Sleep disturbance: a forgotten syndrome in patients with Chiari I malformation.

INTRODUCTION: Chiari type I malformation (CM-I) is characterised by caudal ectopia of the cerebellar tonsils through the foramen magnum. This is associated with brain stem, high spinal cord, and cranial nerve compression phenomena. The most frequent symptoms are occipital headaches and dizziness. Less well-known symptoms are sleep disorders and nocturnal respiratory abnormalities. SOURCES: MEDLINE and information from patients evaluated at the Neurosurgery and Clinical Neurophysiology Departments at Hospital Universitario Vall d'Hebron. DEVELOPMENT: Review article based on data obtained from MEDLINE articles since 1966, using combinations of the following keywords: «Chiari malformation¼ or «Arnold-Chiari malformation¼ and «sleep apnea¼ or «sleep disorders¼. CONCLUSIONS: CM-I patients show a higher prevalence of sleep disorders than that observed in the general population. Some studies report a 50% prevalence of sleep apnea-hypopnea syndrome (SAHS), probably associated with sudden death in some cases. These results support analysing sleep respiratory parameters in theses patients. Identifying SAHS symptoms may help optimise treatment, thereby improving quality of life and prognosis.

Fibrosis endomiocárdica tropical en nuestro medio / Tropical endomyocardial fibrosis in Spain

La fibrosis endomiocárdica es una miocardiopatía restrictiva de etiología desconocida, prevalente en regiones tropicales. Se caracteriza por la obliteración fibrótica del ápex de uno o ambos ventrículos, que puede extenderse a la cámara de entrada ventricular. Afecta predominantemente a niños y adultos jóvenes. No existe un tratamiento específico y suele tener un pobre pronóstico. No es una entidad frecuente en nuestro medio pero debido a la inmigración estamos presenciando un auge de la misma. Presentamos 4 casos clínicos de fibrosis endomiocárdica en niños diagnosticados y tratados en nuestro medio, y realizamos una revisión de la misma (AU)

Endomyocardial fibrosis is a restrictive cardiomyopathy of unknown etiology prevalent in tropical regions. It is characterized by fibrotic obliteration of the apex of one or both ventricles, which can be extended to the ventricular inlet chamber. It predominantly affects children and young adults. There is no specific treatment and usually has a poor prognosis. It is uncommon in Spain, but due to immigration, it appears to be increasing. Four cases of endomyocardial fibrosis in children diagnosed and treated in Spain are presented, as a well as a review of this disease (AU)

[Tropical endomyocardial fibrosis in Spain]. / Fibrosis endomiocárdica tropical en nuestro medio.

Endomyocardial fibrosis is a restrictive cardiomyopathy of unknown etiology prevalent in tropical regions. It is characterized by fibrotic obliteration of the apex of one or both ventricles, which can be extended to the ventricular inlet chamber. It predominantly affects children and young adults. There is no specific treatment and usually has a poor prognosis. It is uncommon in Spain, but due to immigration, it appears to be increasing. Four cases of endomyocardial fibrosis in children diagnosed and treated in Spain are presented, as a well as a review of this disease.

[Cognitive event-related brain potentials (P300) in patients with normal pressure hydrocephalus. Results of a prospective study]. / Potenciales evocados cognitivos (P300) en pacientes con hidrocefalia de presión normal.

OBJECTIVE: To determine the response to cognitive event-related potentials (P300) in patients with normal-pressure hydrocephalus (NPH) and their relationship with clinical and cognitive status before and after shunt surgery. METHODS: We performed a prospective study in a series of 26 patients with NPH who underwent clinical and cognitive assessment before surgery and 6 months afterwards. Visual P300 potentials obtained before and after treatment were also compared with those obtained in 18 healthy volunteers. RESULTS: Before shunting, the P300 wave was detected in 11 (42.3%) NPH patients, compared with the 18 (100%) volunteers. Six months after shunting, the P300 wave was found in 20 (76.9%) NPH patients. P300 latency was significantly longer in NPH patients than in the control group before surgery, but not at 6 months after surgery. No significant differences in neuropsychological studies or in the level of dependence for daily life activities were found between the subgroups of NPH patients with and without pre-surgical P300 waves, or between changes in P300 parameters and clinical and cognitive changes. CONCLUSIONS: The P300 wave was delayed or undetectable in a substantial percentage of patients with NPH before surgery. These alterations can be reversed by shunting. P300 analysis and neuropsychological tests could be complementary measures to evaluate functional status in patients with NPH.

