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We assessed the validity of an Italian language version of the Epworth sleepiness scale (ESS). The translated ESS was compared to the multiple sleep latency test (MSLT), considered the gold standard for the diagnosis of excessive daytime sleepiness (EDS). Within the context of a multicentric national study on narcolepsy (Gruppo Italiano Narcolessia Studio Epidemiologico Nazionale, GINSEN) involving 17 Italian sleep centres, we compared the two diagnostic tests on 91 prospectively recruited subjects with suspected EDS (34 with narcolepsy, 16 with obstructive sleep apnea syndrome, 19 with idiopathic hypersomnia, and 22 with other sleep, neurologic or psychiatric disorders). ESS scores were inversely correlated with mean sleep latency values, as measured with MSLT (rho = −0.31, p

Background Sleep disorders are increasingly recognized in the symptomatology of many neurodegenerative diseases. Gerstmann-Straussler-Scheinker (GSS) disease is a hereditary prion disease featuring cerebellar ataxia, akinetic parkinsonism, pyramidal signs and cognitive decline. Methods We performed a polysomnographic study (PSG) of sleep and body core temperature (BcT°) in two sisters with GSS. Results Our study showed protracted nocturnal awakenings, reduced sleep efficiency and brief daytime naps but also qualitatively preserved slow-wave and REM sleep and substantially normal arousal and periodic limb movements in sleep indices and BcT° rhythm. Conclusions These findings conflict with those in multiple system atrophy and other prion diseases such as fatal familial insomnia, which enter the differential diagnosis of GSS and are characterized by prominently disrupted sleep-wake and BcT° cycles.

GUILLEMINAULT ET AL RECENTLY REPORTED IN SLEEP A SERIES OF SEVEN NON OBESE YOUNG WOMEN AFFECTED WITH SLEEP-RELATED RESPIRATORY noises that they diagnosed as cases of catathrenia (sleep-related groaning).1 In their patients sleep-related respiratory noises occurred mainly during light NREM sleep and diminished during REM sleep. Respiratory noise in these patients was expiratory. In view of the presence of a significant, mainly inspiratory flow limitation, with anatomic evidence of a small upper airway and small jaws, the authors put their patients on nasal CPAP treatment, plus successive surgical intervention or oral device appliance in four of them, that abolished/reduced sleep-related hypopnoeas and inspiratory flow limitation with resolution of the respiratory noise. They argued therefore that catathrenia may have different subtypes related to sleep stage specificity or to the presence of sleep disordered breathing. Together with a recent report of continuous airway pressure benefit in a case of nocturnal groaning associated with mild obstructive sleep apnoea, oxygen desaturation and irregular abdominal wall movements,2 Guilleminault et al. raise the question regarding the definition, the heterogeneity and the pathophysiology of catathrenia.1 Catathrenia was first reported as sleep-related expiratory groaning during REM sleep in abstract form.3 Pevernagie et al. described apparently the same phenomenon as “vocalization during prolonged expiration during REM sleep”,4 and, in our description of nocturnal groaning, we proposed the term of catathrenia (meaning “groaning”) in 4 cases in which the groaning sounds, though sometimes present also during NREM, were prevalent during REM sleep.5 The same prevalence, or even the recurrence solely during REM sleep, was emphasized by Oldani et al. in their report of 21 patients, 16 examined by polysomnography (PSG).6 In all of the PSG performed in catathrenia, the features were quite similar and stereotyped: a deep inspiration without sound production was followed by a prolonged expiration with groaning, usually lasting from at least 2 s to 20 seconds; there was bradypnea during catathrenia, no evidence of respiratory muscular effort and no oxygen desaturation.5,6 Thus, the hallmark of catathrenia is that inspiration is followed by protracted expiration during which a prolonged or fragmented sound is produced, and this recurs especially during REM sleep. Vetrugno et al. recently reported ten patients with catathrenia and without any evidence of other facial, airway or lung disease in whom intrathoracic pressure recordings was performed.7 During the expiratory groaning sounds, neither the diaphragm nor the intercostalis EMG were active and endoesophageal pressure, monitored in all patients, showed a slight initial positive rise subsequently returning to values around 0 cm H2O. Strikingly, during the groaning, the respiratory pattern was distinctly abnormal, with a substantial slowing of the respiratory rate (breathing rate was actually decreased by 2/3) and a disproportionate increase in the length of expiration. Remarkably again, nocturnal groaning alternated with normal breathing in the same night, sometimes in the same sleep state.7 This respiratory pattern, that we consider as typical for catathrenia, is clearly different from what reported by Guilleminault et al.1 In their seven patients, the produced sound was expiratory but short lasting (about 1 s in their figure 1, and again 1–1.5 s in figure 2, compared to 2–20 s in our patients) and occurred at the peak of expiration; in particular, expiration was not prolonged, and there was no bradypnea during the “groaning” (see Figures 1 and 2 in Guilleminault et al.);1 sometimes, a noise could be detected also at the peak of inspiration (see Figure 1 in Guilleminault et al);1 finally, the sounds were not produced during REM sleep.1 All of these differences are substantial, and indicate that the case of Guilleminault et al1 are not catathrenia, but rather instances of expiratory snoring. Figure 1 Catathrenia (A) compared to expiratory snoring in a snorer patient (B). The recordings are shown at the same time scale. Please note: 1) the different duration of the groaning sounds (microph.), 2) the prolonged expiration (oral, thoracic and abdominal ... In order to show that these two phenomena are quite distinct, we provide a picture comparing the polysomnographic features of a catathrenic patient of ours versus expiratory snoring, both displayed at the same time scale. The differences are readily evident (Figure 1). Narrowing of the upper airway and flow limitation is not solely an inspiratory phenomenon but may also occur with expiration in healthy subjects, in snorers, in patients with upper airway resistance syndrome (UARS) and in patients with obstructive sleep apnoea syndrome (OSAS) (Stanescu et al, 1993).8 Observations of expiratory obstruction during sleep are not new. Weitzman et al. first postulated expiratory obstruction9 and Lugaresi et al. provided an illustration of expiratory flow limitation in a snorer.10 Gravity (mainly during the supine body position) together with relaxation of pharingeal dilator muscles, such as the tensor palatini and genioglossus, have been considered to promote local upper airway narrowing during expiration more frequently at the supraglottic/retroglossal level. Isolated expiratory flow limitation has been reported to recur in breaths and to be coupled with inspiratory flow limitation in sleep.8 Whether catathrenia is an abnormal, possibly vestigial, central respiratory pattern during sleep, due to abnormality in brainstem respiratory centers, as we proposed,7 and whether narrowing of the airways occurs during catathrenia, must be left to further studies. We also consider that whether catathrenia is a parasomnia rather than a sleep-related breathing disorder is just a matter of definition. In the previous ICSD-1 classification indeed, at the time of most reports of catathrenia, snoring too, itself a respiratory phenomenon, was classified as a parasomnia. What we are really concerned about and wish to emphasize with this letter is that catathrenia presents with a distinctive pattern on the PSG, and should be clearly differentiated from other respiratory disturbances during sleep. This has clear and important consequences on prognosis and treatment.

