Our data do not support a role for leptin in mediating increased body mass index in narcolepsy. A moderate but selective increase in C-reactive protein in hypocretin-1 deficient subjects should prompt research on inflammation in narcolepsy.

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.

Excessive fragmentary myoclonus during sleep consists of high amounts of brief twitch-like movements occurring asynchronously and asymmetrically in different body areas and has been reported to occur in association with a number of sleep disorders. It was analyzed using a new technique of quantification, the fragmentary myoclonus index (FMI). The FMI exhibited high rates in all stages of sleep but with a somewhat lower frequency in slow wave sleep explaining, as well, a significantly lower rate in the first hour after sleep onset compared to later hours. There was no evidence for greater sleep fragmentation or lighter sleep compared to a matched patient group in whom it had not been noted. RESUME: Myoclonic fragmentaire excessive: horaire nocturne et stades du sommeil. La myoclonic fragmentaire excessive pendant le sommeil consiste en un nombre considerable de mouvements fasciculaires se produisant de facjon asynchrone et symetrique dans differentes regions du corps. Ce phenomene a ete rapporte en association avec un grand nombre de troubles du sommeil. Nous l'avons analyse au moyen d'une nouvelle technique de quantification, l'indice de myoclonie fragmentaire (IMF). L'IMF etait eleve dans tous les stades du sommeil, avec une tendance vers des frequences moins elevees pendant le sommeil a ondes lentes, ce qui explique egalement des frequences significativement plus basses dans la premiere heure apres l'endormissement par rapport aux heures suivantes. II n'existait pas d'evidence de fragmentation du sommeil ou de sommeil plus leger compare a un groupe de patients apparies, chez qui ce phenomene n'avait pas ete note.Can. J. Neurol. Sci. 1993; 20: 142-146 Physiological fragmentary myoclonus occurring in normal human subjects was first described by De Lisi 1 as consisting of brief, fine, twitch-like movements involving various body areas in asynchronous and asymmetric fashion and observable at sleep onset and at times during sleep. Using surface EMG electrodes, normal fragmentary myoclonus has been recorded as muscle potentials of 50-200 ^tV in amplitude and less than 150 msec in duration most prominent in the distal limbs and facial areas and predominating during REM sleep and at sleep onset. 2 -3 Fragmentary myoclonus (FM) must be distinguished from the normal phenomenon of so-called sleep starts or hypnic jerks, which also occur at sleep onset, but consist of bilaterally synchronous gross body movements. 4 -5 It also differs from several pathological forms of sleep-related myoclonus. Epileptic myoclonus can occur during sleep and is associated with a EEG discharge. 6 Periodic limb movement disorder 7 -8 is easily distinguished by the more sustained nature and pseudorhythmic repetition of the jerks.Excessively intense and frequent FM has been reported to occur in association with a variety of clinical diagnoses, almost always in males and in combination with high amounts of sleep fragmentation. 9 It has also been reported in patients in whom excessive daytime sleepiness (EDS) was the sole complaint ...

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