Population measurement of the 3p(5)4s configuration levels in an argon microwave plasma at atmospheric pressure.

Metastable atoms, due to their intrinsic properties, are very useful to characterize plasma and to control the scientific and technological applications carried out with it. This paper describes the implementation of a method to determine the densities of the argon 3p(5)4s metastable and resonant levels in a microwave plasma at atmospheric pressure. Because the method is based on the self-absorption of the radiation emitted by the plasma, no external radiation source is needed. Using this method, the populations of the four levels of the 3p(5)4s argon configuration were found to be in the range 10(10)-10(12) cm(-3), in agreement with the values found in the literature.

Abel inversion applied to a small set of emission data from a microwave plasma.

In this work we propose a criterion to apply the Abel inversion in the case of a small set of experimental data to be used in laboratory plasmas. The Nestor-Olsen method, spline interpolation, and Fourier transform Abel inversion have been compared in order to study the influence of statistical noise and the number of sampled data. The application of this criterion permits us to obtain a radial distribution of the plasma parameters (densities and temperatures) from the spectral line profiles emitted by the discharge. The proposed criterion has been tested using the lateral intensities of several lines emitted by a microwave helium plasma column generated at atmospheric pressure.

Spectroscopic characterization of two different microwave (2.45 GHz) induced argon plasmas at atmospheric pressure.

A surface-wave-sustained discharge created by using a surfatron device in a tube open to the atmosphere can be used to maintain a microwave (2.45 GHz) plasma at atmospheric pressure at powers of less than 300 W. The TIA (Torche a Injection Axiale) is a device also producing a plasma that, moreover, permits us to work at high power (higher than 200 W and up to 1000 W). A study of the departure from the thermodynamic equilibrium existing in the argon plasmas created by both devices has been done by using optical emission spectroscopy techniques in order to characterize them and to evaluate their possible advantages when they are used for applied purposes.

Experimental method for determining the damping parameter of spectral lines emitted by a microwave plasma at atmospheric pressure.

In this work, a simple method for experimentally obtaining the value of the damping parameter or a-parameter of the spectral lines emitted by an argon plasma generated at atmospheric pressure is presented. The value of this coefficient indicates the proportion existing between the Lorentzian and Doppler components of the total line profile, which can be approximated to a Voigt function for our experimental conditions. The a-parameter values found were within the value interval recorded in the literature. The results obtained showed that the damping coefficient of the lines next to the fundamental level remains practically constant along the plasma column, whereas for the spectral lines involving high-lying levels, the a-parameter is sensitive to the changes in the electron density in the plasma. In this work it is also proved that the self-absorption phenomenon induces errors in the calculation of a, due to an increase in the broadening of the line profile produced by this phenomenon.

Electron density and gas temperature from line broadening in an argon surface-wave-sustained discharge at atmospheric pressure.

We have used the collisional broadening of neutral argon lines to determine the electron density and gas temperature of a microwave discharge at atmospheric pressure. The gas temperature can be obtained from the Van der Waals broadening, provided that the Stark broadening is negligible. This can be achieved by using lines from low-lying levels (close to the ground state). On the other hand, lines corresponding to transitions from high-lying levels, which are more sensitive to Stark (quadratic) broadening, can be utilized to determine electron density. The electron density values obtained from the quadratic Stark broadening of argon atoms are in reasonable agreement with those derived from the linear Stark broadening of the H(beta) line. The proposed method ensures perturbation-free access to plasma parameters, which is not the case when adding hydrogen to the discharge, even in a small amount, to observe the Balmer series lines.