Sleep-related breathing disorders are common causes of excessive daytime sleepiness, a socially and clinically relevant problem. Mechanisms responsible for daytime sleepiness are still largely unknown. We investigated whether specific alterations in autonomic cardiac modulation during sleep, commonly associated with sleep-related breathing disorders, are related to excessive daytime sleepiness. Fifty-three patients with sleep-related breathing disorders underwent nocturnal polysomnography. Excessive daytime sleepiness was diagnosed as a Multiple Sleep Latency Test response less than or equal to 600 s. We explored the relation of excessive daytime sleepiness, objectively determined, with indices of autonomic cardiac regulation, such as baroreflex sensitivity and heart rate variability, with polysomnographic indices of the severity of sleep-related breathing disorders and with quality of sleep. Patients with excessive daytime sleepiness, when compared with patients without, had significantly lower baroreflex sensitivity and significantly higher low-to-high frequency power ratio of heart rate variability during the different stages of nocturnal sleep. By contrast, no differences were found in indices quantifying the severity of sleep-related breathing disorders or sleep quality. We demonstrated that excessive daytime sleepiness is accompanied by a deranged cardiac autonomic control at night, the latter probably reflecting autonomic arousals not detectable in the EEG. As abnormal autonomic regulation is also known to be associated with increased cardiovascular risk, a possible relation between excessive daytime sleepiness and cardiovascular events in patients with sleep-related breathing disorders deserves to be investigated in future studies. © 2008 European Sleep Research Society.

Narcolepsy is a clinical condition characterized mainly by excessive sleepiness and cataplexy. Hypnagogic hallucinations and sleep paralysis complete the narcoleptic tetrad; disrupted night sleep, automatic behaviors and weight gain are also usual complaints. Different studies focus on autonomic changes or dysfunctions among narcoleptic patients, such as pupillary abnormalities, fainting spells, erectile dysfunction, night sweats, gastric problems, low body temperature, systemic hypotension, dry mouth, heart palpitations, headache and extremities dysthermia. Even if many studies lack sufficient standardization or their results have not been replicated, a non-secondary involvement of the autonomic nervous system in narcolepsy is strongly suggested, mainly by metabolic and cardiovascular findings. Furthermore, the recent discovery of a high risk for overweight and for metabolic syndrome in narcoleptic patients represents an important warning for clinicians in order to monitor and follow them up for their autonomic functions. We review here studies on autonomic functions and clinical disturbances in narcoleptic patients, trying to shed light on the possible contribute of alterations of the hypocretin system in autonomic pathophysiology.

