NREM Parasomnia Research Articles

Quetiapine-induced behavioral disorder during sleep.

Quetiapine-induced behavioral disorder during sleep.

Parasomnia versus epilepsy: An affair of the heart?

Differential diagnosis between sleep-related epileptic seizures and NREM parasomnia remains challenging. Pathophysiological markers to differentiate both conditions are needed. Activation of autonomic system is present in arousal reactions and epileptic seizures. Our study investigated whether cardiac reactivity differed between arousal disorders and sleep-related seizures. Nocturnal recordings of 50 patients (10 in each group: frontal lobe epilepsy [FLE], temporal lobe epilepsy [TLE], nocturnal terror [NT], confusional arousal [CA], normal arousals [NA]) were retrospectively selected. Timing of the first motor sign and description of behaviours were assessed. Beat-to-beat RR intervals (RRI) as well as heart rate variability were analysed over a period of 60 heartbeats before and after first motor manifestation. The slope and area under the curve (AUC) of RRI were calculated. A significant decrease in RRI was observed at the onset of all events. Statistical analysis revealed: lower RRI in TLE than in other groups; higher slope of RRI in FLE than in NA and TLE, and higher AUC in NT and FLE than in TLE, and in NT than in NA. Sleep stage, episode duration, vocalization characteristics, lateralized or abnormal posture, exploratory behaviour differed between epileptic and parasomnia groups. Analysis of cardiac reactivity seems to be a useful objective tool, together with clinical features, for diagnosing abnormal behaviour during sleep. Cardiac reactivity in TLE could reflect epileptic discharge propagation to brain areas involved in autonomic control, while FLE and NT might involve a strong non-specific activation of the sympathetic system.

Relationship Between Sleep-related Violence and NREM Parasomnia: A Case Report

Relationship Between Sleep-related Violence and NREM Parasomnia: A Case Report

P 121 Sleep stage sequence of sleep onset REM periods predicts narcolepsy in patients with excessive daytime sleepiness

Introduction Sleep onset REM periods (SOREMP) and sleep latency on the Multiple Sleep Latency Test (MSLT) are crucial to distinguish narcolepsy from other conditions with excessive daytime sleepiness (EDS) but SOREMP may also be found in behaviorally induced inadequate sleep syndrome (BIIS), sleep deprivation, and circadian rhythm disorders. Sleep stage sequence analysis in MSLT recordings has been reported to be useful in identifying patients with narcolepsy. We aimed to confirm this hypothesis in a large cohort with special regard to HLA haplotype and CSF hypocretin (Hcrt) levels. Patients and methods 458 patients with EDS (and sleep-disordered breathing/SDB excluded) underwent nocturnal polysomnography (PSG) and MSLT. Hcrt was measured in 181 patients, and HLA haplotyping results were available in 254 individuals. A total of 1887 naps was recorded containing 272 SOREMP. Results Mean age was 36.2 (13.5) years, and 57.4% of patients were female. According to ICSD-3 criteria, 47 patients were diagnosed with type 1 narcolepsy (NL1), 30 individuals with type 2 narcolepsy (NL2), 78 patients with idiopathic hypersomnia, 9 with BIIS, 11 with circadian rhythm disorders, and 70 with other sleep disorders such as NREM parasomnia, insomnia, periodic limb movements, or unexpected SDB. Secondary CNS hypersomnia was diagnosed in 16 patients, 67 patients had hypersomnia associated with a psychiatric disorder, and in 102 participants, EDS was unexplained. SOREMP arising from sleep stage N1 (N1-SOREMP) was extremely rare in non-narcoleptic patients (n = 2), but was present in 45.5% of patients with narcolepsy. The number of N1-SOREMP was significantly higher in patients with NL1 than with NL2. 42.1% of SOREMP in the NL1 group arose from N1 compared to 15.5% in the NL2 group. In patients with narcolepsy, the number of N1-SOREMP showed negative correlation with Hcrt levels, mean sleep latency on MSLT, and REM latency on PSG. Furthermore, it was significantly associated with HLA DQB1*06:02. N1-SOREMP occurred in 69% patients with Hcrt 110 pg/ml. Conclusion Both occurance and frequency of SOREMP arising from sleep stage N1 have high sensitivity for the diagnosis of narcolepsy and type 1 narcolepsy, in particular. The number of N1-SOREMP appears to be a marker of disease severity or instability of REM sleep, respectively. Sleep stage sequence analysis of SOREMP periods may support phenotyping of patients with central disorders of hypersomnolence.