Miocardiopatía dilatada neonatal: a propósito de un caso / Neonatal dilated cardiomyopathy: a case report

Se comunica un nuevo caso de miocardiopatía dilatada neonatal en una recién nacida que, a las pocas horas del parto, comenzó con un cuadro de insuficiencia cardiaca congestiva (ICC) con tendencia al colapso cardiovascular. Las ecocardiografías 2D y Doppler demostraron una función del ventrículo izquierdo muy deprimida, con una fracción de acortamiento del 12 por ciento y una fracción de eyección del 27 por ciento. No se pudo determinar la etiología de la alteración, aunque no se descartó una base génica o familiar. La paciente, que reunía criterios de mal pronóstico evolutivo, se mantuvo con digoxina, furosemida, ácido acetilsalicílico (AIS) y enalapril en una situación de ICC crónica, falleciendo a los tres meses de vida a la espera de un trasplante cardiaco. En el presente trabajo se revisan aspectos de interés de esta afección (AU)

Diagnóstico prenatal de rabdomioma cardiaco / Prenatal diagnosis of cardiac rhabdomyoma

Presentamos un caso clínico de diagnóstico precoz prenatal de rabdomioma cardiaco. El seguimiento ecográfico prenatal revela un aumento progresivo de tamaño de la masa tumoral. Ante la presencia de derrame pericárdico, se programa cesárea a las 38 semanas de gestación. Durante el periodo neonatal ha permanecido estable (AU)

Self-reported sleepiness while driving as a risk factor for traffic accidents in patients with obstructive sleep apnoea syndrome and in non-apnoeic snorers.

The obstructive sleep apnoea syndrome (OSAS) is a condition causing daytime sleepiness and has been related to an increased risk for traffic accidents. However, the evidence linking severity of OSAS to a higher rate of automobile crashes is based on limited data. The aims of this study were to study the traffic accident rate in the last 5 years in patients referred to our sleep clinic because of clinical suspicion of OSAS and to analyse variables related to an increased risk for traffic accidents. A series of 189 consecutive patients with a driving license referred for a sleep study because of OSAS clinical suspicion and a control group (CG) of 40 hospital staff workers who denied snoring, matched for age and sex with the study population, were studied. Patients underwent a full-night polysomnography and both patients and the CG completed a self-answered questionnaire. One hundred and twenty-two patients were diagnosed as OSAS and 67 patients as non-apnoeic snorers (NAS). The self-reported number of accidents was significantly higher in OSAS patients compared with CG. The self-reported number of times off the road was significantly higher in OSAS patients compared with NAS and with CG. Variables associated with an increased risk for traffic accidents were self-reported sleepiness while driving (OR 5, 95%CI 2.3-10.9), having quit driving because of sleepiness (OR 3, 95%CI 1.1-8.6) and being currently working (OR 2.8, 95%CI 1.1-7.7). We conclude that self-reported sleepiness while driving is associated with an increased risk for traffic accidents in OSAS patients and in NAS. We suggest that this symptom can be used to alert patients and to give priority in the sleep clinic for study and treatment.

[Modifications in sleep with aging]. / Modificaciones del sueño en el envejecimiento.

INTRODUCTION: Literature reviewed shows that aging sleep is characterized by a decrease in the ability to stay asleep, resulting in a more fragmented sleep and a decrease in daytime alertness. It exists also an advance of phase, a shortening of wake-sleep period and a desincronitation of circadian rhythms. DEVELOPMENT AND CONCLUSIONS: Changes in circadian rhythms are associated with a decrease of sleep quality. There are a marked reduction of deep slow wave sleep, sleep spindles are less frequent, less ample and shorter. REM sleep appearance is almost uniform during night and REMs density does not increase toward the end of the sleep period. The sleep-wake circadian rhythm is advanced, the temperature rhythm is advanced and the rhythm of cortisol secretion is also advanced. The GH and melatonin peaks of secretion are decreased in elderly.