Catathrenia (nocturnal groaning) is a rare condition characterized by monotonous irregular groans occurring during sleep. Ten patients (five women; mean age: 27 +/- 7.4 years, range: 15-41) with sleep-related groaning persisting for years or decades and normal daytime fibreoptic laryngoscopy and respiratory function tests underwent videopolysomnographic recording (VPSG) analysing their respiratory patterns during sleep. After the VPSG, all patients were clinically followed up for a mean period of 4.9 +/- 3.5 years. On VPSG, all patients showed nocturnal groaning during NREM sleep and particularly during REM sleep stages. Groaning was associated with disproportionate prolonged expiration causing reduced breathing rate without oxygen desaturation. The breathing pattern with prolonged expiration and sound production alternated with a normal respiratory pattern without groaning. Endoesophageal pressure during groaning showed mildly positive swings at the initial phase of expiration suggesting a partial mild expiratory upper airway obstruction. At the end of the follow-up period, all patients reported persistent nocturnal groaning but no other clinical manifestations. Groaning confined to sleep alternating with normal breathing and the absence of long-term clinical consequences suggest that catathrenia is because of an abnormality of the internal respiratory drive system, possibly related to persistence of a neonatal (vestigial) type of breathing pattern.

To estimate the interobserver reliability (IR) of the minimal diagnostic criteria for the parasomnias provided in the International Classification of Sleep Disorders Revised (ICSD-R).Fifty consecutive subjects underwent a structured interview by three doctors based on the ICSD-R minimal criteria for the diagnosis of 13 parasomnias at any time in life. IR was calculated by Kappa statistics and interpreted according to conventional categories.In the group of Arousal Disorders, IR was "substantial" (kappa 0.74) for confusional arousals, "slight" (kappa 0.36) for sleepwalking, "fair" (kappa -0.02) for sleep terrors. In the group of Wake-Sleep Transition Disorders, IR was "substantial" to "almost perfect", but "moderate" for sleep starts (kappa 0.41). In the group of Parasomnias usually associated with REM Sleep, IR was "substantial" (kappa 0.69) for sleep paralysis, "moderate" (kappa 0.46) for RBD, "fair" (kappa 0.25) for nightmares. In the group of Other Parasomnias, IR was "substantial" to "almost perfect" (kappa between 0.73 and 0.93).When the clinical diagnosis of parasomnias is based on the ICSD-R: 1) the majority of Arousal Disorders and REM sleep parasomnias showed only a "fair" to "moderate" IR; 2) all of the other parasomnias showed a "substantial" to "almost perfect" IR. Nosological entities with unsatisfactory IR share complex motor phenomenology associated with a mental state difficult for the patient to define. The source of disagreement probably lies in the difficulty in interpreting patients' reports. For these parasomnias IR must be verified and possibly improved with training.

Involuntary patterned motor activity may occur during seizures, especially those of frontal lobe origin, and during transient ischemic attacks. Paroxysmal patterned motor activity in frontal lobe epilepsy has been attributed to direct involvement of mesial frontal regions by the epileptic discharge. Paroxysmal bipedal frenetic activity occurred during an episode of syncope in a patient with a carotid body tumor. The presence of rhythmic motor patterns similar to the epileptic ones also during syncope and cerebral ischemia suggest a phenomenon of release from neocortical inactivation, probably of innate motor behaviors generated by neural networks referred to as central pattern generators (CPGs).

This section, IMAGES IN SLEEP MEDICINE, is intended to tap a relatively unique feature of sleep science: images that have great educational and conceptual content. These could be, for example, electroencephalograms, electromyograms, polysomnograms, portable devices, actigrams, scans including functional images, pathology specimens, brain slice preparations, fluorescent microscopy and other cutting edge techniques. The source may be human or nonhuman, but the clinical relevance should be clear. Illustrations must consist of clear and high-quality, blackand-white or color-digitized images. At this stage, videos can be submitted only for online publication. The message accompanying the picture may be a maximum of 500 words, with no more than 5 references. It is especially important not to try and make a case report out of these submissions, and detailed clinical (as contrasted to image) analysis should be avoided. The material should be submitted on the Sleep Medicine website (www.ees.elsevier.com/sleep). Images can be embedded within a text document such as Microsoft Word, a slide program such as Microsoft PowerPoint, or converted to Acrobat files. Authors should expect that the images will be available for use (with acknowledgement) to the general sleep community for teaching purposes, and so copyrighted/ patentable material should be avoided. In its final form, these images will be a freely available, searchable digital teaching image library. Comments regarding the images should be sent by e-mail to the section co-editors within a month of the publication date. These comments will be summarized and posted, if determined to be of educational content, with the original image on the journal’s web site, expected to be active for content soon. We hope this section will be enriched by the contributions of our colleagues who wish to offer stimulating opportunities for discussion and new insights in the field of sleep.

