Is there a polysomnographic signature of augmentation in restless legs syndrome?

OBJECTIVE: Augmentation of restless legs syndrome (RLS) is a potentially severe side-effect of dopaminergic treatment. Data on objective motor characteristics in augmentation are scarce. The aim of this study was to investigate in detail different variables of leg movements (LM) in untreated, treated, and augmented RLS patients. METHODS: Forty-five patients with idiopathic RLS [15 untreated, 15 treated (non-augmented), 15 augmented] underwent RLS severity assessment, one night of video-polysomnography with extended electromyographic montage, and a suggested immobilization test (SIT). RESULTS: Standard LM parameters as well as periodicity index (PI) and muscle recruitment pattern did not differ between the three groups. The ultradian distribution of periodic leg movements (PLM) in sleep during the night revealed significant differences only during the second hour of sleep (P <0.05). However, augmented patients scored highest on RLS severity scales (P <0.05) and were the only group with a substantial number of PLM during the SIT. CONCLUSION: This study demonstrates that polysomnography is of limited usefulness for the diagnosis and evaluation of RLS augmentation. In contrast, the SIT showed borderline differences in PLM, and differences on subjective scales were marked. According to these results, augmentation of RLS is a phenomenon that predominantly manifests in wakefulness.

Can observers link dream content to behaviours in rapid eye movement sleep behaviour disorder? A cross-sectional experimental pilot study.

Motor activity in rapid eye movement (REM) sleep behaviour disorder (RBD) has been linked to dream content. Systematic and controlled sleep laboratory studies directly assessing the relation between RBD behaviours and experienced dream content are, however, largely lacking. We aimed to investigate whether a link can be established between RBD behaviours and dream content when both are systematically sampled in a controlled setting. We investigated six patients with Parkinson syndrome and RBD who underwent 2-3 nights of video-polysomnographic recording during which they were awakened from REM sleep (10 min after the onset of the second and successive REM periods). Spontaneous free-worded dream reports and a structured dream questionnaire were obtained. Video recordings of motor manifestations were each combined with four dream reports, and seven judges had to match the video clip with the correctly reported dream content from a choice of four possibilities. Of the 35 REM sleep awakenings performed, a total of 17 (48.6%) motor-behavioural episodes with recalled dream content were obtained. The mean of correctly identified video-dream pairs was 39.5% (range 0-100%). Our data showed that reported dream content can be linked to motor behaviours above chance level. Matching accuracy was affected mainly by the clarity of dream reports and the specific nature of movements manifest in video recordings.

Genome-wide association study identifies novel restless legs syndrome susceptibility loci on 2p14 and 16q12.1.

Restless legs syndrome (RLS) is a sensorimotor disorder with an age-dependent prevalence of up to 10% in the general population above 65 years of age. Affected individuals suffer from uncomfortable sensations and an urge to move in the lower limbs that occurs mainly in resting situations during the evening or at night. Moving the legs or walking leads to an improvement of symptoms. Concomitantly, patients report sleep disturbances with consequences such as reduced daytime functioning. We conducted a genome-wide association study (GWA) for RLS in 922 cases and 1,526 controls (using 301,406 SNPs) followed by a replication of 76 candidate SNPs in 3,935 cases and 5,754 controls, all of European ancestry. Herein, we identified six RLS susceptibility loci of genome-wide significance, two of them novel: an intergenic region on chromosome 2p14 (rs6747972, P = 9.03 × 10(-11), OR = 1.23) and a locus on 16q12.1 (rs3104767, P = 9.4 × 10(-19), OR = 1.35) in a linkage disequilibrium block of 140 kb containing the 5'-end of TOX3 and the adjacent non-coding RNA BC034767.

Usefulness of the SINBAR electromyographic montage to detect the motor and vocal manifestations occurring in REM sleep behavior disorder.

OBJECTIVE: In a previous study we showed that simultaneous electromyographic (EMG) recording of the mentalis, flexor digitorum superficialis and extensor digitorum brevis (SINBAR EMG montage) detected the highest rates of rapid eye movement (REM) sleep phasic EMG activity in subjects with REM sleep behavior disorder (RBD). As a next step, in the present study we evaluated the usefulness of the SINBAR EMG montage to detect the movements and vocalizations occurring in RBD. METHODS: Polysomnographic studies with synchronized audiovisual monitoring of 11 patients with idiopathic RBD were analyzed. Phasic EMG activity in REM sleep was scored and quantified in 3-s mini-epochs while the video was reviewed to detect motor events and vocalizations. RESULTS: A total of 64.8% (11,562 out of 17,848) of all mini-epochs contained phasic EMG activity, whereas 28.8% (5135 out of 17,848) contained movements or vocalizations. Using the SINBAR EMG montage, 94.4% of the mini-epochs containing behavioral events were linked to phasic EMG activity. The sensitivity of the SINBAR EMG montage was 94.4%, specificity was 47.2%, negative predictive value was 95.4% and positive predictive value was 41.9%. Isolated EMG recording of the mentalis did not show phasic EMG activity in 35.5% of the behavioral events seen in the video. CONCLUSIONS: The SINBAR EMG montage is a useful approach for the diagnosis of RBD showing that simultaneous EMG recording of the mentalis, flexor digitorum superficialis and extensor digitorum brevis muscles detected the majority (94.4%) of the motor and vocal manifestations occurring in RBD. For clinical purposes, this means that it is efficient to screen the video when increased phasic EMG activity is seen on the polysomnography.

