- Email: [email protected]
M-N-124 PSYCHOPATHOLOGICAL PROFILE IN PATIENTS WITH PRIMARY HYPERSOMNIA
Abstracts of 4th International Congress of WASM & 5th Conference of CSS / Sleep Medicine 12, Suppl. 1 (2011) S1–S130
sporadic cases, and 7 cases from the same family]) with a mean age at the onset of sleep episodes of 22.19±9.1 yrs (range, 6-36) and mean age at diagnosis of NC of 39.2±13.2 yrs (range, 18-61). The most frequent environmental risk factor was a major change in sleeping habits (31.3%), and the difference was statistically significant compared with the control group (p=0.029). This was followed by death of a close friend or family member (31.3%) and major change in working hours or conditions (21.9%), although the differences were not significant. Common cold was the most frequent infectious disease (48.3%), although, once again, the difference between the groups was not significant. Conclusion: In line with previous studies, the only significant difference in risk factors in our NC series was major change in sleeping habits.
M-N-121
FAVORABLE EFFECT OF STEROID THERAPY FOR AN 11-YEARS-OLD GIRL WITH ACUTE ONSET NARCOLEPSY
Rie Miyata 1 , Makoto Honda 2 , Takashi Kanbayashi 3 , Jun Kohyama 4 . Department of Pediatrics, Tokyo-Kita Social Insurance Hospital, Japan; 2 Department of the Sleep Research Project, Tokyo Metropolitan Institute of Medical Science, Japan; 3 Department of Neuropsychiatry, Akita University School of Medicine, Japan; 4 Department of Pediatrics, Tokyo Bay Urayasu-Ichikawa Medical Center, Japan 1
Introduction and Objectives: Introduction: The autoimmune mechanism has been hypothesized to underlie the pathogenesis of narcolepsy. Here we reported favorable effect of steroid on a girl with very recent onsetnarcolepsy with cataplexy. Case Report: An eleven-years-old girl visited our clinic because of sleepiness and cataplexy. On the day when her second menstruation occurred, she abruptly developed difficulty walking due to frequent knee bucklings. Soon severe daytime sleepiness followed and she could not keep awake for 15 minutes during daytime, sometimes became stuporous in school or at home. She collapsed on the floor with her jaw sagging when she laughed. Frequent nocturnal awaking together with muscle twitches and sleep talking also started. Her medical histories were unremarkable except for mild bronchial asthma from childhood. Initial nocturnal polysomnography (nPSG) revealed seven sleep cycles and 4 slow wave sleep (SWS) periods distributed throughout the night, predominantly in the morning. The hypocretin-1 level in her cerebrospinal fluid (CSF) was undetectable and other inflammatory cytokines were not elevated. HLA-typing revealed she carried HLA-DQB1*0602 allele. From the 32nd day of her illness, she was medicated with full dose of predonisolone for two weeks with three weeks tapering off period. Her frequent knee bucklings decreased and she could walk by herself. She could stay awake longer in daytime. Her Epworth Sleepiness Score reduced from 19 to 9, though sleepiness and cataplexy remained. Second measurement of CSF hypocretin-1 and cytokine levels showed no change. Discussion: Steroid therapy reduced her cataplexy frequency and subjective daytime sleepiness, though CSF cytokines and hypocretin-1 levels were not changed. Change in nocturnal sleep structure might contribute to the improvement. The steroid therapy could be effective for symptoms of narcolepsy. Steroid could be one therapeutic option for acute onset narcolepsy.
