The Spanish Society of Neurology's official clinical practice guidelines for epilepsy. Special considerations in epilepsy: comorbidities, women of childbearing age, and elderly patients.

INTRODUCTION: The characteristics of some population groups (patients with comorbidities, women of childbearing age, the elderly) may limit epilepsy management. Antiepileptic treatment in these patients may require adjustments. DEVELOPMENT: We searched articles in Pubmed, clinical practice guidelines for epilepsy, and recommendations by the most relevant medical societies regarding epilepsy in special situations (patients with comorbidities, women of childbearing age, the elderly). Evidence and recommendations are classified according to the prognostic criteria of Oxford Centre of Evidence-Based Medicine (2001) and the European Federation of Neurological Societies (2004) for therapeutic interventions. CONCLUSIONS: Epilepsy treatment in special cases of comorbidities must be selected properly to improve efficacy with the fewest side effects. Adjusting antiepileptic medication and/or hormone therapy is necessary for proper seizure management in catamenial epilepsy. Exposure to antiepileptic drugs (AED) during pregnancy increases the risk of birth defects and may affect fetal growth and/or cognitive development. Postpartum breastfeeding is recommended, with monitoring for adverse effects if sedative AEDs are used. Finally, the elderly are prone to epilepsy, and diagnostic and treatment characteristics in this group differ from those of other age groups. Although therapeutic limitations may be more frequent in older patients due to comorbidities, they usually respond better to lower doses of AEDs than do other age groups.

[Progressive Brown-Sequard syndrome secondary to idiopathic spinal cord herniation: clinico-radiological and surgical correlations]. / Síndrome de Brown-Séquard progresivo secundario a hernia medular idiopática: correlación clinicorradiológica y quirúrgica.

INTRODUCTION: Idiopathic medullary herniation is an infrequent disease, which shows up in clinical form as a progressive mielopathy, most commonly known as the Brown-Sequard syndrome. Its anatomical base is a dural defect where a portion of anterior spinal cord gets progressively incarcerated. The MRI and myelo-CT scan show a bending of the spinal cord in the form of a «bell tent¼ towards the anterior dural sheath at the mid-dorsal portion mainly. CASE REPORT: A 37 year old male, who was diagnosed of idiopathic medullary herniation and surgically treated by our own developed technique, reporting its neuroradiological, anatomo-surgical and clinical correlation. CONCLUSION: Treatment should be individualized, as no standard surgical technique has been established up to the present.

TITLE: Síndrome de Brown-Séquard progresivo secundario a hernia medular idiopática: correlación clinicorradiológica y quirúrgica.Introducción. La hernia medular idiopática es una patología infrecuente que cursa clínicamente con una mielopatía progresiva, la mayoría de las ocasiones en forma de síndrome de Brown-Séquard. Su base anatómica es un defecto dural por el que se incarcera progresivamente una porción del cordón medular anterior. La resonancia magnética y la mielotomografía demuestran un acodamiento medular en «tienda de campaña¼ hacia la cara anterior del estuche dural, a nivel dorsal medio fundamentalmente. Caso clínico. Varón de 37 años, diagnosticado de hernia medular idiopática e intervenido quirúrgicamente mediante una técnica propia; se demuestra su correlación neurorradiológica, anatomoquirúrgica y evolutiva. Conclusión. El tratamiento debe ser individualizado, pues no existe una técnica quirúrgica universalmente establecida.

[Sleep-related movement and behavioural disorders in adults]. / Trastornos del movimiento y de la conducta durante el sueño en el adulto.

Sleep-related movement and behaviour disorders may have an impact on sleep quality and lead to daytime symptoms. These groups of conditions include diseases such as restless legs syndrome, periodic leg movements, and REM and NREM parasomnias. The knowledge of their clinical features and management is of utmost importance for the neurologist and sleep specialist. Frequently, these patients are referred to such specialists and it is relevant to know that certain sleep disorders may be associated with other neurological conditions.

TITLE: Trastornos del movimiento y de la conducta durante el sueño en el adulto.Los trastornos del movimiento y de la conducta durante el sueño pueden tener un impacto en la calidad del sueño del paciente y dar lugar a síntomas diurnos. En estos grupos de enfermedades se incluyen entidades como el síndrome de piernas inquietas, los movimientos periódicos de las piernas y las parasomnias del sueño de movimientos oculares rápidos (REM) y no REM. El conocimiento de sus características clínicas y nociones sobre su manejo es de gran importancia para el neurólogo y especialista en sueño por su frecuencia e impacto en la calidad del sujeto. Con frecuencia, estos pacientes son referidos a dichos especialistas, y es relevante conocer que ciertos trastornos del sueño pueden asociarse a otras enfermedades neurológicas.