Motor disturbances during non-REM and REM sleep in narcolepsy-cataplexy: a video-polysomnographic analysis.

Motor events during sleep can be frequently observed in patients with narcolepsy-cataplexy. We hypothesized that increased motor events and related arousals contribute to sleep fragmentation in this disease. We aimed to perform a detailed whole-night video-polysomnographic analysis of all motor events during non-rapid eye movement and rapid eye movement sleep in a group of narcolepsy-cataplexy patients and matched controls, and to assess the association with arousals. Video-polysomnographic registrations of six narcolepsy-cataplexy patients and six sex- and age-matched controls were analysed. Each motor event in the video was classified according to topography, number of involved body parts, duration and its association with arousals. The mean motor activity index was 59.9 ± 23.0 h(-1) in patients with narcolepsy-cataplexy compared with 15.4 ± 9.2 h(-1) in controls (P = 0.004). Distribution of motor events was similar in non-rapid eye movement and rapid eye movement sleep in the patient group (P = 0.219). In narcolepsy-cataplexy, motor events involved significantly more body parts (≥ 2 body regions: 38.2 ± 15.6 versus 14.9 ± 10.0; P = 0.011). In addition, the proportion of motor events lasting longer than 1 s was higher in patients than controls (88% versus 44.4%; P < 0.001). Both total and motor activity-related arousal indices were increased in narcolepsy-cataplexy (total arousal index: 21.6 ± 9.0 versus 8.7 ± 3.5; P = 0.004; motor activity-related arousal index: 17.6 ± 9.8 versus 5.9 ± 2.3; P = 0.002). Motor activity and motor activity-related arousal indices are increased in both non-rapid eye movement and rapid eye movement sleep in narcolepsy-cataplexy compared with controls. This supports the concept of a general sleep motor dysregulation in narcolepsy-cataplexy, which potentially contributes to or even underlies sleep fragmentation in this disease.

Video-EEG monitoring: safety and adverse events in 507 consecutive patients.

PURPOSE: Video-electroencephalography (EEG) monitoring plays a central role in the presurgical evaluation of medically refractory epilepsies and the diagnosis of nonepileptic attack disorders (NEADs). The aim of this study was to analyze safety and adverse events (AEs) during video-EEG monitoring. METHODS: We retrospectively evaluated 596 video-EEG sessions in 507 patients (233 men, mean age 36 years, standard deviation = 14, range 9-80 years) within a 6-year period. AEs were examined in detail and their risk factors were assessed using multiple logistic regression analysis. KEY FINDINGS: Forty-four patients (9%) experienced 53 AEs: 20 had psychiatric events (17 postictal psychosis, 2 panic attacks, 1 interictal psychosis), 15 had injuries (14 falls with minor injuries, 2 falls with fractures, 2 fractures without fall, 1 fall with epidural hematoma), 10 patients had 13 episodes of status epilepticus (SE), and one AE was treatment-related (valproic acid--induced encephalopathy). Patients with AEs were older (p = 0.036) and had a longer duration of epilepsy (p = 0.019). All AEs resulted in a prolonged hospital stay (p < 0.001). Ninety-one percent of the AEs occurred within the first 4 days of monitoring. Independent risk factors were duration of epilepsy >17 years [odds ratio (OR) 3.096; 95% confidence interval (CI) 1.548-6.189], a previous history of psychiatric illness (OR 16.882; 95% CI 5.469-52.110), a history of seizure-related injuries (OR 3.542; 95% CI 1.069-11.739), or a history of SE (OR 3.334; 95% CI 1.297-8.565). SIGNIFICANCE: The most common AEs were postictal psychosis, falls, and SE. Patients with an older age, long disease duration, psychiatric comorbidity, history of injuries, and SE have a higher risk.

A descriptive analysis of neck myoclonus during routine polysomnography.

