Sleep specialists are frequently referred adults with epilepsy to evaluate their sleep/wake complaints, sometimes to determine whether their paroxysmal nocturnal behaviors are epileptic or not. Many patients with epilepsy have at least one parasomnia (some more than one), and the sleep specialists are often asked to differentiate and treat these. Sleep specialists review which primary sleep disorders are more common in adults with epilepsy and how to evaluate and best treat these. The authors summarize (1) how to evaluate and differentiate parasomnias using video-polysomnography;(2) the value of sleep deprivation and loud auditory stimuli to increase the likelihood of provoking a non-rapid eye movement arousal parasomnia with a single night of video-polysomnography; and (3) how to score excessive muscle activity during rapid eye movement sleep to confirm a diagnosis of rapid eye movement sleep behavior disorder. The clinical semiology and video-polysomnography features of simple and complex sleep-related movement disorders and parasomnias are reviewed.

Axial myoclonus (AM) is characterized by sudden muscle jerks involving axial and proximal muscles. It includes propriospinal myoclonus (PSM) which consists of trunk flexion or extension jerking with activity arising in axial muscles and spreading to caudal and rostral muscles at low velocity along propriospinal pathways. We report on two patients displaying flexion AM jerks in the absence of structural lesion of the central nervous system or electrophysiological evidence of organic origin. A conversion disorder was diagnosed. The jerks disappeared after psychoeducation with the patients remaining symptom free in 6-year long follow-up. The diagnoses of psychogenic axial (propriospinal-like) myoclonus were established. The literature on psychogenic axial (propriospinal-like myoclonus) is limited to a case report. Our cases demonstrate a good response to psychotropic medication and psychoeducation and fulfill the psychogenic movement disorder criteria. The phenomenology of psychogenic abnormal movements is diverse and PSM-like clinical picture may be a novel presentation.

OBJECTIVE: The literature on propriospinal myoclonus (PSM) is poor and there are no systematic reviews of the subject. We sought to clarify the spectrum of PSM. METHODS: We first prospectively investigated all patients seen in our movement disorders clinic with a firm diagnosis of PSM between 2002 and 2007. All had a standardized interview, detailed clinical examination, laboratory investigations, comprehensive neurophysiologic examination, and spinal cord MRI, including diffusion tensor imaging with fiber tracking (DTI-FT). We also collected drug responses. Finally, we conducted a systematic review of the literature. RESULTS: We enrolled 10 patients meeting the strict criteria for PSM, and also analyzed data on 50 patients from 26 previous reports. PSM occurred predominantly in male and middle-aged patients. The typical clinical picture consisted of myoclonic jerks consistently involving abdominal wall muscles, which worsen in the lying position. A premonitory sensation preceding the jerks and wake-sleep transition phase worsening were frequent. Most patients had a myoclonic generator at the thoracic level, with a myoclonus duration between 200 msec and 2 s. An underlying cause was infrequently found. DTI-FT detected cord abnormalities all of our patients. CONCLUSION: The clinico-physiologic spectrum of propriospinal myoclonus (PSM) is homogenous. Involvement of the abdominal wall muscles, worsening in the lying position, premonitory sensation, and wake-sleep transition phase worsening are helpful clinical clues. Diffusion tensor imaging with fiber tracking appears more sensitive than conventional MRI for detecting associated microstructural abnormalities of the spinal cord. Symptomatic treatment of PSM is not straightforward, and clonazepam is reported to be the most effective drug. Zonisamide may be an interesting option.