M-N-122
MONOZYGOTIC TWINS AFFECTED WITH KLEINE-LEVIN SYNDROME
Rosa Peraita-Adrados 1, José-Luis Vicario 2, Manuel García de León 3 , Mehdi Tafti 4 . 1 Sleep Unit - Clinical Neurophysiology Department, Gregorio Marañón University Hospital, Madrdid, Spain; 2 Histocompatibility, Blood Center of the Community of Madrid, Spain; 3 Sleep Unit, Milagrosa Hospital, Spain; 4 Center for Integrative Genomics (CIG), University of Lausanne, Lausanne, Switzerland Introduction and Objectives: A recent review article on recurrent hypersomnia indicated that the percentage of familial cases of Kleine-Levin syndrome (3.7%) was in the same range as that of narcolepsy with cataplexy. Aim. To report a unique pair of Spanish monozygotic twins who were affected with Kleine-Levin syndrome. Materials and Methods: Both patients underwent a complete physical examination and laboratory work-up including EEG, PSG, MRI, and HLA class II antigen typing
S55
Results: – Case No. 1 was the second-born twin. His first attack occurred at the age 17, after breaking up with his first girlfriend, and his last attack occurred at the age of 31. Clinical features included recurrent bouts of hypersomnia associated with compulsive eating, increased libido, cognitive symptoms, depression and anxiety, profuse sweating, and transient elation and insomnia at the end of episodes. The patient was diagnosed with Kleine-Levin syndrome. – Case No. 2 was the first-born twin. His first attack occurred at the age of 16, in the context of rhinitis, and his last attack at the age 29. Clinical features included recurrent hypersomnia, anorexia, odd behavior, cognitive symptoms, depression and anxiety, transient partial amnesia, and insomnia at the end of episodes. His diagnosis was Kleine-Levin syndrome without compulsive eating. The twin brothers looked very much like each other and genetic markers (HLA and 7 additional highly polymorphic markers on chromosome 11 and 15) revealed identical results, confirming monozygosity. Conclusion: To our knowledge this is the first published report of monozygotic twins with Kleine-Levin syndrome. Although familial cases of KLS are extremely rare, our observation raises the possibility of genuine genetic forms of KLS.
M-N-123
MULTICOMPONENT COGNITIVE BEHAVIORAL TREATMENT EFFICACY FOR NARCOLEPSY (MCBT-N)
Hernán Andrés Marín Agudelo. Universidad Cooperativa de Colombia, Colombia Introduction and Objectives: Narcolepsy treatment includes pharmacological strategies, with high recommendation levels and strong studies that demonstrate the effectiveness. So far there are few studies on the effectiveness of CBT for the treatment of narcolepsy. The objective of this study was to evaluate the effect of a multicomponent cognitive-behavioral treatment of 12 weeks for narcolepsy. Materials and Methods: Randomized study sample of 11 patients, assigned MCBT-N (sleep satiation, scheduling naps, cognitive restructuring, problem solving, systematic desensitization and training in social support), and controls. Care was taken to confirm the diagnosis of narcolepsy and that they were not receiving pharmacological treatment when assigning the intervention protocol. Multiple Sleep Latency, Epworth Sleepiness Scale, the SF36, Ullanlina Narcolepsy scale and the Stanford cataplexy scale (modified) were used at pre-treatment, 6 months and 12 months of treatment. Results: Differences were significant for patients compared to controls (p <0.001) with improvement in TCC-MN at six months and one year after finishing treatment. Dimensions of quality of life related to health (SF-36), excessive daytime sleepiness and symptoms of narcolepsy improved along with improvement in the areas of social function (p <0.005), vitality and role limitations, physical problems both in emotional problems (P <0.001). These areas were more abormal in the patients before the measurements and their improvement has been reported in previous studies. Importantly, the reduction of episodes of excessive daytime sleepiness, the TMLS means (p <0.005), and episodes of cataplexy (p <0.005), were significant at the one year of treatment, without significant differences compared to the six months treatment. Conclusion: Multicomponent cognitive behavioral therapy applied systematically produces appropriate levels of efficacy intervention in narcolepsy, results that significantly impact the improvement of quality of life of patients treated with this protocol. However, it is necessary to study larger samples support the conclusions of this study.
