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Objectives: Low vitamin D serum level has been reported in women with polycystic ovary syndrome (PCOS) compared to controls. A few in vitro studies showed that the bioactive form of vitamin D is able to modulate the expression of the anti-Müllerian hormone (AMH) gene. However, in vivo studies failed to demonstrate clearly whether low vitamin D3 serum level is involved in the AMH excess of PCOS. This prospective study evaluates serum vitamin D3 and AMH levels in women with PCOS and in controls, before and after vitamin D supplementation., Materials and Methods: Among vitamin D deficient patients, 23 patients with PCOS were compared to 27 women with normal ovarian reserve (NOR). The vitamin D deficient patients received a vitamin D supplementation according to the depth of their insufficiency. For the 23 patients with PCOS and the 27 controls, serum AMH assay and serum calciotropic hormone assays [25-hydroxyvitamin D (25[OH]D), 1,25 dihydroxyvitamin D (1,25[OH] 2 D) and parathyroid hormone (PTH)] were performed before and after supplementation., Results: Serum 25(OH)D levels before treatment were statistically lower in PCOS women than in NOR patients (P<0.05), even after adjustment for BMI, age and AMH level, but not after adjustment for waist circumference measurement. No difference in the serum AMH levels before and after treatment was observed neither in PCOS patients nor in NOR patients. In both groups, 25(OH)D serum levels were not related to serum AMH levels, serum 1,25(OH) 2 D and serum PTH levels, before and after treatment., Conclusion: We found no evidence that serum calciotropic hormones are linked to circulating AMH levels, particularly in PCOS., (Copyright © 2016 Elsevier Masson SAS. All rights reserved.)

Chronic renal failure leads to many metabolic disorders affecting reproductive function. For men, hypergonadotropic hypogonadism, hyperprolactinemia, spermatic alterations, decreased libido and erectile dysfunction are described. Kidney transplantation improves sperm parameters and hormonal function within 2 years. But sperm alterations may persist with the use of immunosuppressive drugs. In women, hypothalamic-pituitary-ovarian axis dysfunction due to chronic renal failure results in menstrual irregularities, anovulation and infertility. After kidney transplantation, regular menstruations usually start 1 to 12 months after transplantation. Fertility can be restored but luteal insufficiency can persist. Moreover, 4 to 20% of women with renal transplantation suffer from premature ovarian failure syndrome. In some cases, assisted reproductive technologies can be required and imply risks of ovarian hyperstimulation syndrome and must be performed with caution. Pregnancy risks for mother, fetus and transplant are added to assisted reproductive technologies ones. Only 7 authors have described assisted reproductive technologies for patients with kidney transplantation. No cases of haemodialysis patients have been described yet. So, assisted reproductive technologies management requires a multidisciplinary approach with obstetrics, nephrology and reproductive medicine teams' agreement., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Objectives: In Assisted Reproductive Technologies (ART), impaired ovarian reserve represents a therapeutic challenge. The Anti-Mullerian Hormone (AMH) serum level would be a good marker of ovarian reserve and a predictor of response to stimulation. The objective of this study is to assess into a population of infertile couples where the woman has at least one patent tube and where the man has sperm parameters compatible with insemination, whether AMH level less than 12pmol/L can be used to establish a strategy supporting the couple's infertility by comparing their chances of pregnancy after Intra-uterine insemination (IUI) or in vitro fertilization (IVF)., Materials and Methods: This single-center retrospective study of 1012 patients over 28months compared the pregnancy rates of 2011 ART attempts (1385 IUI and 626 IVF, ICSI excluded) according to the value of serum AMH, either reduced if≤12pmol/L or non-reduced if greater., Results: In IVF, a low AMH reduced pregnancy rate (18.4% vs. 32.9% in the normal AMH group, P<0.0001). Conversely, the AMH value did not influence the success in IUI cycles (14.2% vs. 14.5%, respectively, NS). In cases with low AMH, the pregnancy rate per initiated cycle in IVF (18.4%) was not significantly greater than in IUI cycles (14.2%). Converting an IVF attempt in IUI did not impair the pregnancy rate (13.5% vs. 14.5% after immediate IUI, NS)., Conclusion: When the serum AMH level is less than 12pmol/L, IUI may be an interesting option in case of IVF failure. However, its place remains to be defined: converting IVF in IUI, IUI in relay of failed IVF, or even as first line therapy when the chances with IVF appear to be minimal., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Fatigue and sleep disorders impact the quality of life of cancer patients. They do not put the vital prognosis at stake, but are debilitating and as a whole poorly treated. This article is oriented toward the fatigue and sleep disorders of patients with colorectal cancer. Special emphasis is put on the necessary clinical work up, on various available scales, indexes, inventories, questionnaires and on actigraphy and polysomnography, on the semiology of these disorders, on their mechanisms and on the recent therapeutic methods which are still insufficiently distributed., (Copyright © 2011 Elsevier Masson SAS. All rights reserved.)