Recent evidence suggests that the motor system undergoes very specific modulation in its functional state during the different sleep stages. Here we test the hypothesis that changes in the functional organisation of the motor system involve both cortical and subcortical levels and that these distributed changes are interrelated in defined frequency bands. To this end we evaluated functional connectivity between motor and non-motor cortical sites (fronto-central, parieto-occipital) and the Globus pallidus (GP) in human non-REM sleep in seven patients undergoing deep brain stimulation (DBS) for dystonia using a variety of spectral measures (power, coherence, partial coherence and directed transfer function [DTF]). We found significant coherence between GP and fronto-central cortex as well as between GP and parieto-occipital cortex in circumscribed frequency bands that correlated with sleep specific oscillations in "light sleep"(N2) and "slow-wave sleep"(N3). These sleep specific oscillations were also reflected in significant coherence between the two cortical sites corroborating previous studies. Importantly, we found two different physiological activities represented within the broad band of significant coherence between 9.5-17Hz. One component occurred in the frequency range of sleep spindles (12.5-17 Hz) and was maximal in the coherence between fronto-central and parieto-occipital cortex as well as between GP and both cortical sites during N2. This component was still present between fronto-central and parieto-occipital cortex in N3. Functional connectivity in this frequency band may be due to a common input to both GP and cortex. The second component consisted of a spectral peak over 9.5-12.5 Hz. Coherence was elevated in this band for all topographical constellations in both N2 and N3, but especially between GP and fronto-central cortex. The DTF suggested that the 9.5-12.5 Hz activity consisted of a preferential drive from GP to the fronto-central cortex in N2, whereas in N3 the DTF between GP and fronto-central cortex was symmetrical. Partial coherence supported distinctive patterns for the 9.5-12.5 and 12.5 and 17 Hz component, so that only coherence in the 9.5-12.5 Hz band was reduced when the effects of GP were removed from the coherence between the two cortical sites. The data suggests that activities in the GP and fronto-central cortex are functionally connected over 9.5-12.5, possibly as a specific signature of the motor system in human non-REM sleep. This finding is pertinent to the longstanding debate about the nature of alpha-delta sleep as a physiological or pathological feature of non-REM sleep.

Propriospinal myoclonus is an unusual movement disorder. Here we reported a patient with electrophysiological data. The 32-year-old man had spontaneous, intermittent and brief jerks over the trunk, hip and lower limbs muscles for 4 years. The jerks were not sensitive to external stimuli and usually exacerbated whenever the patient felt relaxed, particularly preceding the onset of sleep. Electromyographic (EMG) recordings showed a long duration in each EMG burst (over 200 milliseconds) with a slow propagating pattern initiating from the rectus abdominis muscles. There was no time-locked cortical potential before the spontaneous jerks; however, we found movement-related cortical potential, while the patient was requested to mimic the jerks voluntarily. These results provided evidence to demonstrate that the jerks were not cortical origin and supported the diagnosis of propriospinal myoclonus. A comprehensive clinical evaluation including distinctive electrophysiological findings is important for the diagnosis.

BACKGROUND AND PURPOSE   The aim of this study was to evaluate the presence of abnormalities in the brain of patients with restless legs syndrome (RLS) using voxel-based morphometry and diffusion tensor imaging (DTI). METHODS   Twenty patients and twenty controls were studied. Voxel-based morphometry analysis was performed using statistical parametric mapping (SPM8) and FSL-VBM software tools. For voxel-wise analysis of DTI, tract-based spatial statistics (TBSS) and SPM8 were used. RESULTS   Applying an appropriate threshold of probability, no significant results were found either in comparison or in correlation analyses. CONCLUSIONS   Our data argue against clear structural or microstructural abnormalities in the brain of patients with idiopathic RLS, suggesting a prevalent role of functional or metabolic impairment.

OBJECTIVE We investigated the prevalence of nocturnal smoking (NS) in patients with RLS. METHODS One hundred RLS patients living in Emilia-Romagna (Northern Italy) and 100 matched controls, randomly selected from the general population, underwent interviews for the presence of nocturnal smoking and for obsessive-compulsive traits, depression, excessive daytime sleepiness (EDS) and subjective sleep quality. RESULTS NS was more prevalent in RLS patients than controls (lifetime prevalence: 12% vs. 2%, P=0.012). Patients with NS had more frequently Sleep-Related Eating Disorders (SRED) than patients without NS (83.3% vs. 26.1%, P=0.0002). Pathological and borderline Maudsley Obsessive-Compulsive Inventory (MOCI) values as well as pathological values at the Beck Depression Inventory (BDI) increased from controls to RLS patients without NS to RLS patients with NS (P=0.005 and P=0.01, respectively). CONCLUSIONS We demonstrate an increased prevalence of NS in patients with RLS, in many cases associated with increased SRED. NS may be associated with psychopathological traits in RLS and may be relevant in the management of RLS patients.