M-N-124
PSYCHOPATHOLOGICAL PROFILE IN PATIENTS WITH PRIMARY HYPERSOMNIA
Ana Teijeira-Azcona 1 , Carmen Rosa-Aguilar 2 , Jose Corredera-Rodriguez 3, Fernando Ayuga-Loro 4 , Rafael Cuena-Boy 5 , Jose Teijeira-Alvarez 1 . 1 Servicio de Neurofisiologia Clinica, Complejo Hospitalario de Toledo, Spain; 2 Dirección General de Atención Sanitaria y Calidad, SSCC SESCAM, Spain; 3 Clinical Neurophysiology Department, Complejo Hospitalario de Toledo, Spain; 4 Servicio de Neurología, Hospital Nacional de Paraplejicos, Spain; 5 Unidad de Investigación, Complejo Hospitalario de Toledo, Spain Introduction and Objectives: Psychopathological profile in patients with Primary Hypersomnia has not been systematically studied, probably due to the complexity and chronicity of this disease and to the possible side-effects
S56
Abstracts of 4th International Congress of WASM & 5th Conference of CSS / Sleep Medicine 12, Suppl. 1 (2011) S1–S130
of the drugs used for treatment. The Minnesota Multiphasic Personality Inventory (MMPI) is the clinical test used most frequently to identify personality profiles and for diagnosing different psychopathologies. It is composed of multiple scales, including six critical variables with a greater influence in the treatment decision. Materials and Methods: We have performed a comparative and observational study using the latest version of the MMPI-2RF in 10 patients in our sleep laboratory diagnosed with Primary Hypersomnia. The range of age was 18 to 40 years. 7 of them had Narcolepsy, 3 of them with Cataplexy, and 3 had Idiophatic Hypersomnia. None of them had a history of psychiatric or somatic problems. The education level of the patients was at least the high school level. We compared the results with 10 healthy controls with similar demographic characteristics but without sleep disorder. Previous informed consent was obtained from all patients. Results: Preliminary results showed in patients a high level of demoralization, somatization, absence of positive emotions, hopelessness and no motivation for change. Many patients showed a propensity for violence, familial conflicts and impulsivity. None of these characteristics were identified in the control group. We performed a statistical analysis with the data. In a discriminant analysis between the critical variables, we found that a combination of two factors, specifically anxiety (AXY) and defenselessness (HLP), were capable of separating patients from the control subjects. Conclusion: Based on preliminary data we have found a specific psychopathological profile in patients with Primary Hypersomnia but a more detailed and extensive study is needed to provide consistent data. In our experience, we suggest that these patients need specific psychological support.
M-N-125
REACTION TIME IN A MONETARY INCENTIVE TASK DOES NOT DIFFER BETWEEN NARCOLEPSY PATIENTS AND HEALTHY CONTROLS
Armand Mensen 1 , Rositsa Poryazova 2 , Nina Zollinger 2 , Christopher Tsatsaris 2 , Tanja Eberle 1 , Christian Baumann 2 , Ramin Khatami 1 . 1 Sleep Center, Klinik Barmelwed, Switzerland; 2 University Hospital Zurich, Switzerland Introduction and Objectives: Hypocretin deficiency in narcolepsy – cataplexy patients has been linked to disturbed emotional processing, especially reward. We aimed to assess reaction time in narcolepsy patients and healthy controls in an incentive monetary task and to compare outcomes to those in healthy controls. Materials and Methods: Nine HLA positive, hypocretin-deficient (7/7 tested) unmedicated (8/9) narcolepsy patients with cataplexy (mean age 39 years) and nine healthy controls (mean age 31 years) performed an incentive monetary task using different value and valence cues. The participants had to press a button as fast as possible while a picture of a landscape was presented on the screen in order to gain or not lose money. On each trial, the picture was preceded by one of four possible cues: potential gains (+1/+5 points) or losses (-1/-5 points). The duration of the target presentation was adapted online based on the participant’s performance on the previous trials to ensure a balanced amount of won and lost trials in each participant. Data was analyzed using repeated measures ANOVA (with group as a between subject factor and valence – positive versus negative, and value – small versus big cues, as within subject factors) and unpaired t-tests. Results: Reaction time for successful trials did not differ between patients (mean ± SD, ms 313±66) and controls (277±61) in general. There were no differences in reaction times neither for small versus large cues nor for positive versus negative cues. Conclusion: Narcolepsy patients achieve normal reaction times in highly motivational game-like tasks. Acknowledgements: The study is supported by a Swiss National Foundation (SNF) Grant.