PURPOSE: This study aims to report on catathrenia occurring in narcolepsy with cataplexy (NC) patients under sodium oxybate (SO) treatment. Catathrenia is a parasomnia characterized by groaning and an abnormal respiratory pattern during sleep. METHODS: Fifty-one patients with NC and starting SO therapy underwent a baseline overnight polysomnography (PSG) to detect any sleep-related breathing disorders (SRBD). To avoid risks due to a possible central respiratory control depression by SO, all patients with concomitant obstructive sleep apnea (OSA) were treated with a nasal continuous positive airway pressure (nCPAP) device. After 2 months of treatment with SO, all patients underwent a follow-up overnight PSG to investigate possible newly occurring SRBD. They also underwent a semi-structured clinical interview to monitor other potential SO side effects. RESULTS: At baseline, four out of 51 patients showed simple snoring, and eight, mild to severe OSA. After a titration PSG night, patients with OSA received a nCPAP device. After 2 months of SO treatment, 28 patients (54.9%) showed SO-related side effects, including SRBD in 11 (21.6%). The follow-up PSG showed a respiratory pattern characteristic of catathrenia in seven patients (13.7%) as a newly observed and possibly benign SO side effect, and ruled out a worsening of OSA. CONCLUSIONS: Catathrenia should be considered a possible side effect in NC patients under SO treatment and should be accurately identified to prevent unnecessary SO withdrawal.

While dreaming amputees often experience a normal body image and the phantom limb may not be present. However, dreaming experiences in amputees have mainly been collected by questionnaires. We analysed the dream reports of amputated patients with phantom limb collected after awakening from REM sleep during overnight videopolysomnography (VPSG). Six amputated patients underwent overnight VPSG study. Patients were awakened during REM sleep and asked to report their dreams. Three patients were able to deliver an account of a dream. In all dreaming recalls, patients reported that the amputated limbs were intact and completely functional and they no longer experienced phantom limb sensations. Phantom limb experiences, that during wake result from a conflict between a pre-existing body scheme and the sensory information on the missing limb, were suppressed during sleep in our patients in favour of the image of an intact body accessed during dream.

This section is a new feature of Sleep Medicine allowing readers to submit unique sleep related cases with video segments as a way to enhance educational value in a manner unattainable through paper format. Please see our web site’s (http://ees.elsevier.com/ sleep/) Guide for Authors for instructions. We hope this section will be enriched by the contributions of our colleagues who wish to offer stimulating opportunities for discussion and new insights into the field of sleep.

Objective To describe the unique case of a middle-aged woman with severe insomnia recurring with a regular infradian period without any other significant clinical condition. To infer the existence of a circadian dysfunction modeled according to the physical phenomenon of the “beats.” Patient/Methods A two-year prospective observation by means of a sleep log was performed during the patient’s normal life. She underwent one month of motor activity recording and also polysomnography, circadian rhythm of body core temperature and psychiatric evaluation during periods with and without insomnia. Results Visual inspection of the 293-day plot of the sleep log disclosed a regular 42-day rhythm of insomnia recurrence confirmed by a Discrete Fourier Transform. During the periods of insomnia, lasting 5–7 days, only moderate mood symptoms (depressive overlapping hypomaniac symptoms) were present. Treatment with sodium valproate was effective in curtailing insomnia. Conclusion The wax and wane infradian modulation of the sleep length suggested the presence of a basic mechanism similar to the physical phenomenon of the “beats,” i.e., a long period modulation of the amplitude of an oscillating system due to the interference of two uncoupled oscillators with a slightly different oscillation frequency. Hypothesizing a dysfunction of the circadian component of sleep, namely two uncoupled circadian cycles, a simple mathematical model estimated the difference of their periods of oscillation |34 ± 2 min| and reproduced the sleep-log data of the drug-free period of observation.

Peduncular hallucinosis (PH) consists of formed and coloured visual images, which the patient knows are unreal; it is often associated with lesions of the pons, midbrain and diencephalon. A 72-year-old man had noted the sudden onset of visual hallucinations one year before, specifying the time and body position in a 4-week, 24-h diary. Thereafter, he underwent video-polysomnography (VPSG), brain magnetic resonance imaging (MRI), angiography (MRA), proton spectroscopy ((1)H MRS), and single photon emission tomography (SPECT). Patient's diaries and VPSG showed a strong clustering of hallucinatory experiences during the evening/night time while lying in supine position, similar to hypnagogic hallucination and sleep paralysis in supine position. Repeated episodes of REM sleep behaviour disorder (RBD) occurred during the night. MRI and MRA showed an elongated and dilated left internal carotid artery displacing the left subthalamus upwards, and (1)H MRS relatively decreased N-acetyl-aspartate in the left subthalamus. Brain SPECT during PH revealed hypoperfusion in the right temporal region and hyperperfusion in the left occipital and right opercular regions (the latter possibly related to the patient's awareness of unreality). PH resolved with serotonergic (citalopram) therapy.