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.

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.

OBJECTIVE To study sleep-wake and body core temperature (BCT) circadian rhythms in patients with multiple sclerosis (MS)-associated with chronic fatigue. METHODS Six relapsing-remitting MS patients with chronic fatigue underwent 48 consecutive hours polysomnography (PSG) with BCT measurement, followed by a Multiple Sleep Latency Test (MSLT). All patients were relapse- and drug-free. Mood depression, brain and cervical cord enhanced MRI, dynamic spirometry and Fatigue Severity Scale (FSS) were assessed just before PSG. RESULTS In all patients mood depression was absent and dynamic spirometry normal, but FSS confirmed fatigue. MRI showed non-enhancing lesions. Nocturnal sleep was characterized by normal architecture and mean sleep efficiency was only slightly reduced. Arousal index was normal and periodic limb movements during sleep (PLMS) were present in four patients, with an increased index (PLMS-I) in only two of them. Upon MSLT, mean sleep latency was normal in all patients with one sleep onset REM period in one patient. All patients displayed a normal BCT 24-h rhythm. Mesor, amplitude and acrophase of BCT rhythm did not show significant differences between MS and controls. CONCLUSIONS We found substantially normal sleep-wake and BCT rhythmicity in six patients with MS and fatigue. Non-restorative sleep and abnormal BCT regulation were unlikely mechanisms of chronic fatigue in our MS patients. SIGNIFICANCE Subjective fatigue and abnormal sleep and BCT can be independent manifestation in MS patients. The findings support the notion that objective measures of fatigue comparable to the MSLT for sleepiness do not exist.

Obtructive sleep apnoea syndrome (OSAS) is a common disorder in the general population with an estimated prevalence in an adult population of 2% in women and 4% in men. Although several studies have suggested that headaches, particularly morning headaches, are more common in patients with OSAS than in normal subjects, others have yielded contradictory findings. When the sleep-related breathing disorder was treated with success, the headache generally disappeared, supporting a causal role of the sleep disorder for headache. Several hypotheses have been proposed to explain the relationship between OSAS and the occurrence of headache, particularly on awakening. Night-time fluctuations of oxygen saturation during the night with hypercapnia, vasodilatation, increased intracranial pressure and impaired sleep quality are all considered contributing factors. However the exact mechanisms of headache pathogenesis and the relationship between OSAS, headache and morning headaches in particular remain controversial.

Different pathological conditions may lead to somnambulic automatisms arising from nocturnal sleep. Video polysomnography represents the diagnostic tool but, due to the difficulty of capturing complex episodes in the sleep laboratory, audio-video recordings at home of the episodes may help in the differential diagnosis also. Sleepwalking is a disorder of arousal in which the subject arises from deep sleep, even displaying long complex behaviour, including leaving the bed and walking, with memory impairment of the event. Disordered arousal mechanisms with an inability of the brain to fully awaken from slow-wave sleep are thought to lead to these motor automatisms. REM sleep behaviour disorders begin during REM sleep and are accompanied by features of REM sleep. The motor behaviour may be violent and injurious to the patient and/or bed partner. In some patients, however, the behaviour may be similar to that observed in sleepwalking and some patients have an overlap syndrome. In nocturnal frontal lobe epilepsy in particular, and in complex partial seizures in general, stereotypic and repetitive motor attacks may recur, at any time, on the same night and on different nights, with a continuum between minimal or minor attacks and major or prolonged episodes up to agitated epileptic nocturnal wanderings.

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.

A patient with nocturnal frontal lobe epilepsy characterized by paroxysmal motor attacks during sleep had brief paroxysmal arousals (PAs), complex episodes of nocturnal paroxysmal dystonia, and epileptic nocturnal wandering since childhood. Ictal SPECT during an episode of PA demonstrated increased blood flow in the right anterior cingulate gyrus and cerebellar cortex with hypoperfusion in the right temporal and frontal associative cortices.

