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WO07 Twin sister with delayed phase disorder
Abstracts of the 13th European Congress of Clinical Neurophysiology / Clinical Neurophysiology 119 (2008), S1–S131 will unveil insufficient nocturnal sleep as the cause of hypersomnia, and in circadian rhythm sleep disorders sleep logs and actigraphy will provide a rather sound basis for the diagnosis. Although the reporting of cataplexia is clearly indicative of narcolepsy as a cause of hypersomnia, polisomnography and MSLT can be considered mandatory to confirm the diagnosis. In this condition, cerebrospinal fluid biochemistry may provide useful data with the finding of low hypocretin levels. In any event, a careful medical history and examination is of the outmost importance, since narcolepsy can also be secondary to several medical conditions. Polysomnography can be useful in idiopathic hypersomnias with long or short sleep time, but in these cases appropriate diagnostic procedures should be undertaken if there in any suspicion of an underlying brain lesion. Related in part to the increased incidence of obesity in developed countries, sleep apnea syndromes often provide the greatest number of patients in the sleep laboratory. Sleep apnea can be primary in the infancy and a causatory role of drugs can be sometimes elicited in the adult. Nonetheless, obstructive sleep apneas are the major players in this chapter. It is important to realize that in this case even if hypersomnia is the complaint leading to a medical consultation, the condition is an important cardiovascular risk factor to be taken into account. Reduction of cessation of the respiratory flow with ongoing respiratory effort is a typical feature of the polysomnographic recordings as is the decline of oxygen saturation. Of course, evaluation of the causes of obstruction by otorhynolaringologists and respiratory experts is mandatory.
WO05 Sympathetic and cardiovascular activity during cataplexy in narcolepsy Vincenzo Donadio 1 , Giuseppe Plazzi 1 , Christian Franceschini 1 , Stefano Vandi 1 , Tomas Karlsson 2 , Pasquale Montagna 1 , Roberto Vetrugno 1 , Emanuel Mignot 3 , Rocco Liguori 1 1 Department of Neurological Sciences, University of Bologna, Bologna, Italy; 2 Department of Clinical Neurophysiology, Inst of Neuroscience and Physiology, University of Goteborg, Sweden; 3 Center for Narcolepsy, Stanford University, Stanford, CA, USA Purpose: Autonomic changes could have a role in the triggering mechanism inducing cataplexy. Our aim was to investigate the time course of sympathetic and cardiovascular activity during cataplexy to establish their role in inducing cataplexy reflexively. Method: We studied under microneurographic recording seven cataplectic episodes from two patients with hypocretin-deficient narcolepsy. During microneurography muscle sympathetic activity (MSNA) from the peroneal nerve was recorded simultaneously to ECG, respiratory movements, arterial finger blood pressure, right and left electro-oculogram and superficial electromyogram of mylohyoideus muscle. Results: Our findings showed no significant autonomic changes before the onset of cataplexy. Cataplectic attacks were characterized by muscle atonia (p< 0.001) and alpha activity similar to wakefulness. During the episodes MSNA incidence and amplitude increased (p< 0.01) together with SBP increase (p< 0.01) and HR decrease (p< 0.001) whereas DBP did not change significantly compared to baseline. These changes were most evident at the beginning of cataplectic attacks and returned to baseline during post-cataplexy. Conclusion: Our findings did not display significant changes of autonomic nerve activity prior to cataplexy onset, ruling out a role of this system in the triggering of cataplexy. Interestingly, however, we found that cataplexy was associated with a co-activation of sympathetic and parasympathetic nervous systems.
WO06 The forced awakening test in 120 consecutive patients complaining of excessive daytime sleepiness Bastuji 1 ,
Perrin 2 ,
Petitjean 3 ,
Garcia-Larrea 1
Hélène Fabien Thierry Luis 1 INSERM U 879, Lyon, France; 2 CNRS UMR 5020, Lyon, France; 3 Laboratoire des Troubles Respiratoires du Sommeil, Hôpital de la Croix Rousse, Lyon, France
Purpose: The comparison of event related potentials (ERPs) obtained during wakefulness and after 3 minutes of sleep (the “forced awakening test”)
S21
has been recently proposed to assess simultaneously sleep propensity and sleep inertia in patients with excessive daytime sleepiness (EDS) (Bastuji et al., 2003). Methods: We report our experience in 120 consecutive EDS patients, aged 16-73 years, 50% women, whose etiological diagnostic was narcolepsy (27%), idiopathic hypersomnia (30%), sleep apnea syndrome (SAS) (26%) and EDS associated with psychiatric disorders (17%). Results: On forced awakening, ERPs were abnormal (i.e. presented sleep negativities and/or P300 delay) in 75% of narcoleptics, 74% of patients with idiopathic hypersomnia, and 43% of patients with SAS. On the contrary, forced awakening ERPs were normal in EDS associated with psychiatric disorders. The ERP pattern was significantly related to the aetiology of EDS (χ2 : 36.7, p=0.0001). The presence of sleep negativites on the ERPs was a sign of severe acute sleep inertia in narcolepsy (58%) and idiopathic hypersomnia (59%). The behavioural performances were significantly less effective than ERPs to discriminate EDS aetiology. Sleep latencies were abnormal (<7 min) in 87% of narcoleptics, 74% of patients with idiopathic hypersomnia, 78% of patients with SAS and 12.5% of patients with psychiatric disorders. The ERP pattern was not correlated with cataplexy in narcoleptic patients, but rather with the amount of psychostimulant drugs needed during the follow-up. Conclusions: The significant correlation between result on the test and the severity of EDS suggests that the FA test is useful in clinical routine, not only for accurate diagnostic classification, but also as a reliable estimation of the overall severity of EDS.