Hypospermia is a semen volume lower than 2 mL on at least two semen analyses. The etiologies of hypospermia are many and may be divided into two pathophysiologic sub-groups: disturbances of ejaculation reflex leading to partial retrograde ejaculation and seminal glands and ducts anatomic and functional anomalies. In this last pathologic mechanism, the mutations of CFTR gene, involved in many different forms of cystic fibrosis, represent a possible cause of hypospermia. The molecular anomaly of CFTR gene's screening is very important for the potential descendents and for the patient himself. It must be considered any time clinic and/or paraclinic context is evocative.

Unlabelled: Narcolepsy is a chronic debilitating sleep disorder first described in the late 19th century. It is characterized by two major symptoms, excessive daytime sleepiness and cataplexy, and two so-called auxiliary symptoms, hypnagogic hallucinations and sleep paralysis. The final diagnosis relies on polysomnography showing the presence of sleep onset rapid eye movement periods (SOREMPs) during the multiple sleep latency test. The presence of HLA DQA1*0102-DQB1*0602 is supportive of the diagnosis. The pathophysiology of the disorder is still unknown but an imbalance between monoamines and acetylcholine is generally accepted. Recent findings in narcoleptic dogs, a natural model of narcolepsy, and in knockout mice revealed that a mutation of type 2 hypocretin receptor plays a major role in the etiology of narcolepsy. Up to now, no mutation has been found in humans except a case of early onset and atypical narcolepsy. However, a marked reduction of hypocretin type 1 has been found in the cerebrospinal fluid (CSF) of a majority of patients and a global loss of hypocretins was noted in post-mortem brain tissue of narcoleptic subjects. Conversely, no hypocretin neuron degeneration has been observed in the genetic form of narcolepsy in dogs but no trace of hypocretin was seen in the brain or the CSF in cases of sporadic canine narcolepsy. This suggests that different hypocretinergic mechanisms are involved in sporadic and genetic forms of canine narcolepsy. Treatment has not evolved significantly over the last few years. However, new drugs, such as hypocretin agonists, are currently being developed., Significance: After the discovery of the type 2 hypocretin receptor mutation in canine narcolepsy and the finding of a CSF hypocretin-1 deficiency in human narcolepsy, the major stream of research has involved the hypocretinergic system. However, other lines of research deserve to be pursued simultaneously, in view of comprehensive advancements in the understanding of narcolepsy.

Objective: A high degree of sleep fragmentation by arousals related to respiratory events would result in an abnormal distribution of slow wave activity (SWA) and a decrease in sleep spindle density in sleep disordered breathing (SDB) patients when compared to controls., Methods: Eighteen mild SDB subjects (6 females and 12 males), aged 18-56 years with (5

Case Report: A three-year-old girl was admitted for persistent fever, erythermatous rash with subsequent desquamation, stomatitis, cheleitis and cervical lymphadenopathy following development of a buttock abscess secondary to an insect bite. A TSS-positive Staphylococcus aureus strain was isolated from the abscess., Comments: Both clinical and bacteriological features led to discuss a "toxic shock syndrome without shock", an atypical form of Kawasaki syndrome without thrombocytosis and coronary arteritis or a staphylococcal skin syndrome. An early treatment with antibiotics could have limited the toxin production explaining both symptomatology and favourable course of the disease.

