disorders during undergraduate and residency training is limited. This study assesses the knowledge, attitude and exposure of medical students to FNDs before completing their medical degree. PATIENTS AND METHODS: We conducted a 15-item survey to explore understanding, exposure and attitudes towards FNDs among sixth-year medical students at four Spanish universities. RESULTS: A total of 118 students (mean age 23.6 ± 1.2 years; 71.2% female) returned the survey. Of these, 88 (74.6%) were aware of the concept of FNDs and 78 (66.1%) had studied them in psychiatry classes. The term 'psychosomatic' was chosen by 54.1% of the students as the most appropriate term to refer to these disorders, and 111 (94.1%) believed that a history of sexual or physical abuse was common among FND patients. Fifty-seven students (48.3%) assumed that the diagnosis of FND was mostly a clinical diagnosis of exclusion and 63 (53.4%) indicated that it is managed only by psychiatry. One hundred and one students (85.6%) considered that adequate training on FNDs is an important aspect of their medical training. CONCLUSIONS: Medical students are aware of the existence of FNDs, but their preferred terminology, as well as the perceived aetiological factors, reflect that the historical view of these disorders is still deeply rooted. Medical students feel that they should receive adequate education on FNDs from specialists in neurology and psychiatry as part of their training.

TITLE: ¿Qué piensan los estudiantes de Medicina sobre los trastornos neurológicos funcionales?Introducción. Los trastornos neurológicos funcionales (TNF) son un motivo de consulta frecuente en neurología. Sin embargo, la formación específica sobre estos trastornos durante la formación universitaria y el período de residencia es limitada. En este estudio se evalúan los conocimientos, la actitud y la exposición de los estudiantes de Medicina a los TNF antes de terminar el grado de Medicina. Sujetos y métodos. Realizamos una encuesta de 15 ítems para explorar la comprensión, la exposición y las actitudes hacia los TNF entre los estudiantes de Medicina de sexto año en cuatro universidades españolas. Resultados. Devolvieron la encuesta 118 estudiantes (edad media 23,6 ± 1,2 años; 71,2%, mujeres). De ellos, 88 (74,6%) conocían el concepto de TNF y 78 (66,1%) los habían estudiado en las clases de psiquiatría. El 54,1% de los estudiantes eligió el término 'psicosomático' como el más adecuado para referirse a estos trastornos, y 111 (94,1%) creían que una historia de abuso sexual o físico era común entre los pacientes con TNF. Cincuenta y siete estudiantes (48,3%) asumieron que el diagnóstico de TNF era mayoritariamente un diagnóstico clínico de exclusión y 63 (53,4%) señalaron que el manejo se realiza únicamente desde psiquiatría. Ciento un estudiantes (85,6%) consideraron que una formación adecuada sobre los TNF es un aspecto importante de su formación médica. Conclusiones. Los estudiantes de Medicina son conscientes de la existencia de los TNF, pero la terminología preferida por ellos, así como los factores etiológicos percibidos, reflejan que la visión histórica acerca de estos trastornos está aún arraigada. Los estudiantes de Medicina consideran que deberían recibir una educación adecuada sobre los TNF como parte de su formación por parte de los especialistas en neurología y psiquiatría.

[Clinical experience in the treatment of motor fluctuations in Parkinson's disease. Delphi consensus of a group of experts in movement disorders]. / Experiencia clínica en el tratamiento de las fluctuaciones motoras en la enfermedad de Parkinson. Consenso Delphi de un grupo de expertos en trastornos del movimiento.

