[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.

[Healthy sleep: evidence and guidelines for action. Official document of the Spanish Sleep Society]. / Sueño saludable: evidencias y guias de actuacion. Documento oficial de la Sociedad Española de Sueño.

One of the main objectives of the Spanish Sleep Society is to promote healthy sleep in both the general population and in health professionals. This document aims to conduct a review of the current scientific literature on sleep habits that can serve as the basis on which to establish a set of general recommendations, regarding healthy sleep, for use by the general population in Spain as well as to identify the main challenges faced by research into sleep habits. The document has been developed by a multidisciplinary team made up of members of the Spanish Sleep Society who are experts in paediatric sleep medicine, clinical neurophysiology, pulmonology, neurology, chronobiology, physiology and psychology. The existing scientific literature dealing with sleep habits in the general population was reviewed, and the following aspects were addressed: the current state of sleep habits in the Spanish population; a generic review of the optimum number of hours of sleep; the impact of the environmental setting (noise, temperature, illumination, etc.), hours of sleep, diet and sport, together with several specific sections for children and teenagers, shift-workers and drivers of different vehicles. The conclusions from all the aspects addressed in this document have resulted in a set of final general recommendations that will serve as a guide for the general population and health professionals. Likewise, the principal environmental challenges and future lines of research are also discussed.

Trastorno de retraso de la fase del sueño y del despertar. Síndrome de retraso de fase / Delayed sleep wake phase disorder (DSWPD)

El trastorno de retraso de la fase del sueño y del despertar o síndrome de retraso de fase (SRF) es la alteración del ritmo circadiano de sueño más frecuente y suele manifestarse en la adolescencia. Se caracteriza por un retraso estable, habitualmente de más de dos horas, del inicio y del final del sueño respecto a los horarios convencionales. Clínicamente los pacientes presentan insomnio a la hora de acostarse, con gran dificultad para levantarse por la mañana en la hora deseada. Entre semana, debido a las obligaciones escolares o sociales, los niños con SRF duermen pocas horas, generándose una privación crónica de sueño que se manifestará con somnolencia diurna, fatiga, falta de atención, afectación del rendimiento escolar o absentismo escolar. Característicamente, el fin de semana o durante las vacaciones, cuando están libres de horarios, retrasan el sueño, siendo este de características normales y levantándose descansados. Es importante realizar un diagnóstico precoz para iniciar un tratamiento temprano que minimice las consecuencias del SRF. Por la imposibilidad de seguir unos horarios regulares de estudio ni de trabajo, son jóvenes a los que se califica de noctámbulos o de vagos, a pesar de sus esfuerzos repetidos por adaptarse a unos horarios convencionales, lo que aboca en altos índices de depresión, ansiedad y abuso de sustancias. El retraso de fase de sueño se confirma mediante las agendas de sueño, la actigrafía y los marcadores de fase circadianos. La higiene del sueño, la cronoterapia, la fototerapia y la administración de melatonina son los posibles tratamientos del SRF (AU)

Delayed sleep wake phase disorder (DSWPD) or delayed sleep phase disorder is the most frequent circadian rhythm sleep disorder and is commonly seen in adolescents. DSWPD is characterized by habitual by sleep onset and wake times that are usually delayed more than two hours relative to conventional sleep-wake times. Clinically, affected subjects experience difficulty falling asleep and arising at socially acceptable wake time. Enforced conventional wake times (during the school or working days), may result in chronically insufficient sleep manifested as excessive daytime sleepiness, fatigue, repetitive school absences with negative impact on their attention and academic performance. When allowed to follow their preferred schedule (during the weekends or vacation periods), the patient’s timing of sleep is delayed with normal and restoring sleep. It is very important to make an early diagnosis to initiate treatments that minimize consequences of DSWPD. Although their repetitive attempts to adapt to conventional times, their difficulties to maintain regular school or work timings leads these adolescents to be seen as lazy and not motivated, which usually results in an increase in mood disorders and drug abuse. Delay sleep phase is demonstrated by sleep log, actigraphy monitoring and in the timing of other circadian rhythms. Sleep hygiene, chronotherapy, bright light therapy or melatonin administration are the most habitual treatment of the DSWPD (AU)

Consenso sobre el uso de melatonina en niños y adolescentes con dificultades para iniciar el sueño / Consensus document on the clinical use of melatonin in children and adolescents with sleep-onset insomnia