Objectives: A very strong SWS intensity reflected by both an increased level of SWA and an abnormal sleep spindles distribution would be responsible for the major difficulty of parasomniac subjects in waking up from SWS, leading to episodes of parasomnia., Methods: Eleven adult parasomniac subjects, 6 females and 5 males, with sleepwalking (SW) and/or sleep terrors (ST) and 11 age- and sex-matched control subjects underwent polysomnography (PSG) during 2 consecutive nights. After an habituation and selection night followed by a 16 h period of controlled wakefulness, the sleep EEGs of the parasomniac and control subjects were analyzed on the second night by computer-aided visual scoring (integrated digital filtering analysis, IDFA) and spectral analysis (fast Fourier transform, FFT). Throughout the night subject behaviour was controlled and recorded by means of a video infra-red camera and videotape recorder., Results: Fifteen episodes of parasomnia were recorded during the second night in the 11 subjects. Sleep analysis showed significantly (P<0.05) decreased sleep efficiency and stage 2 sleep (absolute values and percentage of total sleep time) and increased (P<0.05) slow wave sleep (absolute values and percentage of total sleep time). Arousal index and wake-time after sleep onset were significantly higher in parasomniac subjects. Sleep fragmentation was mainly concentrated in stages 3 and 4. The slow wave activity (SWA) absolute values averaged during the 2 min immediately preceding an episode of parasomnia were significantly higher than the SWA averaged during 2 min in the same stage 10 min before an episode of parasomnia. Moreover, SWA was higher in the slow wave sleep (SWS) episodes preceding the episode of parasomnia than in the episodes preceding an awakening without an episode of parasomnia. The temporal course of SWA showed a slower exponential decay in both groups, but the time constant of the curve was larger in parasomniacs than in controls. Finally, in control subjects the sleep spindle index increased from the beginning to the end of the night while it was equally distributed in parasomniacs., Conclusions: An abnormal deep sleep associated with a high SWS fragmentation might be responsible for the occurrence of SW or ST episodes.

The calculation of the correlation dimension (D2) was applied to the study of the profiles of EEG slow-wave activity in nine narcoleptic subjects and nine sex- and age-matched control subjects who, following a baseline night recording, were maintained on 16 h of diurnal sleep deprivation and, thereafter, submitted to a 32-h bed rest protocol. The reversibility test allowed us to reject the null hypothesis that the time series considered in our study were generated by a static transformation of a linear Gaussian random process. Similarly, all profiles showed a positive largest Lyapunov exponent. Finally, the computation of D2 showed an average value of 5.27 (0.68 S.D.) in normal controls and 4.05 (1.49 S.D.) in narcoleptic patients (p = 0.067). Four of the narcoleptic patients showed values of D2 lower than 4, this was never observed in the normal controls (p = 0.0294). This study indicates that the mechanism of sleep-wake regulation in narcolepsy shows a somewhat lower degree of complexity as compared to normal controls. In particular, these data seem to confirm the already suggested different and simpler coupling between the homeostatic process of sleep regulation and the circadian and ultradian drives to sleep that occurs in bed rest condition in this disease.

Objectives: A low level of process 5 at bed time would be responsible for a reduced amount of slow-wave activity (SWA) leading to increased alpha activity and awakenings at the end of the night., Methods: Following a base-line night (BLN) recording, 7 sleep-maintenance insomnia (SMI) subjects and 7 sex- and age-matched controls were maintained on 21 h of sleep deprivation. Thereafter, a recovery night (RN) was performed from 2300 h until spontaneous awakening. SWA (power density of the EEG delta band between 0.75 and 4.5 Hz) was monitored by means of spectral analysis (FFT). Sleep spindles and the occupation ratio of Rechtschaffen and Kales EEG bands were observed by integrated digital filtering analysis., Results: SWA was lower in SMI subjects than in controls during RN but was higher than in BLN indicating that the homeostatic process was operating, but weaker in SMI subjects. On the other hand in SMI subjects the sleep spindle index (SSI) did not decrease during slow-wave sleep and was significantly lower than in controls. Moreover during RN the SSI decreased significantly during the first sleep cycle in controls and not in SMI subjects. The existence of an inverse relationship between SWA and SSI was therefore not observed in insomniacs. Finally the mean duration of alpha frequency significantly increased in SMI subjects., Conclusions: It is hypothesised that in SMI subjects, an alteration of the homeostatic process is responsible for insufficient sleep pressure leading to an inability to maintain sleep for an extended period.