M-N-126
RETROSPECTIVE EVALUATION OF PREGNANCIES IN EUROPEAN NARCOLEPTIC WOMEN
Eszter Maurovich Horvat 1 , David Kemlink 1 , Birgit Högl 2 , Birgit Frauscher 2 , Laura Ehrmann 2 , Peter Geisler 3 , Katharina Ettenhuber 3 , Geert Mayer 4 , Rosa Peraita-Adrados 5, Elena Calvo 5 , Gert Jan Lammers 6 , Astrid van der Heide 6 , Luigi Ferini Strambi 7 , Francesca Poli 8 , Yves Dauvilliers 9 , Francisco Javier Puertas Cuesta 10 , Poul Jennum 11 , Helle Leonthin 11 , Mathis Johannes 12 . 1 1st Faculty of Medicine, Department of Neurology, Charles University, Czech Republic; 2 Sleep Disorders Unit, Department of Neurology, Innsbruck Medical University, Austria; 3 University Medical Center Regensburg, Department of Psychiatry, Germany; 4 Hephata-Klinik, Germany; 5 Sleep and Epilepsy Uni-Clinical Neurophysiology Department Gregorio Marañón University Hospital, Spain; 6 Leiden University Medical Center, Department of Neurology, Netherlands; 7 Sleep Disorders Center, Universitr Vita-Salute San Raffaele, Italy; 8 Department of Neurological Sciences, University of Bologna, Italy; 9 Service de Neurologie, Hôpital Gui-de-Chauliac, France; 10 Sleep Disorders Unit University Hospital La Ribera, Alzira, Department of Physiology, Universty of Valencia, Spain; 11 Danish Center for Sleep Medicine, Department of Clinical Neurophysiology, Center of Healthy Ageing Faculty of Health, University of Copenhagen Glostrup Hospital, Denmark; 12 Zentrum für Schlafmedizin, Abt. Klin. Neurophysiologie, Bern, Switzerland Introduction and Objectives: Narcolepsy is a chronic disease that causes patients many complications in everyday life. Although there have been advancements in recent years in understanding this disorder, its effects on pregnancy and childbirth are still largely unknown. To address this problem we ran an international study. Materials and Methods: We carried out a retrospective questionnaire study with patients suffering from narcolepsy in 10 European countries. The data were collected from 245 mothers with a total of 373 children Results: 213 patients were diagnosed with narcolepsy and cataplexy (NC) and 32 with narcolepsy without cataplexy (Nw/outC). The patients were aged during pregnancy on average 27.4±6.2 years old. The mean BMI before pregnancy was 28,2±5,6, the patients gain on average 14,7±7,8 kg. Women with NC gained significantly more weight than women without cataplexy. Ten percent of patients suffered from complications as impaired glucose tolerance, type 2 diabetes mellitus, hypertension or preeclampsy. Twenty women took medications against the symptoms of narcolepsy (Ephedrin, Fenmetrazin, Modafinil, Sodium Oxybate) without any reported complication. Duration of pregnancy was 39.3±5.1 weeks, 78% of patients delivered spontaneously. Two patients with NC experienced cataplexy during the delivery. Women with NC reported significantly higher rate of Caesarean sections, than patients without cataplexy. Weight of newborns was 3328±826 g and their height was 50.7±3.0 cm. From 373 newborns, there were 207 boys and 166 girls. 64% of babies endured reduced levels of care due to mothers afflicted with one or more symptoms of narcolepsy Conclusion: Overall, no clinically relevant adverse effects of narcolepsy on pregnancy, childbirth or the newborn were disclosed. Coping with baby care is more difficult due to symptoms of narcolepsy.
M-N-127
SUBJECTIVE RATING OF ATTENTION AND MOOD IN PATIENTS WITH NARCOLEPSY-CATAPLEXY
Birgit Högl, Johanna Wenter, Birgit Frauscher, Laura Zamarian, Laura Ehrmann, Viola Gschliesser, Elisabeth Brandauer, Margarete Delazer. Department of Neurology, Austria Introduction and Objectives: Narcolepsy-cataplexy is a neurological disease due to hypocretin deficiency. Hypocretin is a neurotransmitter which is especially important for sleep wake regulation, motor control in sleep and food intake. Findings about cognitive functioning in narcolepsy-cataplexy are inconsistent. Deficits have been described in demanding tasks assessing attention or executive functions. Patients themselves frequently report difficulties in cognitive domains including attention and memory. Furthermore, a high proportion of anxiety disorders (Droogleever Fortuyn et al., 2010) or of depressive symptoms (Dauvilliers et al., 2009) has been reported. The present study investigates how subjective ratings of attention relate to objectively measured cognitive functions and to ratings of depression and anxiety. Materials and Methods: Thirty consecutive patients with narcolepsycataplexy according to ICSD-2 criteria completed the FEDA questionnaire assessing everyday attention in three different domains (distractibility and
sporadic cases, and 7 cases from the same family]) with a mean age at the onset of sleep episodes of 22.19±9.1 yrs (range, 6-36) and mean age at diagnosis of NC of 39.2±13.2 yrs (range, 18-61). The most frequent environmental risk factor was a major change in sleeping habits (31.3%), and the difference was statistically significant compared with the control group (p=0.029). This was followed by death of a close friend or family member (31.3%) and major change in working hours or conditions (21.9%), although the differences were not significant. Common cold was the most frequent infectious disease (48.3%), although, once again, the difference between the groups was not significant. Conclusion: In line with previous studies, the only significant difference in risk factors in our NC series was major change in sleeping habits.