We report the case of a 47-year-old man with idiopathic central sleep apnoeas recurring during wake-sleep transitions at night or at diurnal naps. Symptoms associated with central apnoeas were sudden awakening with shortness of breath, sleep fragmentation and daytime tiredness. Following the failure of CPAP and BiPAP, we treated the patient with zolpidem which reduced central apnoea events, improved sleep and eliminated the patient's nocturnal disturbing symptoms. Beneficial effects were still present after two years of treatment. Our case demonstrates that zolpidem may be beneficial in the long term treatment of idiopathic central sleep apnoea syndrome.

Objective We present two patients, a 52-year-old man and a 56-year-old woman, with rapid eye movement sleep behaviour disorder (RBD) since the age of 50. Method In both the patients RBD was videopolysomnographically documented. Results Both patients developed, with time, autonomic and motor symptoms consistent with the diagnosis of multiple system atrophy. During the course of the disease RBD episodes diminished in frequency but the patients’ sleep became even more abnormal, with nearly continuous motor and verbal abnormal behaviours and ambiguous and rapid oscillations of state-determining polysomnographic variables, now consistent with status dissociatus (SD). Conclusion If SD represents the evolution and most extreme form of RBD it should be investigated longitudinally in neurodegenerative diseases.

SUMMARY The aim of this study was to investigate the relationships among sympathetichyperactivity, excessive daytime sleepiness (EDS) and hypertension in obstructive sleepapnoea syndrome (OSAS). Ten newly diagnosed OSAS patients with untreated EDSand daytime hypertension underwent polysomnography (PSG) and daytime measure-ments of plasma noradrenaline (NA), ambulatory blood pressure (BP), musclesympathetic nerve activity (MSNA) by microneurography and objective assessmentof EDS before and during 6 months of compliance-monitored continuous positiveairway pressure (CPAP) treatment. One month after the start of CPAP, BP, MSNAand NA were significantly lowered, remaining lower than baseline also after 3 and6 months of treatment. CPAP use caused a significant improvement of sleep structures,and reduced EDS. A statistical correlation analysis demonstrated that EDS was notcorrelated with sleep measures obtained from baseline PSG (% sleep stages, apnoea andarousal index, mean oxygen saturation value), whereas daytime sleepiness wassignificantly correlated with MSNA. Furthermore, MSNA and BP showed nocorrelation. Our data obtained from selected patients suggest that the mechanismsinducing EDS in OSAS are related to the degree of daytime sympathetic hyperactivity.Additionally, resting MSNA was unrelated to BP suggesting that factors other thanadrenergic neural tone make a major contribution to OSAS-related hypertension. Theresults obtained in this pilot study need, however, to be confirmed in a larger studyinvolving more patients.keywords continuous positive airway pressure, hypertension, muscle sympatheticnerve activity, obstructive sleep apnoea syndrome, sleepiness

Objective We describe six patients affected by frequent episodes from sleep associated with compulsive smoking and/or eating. Patients woke up with a desire to smoke and/or eat because of an “inner” drive. Method Video-polysomnography (VPSG) was performed in three patients. Results VPSG documented a normal sleep structure with an increased arousal index. Conclusion Compulsive eating during sleep has been classified as sleep-related eating syndrome or Nocturnal eating syndrome, but its association with compulsive smoking has not been previously reported.

Restless legs syndrome (RLS) augmentation, defined as a kind of suppression of the circadian rhythm of the disease in which sensory and motor symptoms appear earlier during the day (and over previously unaffected body parts), with a progressive phase advance until, backwards, the symptoms may cover the entire day, has been described only after treatment with dopaminergic drugs. We report clinical and polysomnographic accounts of a patient developing RLS augmentation after long-term treatment with tramadol, an opioid agonist with selectivity for mu-receptor and added norepinephrine and serotonin reuptake inhibition properties. Polysomnographic measures showed an improvement of RLS and a disappearance of diurnal sensory and motor RLS symptoms after tramadol was stopped. Our case confirms a recent retrospective report of augmentation of RLS after treatment with tramadol, and begs the question whether augmentation is truly restricted to dopaminergic drugs.