STUDY OBJECTIVES: Although episodes of neck myoclonus (head jerks) in REM sleep have a characteristic appearance, they have so far not been described systematically in video-polysomnography. This study assesses the occurrence, frequency, and characteristics of neck myoclonus in REM sleep in a prospective sleep disorder cohort, and investigates clinical correlates and associations with medication. SETTING: University hospital sleep disorders center. PARTICIPANTS: Two-hundred twenty-eight mixed sleep disorder patients. INTERVENTIONS: Not applicable. MEASUREMENTS AND RESULTS: REM sleep was screened visually for short "stripe-shaped" movement-induced artifacts visible vertically over the EEG leads in polysomnographic registration. If such artifact was present, the synchronized video was inspected for the presence of neck myoclonus. Out of 205 patients, 54.6% (n = 112) had neck myoclonus during REM sleep. The mean neck myoclonus index was 1.0 +/- 2.7/h REM sleep. Younger patients had a higher neck myoclonus index than older patients (< 45 years versus 45-60 years versus > 60 years: 1.8 +/- 4.2 versus 0.6 +/- 1.1 versus 0.5 +/- 1.1; P = 0.004). Ninety-five percent of subjects < 45 years had a neck myoclonus index between 0 and 9.4/h; 95% of subjects > 45 years had a neck myoclonus index between 0 and 2.7/h. Patients on benzodiazepine treatment had no neck myoclonus (0/112 vs. 13/93; P < 0.001). In 23 patients, additional surface neck EMG was performed. EMG activation associated with neck myoclonus had a mean duration of 0.6 +/- 0.4 sec. Correlation between duration of neck EMG activation and movement-induced EEG artifact duration was very high (rho = 0.96; P < 0.001). CONCLUSIONS: Neck myoclonus is common during REM sleep and more frequent in younger individuals. This could indicate that neck myoclonus during REM sleep is a physiological phenomenon. If there is a cut-off distinguishing normal from excessive has to be investigated in further studies.

Asymmetric seizure termination in primary and secondary generalized tonic-clonic seizures.

PURPOSE: In temporal lobe epilepsies an asymmetric termination (AST) of the clonic phase of secondary generalized tonic-clonic seizures (sGTCS) reliably lateralizes the side of seizure onset. The last clonic activity occurs ipsilateral to the side of the seizure onset zone. We compared the prevalence and lateralizing value of AST in sGTCS of frontal and temporal lobe origin as well as in primary generalized tonic-clonic seizures (pGTCS). METHODS: We analyzed 177 seizures in 84 consecutive patients. Forty-one patients had temporal lobe epilepsy (TLE), 24 frontal lobe epilepsy (FLE), and 19 had nonfocal (primary) generalized epilepsies (GE). All patients underwent intensive video-EEG (electroencephalography) monitoring, high-resolution magnetic resonance imaging (MRI), neuropsychological testing, and single photon emission computed tomography/positron emission tomography (SPECT/PET) when feasible. Two investigators blinded for diagnosis, EEG, and imaging data assessed frequency and side of the last clonic jerk. RESULTS: AST occurred in 63% of patients with TLE (47% of seizures), in 71% with FLE (60% of seizures), and in 42% with GE (21% of seizures). These results were not significant for patients, but significant for seizures in TLE versus GE and in FLE versus GE (p < 0.001). The positive predictive value (PPV) for the side of seizure onset was 74% (p = 0.003) in TLE and 75% (p = 0.008) in FLE. DISCUSSION: AST in sGTCS lateralizes the side of seizure onset in TLE and in FLE to the ipsilateral hemisphere with a high PPV. However, AST was also observed in GE. Therefore, asymmetric clinical signs should not inevitably lead to the assumption of focal epilepsy syndromes.

Successful surgical treatment of insular epilepsy with nocturnal hypermotor seizures.

Nocturnal hypermotor seizures (NHSs) suggest seizure onset in the frontal lobe. We present a patient with NHSs and insular seizure onset who underwent successful surgical treatment. A 29-year-old right-handed man suffered from intractable NHSs since the age of 12 years. High-resolution MRI, [(18)F]FDG-PET, and neuropsychological examination gave normal results, ictal EEG was obscured by artifacts. Ictal [(99m)Tc]HMPAO-SPECT revealed hyperperfusion in the right anterior part of the insula and right frontal operculum. The seizure onset zone was localized in the right anterior insula based on invasive recordings. Electrical stimulation in that area elicited habitual seizures. A limited resection of the anterior part of the right insula and the right frontal operculum was performed rendering the patient seizure-free (follow-up 1 year). To our knowledge, this is the first reported nonlesional patient with an insular seizure onset and NHSs who underwent successful epilepsy surgery.