M-N-121
FAVORABLE EFFECT OF STEROID THERAPY FOR AN 11-YEARS-OLD GIRL WITH ACUTE ONSET NARCOLEPSY
Rie Miyata 1 , Makoto Honda 2 , Takashi Kanbayashi 3 , Jun Kohyama 4 . Department of Pediatrics, Tokyo-Kita Social Insurance Hospital, Japan; 2 Department of the Sleep Research Project, Tokyo Metropolitan Institute of Medical Science, Japan; 3 Department of Neuropsychiatry, Akita University School of Medicine, Japan; 4 Department of Pediatrics, Tokyo Bay Urayasu-Ichikawa Medical Center, Japan 1
Introduction and Objectives: Introduction: The autoimmune mechanism has been hypothesized to underlie the pathogenesis of narcolepsy. Here we reported favorable effect of steroid on a girl with very recent onsetnarcolepsy with cataplexy. Case Report: An eleven-years-old girl visited our clinic because of sleepiness and cataplexy. On the day when her second menstruation occurred, she abruptly developed difficulty walking due to frequent knee bucklings. Soon severe daytime sleepiness followed and she could not keep awake for 15 minutes during daytime, sometimes became stuporous in school or at home. She collapsed on the floor with her jaw sagging when she laughed. Frequent nocturnal awaking together with muscle twitches and sleep talking also started. Her medical histories were unremarkable except for mild bronchial asthma from childhood. Initial nocturnal polysomnography (nPSG) revealed seven sleep cycles and 4 slow wave sleep (SWS) periods distributed throughout the night, predominantly in the morning. The hypocretin-1 level in her cerebrospinal fluid (CSF) was undetectable and other inflammatory cytokines were not elevated. HLA-typing revealed she carried HLA-DQB1*0602 allele. From the 32nd day of her illness, she was medicated with full dose of predonisolone for two weeks with three weeks tapering off period. Her frequent knee bucklings decreased and she could walk by herself. She could stay awake longer in daytime. Her Epworth Sleepiness Score reduced from 19 to 9, though sleepiness and cataplexy remained. Second measurement of CSF hypocretin-1 and cytokine levels showed no change. Discussion: Steroid therapy reduced her cataplexy frequency and subjective daytime sleepiness, though CSF cytokines and hypocretin-1 levels were not changed. Change in nocturnal sleep structure might contribute to the improvement. The steroid therapy could be effective for symptoms of narcolepsy. Steroid could be one therapeutic option for acute onset narcolepsy.