Seizures, namely in certain epileptic conditions, may be precipitated by sleep. Nocturnal frontal lobe epilepsy seizures, characterized by bizarre motor behaviour and autonomic activation, appear almost exclusively during sleep. The differential diagnosis between this condition and sleep-related non-epileptic paroxysmal motor phenomena, in particular the parasomnias, is arduous. Moreover, accepted criteria for the diagnosis of nocturnal frontal lobe seizures are lacking and even ictal scalp EEG recording could fail to disclose paroxysmal abnormalities. The clinical and polygraphic features of the different types of seizures in nocturnal frontal lobe epilepsy and of the more common non-epileptic paroxysmal events during sleep are described. The main differentiating features characterizing nocturnal frontal seizures are: onset at any age, several attacks per night at any time during the night, brief duration (s) with stereotyped motor pattern. As video-polysomnographic recordings of the attack, the gold-standard for diagnosis, are expensive and not readily available everywhere, home-made video recordings may be helpful. Further investigations on pathophysiology, genetics and epidemiology are needed to clarify the relationship between epileptic and non-epileptic sleep related paroxysmal phenomena.

Objective To evaluate the structure and quality of sleep and the circadian rhythm of body core temperature (BcT degrees ) in patients with drug-resistant chronic cluster headache (CH) before and during deep brain stimulation (DBS) of the posterior hypothalamus. Background Chronic CH is a severe primary headache and frequently associated with disturbances in sleep. Posterior hypothalamus DBS is performed as an effective treatment of drug-resistant chronic CH. The effects of posterior hypothalamus DBS on sleep and the circadian rhythm of BcT degrees are unknown. Methods Three male patients with chronic drug-resistant CH underwent 48-hour consecutive polysomnography (PSG) by means of the VITAPORT system with determination of BcT degrees by means of a rectal probe. Recordings were done before electrode implantation in the posterior hypothalamus and after optimized DBS of posterior hypothalamus. Results Before electrode implantation PSG showed nocturnal CH attacks, reduced sleep efficiency, fragmented sleep and increased periodic limb movements in sleep (PLMS). During DBS nocturnal CH attacks were abolished and sleep efficiency and PLMS improved. BcT degrees circadian rhythm was normal both before and during DBS. Conclusions Our data show that DBS of posterior hypothalamus in drug-resistant chronic CH is effective in curtailing nocturnal CH attacks, and is associated with improved sleep structure and quality. Chronic CH displays a normal circadian rhythm of BcT degrees, unchanged during hypothalamic DBS.

Previously referred to as “nocturnal myoclonus”, periodic limb movements in sleep (PLMS) is regarded as a distinct nosologic entity, although it overlaps a great deal with restless legs syndrome and other sleep disorders. While there is a lack of complete consensus on this matter, PLMS are regarded as pathological if more than five events occur per hour of sleep, and as a disorder (PLMD) if there is comorbid insomnia or daytime sleepiness in the absence of other symptoms. PLMS are common in healthy elderly individuals and have been reported to increase with age. The pathogenesis of PLMS is not clear. Lesion, imaging and laboratory studies indicate neuronal hyperexcitability with involvement of brain stem and spinal cord structures, in particular the central pattern generator for gait, and decreased dopaminergic transmission. Dopaminergic drugs, dopamine agonists, opiates, benzodiazepines and anticonvulsants are all used, with dopaminergic drugs or dopamine agonist regarded as first-line treatment.

Sleep-disordered breathing and sleep-related motor phenomena are part of the clinical spectrum of multiple system atrophy (MSA). Stridor has been attributed to denervation of laryngeal muscles or instead to dystonic vocal cord motion. We studied 3 patients with nocturnal stridor in the setting of MSA. All patients underwent nocturnal videopolysomnography (VPSG) with breathing and heart rate, O(2) saturation and intra-esophageal pressure recordings, and simultaneous EMG recordings of the posterior cricoarytenoid, cricothyroid, and thyroarytenoid muscles and continuous vocal cord motion evaluation by means of fiberoptic laryngoscopy. VPSG/EMG and fiberoptic laryngoscopy documented normal vocal cord motion without denervation during wake and stridor only during sleep when hyperactivation of vocal cords adductors appeared in the absence of significant O(2) desaturation. All patients had tachycardia and tachypnea and paradoxical breathing during sleep, erratic intercostalis and diaphragmatic EMG activity and Rem sleep behavior disorder. One of the patients had restless legs syndrome with periodic limb movement during sleep and excessive fragmentary hypnic myoclonus. In conclusion, our patients with MSA had nocturnal stridor due to sleep-related laryngeal dystonia. Stridor was associated with other abnormal sleep-related respiratory and motor disorders, suggesting an impairment of homeostatic brainstem integration in MSA.