Las dificultades para dormir en los niños y adolescentes son muy prevalentes en nuestro medio. El tratamiento se basa principalmente en terapias cognitivo-conductuales y modificación de hábitos. Sin embargo, el uso de medicamentos y sustancias para facilitar el sueño es elevado, sin existir guías clínicas que lo apoyen. La melatonina exógena es una neurohormona comercializada como suplemento nutricional que se utiliza cada vez más en los problemas de sueño, sin existir regulación sobre su uso. Se presenta el documento de consenso sobre el uso de melatonina en el insomnio de inicio, elaborado por representantes de la Asociación Española de Pediatría, la Sociedad Española de Sueño, la Sociedad Española de Pediatría Extrahospitalaria y de Atención Primaria, la Sociedad Española de Medicina de la Adolescencia, la Sociedad Española de Psiquiatría Infantil y la Sociedad Española de Neurología Pediátrica

Sleep problems are highly prevalent among our children and adolescents. Its treatment is mainly based on cognitive behavioural therapies and habit modification procedures. However, the use of sleep promoting drugs and substances is widespread without being supported by clinical guidelines. Exogenous melatonin is a neurohormone marketed as a nutritional supplement that is being increasingly used in the management of sleep problems, and with no control over its use. The consensus document is presented on the use of melatonin in sleep-onset insomnia prepared by representatives of the Spanish Paediatric Association, the Spanish Society of Sleep, the Spanish Society of Paediatric Outpatients and Primary Care, the Spanish Society for Adolescent Medicine, the Spanish Society of Child Psychiatry, and the Spanish Society of Paediatric Neurology

[Consensus document on the clinical use of melatonin in children and adolescents with sleep-onset insomnia]. / Consenso sobre el uso de melatonina en niños y adolescentes con dificultades para iniciar el sueño.

Sleep problems are highly prevalent among our children and adolescents. Its treatment is mainly based on cognitive behavioural therapies and habit modification procedures. However, the use of sleep promoting drugs and substances is widespread without being supported by clinical guidelines. Exogenous melatonin is a neurohormone marketed as a nutritional supplement that is being increasingly used in the management of sleep problems, and with no control over its use. The consensus document is presented on the use of melatonin in sleep-onset insomnia prepared by representatives of the Spanish Paediatric Association, the Spanish Society of Sleep, the Spanish Society of Paediatric Outpatients and Primary Care, the Spanish Society for Adolescent Medicine, the Spanish Society of Child Psychiatry, and the Spanish Society of Paediatric Neurology.

Narcolepsia con y sin cataplejia: una enfermedad rara, limitante e infradiagnosticada / Narcolepsy with and without cataplexy: An uncommon disabling and unrecognized disease

Aunque la narcolepsia es una enfermedad relativamente rara, su impacto en la vida del niño puede ser considerable. La narcolepsia está caracterizada por somnolencia diurna excesiva (SDE), con “ataques de sueño” en momentos inapropiados, y habitualmente acompañada de cataplejia (pérdida brusca del tono muscular y caída al suelo, frecuentemente desencadenada por risa, con preservación de la conciencia). Otros síntomas asociados son las parálisis del sueño (sensación de imposibilidad para moverse o hablar sin pérdida de conciencia), las alucinaciones hipnagógicas (sueños “vividos”, con experiencias difíciles de distinguir de la realidad) o el sueño nocturno fragmentado. Algunos niños también tienen síntomas depresivos y sobrepeso-obesidad. Esta enfermedad se ha estudiado ampliamente, pero la causa exacta no se conoce con precisión. En la narcolepsia parece existir un trastorno de las estructuras cerebrales responsables de los mecanismos de vigilia y sueño, que implica al hipotálamo dorsolateral y la hipocretina. Aunque se ha postulado un origen genético, existe una baja prevalencia de casos familiares. En términos generales, se piensa que existe una etiología multifactorial: un grupo de genes se combina con factores externos y causa finalmente la enfermedad. El tratamiento eficaz de la narcolepsia requiere no solo medicación (estimulantes, antidepresivos y oxibato sódico, principalmente) sino también realizar algunos ajustes en la vida diaria, mediante siestas programadas. El tratamiento de esta enfermedad en los niños exige un abordaje integral del paciente, que incluye un diagnóstico correcto, un tratamiento farmacológico y no farmacológico y ajustes en el entorno. Estas medidas pueden mejorar la autoestima del niño y la capacidad para conseguir una buena escolarización (AU)