Clinical guide for the diagnosis and follow-up of myotonic dystrophy type 1, MD1 or Steinert's disease. / Guía clínica para el diagnóstico y seguimiento de la distrofia miotónica tipo 1, DM1 o enfermedad de Steinert.

BACKGROUND AND OBJECTIVES: Steinert's disease or myotonic dystrophy type 1 (MD1), (OMIM 160900), is the most prevalent myopathy in adults. It is a multisystemic disorder with dysfunction of virtually all organs and tissues and a great phenotypical variability, which implies that it has to be addressed by different specialities with experience in the disease. The knowledge of the disease and its management has changed dramatically in recent years. This guide tries to establish recommendations for the diagnosis, prognosis, follow-up and treatment of the complications of MD1. MATERIAL AND METHODS: Consensus guide developed through a multidisciplinary approach with a systematic literature review. Neurologists, pulmonologists, cardiologists, endocrinologists, neuropaediatricians and geneticists have participated in the guide. RECOMMENDATIONS: The genetic diagnosis should quantify the number of CTG repetitions. MD1 patients need cardiac and respiratory lifetime follow-up. Before any surgery under general anaesthesia, a respiratory evaluation must be done. Dysphagia must be screened periodically. Genetic counselling must be offered to patients and relatives. CONCLUSION: MD1 is a multisystemic disease that requires specialised multidisciplinary follow-up.

[The importance of adherence to treatment in control of epilepsy]. / Relevancia de la adhesión al tratamiento en el control de la epilepsia.

TITLE: Relevancia de la adhesión al tratamiento en el control de la epilepsia.

[«Apuntes en Neurologia¼ (Notes in Neurology): a synthesis of the evidence on common paroxysmal neurological disorders and on neurodegenerative disorders]. / «Apuntes en Neurologia¼: una sintesis de la evidencia en trastornos neurologicos comunes paroxisticos y en trastornos neurodegenerativos.

«Apuntes en Neurologia¼ is an initiative in which prominent national and international leaders, with broad academic recognition, came together to synthesise the most outstanding clinical aspects within their area of interest and to discuss the latest developments in a more accessible language. Understanding the factors that affect the onset and progression of any neurological disease through a review is important to be able to develop strategies to reduce the burden of these diseases. Moreover, knowledge of the clinical aspects is essential to solve the problems of daily clinical practice. The data collected here reflect the weight of evidence and some of them anticipate a promising future in the treatment of these diseases. This first edition focuses on common paroxysmal neurological disorders such as migraine, epilepsy and sleep disorders, as well as neurodegenerative disorders such as Parkinson's disease and cognitive impairment. These are clearly different pathologies, although some of them such as migraine and epilepsy, may share clinical symptoms. Sleep disorders, however, are important manifestations of neurodegenerative diseases that are sometimes clinically apparent long before the onset of other neurological symptoms. After recalling pathophysiology and diagnosis, the current review focuses on bringing together the main advances in five of the major neurological diseases.

TITLE: «Apuntes en Neurologia¼: una sintesis de la evidencia en trastornos neurologicos comunes paroxisticos y en trastornos neurodegenerativos.«Apuntes en Neurologia¼ es una iniciativa en la cual lideres de primera linea nacional e internacional, con amplio reconocimiento academico, se reunieron para sintetizar los aspectos clinicos mas destacables dentro de su area de interes y acercar las novedades en una lengua mas proxima. Entender los factores que afectan al inicio y progresion de cualquier enfermedad neurologica a traves de una revision es importante para el desarrollo de estrategias en pro de reducir la carga de estas enfermedades, y conocer los aspectos clinicos es esencial para poder resolver los problemas de la practica clinica diaria. Los datos aqui recogidos reflejan el peso de la evidencia y algunos de ellos anticipan un futuro prometedor en el tratamiento de estas enfermedades. Esta primera edicion se centra en trastornos neurologicos comunes paroxisticos como la migraña, la epilepsia y las alteraciones del sueño, y en trastornos neurodegenerativos como la enfermedad de Parkinson y el deterioro cognitivo. Se trata de patologias claramente diferentes, si bien algunas de ellas, como la migraña y la epilepsia, pueden compartir sintomatologia clinica. Los trastornos del sueño, por su parte, son manifestaciones importantes de enfermedades neurodegenerativas que, en ocasiones, son clinicamente evidentes mucho antes del inicio de otros sintomas neurologicos. Tras recordar la fisiopatologia y el diagnostico, la revision actual se centra en acercar los principales avances en cinco de las principales enfermedades neurologicas.