M-N-122
MONOZYGOTIC TWINS AFFECTED WITH KLEINE-LEVIN SYNDROME
Rosa Peraita-Adrados 1, José-Luis Vicario 2, Manuel García de León 3 , Mehdi Tafti 4 . 1 Sleep Unit - Clinical Neurophysiology Department, Gregorio Marañón University Hospital, Madrdid, Spain; 2 Histocompatibility, Blood Center of the Community of Madrid, Spain; 3 Sleep Unit, Milagrosa Hospital, Spain; 4 Center for Integrative Genomics (CIG), University of Lausanne, Lausanne, Switzerland Introduction and Objectives: A recent review article on recurrent hypersomnia indicated that the percentage of familial cases of Kleine-Levin syndrome (3.7%) was in the same range as that of narcolepsy with cataplexy. Aim. To report a unique pair of Spanish monozygotic twins who were affected with Kleine-Levin syndrome. Materials and Methods: Both patients underwent a complete physical examination and laboratory work-up including EEG, PSG, MRI, and HLA class II antigen typing
S55
Results: – Case No. 1 was the second-born twin. His first attack occurred at the age 17, after breaking up with his first girlfriend, and his last attack occurred at the age of 31. Clinical features included recurrent bouts of hypersomnia associated with compulsive eating, increased libido, cognitive symptoms, depression and anxiety, profuse sweating, and transient elation and insomnia at the end of episodes. The patient was diagnosed with Kleine-Levin syndrome. – Case No. 2 was the first-born twin. His first attack occurred at the age of 16, in the context of rhinitis, and his last attack at the age 29. Clinical features included recurrent hypersomnia, anorexia, odd behavior, cognitive symptoms, depression and anxiety, transient partial amnesia, and insomnia at the end of episodes. His diagnosis was Kleine-Levin syndrome without compulsive eating. The twin brothers looked very much like each other and genetic markers (HLA and 7 additional highly polymorphic markers on chromosome 11 and 15) revealed identical results, confirming monozygosity. Conclusion: To our knowledge this is the first published report of monozygotic twins with Kleine-Levin syndrome. Although familial cases of KLS are extremely rare, our observation raises the possibility of genuine genetic forms of KLS.
M-N-123
MULTICOMPONENT COGNITIVE BEHAVIORAL TREATMENT EFFICACY FOR NARCOLEPSY (MCBT-N)
Hernán Andrés Marín Agudelo. Universidad Cooperativa de Colombia, Colombia Introduction and Objectives: Narcolepsy treatment includes pharmacological strategies, with high recommendation levels and strong studies that demonstrate the effectiveness. So far there are few studies on the effectiveness of CBT for the treatment of narcolepsy. The objective of this study was to evaluate the effect of a multicomponent cognitive-behavioral treatment of 12 weeks for narcolepsy. Materials and Methods: Randomized study sample of 11 patients, assigned MCBT-N (sleep satiation, scheduling naps, cognitive restructuring, problem solving, systematic desensitization and training in social support), and controls. Care was taken to confirm the diagnosis of narcolepsy and that they were not receiving pharmacological treatment when assigning the intervention protocol. Multiple Sleep Latency, Epworth Sleepiness Scale, the SF36, Ullanlina Narcolepsy scale and the Stanford cataplexy scale (modified) were used at pre-treatment, 6 months and 12 months of treatment. Results: Differences were significant for patients compared to controls (p <0.001) with improvement in TCC-MN at six months and one year after finishing treatment. Dimensions of quality of life related to health (SF-36), excessive daytime sleepiness and symptoms of narcolepsy improved along with improvement in the areas of social function (p <0.005), vitality and role limitations, physical problems both in emotional problems (P <0.001). These areas were more abormal in the patients before the measurements and their improvement has been reported in previous studies. Importantly, the reduction of episodes of excessive daytime sleepiness, the TMLS means (p <0.005), and episodes of cataplexy (p <0.005), were significant at the one year of treatment, without significant differences compared to the six months treatment. Conclusion: Multicomponent cognitive behavioral therapy applied systematically produces appropriate levels of efficacy intervention in narcolepsy, results that significantly impact the improvement of quality of life of patients treated with this protocol. However, it is necessary to study larger samples support the conclusions of this study.