Neuropathological criteria of anti-IgLON5-related tauopathy.

We recently reported a novel neurological syndrome characterized by a unique NREM and REM parasomnia with sleep apnea and stridor, accompanied by bulbar dysfunction and specific association with antibodies against the neuronal cell-adhesion protein IgLON5. All patients had the HLA-DRB1*1001 and HLA-DQB1*0501 alleles. Neuropathological findings in two patients revealed a novel tauopathy restricted to neurons and predominantly involving the hypothalamus and tegmentum of the brainstem. The aim of the current study is to describe the neuropathological features of the anti-IgLON5 syndrome and to provide diagnostic levels of certainty based on the presence of associated clinical and immunological data. The brains of six patients were examined and the features required for the neuropathological diagnosis were established by consensus. Additional clinical and immunological criteria were used to define “definite”, “probable” and “possible” diagnostic categories. The brains of all patients showed remarkably similar features consistent with a neurodegenerative disease with neuronal loss and gliosis and absence of inflammatory infiltrates. The most relevant finding was the neuronal accumulation of hyperphosphorylated tau composed of both three-repeat (3R) and four-repeat (4R) tau isoforms, preferentially involving the hypothalamus, and more severely the tegmental nuclei of the brainstem with a cranio-caudal gradient of severity until the upper cervical cord. A “definite” diagnosis of anti-IgLON5-related tauopathy is established when these neuropathological features are present along with the detection of serum or CSF IgLON5 antibodies. When the antibody status is unknown, a “probable” diagnosis requires neuropathological findings along with a compatible clinical history or confirmation of possession of HLA-DRB1*1001 and HLA-DQB1*0501 alleles. A “possible” diagnosis should be considered in cases with compatible neuropathology but without information about a relevant clinical presentation and immunological status. These criteria should help to identify undiagnosed cases among archival tissue, and will assist future clinicopathological studies of this novel disorder.Electronic supplementary materialThe online version of this article (doi:10.1007/s00401-016-1591-8) contains supplementary material, which is available to authorized users.

Not Only Sleepwalking But NREM Parasomnia Irrespective of the Type Is Associated with HLA DQB1*05:01.

Despite the high prevalence and clinical relevance of NREM parasomnias, data on supportive genetic markers are scarce, and mainly refer to sleepwalking only. We retrospectively analyzed clinical, polysomnographic, and HLA findings of 74 adults (37 men) with NREM parasomnia gathered from four neurological sleep centers. Parasomniac events were classified according to ICSD-2 criteria. HLA DQB1 genotyping was compared to regional-matched reference allele-frequencies. Fifty-six patients had more than 2 different parasomnia type: 11 sleepwalking, 4 sleep terrors, 3 confusional arousals only. Parasomniac events were documented during video-polysomnography (V-PSG) in 70% (49/70) of subjects (71.4% confusional arousals, 8.2% sleep terrors, 4.1% sleepwalking, 16.3% ≥ 2 NREM parasomnia types). Violent behavior during V-PSG occurred in 8.5% (6/71). NREM parasomnia onset was reported after the age of 30 years in 6.8% (5/74). The HLA DQB1*05:01 allele was present in 41% (29/71) compared to 24.2% in the regional-matched reference allele group (p < 0.05). This haplotype prevalence did not differ within the NREM parasomnia type. Epworth Sleepiness Score was 10 or higher in 28.6%. This is a large polysomnography-based case series of patients with NREM parasomnia. In patients with suspected sleepwalking or sleep terrors, polysomnography is highly useful in detecting arousals from NREM sleep as a marker of NREM parasomnia. We confirmed previous findings by demonstrating a high prevalence of the HLA DQB1*05:01 genotype for different types of NREM parasomnias. Our findings therefore support a common genetic background, and corroborate the importance of video-polysomnography in the work-up of parasomnia.

Distinctive features of NREM parasomnia behaviors in parkinson's disease and multiple system atrophy.