Different challenges, constraints for quality of sleep and wakefulness, sleep in extreme conditions, search for a better identification of disorders of sleep and wakefulness, spur researches on the circadian rhythm of sleep and on its disorders. Any advance in that field rests on the understanding of different physiologic phenomena: the intrinsic period of our internal clock is not 24-hours but a little less than 25-hours and it must be reset daily by the Zeitgebers. A sudden phase shift provokes an external desynchronisation. In addition not all rhythms resynchronize together resulting in an internal desyncronization. There is a close relationship between quantities of total sleep and REM sleep and the time of sleep onset within the 24-hours. The propensity to sleep follows a circasemidian rhythm with a major peak during the night and a secondary peak in the midafternoon. Disorders of the circadian rhythm of sleep are of two types: related to the misalignment of the sleep-wake schedule and the synchronizer-controlled rest-activity rhythm (shift work sleep disorder and time zone change syndrome) or related to an abnormal escape of the individual rest-activity rhythm from synchronizer control (delayed sleep phase syndrome, advanced sleep phase syndrome and non 24-hour sleep-wake syndrome.

Following a baseline night recording, 9 narcoleptic subjects and 9 sex and age-matched control subjects were maintained on 16 hours of diurnal sleep deprivation. Thereafter subjects were submitted to a 32 hour bed rest protocol in a sound-light attenuated room. The EEG was recorded and processed using a Fast Fourier Transform. Narcoleptic patients did not differ from control subjects in total sleep time over the whole time-span. An ultradian tendency to sleep seems to be predominant in narcoleptic patients and evidence of a strong basic rest activity cycle is shown. The coupling between the homeostatic process of sleep regulation and an ultradian drive to sleep would explain the peculiar 4 hour distribution pattern of SWA in narcoleptic patients.

One hundred and forty patients (104 male and 36 female) aged 42.26 +/- 19.19 (range = 8 to 79.5 years) with narcolepsy-cataplexy were given modafinil (200 to 400 mg) at the Montpellier sleep disorders center from 1984 onwards. The follow-up focused on the reduction of excessive daytime somnolence (EDS), side effects and duration of treatment. In order to determine if any clinical aspect of narcolepsy could be involved in modafinil discontinuation, patients were divided into two groups according to continued or interrupted treatment. When modafinil effect on EDS was evaluated according to a scale varying from 0 (no effect) to 3 (excellent effect), 64.1% of the subjects, scored good or excellent. The mean duration of treatment was 22.05 months +/- 24.9, ranging from 1 to 114 months. Dependency signs were never observed.

Following a baseline night recording, 9 narcoleptic subjects and 9 sex- and age-matched control subjects were maintained on 16 h of diurnal sleep deprivation. Thereafter subjects were submitted to a 32 h bed rest protocol in a sound- and light-attenuated room. The EEG was recorded and processed by a Fast Fourier Transform. Narcoleptics did not differ from controls in total sleep time over the whole 32 h, but spent more time sleeping during the daytime (DT). In both groups slow wave activity (SWA) showed an exponential decaying trend during the first night (N1); a similar exponential trend during the second night (N2) was evident only in controls. In controls SWA showed a circadian-circasemidian distribution that was hardly detectable in nacroleptics. Narcoleptics showed an ultradian distribution of SWA with periodic emergence every 4 h during DT and N2. Our data confirm that a homeostatic mechanism is evident in narcolpetics when stimulated by diurnal sleep deprivation, while circadian and circasemidian mechanisms are less evident during DT and N2. These findings suggest a different coupling between homeostatic sleep regulating and circadian drives to sleep in narcoleptics. Ultradian drives to sleep seem to be predominant in these patients, thus probably acting as a means for the avoidance of stressful attempts to counteract a weaker waking state maintenance mechanism.