M-N-124
PSYCHOPATHOLOGICAL PROFILE IN PATIENTS WITH PRIMARY HYPERSOMNIA
Ana Teijeira-Azcona 1 , Carmen Rosa-Aguilar 2 , Jose Corredera-Rodriguez 3, Fernando Ayuga-Loro 4 , Rafael Cuena-Boy 5 , Jose Teijeira-Alvarez 1 . 1 Servicio de Neurofisiologia Clinica, Complejo Hospitalario de Toledo, Spain; 2 Dirección General de Atención Sanitaria y Calidad, SSCC SESCAM, Spain; 3 Clinical Neurophysiology Department, Complejo Hospitalario de Toledo, Spain; 4 Servicio de Neurología, Hospital Nacional de Paraplejicos, Spain; 5 Unidad de Investigación, Complejo Hospitalario de Toledo, Spain Introduction and Objectives: Psychopathological profile in patients with Primary Hypersomnia has not been systematically studied, probably due to the complexity and chronicity of this disease and to the possible side-effects
S56
Abstracts of 4th International Congress of WASM & 5th Conference of CSS / Sleep Medicine 12, Suppl. 1 (2011) S1–S130
of the drugs used for treatment. The Minnesota Multiphasic Personality Inventory (MMPI) is the clinical test used most frequently to identify personality profiles and for diagnosing different psychopathologies. It is composed of multiple scales, including six critical variables with a greater influence in the treatment decision. Materials and Methods: We have performed a comparative and observational study using the latest version of the MMPI-2RF in 10 patients in our sleep laboratory diagnosed with Primary Hypersomnia. The range of age was 18 to 40 years. 7 of them had Narcolepsy, 3 of them with Cataplexy, and 3 had Idiophatic Hypersomnia. None of them had a history of psychiatric or somatic problems. The education level of the patients was at least the high school level. We compared the results with 10 healthy controls with similar demographic characteristics but without sleep disorder. Previous informed consent was obtained from all patients. Results: Preliminary results showed in patients a high level of demoralization, somatization, absence of positive emotions, hopelessness and no motivation for change. Many patients showed a propensity for violence, familial conflicts and impulsivity. None of these characteristics were identified in the control group. We performed a statistical analysis with the data. In a discriminant analysis between the critical variables, we found that a combination of two factors, specifically anxiety (AXY) and defenselessness (HLP), were capable of separating patients from the control subjects. Conclusion: Based on preliminary data we have found a specific psychopathological profile in patients with Primary Hypersomnia but a more detailed and extensive study is needed to provide consistent data. In our experience, we suggest that these patients need specific psychological support.
M-N-125
REACTION TIME IN A MONETARY INCENTIVE TASK DOES NOT DIFFER BETWEEN NARCOLEPSY PATIENTS AND HEALTHY CONTROLS
Armand Mensen 1 , Rositsa Poryazova 2 , Nina Zollinger 2 , Christopher Tsatsaris 2 , Tanja Eberle 1 , Christian Baumann 2 , Ramin Khatami 1 . 1 Sleep Center, Klinik Barmelwed, Switzerland; 2 University Hospital Zurich, Switzerland Introduction and Objectives: Hypocretin deficiency in narcolepsy – cataplexy patients has been linked to disturbed emotional processing, especially reward. We aimed to assess reaction time in narcolepsy patients and healthy controls in an incentive monetary task and to compare outcomes to those in healthy controls. Materials and Methods: Nine HLA positive, hypocretin-deficient (7/7 tested) unmedicated (8/9) narcolepsy patients with cataplexy (mean age 39 years) and nine healthy controls (mean age 31 years) performed an incentive monetary task using different value and valence cues. The participants had to press a button as fast as possible while a picture of a landscape was presented on the screen in order to gain or not lose money. On each trial, the picture was preceded by one of four possible cues: potential gains (+1/+5 points) or losses (-1/-5 points). The duration of the target presentation was adapted online based on the participant’s performance on the previous trials to ensure a balanced amount of won and lost trials in each participant. Data was analyzed using repeated measures ANOVA (with group as a between subject factor and valence – positive versus negative, and value – small versus big cues, as within subject factors) and unpaired t-tests. Results: Reaction time for successful trials did not differ between patients (mean ± SD, ms 313±66) and controls (277±61) in general. There were no differences in reaction times neither for small versus large cues nor for positive versus negative cues. Conclusion: Narcolepsy patients achieve normal reaction times in highly motivational game-like tasks. Acknowledgements: The study is supported by a Swiss National Foundation (SNF) Grant.