ObjectiveTo characterize parasomnia behaviors on arousal from NREM sleep in Parkinson’s Disease (PD) and Multiple System Atrophy (MSA).MethodsFrom 30 patients with PD, Dementia with Lewy Bodies/Dementia associated with PD, or MSA undergoing nocturnal video-polysomnography for presumed dream enactment behavior, we were able to select 2 PD and 2 MSA patients featuring NREM Parasomnia Behviors (NPBs). We identified episodes during which the subjects seemed to enact dreams or presumed dream-like mentation (NPB arousals) versus episodes with physiological movements (no-NPB arousals). A time-frequency analysis (Morlet Wavelet Transform) of the scalp EEG signals around each NPB and no- NPB arousal onset was performed, and the amplitudes of the spectral frequencies were compared between NPB and no-NPB arousals.Results19 NPBs were identified, 12 of which consisting of ‘elementary’ NPBs while 7 resembling confusional arousals. With quantitative EEG analysis, we found an amplitude reduction in the 5-6 Hz band 40 seconds before NPBs arousal as compared to no-NPB arousals at F4 and C4 derivations (p<0.01).ConclusionsMany PD and MSA patients feature various NREM sleep-related behaviors, with clinical and electrophysiological differences and similarities with arousal parasomnias in the general population.SignificanceThis study help bring to attention an overlooked phenomenon in neurodegenerative diseases.

P97: Frontal lobe epilepsy versus NREM sleep parasomnia: clinical and EEG characteristics

s of Poster Presentations / Clinical Neurophysiology 125, Supplement 1 (2014) S1–S339 S75 ization as focal or generalized, as presentation was mixed. The convulsions seldom qualified as simple generalized tonic-clonic. Nearly half of our patients had generalized convulsive seizures followed by postictal EEG suppression, a possible biomarker for sudden unexpected death in epilepsy. P97 Frontal lobe epilepsy versus NREM sleep parasomnia: clinical and EEG characteristics P. Meritam1,2, M. Lauritzen1,3, B. Wanscher1, P. Jennum1,4, K. Benedek1 1Glostrup Hospital, Clinical Neurophysiology, Glostrup, Copenhagen, Denmark; 2Danish Epilepsy Center, Clinical Neurophysiology, Dianalund, Denmark; 3Copenhagen University, Neuroscience and Pharmacology, Copenhagen, Denmark; 4Copenhagen University, Copenhagen, Denmark Background: Nocturnal paroxysmal events may be characterized clinically, and by video EEG and video polysomnographic recordings, but even when applied rigorously it is often difficult to distinguish between frontal lobe epilepsy (FLE) and NREM parasomnia (NREM-P). Aim of study: To assess the value of high frequency EEG synchronization during pre-ictal state in order to differentiate between FLE and NREM-P. Methods: We analyzed the clinical features as well as macroscopic EEG and polysomnografic recordings of 12 patients with frontal lobe epilepsy and 12 patients with NREM parasomnia. Short time Fast Fourrier Transformation (FFT), spectral data analysis as well as spectral coherences were evaluated among all electrodes at 1-10 seconds prior to the nocturnal episode in both patient groups. Results: Evaluation of clinical features of frontal lobe seizures and NREM parasomnia events showed significant differences between the two groups. FLE seizures had shorter duration (<2 min), higher frequency per night (mean 9.75±3.9) as well as per month (mean 22.75±8) as compared to NREM-P events (mean 4.33±3.9 and 15.6±12.6 respectively). The semiology of FLE seizures were more stereotyped (p<0.0001) than NREM-P attacks with higher incidence of dystonic or tonic posturing and abrupt offset with recollection (p=0.011) of the event. In comparison, the semiology of NREMP events was heterogenous, with a more gradual offset and with the patient often going back to sleep and having only a vague or no recollection of the event. Our results were consistent with a higher positive FLEP scale in FLE (mean 5.42±2.3) as compared to NREM-P (mean −2.65±3.6). No abnormal rhythmic activity was observed in the macroscopic EEG recordings of most patients (FLE 58% n=7, NREM-P 92% n=11). In contrast, FFT analysis of the EEG signal showed remarkable increases of phase coherence in the high frequency beta and gamma range, mainly occurring around the frontal areas in patients with FLE prior to seizures. This was not observed in patients with NREM sleep parasomnia patients. Conclusion: The clinical features of FLE and NREM sleep parasomnia differ importantly. In addition EEG signal analysis with focus on the highfrequence EEG bands is a useful tool in distinguishing between nocturnal events. Glossary: FLE, frontal lobe epilepsy; REM, rapid eye movement; NREM, non-REM; EEG, electroencephalopraphy; FLEP scale, frontal lobe epilepsy and parasomnia scale. P98 Methionine-enriched diet increases susceptibility of rats to epilepsy: the role of sodium-potassium pump D. Hrncic1, A. Rasic-Markovic1, M. Colovic2, D. Krstic3, R. Obrenovic4, Z. Grubac1, V. Susic5, D. Macut4, D. Djuric1, O. Stanojlovic1 1Belgrade University Faculty of Medicine, Institute of Medical Physiology “Richard Burian”, Lab of Neurophysiology, Belgrade, Serbia; 2Institute for Nuclear Sciences “Vinca”, Belgrade, Serbia; 3Belgrade University Faculty of Medicine, Department of Medical Chemistry, Belgrade, Serbia; 4Clinical Center of Serbia, Belgrade, Serbia; 5Serbian Academy of Sciences and Arts, Belgrade,

