A questionnaire survey has been made of the life effects of narcolepsy in 180 patients, 60 each from North American, Asian and European populations, with 180 similarly distributed age and sex matched controls. Life-effects were attributed by the patients to the primary symptoms of excessive daytime drowsiness, sleep attacks, cataplexy, vivid hypnagogic hallucinations and sleep paralysis, and also to other frequent symptoms such as visual problems (blurring, diplopia) and memory impairment. Occupational problems were prevalent (over 75%) and included statistically significant deleterious effects upon performance, promotion, earning capacity, fear of or actual job loss RESUME: Nous avons e'tudie par questionnaire les effets sur la vie de 180 patients souffrant de narcolepsie (60 d'Amerique du Nord, 60 d'Asie et 60 d'Europe) el de 180 temoins apparilles pour age et sexe. Parmi les causes importants mentionnees par les patients, on note la somnolence diurne excessive, les attaques de sommeil, la cataplexie, les hallucinations hypnagogiques vivides et la paralysie du sommeil. D'autres symptomes frequents furenl les problemes visuels et lespertes de memoire. Des problemes au travail etaient tres frequents (75%) et significativement influent sur la performance, les promotions, la capacite a gagner, la crainte de la perte and increased disability insurance. Driving was greatly affected and patients fell asleep at the wheel more frequently (66%), had near or actual accidents from drowsiness or falling asleep at the wheel (67%), and could experience cataplexy (29%) or sleep paralysis (12%) while driving. Work or home accidents attributed to sleepiness or sleep (49%) or related to smoking (49%) were much more common in patients. There were also deleterious effects on education, recreation and personality related to the disease. Narcolepsy can produce a variety of life-effects probably more serious and pervasive than, for instance, those of epilepsy, therefore emphasizing the importance of early diagnosis and treatment. d'emploi ou la perte re'elle de I'emploi et I'assurance invalidite. La conduite d'un vehicule moteur etait souvent difficile; endormissement a la roue (66%), accidents apres somnolence (67%), cataplexie (29%) ou paralysie du sommeil (12%) avec le vehicule en marche. Les accidents a la maison ou au travail etaient egalement plus frequents, en rapport avec le sommeil (49%) ou le fait de fumer (49%). La narcolepsie eut egalement des effets nocifs sur /'education, la recreation et la personality. La narcolepsie est done apte a causer de nombreux problemes, ce qui devrait inciter a faire le diagnostic tot pour entreprendre rapidement le traitement.

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SUMMARY:A recent questionnaire survey of the life-effects of narcolepsy in 180 patients, 60 each from North American, Asian and European populations, compared to similarly distributed age and sex matched controls, documented multiple and marked effects on work, education, driving, accidents, recreation, personality, memory and other parameters. The data have now been further analysed according to the patients’ geographic (culturo-genetic) origin and to a number of other patient and illness variables. The three different geographic populations showed few significant differences for the some 160 life-effects items in the questionnaire. This strongly indicates that these are an integral part of the disease or of the human reaction to it. Most of the few significant population differences appeared cultural in origin (e.g., concerning driving records, personality changes), although a few may possibly reflect genetic differences (e.g., visual problems). Analysis of the pooled data according to respondees’ age, sex, age at illness onset, duration of illness and treatment led again to relatively few significant findings. It is concluded that, in general, once the disease has been diagnosed, all the major life effects are present and remain so. The results strongly support the contention that most life-effects are not related to the diagnostic ‘tetrad’ symptoms themselves but rather to excessive daytime sleepiness, the symptom most resistant to anti-narcoleptic treatment.

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Summary: This article describes a quantitative assessment of pathological diurnal sleepiness in three groups of patients with excessive daytime sleepiness: narcoleptic patients, idiopathic hypersomniac patients, hypersomniac patients with sleep apnea syndrome. We analyzed polygraphic diurnal recordings of 45 min duration obtained under standardized conditions. We called the percentage of total sleep time during the 45-min recording the polygraphic index of sleepiness. The polygraphic score of sleepiness is determined by the latencies and total durations of the individual sleep stages. Because deeper sleep stages correspond to more pronounced sleepiness than do superficial sleep stages, we introduced coefficients for each sleep stage. We present a formula for calculating a score in a single figure that gives a good indication of the patient's sleepiness and makes inter-and intraindividual comparison possible. Separate REM and NREM sleep scores are also given. Key Words: Sleep polygraphy-Sleepiness index-Sleepiness scoreNarcolepsy-Hypersomnia.The quantitative evaluation of pathological diurnal sleepiness has only recently received attention. In 1982, Dement and Carskadon (1) noted that research was needed in this area and organized a symposium devoted to this question. The findings presented at that symposium (2) were based, for the most part, on the multiple sleep latency test (MSLT) and a modified version of this test, the Repeated Test of Sustained Wakefulness (3). Although these articles presented new, useful information, they used almost exclusively the latency of the first sleep stage in the recording for the quantitative evaluation of sleepiness.We have tried another approach. Using 45-min polygraphic recordings, we evaluated the latencies and the total durations of all sleep stages, i.e., nearly all the information on wakefulness and sleep in the recording. This test has the advantage of being relatively short while giving much quantified information on the patients' diurnal sleepiness. MATERIALS AND METHODSWe examined polygraphically 10 patients with idiopathic narcolepsy-cataplexy, 10 patients suffering from idiopathic hypersomnia, 10 patients with hypersomnia-sleep apnea