[Diagnosis and treatment of the phobia due to treatment with air using nasal continuous pressure]. / Diagnóstico y tratamiento de la fobia a la terapia con presión nasal positiva continua de aire.

INTRODUCTION: The obstructive sleep apnoea syndrome (OSAS) is characterized by destructured sleep due to repeated episodes of obstruction of the superior respiratory tract during sleep. Treatment by nasal continuous positive airway pressure (n-CPAP) using air is one of the most common forms of treatment of OSAS, although some patients cannot tolerate it. One of the causes of intolerance of n-CPAP is the phobic reaction of some patients. OBJECTIVE: The objective of this study is to show that phobia is one of the causes of n-CPAP not being accepted by OSAS patients and demonstrates the efficacy of treatment by exposure in cases of phobic fear of mask. Patients and methods. We studied six patients with phobic intolerance of CPAP and established the diagnosis of phobia according to the criteria of DSM-IV. Subsequently, they were treated by real-life exposure, one of the behavior techniques most often used for phobic disorders, and consisting of exposure to the feared object under conditions in which escape or avoidance is impossible. CONCLUSION: Following the diagnosis of phobia of the CPAP mask, it was seen that treatment involving real-life exposure was effective.

[Accidents prevalence in a group of patients with the narcolepsy- cataplexy syndrome]. / Prevalencia de accidentes en un grupo de pacientes afectos de síndrome de narcolepsia-cataplejía.

INTRODUCTION: Recent studies have shown statistically that the proportion of accidents at work, at home and on the roads is greater in narcoleptic patients than in the general population. OBJECTIVE: The object of this study is to show the high prevalence of the risk of accidents in patients diagnosed as having the narcolepsy-cataplexy syndrome. PATIENTS AND METHODS: We studied a group of 35 patients of both sexes, diagnosed in the Sleep Pathology Unit of our hospital between 1994 and 1998 as having the narcolepsy-cataplexy syndrome. They filled out a questionnaire to find the prevalence of accidents. The data obtained regarding the type and number of accidents, their causes, gravity, legal and economic consequences were compared with those obtained in a group of 25 healthy subjects using the chi squared test. RESULTS: The results showed a higher risk of accidents in narcoleptic as compared with normal persons, with a statistically significant difference between the two groups. CONCLUSIONS: This study has shown the greater risk of accidents in these patients and the need for diagnosis as soon as possible so as to establish suitable treatment and thus improve their own personal safety and that of those around them.

Cognitive changes after cerebrospinal fluid shunting in young adults with spina bifida and assumed arrested hydrocephalus.

OBJECTIVES: To establish whether surgery can improve the neuropsychological functioning of young adult patients with spina bifida and apparent clinically arrested hydrocephalus showing abnormal intracranial pressure. METHODS: Twenty three young adults with spina bifida and assumed arrested hydrocephalus (diagnosed as active or compensated by continuous intracranial pressure monitoring) underwent surgery. All patients received neuropsychological examination before surgery and 6 months later. Neuropsychological assessment included tests of verbal and visual memory, visuospatial functions, speed of mental processing, and frontal lobe functions. RESULTS: Shunt placement in this subgroup of patients improves neuropsychological functioning, especially in verbal and visual memory and attention and cognitive flexibility. CONCLUSIONS: Young adults with spina bifida and suspected non-functioning shunt or non-shunted ventriculomegaly should be carefully monitored to identify those who could benefit from shunting.