In this study, restless legs syndrome (RLS) risk factors, RLS-associated behaviors, and the ability to understand and answer an RLS diagnostic interview were investigated. In 23 older adults with early to moderate dementia and nighttime sleep disturbance, the most common risk factors for RLS were a periodic leg movement sleep index > 15 (54.55%), based on polysomnography, and use of selective serotonin reuptake inhibitors (SSRIs)(34.78%). The most common RLS-associated behaviors were repetitious mannerisms (56.52%) and general restlessness (34.78%), according to direct observation from research assistants. Finally, older adults with early to moderate dementia were unable to understand and reliably answer the RLS diagnostic interview. Older persons with mild to moderate dementia and sleep disturbance may require objective diagnostics to identify RLS.

Transcranial magnetic stimulation (TMS) is a new method, developed nearly 20 years ago, that allows the study of cortical excitability. The whole brain undergoes profound changes in sleep. Motor evoked potentials (MEPs) have been used to trace the effects of sleep on cortical excitability and to the corticomotoneuron connections. Although in the past some technical aspects limited the application of TMS in sleep, recently we observed a new explosion of interest in this field. The main body of data was gathered on sleep physiology, but its diseases or syndromes were also studied in detail. Many single and paired pulse-TMS variables were applied. Moreover, TMS variables were investigated as a potential tool for the diagnosis or the differential diagnosis of sleep disorders. In the recent years, the advent of repetitive TMS offered some therapeutic perspectives, which are under current investigation in few of these disorders. Combining repetitive TMS with electroencephalogram (EEG) represents a new and probably useful approach to sleep. Among the main entities classified in the sleep disease group, the following were subject to TMS studies: obstructive sleep apnoea syndrome (OSAS), propriospinal myoclonus, restless legs syndrome (RLS) with periodic limb movement and narcolepsy. For each of these, we examine the applications of TMS separately.

BACKGROUND AND PURPOSE Restless legs syndrome (RLS) is usually associated with periodic leg movements (PLM) occurring during wakefulness and sleep. The PLM index obtained by the polysomnographic method reflects the degree of motor symptoms and their consequences on sleep structure. Automated analysis of PLM using actigraphy can assess this condition and can therefore be used to assess therapeutic effects in clinical trials. In the current study we assessed the reliability of the PAM-RL, an ambulatory device measuring limb movements and PLM with a high-time resolution. PATIENTS AND METHODS Forty-three patients consecutively referred to the sleep laboratory for insomnia and/or excessive daytime sleepiness underwent one or two nights of polysomnography (PSG) with simultaneous bilateral recording of limb activity by the PAM-RL device. The PSG recordings were blinded and manually analyzed for PLM, while limb actimetry was scored automatically based on the manufacturer's algorithm. RESULTS There was a significant correlation between PLM derived from PSG and actimetry (r=0.87, P<0.0001) with good agreement across a wide range of values. The sensitivity and specificity of the PAM-RL device in detecting patients having a polysomnographic PLM index >10 were, respectively, 0.88 and 0.76 with a receiving operating curve having an area under the curve (AUC) of 0.86 for the entire group of patients. All patients with clinically definitive RLS and primary PLM disorder (PLMD) had a PLM index >10 on PSG, but among patients with sleep-related breathing disorders (SRBD) 60% reached this cut-off value. Conversely, only 50% of those patients with an actigraphically assessed PLM index >10 had clinically definitive RLS or PLMD, and 40% had SRBD. CONCLUSIONS We demonstrate that automatic detection of PLM derived from the PAM-RL device is highly reliable when compared to the 'gold standard' of polysomnography in patients with RLS and PLMD. Therefore, limb actigraphy can be used routinely to assess motor restlessness in patients with RLS and PLMD. The higher discrepancy in patients with SRBD and insomnia may preclude the use of the device in these patients.

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.