P98: Methionine-enriched diet increases susceptibility of rats to epilepsy: the role of sodium-potassium pump

s of Poster Presentations / Clinical Neurophysiology 125, Supplement 1 (2014) S1–S339 S75 ization as focal or generalized, as presentation was mixed. The convulsions seldom qualified as simple generalized tonic-clonic. Nearly half of our patients had generalized convulsive seizures followed by postictal EEG suppression, a possible biomarker for sudden unexpected death in epilepsy. P97 Frontal lobe epilepsy versus NREM sleep parasomnia: clinical and EEG characteristics P. Meritam1,2, M. Lauritzen1,3, B. Wanscher1, P. Jennum1,4, K. Benedek1 1Glostrup Hospital, Clinical Neurophysiology, Glostrup, Copenhagen, Denmark; 2Danish Epilepsy Center, Clinical Neurophysiology, Dianalund, Denmark; 3Copenhagen University, Neuroscience and Pharmacology, Copenhagen, Denmark; 4Copenhagen University, Copenhagen, Denmark Background: Nocturnal paroxysmal events may be characterized clinically, and by video EEG and video polysomnographic recordings, but even when applied rigorously it is often difficult to distinguish between frontal lobe epilepsy (FLE) and NREM parasomnia (NREM-P). Aim of study: To assess the value of high frequency EEG synchronization during pre-ictal state in order to differentiate between FLE and NREM-P. Methods: We analyzed the clinical features as well as macroscopic EEG and polysomnografic recordings of 12 patients with frontal lobe epilepsy and 12 patients with NREM parasomnia. Short time Fast Fourrier Transformation (FFT), spectral data analysis as well as spectral coherences were evaluated among all electrodes at 1-10 seconds prior to the nocturnal episode in both patient groups. Results: Evaluation of clinical features of frontal lobe seizures and NREM parasomnia events showed significant differences between the two groups. FLE seizures had shorter duration (<2 min), higher frequency per night (mean 9.75±3.9) as well as per month (mean 22.75±8) as compared to NREM-P events (mean 4.33±3.9 and 15.6±12.6 respectively). The semiology of FLE seizures were more stereotyped (p<0.0001) than NREM-P attacks with higher incidence of dystonic or tonic posturing and abrupt offset with recollection (p=0.011) of the event. In comparison, the semiology of NREMP events was heterogenous, with a more gradual offset and with the patient often going back to sleep and having only a vague or no recollection of the event. Our results were consistent with a higher positive FLEP scale in FLE (mean 5.42±2.3) as compared to NREM-P (mean −2.65±3.6). No abnormal rhythmic activity was observed in the macroscopic EEG recordings of most patients (FLE 58% n=7, NREM-P 92% n=11). In contrast, FFT analysis of the EEG signal showed remarkable increases of phase coherence in the high frequency beta and gamma range, mainly occurring around the frontal areas in patients with FLE prior to seizures. This was not observed in patients with NREM sleep parasomnia patients. Conclusion: The clinical features of FLE and NREM sleep parasomnia differ importantly. In addition EEG signal analysis with focus on the highfrequence EEG bands is a useful tool in distinguishing between nocturnal events. Glossary: FLE, frontal lobe epilepsy; REM, rapid eye movement; NREM, non-REM; EEG, electroencephalopraphy; FLEP scale, frontal lobe epilepsy and parasomnia scale. P98 Methionine-enriched diet increases susceptibility of rats to epilepsy: the role of sodium-potassium pump D. Hrncic1, A. Rasic-Markovic1, M. Colovic2, D. Krstic3, R. Obrenovic4, Z. Grubac1, V. Susic5, D. Macut4, D. Djuric1, O. Stanojlovic1 1Belgrade University Faculty of Medicine, Institute of Medical Physiology “Richard Burian”, Lab of Neurophysiology, Belgrade, Serbia; 2Institute for Nuclear Sciences “Vinca”, Belgrade, Serbia; 3Belgrade University Faculty of Medicine, Department of Medical Chemistry, Belgrade, Serbia; 4Clinical Center of Serbia, Belgrade, Serbia; 5Serbian Academy of Sciences and Arts, Belgrade,