Although narcolepsy is a relatively uncommon condition, its impact on a child's life can be dramatic and disabling. Narcolepsy is characterized by excessive daytime sleepiness (EDS), with brief "sleep attacks" at very unusual times and usually associated with cataplexy (sudden loss of muscle control while awake, resulting in a fall, triggered by laughter). Other symptoms frequently reported are sleep paralysis (feeling of being unable to move or speak, even totally aware), hypnagogic hallucinations (vivid dreamlike experiences difficult to distinguish from reality) or disturbed night time sleep. Some children also experience depression or overweight-obesity. Although narcolepsy has been thoroughly studied, the exact cause is unknown. It appears to be a disorder of cerebral pathways that control sleep and wakefulness, involving dorsolateral hypothalamus and hypocretin. A genetic factor has been suggested, but narcolepsy in relatives is rare. Researchers have suggested that a set of genes combines with additional factors in a person's life to cause narcolepsy. The effective treatment of narcolepsy requires not only medication (usually stimulants, antidepressants and sodium oxybate), but also adjustments in life-style (scheduled naps).Management of this condition in children demands a comprehensive approach to the patient, that includes a correct diagnosis, pharmacological and non-pharmacological treatment and adjustments in the environment. These strategies can improve the child's self-esteem and ability to obtain a good education (AU)

[Narcolepsy with and without cataplexy: an uncommon disabling and unrecognized disease]. / Narcolepsia con y sin cataplejia: una enfermedad rara, limitante e infradiagnosticada.

Although narcolepsy is a relatively uncommon condition, its impact on a child's life can be dramatic and disabling. Narcolepsy is characterized by excessive daytime sleepiness (EDS), with brief "sleep attacks" at very unusual times and usually associated with cataplexy (sudden loss of muscle control while awake, resulting in a fall, triggered by laughter). Other symptoms frequently reported are sleep paralysis (feeling of being unable to move or speak, even totally aware), hypnagogic hallucinations (vivid dreamlike experiences difficult to distinguish from reality) or disturbed night time sleep. Some children also experience depression or overweight-obesity. Although narcolepsy has been thoroughly studied, the exact cause is unknown. It appears to be a disorder of cerebral pathways that control sleep and wakefulness, involving dorsolateral hypothalamus and hypocretin. A genetic factor has been suggested, but narcolepsy in relatives is rare. Researchers have suggested that a set of genes combines with additional factors in a person's life to cause narcolepsy. The effective treatment of narcolepsy requires not only medication (usually stimulants, antidepressants and sodium oxybate), but also adjustments in life-style (scheduled naps). Management of this condition in children demands a comprehensive approach to the patient, that includes a correct diagnosis, pharmacological and non-pharmacological treatment and adjustments in the environment. These strategies can improve the child's self-esteem and ability to obtain a good education.

Episodio nocturno de vómitos y desviación tónica de los ojos / Nocturnal episode of vomiting and tonic deviation of the eyes

No disponible

[Is benign childhood paroxysmal eye deviation a non-epileptic disorder?]. / Desviación ocular paroxística benigna infantil: un trastorno no epiléptico?

INTRODUCTION: Benign childhood paroxysmal eye deviation (BCPED) is classified as a 'non-epileptic paroxysmal disorder'. CASE REPORTS: We report the cases of four patients aged between 6 months and 2 years, who suffered brief episodes of upward conjugate gaze deviation, with no clonic movements or associated cognitive deterioration. These episodes, which lasted several seconds, appeared in short repeated bouts that became worse with fatigue. Results of the neurological exploration, laboratory examinations, neuroimaging (CAT, MRI, brain ultrasonography) and a neurophysiological study, which included EEG-video monitoring and EEG performed during the waking state, were all normal. A nocturnal polysomnographic study was later conducted for 7-8 hours and EEG, EMG and EOG readings were recorded. The trace showed focal or generalised paroxysmal discharges during non-REM sleep in the form of polyspike-wave and spike-wave complexes. Sleep analysis (Reschstaffen and Kales) showed only a shortened REM sleep latency, with no clear clinical meaning. Several cases have been reported in the literature with identical symptoms and normal results in the diagnostic tests, including daytime polysomnography. CONCLUSIONS: The appearance of these epileptic anomalies in the nocturnal study makes it necessary to perform a complete nocturnal polysomnography. In spite of these findings, BCPED courses favourably and has a benign prognosis both with and without antiepileptic treatment. We therefore believe that BCPED should be classed within the group of 'benign idiopathic epilepsies of childhood'.

Desviación ocular paroxística benigna infantil: ¿un trastorno no epiléptico? / Is benign childhood paroxysmal eye deviation a non-epileptic disorder?