WO07 Twin sister with delayed phase disorder María Aguilar Andújar, Paloma Villalobos López, Pablo Medrano, Teresa Herrera, Georgina Botebol Benhamou Spain Introduction: Delayed Sleep Phase Disorder is a circadian rhythm disorder, common during adolescence and early adulthood characterized by a delay in the major sleep episode. In these patients the sleep drive appears late at night, from 02:00 a.m to 06:00 a.m, therefore they have sleep onset insomnia and great difficulty waking in the morning for an academic timetable. This disorder is usually brought on by bad sleep habits. Case report: We present two 14 year old twin girls, with no family history of interest, with a personal history of menstrual cycle regulation disturbance and chronic insomnia from birth. In adolescence their difficulties falling asleep and waking up at the required time increased, interfering with their school life. After interviewing the patients we suspect delayed sleep phase disorder. Reviews: – Sleep diary with an irregular schedule. – Measurement of melatonin by serial blood samples: we found a very striking feature. Both patients have similar abnormal rhythms with two peaks. The highest peak of plasma melatonin secretion is delayed, appearing at 06:00 a.m. (normal about 04:00 a.m.) with the first appearing at about 24:00 p.m. – Plasma values of cortisol were altered. – FSH, LH and estradiol determination: all values were in the normal range. – Prolactin determination: values were increased only in the patient who was taking sulpiride during the weeks leading up to the study. Results: The most significant characteristic is a similar alteration in the melatonin rhythm; both patients present a double peak of melatonin secretion, which could be determined genetically. These peaks would justify the tendency to delay their sleep schedule and their difficulties in waking up early in the morning to attend school
WO08 Polysomnography and MSLT in the differential diagnosis of narcolepsy – a necessity or a waste of time? Aleksandra Wierzbicka, Adam Wichniak, Eugenia Szatkowska, Iwona Musinska, Krystyna Czasak, El˙zbieta Waliniowska, Wojciech Jernajczyk Sleep Disorders Center, Inst. of Psychiatry and Neurology, Warsaw, Poland Purpose: Increasing workload of physicians inclines some of them to
WO05 Sympathetic and cardiovascular activity during cataplexy in narcolepsy Vincenzo Donadio 1 , Giuseppe Plazzi 1 , Christian Franceschini 1 , Stefano Vandi 1 , Tomas Karlsson 2 , Pasquale Montagna 1 , Roberto Vetrugno 1 , Emanuel Mignot 3 , Rocco Liguori 1 1 Department of Neurological Sciences, University of Bologna, Bologna, Italy; 2 Department of Clinical Neurophysiology, Inst of Neuroscience and Physiology, University of Goteborg, Sweden; 3 Center for Narcolepsy, Stanford University, Stanford, CA, USA Purpose: Autonomic changes could have a role in the triggering mechanism inducing cataplexy. Our aim was to investigate the time course of sympathetic and cardiovascular activity during cataplexy to establish their role in inducing cataplexy reflexively. Method: We studied under microneurographic recording seven cataplectic episodes from two patients with hypocretin-deficient narcolepsy. During microneurography muscle sympathetic activity (MSNA) from the peroneal nerve was recorded simultaneously to ECG, respiratory movements, arterial finger blood pressure, right and left electro-oculogram and superficial electromyogram of mylohyoideus muscle. Results: Our findings showed no significant autonomic changes before the onset of cataplexy. Cataplectic attacks were characterized by muscle atonia (p< 0.001) and alpha activity similar to wakefulness. During the episodes MSNA incidence and amplitude increased (p< 0.01) together with SBP increase (p< 0.01) and HR decrease (p< 0.001) whereas DBP did not change significantly compared to baseline. These changes were most evident at the beginning of cataplectic attacks and returned to baseline during post-cataplexy. Conclusion: Our findings did not display significant changes of autonomic nerve activity prior to cataplexy onset, ruling out a role of this system in the triggering of cataplexy. Interestingly, however, we found that cataplexy was associated with a co-activation of sympathetic and parasympathetic nervous systems.