Nocturnal Paroxysmal Dystonia (NPD), two cases report

Nocturnal Paroxysmal Dystonia (NPD) is a rare condition characterized by recurrent attacks during NREM sleep of variable duration (seconds to minutes), with a complex clinical expression: repetitive stereotyped dystonic, ballistic or choreoathetoid movements involving single or all extremities and neck. It is associated with other conditions such as paroxysmal arousals, episodic nocturnal wanderings, dyskinetic or semipurposeful movements and vocalizations, often no ictal epileptic in surface EEG activity, and normal brain neuroimaging. Nowadays, it is clear that most of patients who fit the diagnosis of NPD have a form of sleep-activated focal epilepsy (nocturnal frontal lobe epilepsy). Our aim is to present two patients diagnosed with NPD. It is a descriptive study, enrolling two patients with NPD diagnosis. We report two cases. The first one, a 13 years old boy without relevant personal and family medical history, who debuted at 5 years old with dystonic and dyskinetic attacks during sleep that occurred many times per night. The second case is a 39 years old man, without relevant personal and family medical history. Since aged 31 years, he complained attacks of motor agitation with stereotypical movements every night and often several times per night. Both patients remained without neurological abnormalities in physical examination and brain neuroimaging. The EEGs during wakefulness and sleep were normal. Initially they were diagnosed as NREM parasomnia and followed many years without evidence of developing any neurocognitive disorder or neurological impairment, despite no treatment. The attacks of Nocturnal Paroxysmal Dystonia are a form of nocturnal frontal lobe epilepsy. The application of deep brain electrodes has led to the recognition that ”hypermotor seizures” may also be found in seizures originated from the temporal lobe and the insula. Functional brain imaging in frontal lobe seizures confirms that the peculiar motor patterns involve mesial and especially cingulate, motor areas. It is necessary to keep into consideration this rare and not well-known entity in the differential diagnosis of atypical parasomnias. Although the latest advances in Neurosciences, the polysomnography and the clinical history continue being pillars in the diagnosis of nocturnal frontal lobe epilepsy so the neurophysiological studies must always be considered when the diagnostic of a parasomnia is not well established. Dres. Quintana, Sáez y Paradinas.

Adult-Onset NREM Parasomnia with Hypnopompic Hallucinatory Pain: A Case Report

We report the case of a 43-year-old woman presenting with nocturnal episodes of pain and screaming during sleep starting at age 30. There was no childhood or family history of parasomnia. The events had gradually become more frequent over the years, occurring in the first half of the night within 2 h of sleep onset. There were no triggers, and she had partial amnesia for the events. A diagnosis of adult-onset sleep terrors was made on clinical grounds and supported polysomnographically. Seizures and periodic limb movements were excluded as triggering factors. There was some mild sleep disordered breathing (predominantly non-desaturating hypopnea with a propensity for REM sleep of debatable significance). Imaging of the brain and spine and neurophysiological investigations ruled out lesions, entrapments, or neuropathies as possible causes of pain. Treatment (clonazepam, paroxetine, or gabapentin) was poorly tolerated and made no difference to the nocturnal episodes, while trazodone worsened them. This is the first report of hypnopompic psychic pain in association with a NREM parasomnia. We hypothesize that the pain may represent a sensory hallucination analogous to the more commonly recognized visual NREM parasomnia-associated hypnopompic visual hallucinations and that, as such, it may arise during arousal of the sensory neocortex as confabulatory response.