Introducción. La desviación ocular paroxística benigna infantil (DOPBI) se clasifica como un 'trastorno paroxístico no epiléptico'. Casos clínicos. Presentamos cuatro pacientes de entre 6 meses y 2 años de edad, con breves episodios de desviación conjugada de la mirada hacia arriba, sin movimientos clónicos ni deterioro cognitivo asociado. Estos episodios, con una duración de varios segundos, aparecieron en salvas y se incrementaron con la fatiga. La exploración neurológica, los exámenes de laboratorio, neuroimagen (TAC, RM, ecografía cerebral) y estudio neurofisiológico, incluido registro vídeo-EEG y EEG durante la vigilia, fueron todos ellos normales. Posteriormente, se practicó estudio polisomnográfico nocturno durante 7-8 h, y se registró simultáneamente EEG, EMG y EOG. El trazado mostró descargas paroxísticas, focales o generalizadas, durante el sueño no REM en forma de complejos punta-onda y polipunta-onda. El análisis de sueño (Rechtschaffen y Kales) mostró únicamente una latencia de sueño REM acortada, sin un claro significado clínico. En la bibliografía se han descrito varios casos con síntomas idénticos y normalidad de las pruebas diagnósticas, incluida la polisomnografía diurna. Conclusiones. El hallazgo de estas anomalías epilépticas en el estudio nocturno exige la práctica de polisomnografía nocturna completa. A pesar de estos hallazgos, la DOPBI tiene un curso favorable y un pronóstico benigno con o sin tratamiento antiepiléptico. Por ello, creemos que la DOPBI debería incluirse en el capítulo de 'epilepsias benignas idiopáticas infantiles' (AU)

Introduction. Benign childhood paroxysmal eye deviation (BCPED) is classified as a ‘non-epileptic paroxysmal disorder’. Case reports. We report the cases of four patients aged between 6 months and 2 years, who suffered brief episodes of upward conjugate gaze deviation, with no clonic movements or associated cognitive deterioration. These episodes, which lasted several seconds, appeared in short repeated bouts that became worse with fatigue. Results of the neurological exploration, laboratory examinations, neuroimaging (CAT, MRI, brain ultrasonography) and a neurophysiological study, which included EEG-video monitoring and EEG performed during the waking state, were all normal. A nocturnal polysomnographic study was later conducted for 7-8 hours and EEG, EMG and EOG readings were recorded. The trace showed focal or generalised paroxysmal discharges during non-REM sleep in the form of polyspike-wave and spike-wave complexes. Sleep analysis (Rechtschaffen and Kales) showed only a shortened REM sleep latency, with no clear clinical meaning. Several cases have been reported in the literature with identical symptoms and normal results in the diagnostic tests, including daytime polysomnography. Conclusions. The appearance of these epileptic anomalies in the nocturnal study makes it necessary to perform a complete nocturnal polysomnography. In spite of these findings, BCPED courses favourably and has a benign prognosis both with and without antiepileptic treatment. We therefore believe that BCPED should be classed within the group of ‘benign idiopathic epilepsies of childhood (AU)

Parkinson's disease and sleepiness: an integral part of PD.

OBJECTIVE: To investigate the potential causes of excessive daytime sleepiness in patients with PD-poor sleep quality, abnormal sleep-wakefulness control, and treatment with dopaminergic agents. METHODS: The authors performed night-time polysomnography and daytime multiple sleep latency tests in 54 consecutive levodopa-treated patients with PD referred for sleepiness, 27 of whom were also receiving dopaminergic agonists. RESULTS: Sleep latency was 6.3 +/- 0.6 minutes (normal >8 minutes), and the Epworth Sleepiness score was 14.3 +/- 4.1 (normal <10). A narcolepsy-like phenotype (> or = 2 sleep-onset REM periods) was found in 39% of the patients, who were sleepier (4.6 +/- 0.9 minutes) than the other 61% of patients (7.4 +/- 0.7 minutes). Periodic leg movement syndromes were rare (15%, range 16 to 43/h), but obstructive sleep apnea-hypopnea syndromes were frequent (20% of patients had an apnea-hypopnea index >15/h; range 15.1 to 50.0). Severity of sleepiness was weakly correlated with Epworth Sleepiness score (r = -0.34) and daily dose of levodopa (r = 0.30) but not with dopamine-agonist treatment, age, disease duration, parkinsonian motor disability, total sleep time, periodic leg movement, apnea-hypopnea, or arousal indices. CONCLUSIONS: In patients with PD preselected for sleepiness, severity of sleepiness was not dependent on nocturnal sleep abnormalities, motor and cognitive impairment, or antiparkinsonian treatment. The results suggest that sleepiness-sudden onset of sleep-does not result from pharmacotherapy but is related to the pathology of PD.