Study objectives To describe the clinical and videopolysomnographic characteristics of nocturnal eating episodes in sleep-related eating disorder. Design Descriptive study of outpatients prospectively enrolled in 2 sleep centers. Setting Videopolysomnographic recordings done in the sleep laboratory. Patients Thirty-five consecutive drug-free patients with nocturnal eating. Interventions N/A. Measurements and results Clinical interviews disclosed abnormal compulsory nocturnal eating episodes in all patients associated with a clinical report of sleepwalking (in 1), somniloquy (in 5), restless legs syndrome (in 8), and periodic limb movements during sleep (in 4). Videopolysomnography documented 45 episodes of nocturnal eating in 26 patients. Eating always occurred after complete awakenings from non-rapid eye movement sleep and only in 1 patient from REM sleep and was characterized by electroencephalographic alpha activity with no dissociated features of state-dependent sleep variables. Patients interviewed during the eating episodes were fully conscious and remembered the events the next day. Pathological periodic limb movements during sleep index was recorded in 22 and restless legs syndrome dyskinesias in 5 patients. Recurring chewing and swallowing movements during sleep were a feature in 29 patients, associated in about half of the events with electroencephalographic arousals. Conclusions In our patients, eating episodes occurred with normal consciousness and recall. Chewing or swallowing movements during sleep occurred frequently, resembling rhythmic masticatory-muscle activity in bruxism patients. The presence of periodic limb movements during sleep and chewing activity, the reported efficacy of dopaminergic medications, and the compulsory food-seeking behavior all argue for a dopaminergic dysfunction underlying the pathogenesis of sleep-related eating disorder.

Augmentation, defined as a loss of circadian recurrence with progressively earlier daily onset and increase in the duration, intensity, and anatomy of symptoms, not compatible with the half-life of the drug, is associated with dopaminergic treatment in restless legs syndrome (RLS) patients. The pathogenesis of augmentation is unclear. We describe a patient with idiopathic RLS who developed augmentation after 8 months of levodopa treatment. Videopolysomnographic and pharmacokinetic studies with monitoring of plasma levodopa levels demonstrated marked motor hyperactivity during augmentation, with anarchic discharges of motor unit potentials, tonic grouped discharges and flexor spasms, associated with painful dysesthesia. Symptoms and signs of augmentation were related to low plasma levodopa levels, abating 75 minutes after oral levodopa administration and reappearing after 3 hours, closely mirroring the rapid rise and fall of plasma levodopa concentration. This case is the first report in which RLS augmentation is shown to be characterized by motor hyperkinesias paralleling levodopa plasma pharmacokinetic profile.

Studies comparing the efficacy of continuous positive airway pressure (CPAP) versus surgery in correcting cardiovascular abnormalities in OSAS are lacking. We describe an OSAS patient with hypertension who responded favorably to CPAP treatment, whereas subsequent uvulopalatopharyngoplasty (UPPP) was less successful. While CPAP markedly lowered daytime muscle sympathetic nerve activity (MSNA) and blood pressure (BP), findings after UPPP were comparable to pre-treatment baseline. Thus, parallel changes in MSNA and BP demonstrated treatment efficacy.

Three patients presented with a 25-, 15-, and 5-year history of restless legs syndrome (RLS) and periodic limb movements during sleep (PLMS). For 1, 4, and 5 years, they reported additional involuntary trunk and limbs jerks preceding falling asleep and occasionally during intrasleep wakefulness. Videopolysomnography revealed jerks during relaxed wakefulness arising in axial muscles with a caudal and rostral propagation at a slow conduction velocity, characteristic of propriospinal myoclonus (PSM). Jerk-related EEG-EMG back-averaging did not disclose any preceding cortical potential. During relaxed wakefulness preceding falling asleep and during intrasleep wakefulness, PSM coexisted with motor restlessness and sensory discomfort in the limbs. PSM disappeared when spindles and K-complexes appeared on the EEG. At this time, typical PLMS appeared every 20 to 40 seconds, especially during light sleep stages. PLMS EMG activity was limited to leg, especially tibialis anterior muscles, and did not show propriospinal propagation. In one patient, alternating leg muscle activation was also present. Jerks with a PSM pattern represent another motor phenomenon associated with RLS and different from the more usual PLMS.