Three cases of adenomatous hyperplasia of the endometrium--one of them degenerated--have been reported in young women provided with ovulation stimulation. The association between these hormonal therapies and the adenocarcinoma or its antecedents signs, is particularly disturbing. However, it's difficult to establish a relationship between cause and effect. Indeed the women who suffer from an ovarian sterility are a group exposed to a cancer of the endometrium independently of any other iatrogenic agent. The method of detection is yet to be found, as is the mode of conduct which can reconcile the risk of a carcinoma and the desire of pregnancy.

Since the discovery of an almost 100% association of HLA-DR2 with narcolepsy-cataplexy, many efforts have been made to demonstrate the intervention of immune factors in the pathogeny of the disease. Some epidemiological features could support this hypothesis: age of onset around 25, triggering factors, association with multiple sclerosis. Molecular studies at the DNA level have, up to now, failed to uncover an abnormal gene in the HLA system, which would imply that the DR2 antigen acts through its role in the immune response. However, results have been largely inconclusive as far as classical features of autoimmunity in blood and CSF are concerned. In canine narcolepsy, a linkage with a human immunoglobulin-related gene has recently been shown, and may constitute a counterpart of the HLA association in man. Thus, the hypothesis of a transient and discrete autoimmune aggression may be ruled out.

A meningoencephalitis Behçet's disease revealing is reported. Magnetic resonance imaging is more sensitive than computerized tomography in detecting encephalitic lesions. However, no specific pattern may be retained. This exploration is non invasive and can be used to monitor the evolution after treatment.

CNS stimulants are the most widely used drugs to treat narcolepsy which is characterized by the excessive daytime sleepiness and typically associated with cataplexy. However, a number of side effects may often arise with this therapeutic approach. Thus, investigating new drugs which are efficient but well tolerated is of utmost importance in the treatment of narcolepsy. Although modafinil, an alpha-1 adreno-receptor agonist, has been reported to bring substantial awakening properties in animals, the studies performed in man, particularly in narcoleptic subjects, are few. In the present study, we evaluated the effects of a 300 mg daily dose of modafinil on sleepiness and psychomotor performance of 16 narcoleptic subjects. The major effect of modafinil in narcoleptic subjects was a decrease of daytime sleepiness and corresponding improvement of performances involving attentive functions. However, the learning effect in psychomotor tests may mask the drug effect.

Sleep of 8 narcoleptic and 8 control subjects was recorded under baseline (i.e., prior wakefulness 16 h) and after 24 h without sleep. During both baseline and recovery total sleep time and stage 2 non-REM sleep were significantly decreased in narcoleptic subjects. Slow wave activity (i.e., EEG power density in the range of 0.75-4.5 Hz) decayed exponentially during baseline and after sleep deprivation in both narcoleptic and control subjects. During both baseline and recovery EEG power density in delta and sigma frequencies in non-REM sleep was enhanced in narcoleptic subjects relative to controls. In REM sleep differences in the same direction were present in delta and beta frequencies. After sleep deprivation EEG power density in non-REM sleep was elevated in delta and some higher frequencies in both patients and controls, but the response to sleep deprivation was stronger in narcoleptic subjects. These data show that in narcoleptic subjects regulatory processes underlying non-REM sleep homeostasis are operative and indicate that the response to sleep deprivation is stronger than in control subjects.