Background and purpose: Periodic limb movement disorder (PLMD) is frequently accompanied by awakenings or signs of EEG arousal. However, it is matter of debate whether EEG arousals trigger leg movements or both EEG arousal and leg movements are separate expressions of a common pathophysiological mechanism. Previous studies showed that cardiac and cerebral changes occur in association with periodic limb movements (PLMs), and that a combining increase in delta activity and in heart rate (HR) occurs before the onset of PLMs. Patients and methods: This paper presents some preliminary data, obtained from a sample of 5 subjects with PLMD not associated to restless legs syndrome. To describe the temporal pattern of cardiac and EEG activities changes concomitant with PLMs in NREM sleep we used time frequency analysis technique. Results: PLM onset is heralded by a significant activation of HR and delta activity power, beginning 4.25 and 3 s respectively before PLMs onset, with PLMs onset and arousal onset falling together. Discussion: Delta and HR variations herald PLMs and activation of fast EEG frequencies. Such a stereotyped pattern is common in PLMs and in spontaneous or stimuli-induced arousals. Moreover a similar pattern seems to encompass the CAP phenomenon. The whole of these phenomena can be linked to the activity of a common brainstem system, which receives peripheral inputs, regulating the vascular, cardiac and respiratory activities and synchronizing them to cortical oscillations of EEG.

Besides polysomnographic techniques, other neurophysiological methods have been utilized in order to understand the pathophysiology of restless legs and periodic limb movement syndromes. By using electromyography with nerve conduction velocity (EMG-CV) and somatosensory evoked potentials (SEPs) it has been possible to determine how frequently each muscle was involved in periodic limb movements, 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. The etiology of symptoms does not involve structural lesions since brainstem and spinal pathways are intact. Recent evidence from paired transcranial magnetic stimulation suggests that the pathophysiological key in sleep motor disorders is a modified global excitability of corticospinal pathways. The next neurophysiological goal will be to localize the unresolved anatomical level of sleep disorder movement generators and to describe their mechanism.

The movements of leg muscles in reference to periodic limb movement disorder (PLMD) have only been described in global terms. The sequences of contracting muscles that cause the PLMs are said to be stereotypical. There is, however, doubt about this fixed sequencing in PLMD. Our goal was to define the sequence of muscle movements in PLMs and then analyse their patterns. We recorded with surface EMG all movements of the muscles said to be involved in PLMs (extensor digitorum brevis, EDB; tibialis anterior, TA; biceps femoris, BF; tensor fasciae latae; TFL) as well as the quadriceps (Q) and soleus (S) muscles in 12 patients with restless legs syndrome combined with PLMD. Accompanying polysomnography provided the sleep parameters. In total, 469 movements were analysed. In only 12% was there the appearance of the classic movement (EDB-TA-BF-TFL) or its direct variants. The most frequent sequences were characterised by contraction of only the TA, TA-EDB only, or TA-EDB followed by all other combinations (32%). The pattern EDB only, EDB-TA, or EDB-TA followed by contraction of one or more other muscles, was seen in 18%. All other combinations appeared in much smaller numbers or only once. Eight patients had specific patterns. Three consistently started with the same muscle. One patient always contracted all six muscles. Six patients never contracted more than three muscles. The number of muscles contracted correlated positively with the appearance of arousal from sleep. The interval between onset of contractions within the PLMs varied randomly in a range of 0-1 s. Within PLMs many variations of muscle movements were documented. Patterns were recognisable, individually determined, and related to arousal from sleep.

OBJECTIVES (1) To determine whether clinical information can predict the presence of periodic limb movement disorder (PLMD).(2) To examine whether clinical data correlate with PLMD severity. METHODS Sixty-one adult patients (48 males and 13 females, aged 55.1+/-14.1 years) with PLMD (without a clinical diagnosis of restless legs syndrome) were compared with 61 control patients without PLMD (43 males and 18 females, aged 49.6+/-16.1 years) in this case-control study. All patients completed a detailed questionnaire which included (1) demographics,(2) sleep complaints,(3) medical disorders,(4) use of medication, nicotine, and caffeine, and (5) history of nocturnal motor/sensory leg symptoms. All patients underwent standard polysomnography. RESULTS The PLMD and control groups were similar in the prevalence of insomnia, hypersomnia, diabetes, peripheral neurologic disorders, anemia, spinal disease, antidepressant medication use, smoking, caffeine intake, and leg pain. Compared with the control group, the PLMD group reported more leg kicks (28% vs. 5%, P<0.001) and more crawling or aching sensations in legs (28% vs. 11%, P=0.023). The logistic regression analysis showed that only age (P=0.044), leg kicks (odds ratio (OR) 12.70, 95% confidence interval (CI) 2.80-57.63, P=0.001), and crawling or aching in legs (OR 5.23, 95% CI 1.16-23.44, P=0.029) were significantly related to the presence of PLMD. The positive predictive value of leg kicks in the diagnosis of PLMD was 85% and the negative predictive value was 57%. Within the PLMD group, only age correlated positively with the PLM-index (r=0.47, P<0.001). Both the PLM-index and the PLM arousal-index were negatively correlated with sleep efficiency on polysomnography (P=0.005 and P=0.006, respectively). CONCLUSIONS Clinical data are not sufficiently predictive of the presence of PLMD to rule in or rule out the diagnosis. Polysomnography is required for establishing the diagnosis of PLMD in patients with insomnia or hypersomnia.