[Epilepsy in the elderly]. / Epilepsia en ancianos.

INTRODUCTION: Epilepsy is a frequent pathology in the elderly, but its aetiology, clinical presentation and prognosis are different to those seen in younger patients. DEVELOPMENT: Beyond a certain age physiological modifications take place in the metabolism that alter the pharmacokinetics of the antiepileptic drugs (AED) and increase the risk of pharmacological interactions, which is already higher in these patients owing to the frequency of polypharmacy. Moreover, elderly patients are especially sensitive to certain side effects of AED, such as cognitive disorders, osteoporosis or weight increase. Since the efficacy of the more important AED is a priori quite similar and the epilepsies we have to deal with at this age usually have a good prognosis, the choice of AED will depend more on their pharmacokinetics and their potential to trigger certain side effects than on their effectiveness. CONCLUSIONS: The ones with the most favourable pharmacokinetic profile are levetiracetam and pregabalin, followed oxcarbazepine and lamotrigine. Additionally, these drugs generally have few cognitive effects, do not give rise to osteoporosis and, with the exception of pregabalin, do not alter the patient's weight, all of which makes them first choice drugs for the treatment of epilepsy in the elderly.

[A retrospective study comparing two formulations of valproic acid]. / Estudio retrospectivo comparando dos formulaciones de ácido valproico.

INTRODUCTION: Valproic acid is available in two different presentations, Depakine and Depakine Crono. Equivalences in plasmatic levels, efficacy and adverse effects between these two formulations are not fully established. AIM: To compare plasmatic levels, efficacy and plasmatic levels between Depakine and Depakine Crono. PATIENTS AND METHODS: We studied a retrospective series of 238 patients in treatment with Depakine or Depakine Crono and compared plasmatic levels, efficacy and adverse effects between both formulations. RESULTS: A total of 173 patients had taken one formulation and 54 both. Plasmatic levels were similar in both groups. There were more significantly more patients free of seizures among the Depakine Crono group. Significantly better outcomes were seen with Depakine Crono with respect to secondary effects. Adverse effects are significantly less frequent in men than in women. The higher the dose, the higher the incidence of adverse events. CONCLUSIONS: Depakine Crono seems to obtain similar plasmatic levels, is at least as efficacious and is associated with less adverse events that Depakine.

[A proposal for a model to replace carbamazepine or oxcarbazepine by eslicarbazepine acetate in clinical practice]. / Propuesta de modelo para sustituir carbamacepina u oxcarbacepina por acetato de eslicarbacepina en la practica clinica.

INTRODUCTION: For many years carbamazepine (CBZ) has been the reference drug for the treatment of partial epileptic seizures. However, the problems related with its pharmacokinetics and safety have led to the development of other derivatives, such as oxcarbazepine (OXC) and, more recently, eslicarbazepine acetate (ESL), which do not display these drawbacks. DEVELOPMENT: In clinical practice, the possibility of replacing CBZ or OCX by ESL is a relatively frequent occurrence, the aim being to maintain the efficacy of its predecessors and benefit from the advantages in terms of the pharmacokinetics and safety of this latter derivative. To achieve this, it is essential to have an approximate dose equivalence and exchange protocol. This review offers a practical reasoned model for carrying out the change. CONCLUSIONS: The shift from OXC to ESL can be completed from one day to the next with a dose equivalence of 1-1.5 to 1. Replacement of CBZ by ESL must be more progressive, and the dose equivalence is established as 1-1.3 to 1.

TITLE: Propuesta de modelo para sustituir carbamacepina u oxcarbacepina por acetato de eslicarbacepina en la practica clinica.Introduccion. Durante muchos a˜os, la carbamacepina (CBZ) ha sido el farmaco de referencia para el tratamiento de las crisis epilepticas parciales. Sin embargo, los problemas asociados de su farmacocinetica y tolerabilidad han llevado al desarrollo de otros derivados, como la oxcarbacepina (OXC) y, mas recientemente, el acetato de eslicarbacepina (ESL), que obvien estos inconvenientes. Desarrollo. En la practica clinica, se presenta con relativa frecuencia la posibilidad de sustituir la CBZ o la OXC por ESL, buscando mantener la eficacia de los predecesores y ganar las ventajas en el ambito de farmacocinetica y tolerabilidad que ofrece este ultimo derivado. Para ello es indispensable disponer de una equivalencia aproximada de dosis y un protocolo de intercambio. La presente revision ofrece un modelo practico y razonado para realizar el cambio. Conclusiones. El paso de OXC a ESL se puede realizar de un dia para otro con una equivalencia de dosis de 1-1,5 a 1. La sustitucion de CBZ por ESL debe ser mas progresiva, y la equivalencia de dosis se establece en 1-1,3 a 1.