The relationships between severe developmental dysphasias and epilepsy were analysed in 32 patients with congenital dysphasias. The mean age was 8 years 2 months; 19 of 32 had never had seizures; 9 had had occasional seizures; 4 were epileptic. Twenty-two of 32 had normal repeated standard EEGs, but 10 (2 of which never had seizures) showed epileptic interictal discharges. During prolonged EEG after sleep deprivation, epileptic abnormalities were observed in 13 of the 32 cases (4 of which never had seizures). The overall night sleep recordings showed epileptic abnormalities in 30 of the 32 cases (17 of which had never had seizures). The epileptic interictal abnormalities varied considerably in intensity and aspect in the same patient from one examination to another. Developmentally aphasic children show a higher incidence of abnormal EEG than expected, particularly during overall night recordings. In most cases, the physiopathology of the language disturbance might be identical to that in Landau-Kleffner syndrome.

Sleep is known to facilitate epileptic manifestations but can also protect the sleeper against the recurrence of seizures. This has been demonstrated in studies on sleep deprivation, and is particularly evident in alcoholic epilepsy and matutinal myoclonus epilepsy. Sleep organization in the epileptic patient is permanently altered by frequent awakenings and stage shifts. Nocturnal grand mal and repetitive partial seizures worsen the sleep disorder by reducing total sleep time and decreasing REM percentage by half. The cumulative effect of these sleep disorders may act on day-time vigilance in epileptics, and may even exert an influence on the recurrence of seizures.

Patients with daytime sleepiness present altered states of consciousness. The occurrence of these states impairs their professional, social and familial activities and may threaten life itself. The automatic behavior syndrome is characterized by continuation of mechanical activity and complete amnesia. Episodes lasting from a few seconds to several hours are correlated with repetitive micro-sleep periods. During cataplectic attacks, patients may have a meshing of reality with hallucinatory dream contents. Sleep-induced apnea may lead to abnormal movement and abnormal ambulation during sleep as well as hallucinations in the early morning. These altered states of consciousness must be considered as diagnostic indexes in differentiating epileptic syndromes from syndromes of daytime sleepiness.

Ten healthy volunteers, aged 20 to 39, underwent 2 adaptation nights and 3 sessions of 2 consecutive experimental nights and days at 1 week intervals. In the 3 sessions, subjects received under double blind conditions either Zopiclone 3.75 mg or 7.5 mg or placebo, according to a latin-square design. On nights 1 and 2 of each session, subjects were continuously polygraphically monitored, except for a 45 min provoked wake episode 135 min after sleep onset on night 2. Sleep continuity and architecture were evaluated during night 1, degree of daytime somnolence during day 1 and residual effects during night 2 (0 h 00) and day 2 (8 h 00 and 12 h 00). Sleep continuity was not modified, except for a reduction of the number of night awakenings. NREM sleep stage 1 was reduced and stage 2 was increased (in duration but not in percentage) with Zopiclone 3.75 and 7.5 mg. NREM sleep stages 3 and 4 were increased with Zopiclone 3.75 mg only. REM sleep was reduced (in percentage only) with Zopiclone 3.75 and 7.5 mg. Daytime somnolence varied according to the time but not with the 3 different conditions. One performance test only (choice reaction time test) showed a significant impairment at 0 h 00 with Zopiclone 7.5 mg. From a subjective point of view, sleep quality was improved and night time awakening was reduced with Zopiclone 7.5 mg.

Twenty narcoleptic patients and ten age-matched normals were polygraphically monitored for 58 consecutive hours. All subjects were on regimented sleep (hours between 2230 and 0700). Group A (11 patients and 10 normals) had enforced wakefulness during the day whereas Group B (9 patients) were permitted to sleep (mean = 2 1/2 hr.). On day 2, all subjects were permitted to sleep for 15-min periods every 2 hr. In narcoloptics, sleep recordings demonstrated a reduction of sleep latency, an increase of stage 1, and a decrease in stages 3 and 4 compared to normals, but total REM time and percentage of REM sleep were similar. Groups A and B showed no difference in the incidence of nocturnal awakenings. REM cyclic periodicity was larger in narcoleptics who also demonstrated a REM-sleep fragmentation. This fragmentation became more pronounced as time passed, with several shifts from REM to wakefulness and stage 1. Narcoleptics present REM onset sleep period but also show an inability to remain in REM sleep.