M-N-126
RETROSPECTIVE EVALUATION OF PREGNANCIES IN EUROPEAN NARCOLEPTIC WOMEN
Eszter Maurovich Horvat 1 , David Kemlink 1 , Birgit Högl 2 , Birgit Frauscher 2 , Laura Ehrmann 2 , Peter Geisler 3 , Katharina Ettenhuber 3 , Geert Mayer 4 , Rosa Peraita-Adrados 5, Elena Calvo 5 , Gert Jan Lammers 6 , Astrid van der Heide 6 , Luigi Ferini Strambi 7 , Francesca Poli 8 , Yves Dauvilliers 9 , Francisco Javier Puertas Cuesta 10 , Poul Jennum 11 , Helle Leonthin 11 , Mathis Johannes 12 . 1 1st Faculty of Medicine, Department of Neurology, Charles University, Czech Republic; 2 Sleep Disorders Unit, Department of Neurology, Innsbruck Medical University, Austria; 3 University Medical Center Regensburg, Department of Psychiatry, Germany; 4 Hephata-Klinik, Germany; 5 Sleep and Epilepsy Uni-Clinical Neurophysiology Department Gregorio Marañón University Hospital, Spain; 6 Leiden University Medical Center, Department of Neurology, Netherlands; 7 Sleep Disorders Center, Universitr Vita-Salute San Raffaele, Italy; 8 Department of Neurological Sciences, University of Bologna, Italy; 9 Service de Neurologie, Hôpital Gui-de-Chauliac, France; 10 Sleep Disorders Unit University Hospital La Ribera, Alzira, Department of Physiology, Universty of Valencia, Spain; 11 Danish Center for Sleep Medicine, Department of Clinical Neurophysiology, Center of Healthy Ageing Faculty of Health, University of Copenhagen Glostrup Hospital, Denmark; 12 Zentrum für Schlafmedizin, Abt. Klin. Neurophysiologie, Bern, Switzerland Introduction and Objectives: Narcolepsy is a chronic disease that causes patients many complications in everyday life. Although there have been advancements in recent years in understanding this disorder, its effects on pregnancy and childbirth are still largely unknown. To address this problem we ran an international study. Materials and Methods: We carried out a retrospective questionnaire study with patients suffering from narcolepsy in 10 European countries. The data were collected from 245 mothers with a total of 373 children Results: 213 patients were diagnosed with narcolepsy and cataplexy (NC) and 32 with narcolepsy without cataplexy (Nw/outC). The patients were aged during pregnancy on average 27.4±6.2 years old. The mean BMI before pregnancy was 28,2±5,6, the patients gain on average 14,7±7,8 kg. Women with NC gained significantly more weight than women without cataplexy. Ten percent of patients suffered from complications as impaired glucose tolerance, type 2 diabetes mellitus, hypertension or preeclampsy. Twenty women took medications against the symptoms of narcolepsy (Ephedrin, Fenmetrazin, Modafinil, Sodium Oxybate) without any reported complication. Duration of pregnancy was 39.3±5.1 weeks, 78% of patients delivered spontaneously. Two patients with NC experienced cataplexy during the delivery. Women with NC reported significantly higher rate of Caesarean sections, than patients without cataplexy. Weight of newborns was 3328±826 g and their height was 50.7±3.0 cm. From 373 newborns, there were 207 boys and 166 girls. 64% of babies endured reduced levels of care due to mothers afflicted with one or more symptoms of narcolepsy Conclusion: Overall, no clinically relevant adverse effects of narcolepsy on pregnancy, childbirth or the newborn were disclosed. Coping with baby care is more difficult due to symptoms of narcolepsy.
M-N-127
SUBJECTIVE RATING OF ATTENTION AND MOOD IN PATIENTS WITH NARCOLEPSY-CATAPLEXY
Birgit Högl, Johanna Wenter, Birgit Frauscher, Laura Zamarian, Laura Ehrmann, Viola Gschliesser, Elisabeth Brandauer, Margarete Delazer. Department of Neurology, Austria Introduction and Objectives: Narcolepsy-cataplexy is a neurological disease due to hypocretin deficiency. Hypocretin is a neurotransmitter which is especially important for sleep wake regulation, motor control in sleep and food intake. Findings about cognitive functioning in narcolepsy-cataplexy are inconsistent. Deficits have been described in demanding tasks assessing attention or executive functions. Patients themselves frequently report difficulties in cognitive domains including attention and memory. Furthermore, a high proportion of anxiety disorders (Droogleever Fortuyn et al., 2010) or of depressive symptoms (Dauvilliers et al., 2009) has been reported. The present study investigates how subjective ratings of attention relate to objectively measured cognitive functions and to ratings of depression and anxiety. Materials and Methods: Thirty consecutive patients with narcolepsycataplexy according to ICSD-2 criteria completed the FEDA questionnaire assessing everyday attention in three different domains (distractibility and