Background: Recently different reports on atypical localization of Restless Legs Syndrome (RLS) symptoms (namely one arm, tongue, thorax, and abdomen) beg the question of the boundaries of RLS.

The first polysomnographic recordings with concomitant monitoring of cardiocirculatory parameters demonstrated that obstructive apneas arising during sleep are accompanied by a marked increase in pulmonary and systemic arterial pressure and severe alveolar hypoventilation. Apneas also may give rise to cardiac arrhythmias, namely potentially life-threatening bradyarrhythmias. The long-term repercussions of these nocturnal cardiocirculatory changes on subsequent cardiovascular diseases and the patient's life expectancy are more controversial. There is little doubt that patients with obstructive sleep apnea syndrome (OSAS) have systemic arterial hypertension, ischemic heart disease, transient ischemic attacks, or stroke more often than control populations and have a shorter life expectancy. However, these clinical manifestations may be at least partly due to myriad other risk factors almost always present in OSAS patients (in particular obesity, diabetes, alcoholism, and cigarette smoking). Few multivariate epidemiological surveys have addressed all these confounding factors. The effectiveness of continuous positive airway pressure treatment in reducing the incidence of cardiovascular comorbidity in OSAS patients is not disputed, even though controlled epidemiological surveys on large populations are scant. This overview of cardiovascular disorders and OSAS examines the latest literature findings aimed at establishing the true impact of nocturnal apneas on cardiocirculatory disease (systemic arterial hypertension, ischemic heart disease, stroke, pulmonary hypertension and right heart failure and mortality).

The aim of the study was to determine the clinical frequency and features of REM sleep behaviour disorder (RBD) in a large population of Parkinson's disease (PD) patients using defined diagnostic criteria both for RBD and PD. Six trained neurologists used a semistructured questionnaire based on ICSD-R diagnostic criteria for RBD to evaluate 200 PD patients and their caregivers. Interobserver reliability for the diagnosis of RBD was "substantial" (Kappa 0.65). Five patients were excluded from the study because of an MMSE lower than 25. The demographic and PD clinical features were compared in the clinically defined RBD group and in those without RBD (NRBD). Then the RBD features during the last year were analysed in the affected group. Out of 195 patients, 66 fulfilled the ICSD-R criteria for RBD; 62 patients reported RBD during the last year (frequency 31.8%). RBD features: two or more episodes per week in 35.5%; upper limb movements in 87%; lower limb movements in 79%; vocalisations during events in 85%. RBD onset was before PD in 27% of patients; 69% of the RBD group had injured themselves or their caregivers during sleep. According to multivariate analysis, RBD was associated with male gender, age and PD duration. Brief training and the use of a semistructured questionnaire may help the neurologist in dealing with sleep disturbances in PD patients. The search for RBD symptoms in PD is highly recommended, especially in patients with a long disease duration, the risk of sleep-related injuries being high.

We describe the novel association of Horner syndrome and contralateral paroxysmal facial flushing and sweating (Harlequin syndrome) with overlap parasomnia. A 66 year old woman suffered right jaw and multiple limb fractures when she was 45. Since then she had recurrent sudden onset episodes of right hemifacial flushing and sweating, occurring with physical exertion and heat, rarely spontaneously, and lasting 20 to 30 minutes. Approximately one year after the trauma, her sleep became restless, being interrupted by excessive motor activity, seemingly purposeful and sometimes harmful, associated with vocalisation and a report of dreaming corresponding to the motor manifestations. Hypnagogic hallucinations and sleep paralysis could also rarely occur. Both facial flushing and sleep disturbances worsened after another accident to her face when she was 64. Examination at age 66 showed partial ptosis of the left eyelid, myosis, and iris heterochromia with subtle enophthalmos. Anisocoria increased in dim light, and 0.01% epinephrine eye drops did not modify the size of the left pupil. Brain magnetic resonance imaging (MRI), somatosensory evoked potentials, transcranial magnetic stimulation, blink reflexes and palmar sympathetic skin responses, baseline and tilt test cardiovascular reflexes, and circadian body core temperature rhythm were all normal. Sweating—assessed by the application of alizarin powder during a heating test1—was absent on the left side of the face and reduced on the right upper limb. After heating, only the right side of the face showed flushing. These changes were replicated by physical exercise. There was no …

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.