INTRODUCTION: Motor fluctuations are one of the most common complications of Parkinson's disease and their treatment is still a complex matter. Therefore, from the Neurology Movement Disorders Group we present our clinical experience in the treatment of these complications, with the intention of it being useful in decision-making in daily clinical practice. DEVELOPMENT: Nineteen questions were developed based on a literature review and an open survey answered by members of this group. These issues were discussed in two phases, using the Delphi methodology. Considering the results of the survey, levodopa dose adjustment and dopamine agonists are the option with the best efficacy/tolerability ratio in the treatment of motor fluctuations. Rotigotine is useful in the motor fluctuations associated with gastroparesis, and intermittent subcutaneous apomorphine has positive effects in patients with unpredictable off periods. The most relevant adverse effect associated with dopamine agonists is impulse control disorder. Catechol-O-methyltransferase inhibitors are useful in the initial stages of motor fluctuations, especially in wearing off. Monoamine oxidase inhibitors are generally drugs that are well-tolerated and useful in motor fluctuations. If these measures are not effective, second-line treatments should be indicated on a case-by-case basis. CONCLUSION: The clinical profile of patients with Parkinson's disease is paramount in deciding the most appropriate therapy for the treatment of motor fluctuations.

TITLE: Experiencia clínica en el tratamiento de las fluctuaciones motoras en la enfermedad de Parkinson. Consenso Delphi de un grupo de expertos en trastornos del movimiento.Introducción. Las fluctuaciones motoras son una de las complicaciones más frecuentes en la enfermedad de Parkinson y su tratamiento sigue siendo complejo. Por ello, desde el Grupo de Trastornos del Movimiento de la Asociación Madrileña de Neurología presentamos nuestra experiencia clínica en el tratamiento de estas complicaciones, con la intención de que sea de utilidad en la toma de decisiones en la práctica clínica diaria. Desarrollo. Se elaboraron 19 preguntas a partir de una revisión bibliográfica y una encuesta abierta respondida por los miembros de dicho grupo. Dichas cuestiones se debatieron en dos fases, utilizando la metodología Delphi. Considerando los resultados de la encuesta, el ajuste de la dosis de levodopa y los agonistas dopaminérgicos son la opción con mejor relación eficacia/tolerabilidad en el tratamiento de las fluctuaciones motoras. La rotigotina es útil en las fluctuaciones motoras asociadas a gastroparesia, y la apomorfina subcutánea intermitente, en pacientes con off impredecible. El efecto adverso más relevante asociado a los agonistas dopaminérgicos es el trastorno del control de impulsos. Los inhibidores de la catecol-O-metiltransferasa son útiles en las fluctuaciones motoras de inicio, especialmente en el wearing off. Los inhibidores de la monoaminooxidasa son fármacos, en general, bien tolerados y útiles en las fluctuaciones motoras. En caso de que estas medidas no resulten eficaces, se deben indicar terapias de segunda línea de manera individualizada. Conclusión. El perfil clínico del paciente con enfermedad de Parkinson es primordial para decidir la terapia más adecuada en el tratamiento de las fluctuaciones motoras.

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.

Dopaminergic agonists in Parkinson's disease.

BACKGROUND: Non-ergoline dopamine agonists (DA) are effective treatments for Parkinson's disease (PD). This review presents the pharmacology, evidence of efficacy and safety profile of pramipexole, ropinirole, and rotigotine, and practical recommendations are given regarding their use in clinical practice. RESULTS: Extended-release formulations of pramipexole and ropinirole and transdermal continuous delivery rotigotine patches are currently available; these may contribute to stabilising of plasma levels. In early PD, the three drugs significantly improve disability scales, delay time to dyskinesia and allow a later introduction of levodopa. In late PD they reduced total 'off'-time, improved Unified Parkinson's Disease Rating Scale (UPDRS) in both 'on' and 'off' state and allowed a reduction in total levodopa dosage. A significant improvement in quality of life scales has also been demonstrated. Extended-release formulations have proved to be non-inferior to the immediate release formulations and are better tolerated (ropinirole). Despite a generally good safety profile, serious adverse events, such as impulse control disorder and sleep attacks, need to be routinely monitored. Although combination therapy has not been addressed in scientific literature, certain combinations, such as apomorphine and another DA, may be helpful. Switching from one DA to another is feasible and safe, although in the first days an overlap of dopaminergic side effects may occur. When treatment with DA is stopped abruptly, dopamine withdrawal syndrome may present. Suspending any DA, especially pramipexole, has been linked to onset of apathy, which may be severe. CONCLUSIONS: New non-ergotine DAs are a valuable option for the treatment of both early and late PD. Despite their good safety profile, serious adverse effects may appear; these effects may have a pathoplastic effect on the course of PD and need to be monitored.