Diagnóstico y tratamiento de la fobia a la terapia con presión nasal positiva continua de aire / Diagnosis and treatment of the phobia due to treatment with air using nasal continuous positive pressure

Introducción. El síndrome de apneas obstructivas del sueño (SAOS) se caracteriza por la desestructuración del sueño debido a repetidos episodios de obstrucción de las vías aéreas superiores durante el sueño. El tratamiento por medio de presión nasal positiva continua de aire (n-CPAP, del inglés nasal Continuous Positive Airway Pressure) es uno de los tratamientos más comunes para el SAOS, si bien algunos pacientes que lo requieren no toleran su aplicación. Una de las causas de la intolerancia al n-CPAP es la reacción fóbica que experimentan algunos pacientes. Objetivo. El objetivo del trabajo es poner de manifiesto la fobia como una de las causas de la no aceptación del n-CPAP en los pacientes con SAOS y reflejar la eficacia del tratamiento con exposición frente al miedo fóbico a la mascarilla. Pacientes y métodos. Se estudian seis pacientes que presentan una intolerancia fóbica al CPAP y se establece el diagnóstico de fobia según los criterios DSM-IV. Posteriormente, se les realiza un tratamiento de exposición en vivo, una de las técnicas conductuales más utilizadas para los trastornos fóbicos, consistente en la exposición al objeto temido sin posibilidad de escape o evitación. Conclusión. Tras el diagnóstico de fobia a la mascarilla del n-CPAP se evidencia que el tratamiento mediante exposición en vivo resulta eficaz (AU)

Modificaciones del sueño en el envejecimiento / Modifications in sleep with aging

Introducción. La revisión de la literatura muestra que el sueño de los ancianos se caracteriza por una disminución de la habilidad para permanecer dormidos, lo que da lugar a un aumento de la fragmentación del sueño y a una disminución de la alerta durante el día. Existe también un avance de fase, un acortamiento del período de vigilia-sueño y una desincronización de los ritmos circadianos. Desarrollo y conclusiones. Los cambios en los ritmos circadianos se asocian a una disminución de la calidad del sueño. Existe una marcada disminución del sueño profundo de ondas lentas. Los husos de sueño son menos frecuentes, menos amplios y más cortos. Los sueños REM son más uniformes durante la noche y la densidad REM no se incrementa a medida que transcurre el sueño. El ritmo circadiano vigilia-sueño presenta un avance de fase, así como el ritmo de la temperatura y el de la secreción de cortisol. Los picos de secreción de hormona de crecimiento y de melatonina aparecen disminuidos en la vejez (AU)

Prevalencia de accidentes en un grupo de pacientes afectos de síndrome de narcolepsia-cataplejía / Accident prevalence in a group of patients with the narcolepsy-cataplexy syndrome

Introducción. Estudios recientes han demostrado estadísticamente que la proporción de accidentes laborales, domésticos y de tráfico es mayor en pacientes narcolépticos que en la población general. Objetivo . El objeto de este trabajo es poner de manifiesto la elevada prevalencia del riesgo de accidentes en los pacientes diagnosticados de síndrome de narcolepsia-cataplejía. Pacientes y métodos. Se ha estudiado un grupo de 35 pacientes de ambos sexos, diagnosticados de síndrome de narcolepsia-cataplejía en la Unidad de Patología del Sueño de nuestro hospital, entre 1994 y 1998, que cumplimentaron un cuestionario para conocer la prevalencia de accidentes. Los datos obtenidos sobre tipo y número de accidentes, motivo y gravedad de los mismos, así como las consecuencias legales y económicas, se compararon con los obtenidos en un grupo de 25 sujetos sanos mediante el test estadístico de la ji al cuadrado. Resultados. Los resultados demuestran el elevado riesgo de accidentes en los pacientes narcolépticos al compararlos con los sujetos normales, observándose una diferencia estadísticamente significativa entre ambos grupos. Conclusiones. De este estudio se desprende el elevado riesgo de accidentes que presentan estos pacientes y la necesidad de diagnosticarlos lo más precozmente posible a fin de establecer el tratamiento adecuado con la consiguiente mejora de su seguridad personal y la de su entorno (AU)