Background: The pathophysiology of periodic limb movements in sleep (PLMS) in restless legs syndrome (RLS) is unclear. Objective: The authors neurophysiologically investigated PLMS in patients with idiopathic RLS in order to obtain information on the origin and pathophysiology of the movements. Methods: Ten patients with idiopathic RLS underwent electromyography with nerve conduction velocity (EMG-CV), somatosensory evoked potentials (SEPs), transcranial magnetic stimulation (TMS), nocturnal videopolysomnography, and multiple sleep latency test. The authors analyzed 100 consecutive PLMS for each patient to determine how frequently each muscle was involved in the PLMS; how frequently EMG activity started in a given muscle; and the time delay and pattern of activation between the first and the other activated muscles. Results: EMG-CV, SEPs, and TMS findings were all normal; in PLMS, leg muscles were those more frequently involved, often with alternation of side. Axial muscles were rarely and upper limb muscles sometimes involved. The tibialis anterior was the most frequent starting muscle. There was no constant recruitment pattern from one PLMS episode to another, even in the same patient. There was no ordinate caudal or rostral spread of the EMG activity. Conclusion: The recruitment pattern indicates the engagement of different, independent, and sometimes unsynchronized generators for each PLMS. The authors hypothesize an abnormal hyperexcitability along the entire spinal cord, especially its lumbosacral and cervical segments, as the primary cause of PLMS, triggered by sleep-related factors located at a supraspinal but still unresolved level.

Thirty-eight patients with lone paroxysmal atrial fibrillation (PAF) were monitored during spontaneous sleep. The starts of 111 episodes of atrial fibrillation (AF) occurred in 12 patients. Statistical analysis failed to disclose significant differences in the number of starts per hour between wakefulness and the different sleep stages. Heart rate (HR) in the 60 seconds prior to a PAF episode did not show significant changes with respect to fragments of recording taken away from PAF episodes. As expected, spectral analysis of HR variability during sleep (basal values) showed a reduction of low frequency/high frequency (LF/HF) ratio during slow sleep, while the ratio returned to values similar to wake levels during REM sleep. In the 2 minutes preceding a PAF episode the LF/HF ratio of the spectral power was more unstable with a clear-cut prevalence of sympathetic activity both in wake and in different sleep stages. A shift toward parasympathetic activation was never seen before the start of any of the 17 PAF episodes recorded during sleep.

We describe the sleep evolution of two cases of segmental myoclonus. The first patient had symptomatic palatal myoclonus which, as in most reported cases, persisted during sleep with a slight but significant reduction in frequency. The second patient presented apparently essential spinal myoclonus, which disappeared on falling asleep and recurred for short periods during arousals. This patient also had nocturnal myoclonus involving the legs, as well as those muscles affected by spinal myoclonus. The physiopathological significance of this unusual association is discussed.

After the advent of polygraphic recordings in the 1960s a great deal of interest focused on cardiocirculatory and respiratory activity during sleep. The Bologna sleep laboratory was the first to make direct recordings of systemic arterial pressure, pulmonary arterial pressure and alveolar ventilation in normal subjects, measuring gas-blood values during different sleep stages. In the 1960s, neurophysiologists rediscovered a syndrome known to pneumologists for a decade as Pickwickian Syndrome. Polygraphic studies performed in sleep laboratoires all over Europe (Germany, France and Italy) led to a major discovery: the syndrome was not caused by respiratory overload due to obesity, but usually by the presence of obstructive apnoeas arising during sleep. By means of continuous sleep recordings, our laboratory documented the severe repercussions of apnoeas on ventilatory and cardiocirculatory functions. Hypnologists pointed to tracheostomy as the logical effective treatment for the syndrome. Surgery was first performed in Germany by Kuhlo and coworkers and then in Bologna. In the early 1970s, following the Bologna group's research, there emerged the now accepted concept that obstruction of the upper airways is a continuum stretching from snoring to full-blown sleep apnoea syndrome. The Bologna team was also the first to conduct epidemiological surveys that indicated that snoring is a relevant risk factor for the cardiocirculatory system. Here the trends of haemodynamic and ventilatory parameters during sleep are investigated in syndromes of obstructive respiratory failure. The conclusion is that sleep, particularly REM-sleep, exacerbates all these disorders, and the topic provides a basis for a wider look at how cardiocirculatory activity varies during sleep under normal and pathological conditions.

The idea of a continuum between heavy snoring and complicated forms of obstructive sleep apnea syndrome (OSAS) came to us in the 1970s when we established that all patients with OSAS had been heavy snorers for years [1]. For this reason we have always included heavy snoring in our studies on the syndrome of upper airway obstruction during sleep, which we have proposed to call heavy snorers’ disease [2].

The case of a young woman suffering from a rare cerebrovascular disease associated with livedo reticularis (Sneddon syndrome) is reported. Hemicranic attacks were the first symptom detected. The patient had a progressive clinical course of neurologic symptoms. Cerebral CT scan, NMR and cerebral arteriography revealed a progressive cerebral multinfarctual feature involving middle-size arteries.

A 51-year-old man with type I Arnold-Chiari malformation presented with severe autonomic impairment. Polysomnography disclosed central and obstructive sleep apneas. During REM sleep apneas were accompanied by hypotension and led to convulsive nonepileptic attacks. Marked blood pressure oscillations and central apneas persisted after tracheostomy.