[Some reflections on the pathophysiology of abnormal movements]. / Algunas reflexiones sobre la patofisiología de los movimientos anormales.

INTRODUCTION: The field of abnormal movements is an area that is in continual expansion within Neurology and treatment is currently available for many of them, at least as far as the symptoms are concerned. Yet, the exact mechanisms of operation of the neurological structures involved in movement are not fully understood. DEVELOPMENT: It seems clear that the basal ganglia play an important role, fundamentally in pseudo-automatic movements, but because they are interconnected with many other structures it is difficult to gain a precise understanding of their individual functions. There are theories based solely on anatomical data, which are not enough to account for everything. The theory of the existence of neuronal circuits seems to explain a wider part of movement, although it still has a number of shortcomings. Another theory of movement disorders is that based on neurochemistry, according to which the imbalance of certain neurotransmitters would be the causation of the disease, but this theory does not enable us to explain all the pathologies related to movement either. A number of clinical observations and the use of animal models, however, have made it possible to draw up pathophysiological hypotheses about the generation of some abnormal movements. CONCLUSIONS: All these approaches have enabled researchers to find symptomatic treatments for certain diseases, but our knowledge of the pathophysiology involved is still far from complete and the chances of enhancing the therapeutic capacity available in such cases in the future are therefore immense.

[New therapeutic options in Alzheimer's disease]. / Nuevas opciones terapéuticas en la enfermedad de Alzheimer.

INTRODUCTION: At present, cholinesterase inhibitors constitute the basis for therapy of Alzheimer's disease (AD); these drugs were rationally introduced given the loss of central cholinergic neurotransmission, even though there are many other systems affected in AD, including glutamatergic pathway. DEVELOPMENT: In addition to the loss of central cholinergic neurotransmission, biochemical evidence suggests glutamatergic dysfunction in AD and thus, therapeutic strategies directed at the glutamatergic system may be useful. These drugs include milacemide, cicloserine and ampakines (positive modulation) and memantine (negative modulation). Lithium seems to be a promising agent in AD, although the mechanism of action is poorly understood. Finally anti-inflammatory agents may be another therapeutic approach to AD. CONCLUSION: In addition to drugs acting on the cholinergic system, a large number of drugs with different mechanism could be used for the treatment and prevention of AD.

[Long-term evolution of the doses of Botulinum toxin used in laryngeal and cervical dystonias]. / Evolución a largo plazo de la dosis de toxina botulínica en las distonías laringea y cervical

INTRODUCTION: Dystonia is a neurological condition characterised by involuntary movements that give rise to abnormal postures. Different strategies have been used in the treatment of focal dystonias, but the most widely accepted at the present time involves the use of botulinum toxin type A (BTA) injections. Yet, despite its widespread use, the ideal dosages for long-term treatment are still not known with precision. AIMS: The purpose of this study is to report on our experience with long-term BTA therapy in laryngeal (LD) and in cervical dystonia (CD). PATIENTS AND METHODS: We reviewed the data concerning the dosages of BTA injected in 10 patients with LD who received treatment in our centre over a period of eight years. We also analysed the data regarding the doses of BTA injected over an eight-year period in 17 patients with CD. The data were analysed using an ANOVA for paired data. RESULTS: No significant differences were found in the highest dosages of BTA needed for the treatment of LD throughout the progression of the disease (p=0.84). These data contrast with those obtained from the analysis of the treatment of CD, which do show a gradual increase in the dose of toxin that is required (p<0.0001). CONCLUSIONS: These findings suggest that the long-term response to treatment is different in the two conditions.

[Guidelines for the treatment of spasticity in adults using Botulinum toxin]. / Guía terapéutica de la espasticidad del adulto con toxina botulínica.