BACKGROUND Idiopathic (primary) insomnia can be difficult to treat; only two prior cases responsive to opiate therapy have been reported. A case is now presented of severe, idiopathic, childhood-onset, familial insomnia, with increased libido, absence of psychopathology, tardive emergence of restless legs syndrome (RLS), and selective response to opiate therapy. CASE REPORT A 39-year-old woman was referred in 1981 by her physician who had discovered 3 years earlier that propoxyphene treatment of migraines also controlled her chronic insomnia. She had experienced severe insomnia since childhood, and during early adulthood the insomnia intensified, as she would sleep 0-3 h nightly and never napped. Daily generalized motor restlessness resulted in her frequently walking around the house while feeling exhausted. The quality of her life was considerably compromised by her insomnia, motor restlessness, and by an increased libido that was present since puberty and that was only partially relieved by having sex repeatedly with her husband. RESULTS Nightly opiate therapy for 19 years has controlled the insomnia, motor restlessness, and excessive libido without affecting her normal libido. The insomnia had not responded to treatment with >25 agents covering >10 pharmacologic categories. During her first (unmedicated) polysomnographic (PSG) study in 1981, she slept 0 min while spending 436 min in bed. In 1984, four consecutive PSG studies were conducted in a design that confirmed the efficacy of propoxyphene therapy of her insomnia. In 1990, an ambulatory PSG revealed two runs of EEG rhythmic paroxysmal activity arising from sleep and wakefulness, without clinical correlate. Neurologic history was negative for seizures, but positive for complete right carotid artery occlusion and three transient ischemic attacks. At age 55 years, typical RLS emerged that was controlled with levodopa therapy, and a concurrent relapse of insomnia was controlled with oxycodone replacing propoxyphene. CONCLUSIONS Nightly opiate therapy of severe idiopathic (primary) insomnia can remain effective during very long-term clinical follow-up. Guidelines are provided for when to consider such an unusual treatment in other cases of severe, chronic insomnia.

OBJECTIVE Periodic limb movement in sleep (PLMS) is a common dysfunction of motor control during sleep, occurring either in isolation or associated with a variety of neurological disorders including restless legs syndrome (RLS). Although the PLMS generators have not been established, their occurrence in patients with spinal cord injury and their clinical resemblance to the spinal cord flexor withdrawal reflex (FR) suggest that PLMS may originate in the circuitry that mediates the FR. The significantly increased spinal cord excitability noted in primary RLS/PLMS patients may play an important role in the pathophysiology of primary RLS. The aim of this study is to establish whether the enhanced spinal cord excitability, which is represented by a lower threshold and/or greater spatial spread of the FR, is also true for the RLS/PLMS patients whose RLS is secondary to chronic renal failure (CRF). METHODS Twenty patients with RLS/PLMS secondary to CRF have been compared with matched controls according to the state dependent changes in FR excitability. All patients met the diagnostic criteria for RLS and PLMS. They had CRF for 5.2+/-3.5 years, and were under the hemodialysis treatment. Twenty healthy, age and sex matched subjects were tested as controls. The electrophysiological testing of the FR was performed during wakefulness (9:30-10:30 p.m.) and sleep (beginning of stage II, the first sleep cycle). RESULTS A significant increase in FR excitability was found in RLS/PLMS patients with CRF. This abnormality was prominent during sleep, which was also true for the primary RLS. CONCLUSIONS Our results suggest that similar neuronal pathways are involved in primary and secondary RLS/PLMS patients. Our results also support that RLS/PLMS and FR share a common spinal mechanism.