The Twenty-Four hour Mind: the Role of Sleep and Dreaming in our Emotional LivesRosalind D.Cartwright, editor. Oxford University Press: New York. 2010. 208. $27.95 ISBN: 978-0-19-538683-7.

The Twenty-Four hour Mind: the Role of Sleep and Dreaming in our Emotional LivesRosalind D.Cartwright, editor. Oxford University Press: New York. 2010. 208. $27.95 ISBN: 978-0-19-538683-7.

Nightmares: An Under-Diagnosed and Undertreated Condition?

Nightmares: An Under-Diagnosed and Undertreated Condition?

Can Homemade Video Recording Become More Than a Screening Tool?

Can Homemade Video Recording Become More Than a Screening Tool?

Evidence of Dissociated Arousal States During NREM Parasomnia from an Intracerebral Neurophysiological Study

Arousal parasomnias are expressions of sleep/ wake state dissociations in which wakefulness and NREM sleep seem to coexist. We describe the results of a neurophysiological (intracerebral EEG) investigation that captured an episode of confusional arousal. Observational analysis. Tertiary sleep center. A 20-year-old male with refractory focal epilepsy. The intracerebral EEG findings documented the presence of a local arousal of the motor and cingulate cortices associated with increased delta activity in the frontoparietal associative cortices; these findings were noted preceding the onset and persisting throughout the episode. The presence of dissociated sleep/wake states in confusional arousals is the expression not of a global phenomenon, but rather of the coexistence of different local states of being: arousal of the motor and cingulate cortices and inhibition of the associative ones. Whether this is an exclusive feature of NREM parasomnias, or a common substrate on which other triggering elements act, needs to be clarified.

Evidence of Dissociated Arousal States During NREM Parasomnia from an Intracerebral Neurophysiological Study

Evidence of Dissociated Arousal States During NREM Parasomnia from an Intracerebral Neurophysiological Study

REM sleep behaviour disorder (RBD) and its associations in young patients

Study objectives To retrospectively examine the characteristics of a population of patients <50 years of age with clinical and polysomnographic features diagnostic for RBD. Methods Review of our sleep centre’s database for patients with RBD diagnosed over the last 7 years. Ninety-one patients were separated into two groups according to their age at the time of diagnosis (<50 y and ⩾50 y). Clinical and polysomnographic data were reviewed. Results Sixty-two were male; mean age was 52 ± 19 y. Thirty-nine were <50 y. In the group <50 y there was a male predominance but in a smaller proportion (M:F = 1.4:1) compared with the group ⩾50 (M:F = 3:1). Seventy-six patients complained of abnormal behaviour (AB) during sleep, 12 with narcolepsy complained of excessive daytime sleepiness (EDS) with the AB being elicited only during consultation, and three complained of both EDS and AB. All patients, except one in the group ⩾50, described AB related to vivid dreams with violent content. The majority of the patients had the idiopathic form of RBD in both groups (51.2% group <50, 63.4% group ⩾50). The secondary form was associated with narcolepsy in 38.4% of patients in the group <50 y and with a synucleinopathy in 28.8% of patients in the group ⩾50. A strong association was noted between RBD and non-REM parasomnias. Conclusions In a population of patients with RBD presenting to a regional sleep laboratory, more than one-third of patients were <50 y at time of diagnosis. The commonest associated disorder was narcolepsy in patients <50 y, and synucleinopathy in those ⩾50 y. The coexistence of RBD with a NREM parasomnia was not uncommon in cases of idiopathic RBD affecting patients <50 y.

Non-Rapid-Eye-Movement Parasomnia With Behavior Disorder

I have presented a case of NREM parasomnia with violent aggression against another person. Treatment with clonazepam failed, but imipramine therapy was successful.