WO06 The forced awakening test in 120 consecutive patients complaining of excessive daytime sleepiness Bastuji 1 ,
Perrin 2 ,
Petitjean 3 ,
Garcia-Larrea 1
Hélène Fabien Thierry Luis 1 INSERM U 879, Lyon, France; 2 CNRS UMR 5020, Lyon, France; 3 Laboratoire des Troubles Respiratoires du Sommeil, Hôpital de la Croix Rousse, Lyon, France
Purpose: The comparison of event related potentials (ERPs) obtained during wakefulness and after 3 minutes of sleep (the “forced awakening test”)
S21
has been recently proposed to assess simultaneously sleep propensity and sleep inertia in patients with excessive daytime sleepiness (EDS) (Bastuji et al., 2003). Methods: We report our experience in 120 consecutive EDS patients, aged 16-73 years, 50% women, whose etiological diagnostic was narcolepsy (27%), idiopathic hypersomnia (30%), sleep apnea syndrome (SAS) (26%) and EDS associated with psychiatric disorders (17%). Results: On forced awakening, ERPs were abnormal (i.e. presented sleep negativities and/or P300 delay) in 75% of narcoleptics, 74% of patients with idiopathic hypersomnia, and 43% of patients with SAS. On the contrary, forced awakening ERPs were normal in EDS associated with psychiatric disorders. The ERP pattern was significantly related to the aetiology of EDS (χ2 : 36.7, p=0.0001). The presence of sleep negativites on the ERPs was a sign of severe acute sleep inertia in narcolepsy (58%) and idiopathic hypersomnia (59%). The behavioural performances were significantly less effective than ERPs to discriminate EDS aetiology. Sleep latencies were abnormal (<7 min) in 87% of narcoleptics, 74% of patients with idiopathic hypersomnia, 78% of patients with SAS and 12.5% of patients with psychiatric disorders. The ERP pattern was not correlated with cataplexy in narcoleptic patients, but rather with the amount of psychostimulant drugs needed during the follow-up. Conclusions: The significant correlation between result on the test and the severity of EDS suggests that the FA test is useful in clinical routine, not only for accurate diagnostic classification, but also as a reliable estimation of the overall severity of EDS.
WO07 Twin sister with delayed phase disorder María Aguilar Andújar, Paloma Villalobos López, Pablo Medrano, Teresa Herrera, Georgina Botebol Benhamou Spain Introduction: Delayed Sleep Phase Disorder is a circadian rhythm disorder, common during adolescence and early adulthood characterized by a delay in the major sleep episode. In these patients the sleep drive appears late at night, from 02:00 a.m to 06:00 a.m, therefore they have sleep onset insomnia and great difficulty waking in the morning for an academic timetable. This disorder is usually brought on by bad sleep habits. Case report: We present two 14 year old twin girls, with no family history of interest, with a personal history of menstrual cycle regulation disturbance and chronic insomnia from birth. In adolescence their difficulties falling asleep and waking up at the required time increased, interfering with their school life. After interviewing the patients we suspect delayed sleep phase disorder. Reviews: – Sleep diary with an irregular schedule. – Measurement of melatonin by serial blood samples: we found a very striking feature. Both patients have similar abnormal rhythms with two peaks. The highest peak of plasma melatonin secretion is delayed, appearing at 06:00 a.m. (normal about 04:00 a.m.) with the first appearing at about 24:00 p.m. – Plasma values of cortisol were altered. – FSH, LH and estradiol determination: all values were in the normal range. – Prolactin determination: values were increased only in the patient who was taking sulpiride during the weeks leading up to the study. Results: The most significant characteristic is a similar alteration in the melatonin rhythm; both patients present a double peak of melatonin secretion, which could be determined genetically. These peaks would justify the tendency to delay their sleep schedule and their difficulties in waking up early in the morning to attend school
WO08 Polysomnography and MSLT in the differential diagnosis of narcolepsy – a necessity or a waste of time? Aleksandra Wierzbicka, Adam Wichniak, Eugenia Szatkowska, Iwona Musinska, Krystyna Czasak, El˙zbieta Waliniowska, Wojciech Jernajczyk Sleep Disorders Center, Inst. of Psychiatry and Neurology, Warsaw, Poland Purpose: Increasing workload of physicians inclines some of them to