Objective To ascertain the presence of abnormalities of sleep in patients with continuous motor unit activity with and without symptoms of central nervous system involvement. Methods Five patients with isolated neuromyotonia (Isaacs' syndrome) and 1 patient with Morvan syndrome underwent 24-h videopolysomnographic recording to investigate sleep structure, motor activities and autonomic variables during sleep. Results Macro- and microstructural organization of sleep and of the attending autonomic variables were substantially normal in patients with Isaacs' syndrome. On the contrary, sleep structure was severely disrupted with subcontinuous dream enactment and hallucinations in the patient with Morvan syndrome. The pattern of the neuromyotonic discharges, however, was not different between the patients with Isaacs' syndrome compared to Morvan syndrome, the EMG discharges persisting throughout the 24 h of recording and affecting wakefulness and sleep equally. Conclusions Neuromyotonia is compatible with normal organization of sleep. The severe sleep abnormalities observed in Morvan syndrome cannot be simply attributed to the effects of neuromyotonia of peripheral origin. Significance Even though neuromyotonia is common to both Isaacs' and Morvan syndromes, the two conditions differ significantly in regard to CNS involvement with sleep abnormalities and lumping the two conditions together is not justified on clinical and neurophysiological grounds.

Sleep-related eating disorder (SRED) is characterized by recurrent arousals from sleep associated with compulsive ingestion of food. No controlled therapeutic trials are available for SRED. We assessed the safety, tolerability and efficacy of pramipexole, a dopamine D3-receptor agonist, in the treatment of SRED. Eleven consecutive patients with SRED in the absence of concurrent daytime eating disorders underwent actigraphic recording and subjective sleep diary evaluation for a week before and every week for 2 weeks of treatment with pramipexole 0.18-0.36 mg or placebo, administered in a double-blind crossover randomized sequence. The primary outcomes of the trial were actigraphic measures of night sleep parameters (sleep efficiency, motor activity mean and median, number and duration of wake episodes), secondary outcomes were the number of good sleep nights/weekly, number and duration of nocturnal awakenings/night, and visual analogue preference score. Pramipexole was well tolerated without any patient withdrawing from the study. Pramipexole reduced night-time activity median (P = 0.02) and increased the number of nights of good sleep/week (P = 0.02). All other measurements remained unaffected. Pramipexole at low doses was well tolerated, improving some measures of sleep quality and reducing median night activity in SRED. Further studies with higher dosages and for longer time-periods are warranted.

The article briefly summarizes the milestones leading to current knowledge and the possibility of treating one of the most widespread and perhaps least known diseases, restless legs syndrome (RLS). Until the mid-twentieth century, the syndrome first described by Willis (1685), was sporadically reported in medical literature and in most cases deemed a bizzare condition. It was only with Ekbom's detailed clinical description of the syndrome (1944) and the polygraphic recordings of Coccagna et al. (1962) that RLS became well-recognised clinical entity. Since then, almost all sleep laboratories have devoted much of their research to discovering the pathogenetic mechanisms underlying the disease and devise increasingly specific treatment. Major advances have been made in recent years, but a full understanding of RLS is still a long way off.

A “body image” is an internal construct of a unitary corporeal self that endures in space and time, even after partial amputations of the physical body. A body image provides the substrate for the feeling of “residual” experiences of the missing body part, that is the “phantom” phenomenon.1 Restless legs syndrome (RLS) is characterised by uncomfortable sensations, usually affecting the legs, associated with an urge to move, worsening at rest, in the evening, and at night, and which increase during attempts to remain still.2 We report the case of a patient who developed painful “phantom” phenomena after the amputation of both legs and the upper right forearm. One year later, in the absent portions of his extremities, there began symptoms that he had never experienced before the amputations, and that he clearly distinguished from the “phantom.” These symptoms were congruent with a diagnosis of RLS. A 56-year-old man began haemodialysis at age 39 due to a chronic renal failure secondary to glomerular nephropathy. Thereafter he developed a severe arteriosclerotic obliterative arteriopathy that led to gangrene at the age of 51, necessitating bilateral above-knee and right below-elbow amputations. A few days after the amputations, “phantom” phenomena began consisting of attacks of …

Limb amputation is followed, in approximately 90% of patients, by “phantom limb” sensations during wakefulness. When amputated patients dream, however, the phantom limb may be present all the time, part of the time, intermittently or not at all.1 The absence of the phantom limb when dreaming has been taken as evidence for a pre-existing kinesthetic body scheme, unaffected by the amputation, that is accessible to the patient when asleep.2 Such dreaming experiences in amputees have usually been obtained only retrospectively in the morning and, moreover, dreaming is normally associated with muscular atonia so the motor counterpart of the phantom limb experience cannot be observed directly. REM sleep behaviour disorder (RBD), in which muscle atonia is absent during REM sleep and patients act out their dreams,3 allows a more direct analysis of the “phantom limb” phenomena and their modifications during sleep. A 58-year-old man had a history of erectile failure and abnormal ejaculation, a 5 year history of orthostatic hypotension and frequent somniloquy with excessive motor activity during sleep, usually accompanied by vivid striking dreams, sometimes of violent content but not causing self- or bed-partner injury. At the age of 39 years, his left arm was amputated at the level of the middle-third of the …