[Contributions to epilepsy genetics]. / Aportaciones de la genética a las epilepsias.

INTRODUCTION AND DEVELOPMENT: In recent years the progress made in genetics has revolutionised our knowledge of the physiopathological mechanisms governing epilepsy. Just 20 years ago, none of the genes involved in epilepsy were known. Since then 11 genes have been identified and this has enabled us to better understand the mechanisms that generate the seizures. This is not only of interest to academics but can also have a practical application by helping when it comes to making a rational choice of a drug in a particular epileptic syndrome, or by allowing new drugs to be developed as new therapeutic targets are identified. CONCLUSIONS: Genetics can help us to understand the mechanisms that cause resistance to drugs and to determine the risk of a patient's suffering from idiosyncratic side effects or not. It is therefore to be expected that over the next few years it will become a fundamental body of doctrine for the daily management of epileptic patients.

[Perampanel as an early added therapy in the treatment of epilepsy]. / Perampanel como terapia añadida precoz en el tratamiento de la epilepsia.

TITLE: Perampanel como terapia añadida precoz en el tratamiento de la epilepsia.

[Integrated treatment of the symptoms of narcolepsy-cataplexy syndrome with sodium oxybate]. / Tratamiento integral con oxibato sódico de los síntomas del síndrome narcolepsia-cataplejía.

INTRODUCTION: Treatment of narcolepsy-cataplexy is based on the use of different drugs that either stimulate or modify REM sleep, which makes it possible to improve the symptoms that characterise the disease. AIM: To present the characteristics of sodium oxybate, a new drug that has been approved in the United States and in the European Union for use in treating narcolepsy-cataplexy; the main contribution made by this new agent is the possibility of acting on all the symptoms of the disease at the same time. DEVELOPMENT: In four randomised, double-blind, placebo-controlled studies and with a class A level of evidence, sodium oxybate has proved to be effective in improving the cardinal symptoms of narcolepsy-cataplexy, that is to say, hypersomnia, cataplexy and fragmentation of nocturnal sleep. Treatment was well tolerated and the side effects, consisting mainly of nausea or symptoms related with the nervous system, such as anxiety, depression, confusion or drowsiness, were mild or moderate in most cases and rarely led to the patient's giving up the treatment. The drug has a favourable pharmacokinetic profile, with little potential to interact with other pharmaceuticals. It seems to have a synergic effect with modafinil, boosting its effects in the treatment of excessive sleepiness. Although it is a substance that has been used as a recreational drug, no cases of addiction or drug abuse have been observed in narcoleptic patients treated in a controlled manner. CONCLUSIONS: Sodium oxybate can be considered to be a first-choice drug in the treatment of narcolepsy-cataplexy.

[Prevalence of breathing disorders during sleep in Spain]. / Prevalencia de los trastornos respiratorios durante el sueño en España.

Sleep-disordered breathing are frequent processes in Spain. Among them, the obstructive sleep apneas are the best known although not all studies are performed with the same diagnostic criteria with regard to the percentage of apneas and hypopneas per hour of sleep and the need to consider the presence or absence of clinic symptoms. After a reference to the biggest epidemiologic studies performed in different populations, the data of the major populational studies in our environs are analyzed.

[Analysis of gait and movement of upper limbs in muscular dystrophies]. / Análisis de la marcha y del movimiento de las extremidades superiores en distrofias musculares.

INTRODUCTION: The objective is to analyze gait and upper extremity movement in a sample of healthy individuals and patients with different muscular dystrophies and to determine the relationship of the obtained variables with the pattern of muscular involvement. SUBJECTS AND METHODS: Gait and upper limb movement was analyzed with a three-dimensional photogrammetry system. Convenience sample of 28 healthy volunteers, 40 patients with myotonic dystrophy (MD), 9 patients with facioscapulohumeral dystrophy (FSHD) and 14 patients with limb-girdle muscular dystrophy type 2A (LGMD2A). Spatio-temporal and kinematic variables were analyzed. RESULTS: Patients had lower velocity, cadence and shorter stride duration than healthy subjects. Gait and upper extremity kinematic variables suggested proximal muscular involvement in LGMD2A patients and distal muscular abnormalities in MD patients. The most characteristic finding in FSHD patients was related with dorsiflexor muscles' weakness. Some of the variables were also correlated to the patients' functional stage. CONCLUSIONS: This analysis allows the distinction of kinematic and spatio-temporal patterns of gait and upper extremity movement that correlate with muscular dystrophy's phenotype. The use of this analysis in the clinical setting to assess disease progression or the potential effects of treatments requires further studies.