AIMS: The introduction of Botulinum toxin type A (BTA) in the treatment of spasticity in adults was a large step forward in neurology and it is currently seen as the first choice treatment in focal spasticity. In an attempt to achieve the optimisation of this therapeutic resource, different clinical guidelines have been drawn up which include reviews of the evidence available about the indications and use of BTA. Spasticity is characterised by the presence of involuntary muscular hyperactivity that is often associated to pain, deformity and functional disability. From the clinical point of view, the advantages of BTA are obvious (ease of use and dosage determination, long lasting effects, reversibility should the response be inappropriate, etc.) and far outweigh its drawbacks. It can only be used after a proper selection of patients, of the therapeutic aims and of the muscular areas to be treated, and a tailor-made programme of rehabilitation must also be drawn up. Increasing experience in its use suggests that its early administration is effective in preventing or reducing the complications arising from spasticity. CONCLUSIONS: BTA is effective in the treatment of spasticity and plays a significant role if the clinical objectives involve functional aspects. At present a large amount of well-documented experience concerning its indications, effects and safety in clinical practice is already available.

[Progression of botulinum toxin type A dosage in craniocervical dystonias. An eight year comparative study]. / Evolucion de la dosis de toxina botulinica tipo A en distonias craneocervicales. Estudio comparativo a lo largo de ocho años.

INTRODUCTION: Botulinum toxin type A (BTA) is currently the choice treatment for focal dystonias; yet long term response to therapy is still not known with total accuracy. PATIENTS AND METHODS: In this study we analysed the dose of BTA used in the first eight years' treatment of 17 patients with cervical dystonia and 16 patients with blepharospasm who received treatment at our hospital. RESULTS: It was found that in the patients with cervical dystonia there was a significant increase in the dosage of BTA (41%) which rose in a linear fashion from the fourth year onwards. On the other hand, in the group of patients with blepharospasm, the dosage of BTA tended to drop with time and this reduction (16%) occurred essentially during the first four years of treatment. CONCLUSIONS: These findings clearly highlight the clinical and functional differences between the two types of craniocervical dystonia.

[Spinocerebellar ataxia type 8: the case of a Spanish family]. / Ataxia espinocerebelosa tipo 8: presentación de una familia española.

INTRODUCTION: Dominant autosomic ataxias include a group of neurodegenerative diseases characterized by the abnormal expansion of triplets. CASE REPORT: Male aged 33, with expansion of the SCA 8 gene (100 repetitions), who presented a clinical picture compatible with a pancerebellar syndrome. The patient had been diagnosed 11 years earlier as suffering from previously of histiocytosis X. A clinico genetic study was conducted on the patient and several members of his family (parents and two sisters). Both sisters and the father were found to be carriers of the expansion (110 and 150 repetitions, respectively), and are currently asymptomatic. RESULTS AND DISCUSSION: There is no relation between the number of repetitions and the age of onset of the disease. The normal interval in our population oscillates between 16 37 repetitions, and the pathological interval has not been well determined. There may be a relation between the SCA 8 form and histiocytosis X.

[Distribution of dominant hereditary ataxias and Friedreich's ataxia in the Spanish population]. / Distribución de ataxias hereditarias dominantes y ataxia de Friedreich en la población española.

BACKGROUND: To establish the distribution of the different forms of dominant ataxias and Friedreich ataxia in Spanish population. PATIENTS AND METHODS: We have performed a molecular study in 121 patients presenting ataxia as the first sign of neurodegenerative disease. In these patients, we have performed a molecular study of SCA 1, SCA 2, SCA 3, SCA 6, SCA 7, SCA 8, DRPLA, alpha-TTP (tocopherol transfer protein) and Friedreich's ataxia genes. RESULTS: The study showed that the Friedreich ataxia is the most frequent form representing 34.4% of the total of the hereditary ataxias. One patient presented mutation in alpha-TTP gene. Among the dominant forms SCA 3 was the most frequent (27.3%) followed by SCA 7 (16%), SCA 6 (9%) and SCA 2 (4.5%). We have not found mutations in SCA 1 and DRPLA genes. Two of 60 apparently sporadic cases presented mutations in the SCA 6 and SCA 8. CONCLUSIONS: The genetic analysis is the principal method to distinguish the different clinic forms of ataxia. We have not found mutations in 41.2% of dominant forms and in 43.3% of recessive forms. These results suggest the existence of new candidates genes.