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OBJECTIVE To develop and test wearable monitoring of surface electromyography and motion for detection and quantification of positive and negative myoclonus in patients with progressive myoclonic epilepsy type 1 (EPM1). METHODS Surface electromyography and three-dimensional acceleration were measured from 23 EPM1 patients from the biceps brachii (BB) of the dominant and the extensor digitorum communis (EDC) of the non-dominant arm for 48 hours. The patients self-reported the degree of myoclonus in a diary once an hour. Severity of myoclonus with action was evaluated by using video-recorded Unified Myoclonus Rating Scale (UMRS). Correlations of monitored parameters were quantified with the UMRS scores and the self-reported degrees of myoclonus. RESULTS The monitoring-based myoclonus index correlated significantly (p

Progressive myoclonic epilepsy type 1 (EPM1) is caused by biallelic alterations in the CSTB gene, most commonly dodecamer repeat expansions. Although transcranial magnetic stimulation (TMS)-induced long-interval intracortical inhibition (LICI) was previously reported to be normal in EPM1, short-interval intracortical inhibition (SICI) was reduced. We explored the association between these measures and the clinical and genetic features in a separate group of patients with EPM1.TMS combined with electromyography was performed under neuronavigation. LICI was induced with an inter-stimulus interval (ISI) of 100 ms, and SICI with ISIs of 2 and 3 ms, and their means (mSICIs) were expressed as the ratio of conditioned to unconditioned stimuli. LICI and mSICI were compared between patients and controls. Nonparametric correlation was used to study the association between inhibition and parameters of clinical severity, including the Unified Myoclonus Rating Scale (UMRS); among patients with EPM1 due to biallelic expansion repeats, also the association with the number of repeats was assessed.The study protocol was completed in 19 patients (15 with biallelic expansion repeats and 4 compound heterozygotes), and 7 healthy, age- and sex-matched control participants. Compared to controls, patients demonstrated significantly less SICI (median mSICI ratio 1.18 vs 0.38; p .001). Neither LICI nor SICI was associated with parameters of clinical severity. In participants with biallelic repeat expansions, the number of repeats in the more affected allele (greater repeat number [GRN]) correlated with LICI (rho = 0.872; p .001) and SICI (rho = 0.689; p = .006).Our results strengthen the finding of deranged γ-aminobutyric acid (GABA)ergic inhibition in EPM1. LICI and SICI may have use as markers of GABAergic impairment in future trials of disease-modifying treatment in this condition. Whether a higher number of expansion repeats leads to greater GABAergic impairment warrants further study.

Purpose Obstructive sleep apnea (OSA) is associated with increased risk for stroke, which is known to further impair respiratory functions. However, it is unknown whether the type and severity of respiratory events are linked to stroke or transient ischemic attack (TIA). Thus, we investigate whether the characteristics of individual respiratory events differ between patients experiencing TIA or acute ischemic stroke and matched patients with clinically suspected sleep-disordered breathing. Methods Polygraphic data of 77 in-patients with acute ischemic stroke (n = 49) or TIA (n = 28) were compared to age, gender, and BMI-matched patients with suspected sleep-disordered breathing and no cerebrovascular disease. Along with conventional diagnostic parameters (e.g., apnea-hypopnea index), durations and severities of individual apneas, hypopneas and desaturations were compared between the groups separately for ischemic stroke and TIA patients. Results Stroke and TIA patients had significantly shorter apneas and hypopneas (p p = 0.007) and a trend for higher apnea/hypopnea number ratios (p = 0.091). The prevalence of OSA (apnea-hypopnea index ≥ 5) was 90% in acute stroke patients and 79% in transient ischemic attack patients. Conclusion Stroke patients had different characteristics of respiratory events, i.e., their polygraphic phenotype of OSA differs compared to matched reference patients. The observed differences in polygraphic features might indicate that stroke and TIA patients suffer from OSA phenotype recently associated with increased cardiovascular mortality. Therefore, optimal diagnostics and treatment require routine OSA screening in patients with acute cerebrovascular disease, even without previous suspicion of OSA.

Objective: We conducted an open-label cross-over study assessing the global effect of two high-frequency protocols of electric-field navigated repetitive transcranial magnetic stimulation (rTMS) targeted to functional facial motor cortex and comparing their efficacy and tolerability in patients with chronic facial pain. Outcome predictors were also assessed. Methods: We randomized twenty consecutive patients with chronic facial pain (post-traumatic trigeminal neuropathic pain, n=14; persistent idiopathic facial pain, n=4; secondary trigeminal neuralgia, n=2) to receive two distinct 5-day rTMS interventions (10Hz, 2400 pulses and 20Hz, 3600 pulses) separated by six weeks. The target area was assessed by mapping of lower face representation. The primary endpoint was the change in weekly mean of pain intensity (numeric rating scale, NRS) between the baseline and therapy week (1st week), and follow-up weeks (2nd and 3rd weeks) for each rTMS intervention. Response was defined using a combination scale including the patient’s global impression of change and continuance with maintenance treatment. Results: Overall, pain intensity NRS decreased from 7.4 at baseline to 5.9 ten weeks later, after the second rTMS intervention (p=0.009). The repetition of the treatment had a significant effect (F=4.983, p=0.043) indicating that the NRS scores are lower during the second four weeks period. Eight (40%) patients were responders, 4 (20%) exhibited a modest effect, 4 (20%) displayed no effect, and 4 (20%) experienced worsening of pain. High disability and high pain intensity (>7) predicted a better outcome (p=0.043 and p=0.045). Female gender, shorter duration of pain and low Beck Anxiety Inventory scores showed a trend towards a better outcome (p=0.052, 0.060 and 0.055, respectively). Conclusions: High-frequency rTMS targeted to face M1 alleviates treatment resistant chronic facial pain. Repeated treatment improves the analgesic effect. A protocol with higher frequency (above 10Hz), longer session duration (more than 20 minutes) and higher number of pulses (above 2400 pulses/session) did not improve the outcome. The results support early consideration of rTMS.

Background Vestibular schwannoma (VS) is a benign tumor originating from the vestibulocochlear nerve. The optimal treatment strategy is debated, since surgery may result in iatrogenic facial nerve injury. We report the results of VS surgery in a population-based unselected cohort in a center with access to Cyber Knife (CK) radiosurgery. Methods We reviewed 117 consecutive operations and found 95 patients who had their primary operation due to vestibular schwannoma between 2001 and 2017. Facial nerve function was evaluated with the House-Brackmann (HB) scale and hearing with the EU classification. Results The population consisted of 37 males and 58 females with a median age of 54 years (range 19–79). One year after surgery 67% of patients had a good outcome (HB 1–2). The rate of good outcome was 90% if no facial nerve damage was observed during intraoperative monitoring, the size of the tumor was under 30 mm and no hydrocephalus was present. During the study period, the treatment strategy changed from total to near-total resection after the introduction of CK radiosurgery, which could be used as a second-line treatment in case of residual tumor regrowth. This resulted in an improvement of outcomes (0% HB 5–6) despite the larger tumor sizes (25 ± 14 mm vs. 31 ± 9 mm, p

Purpose: To determine the accuracy of scoring masticatory muscle activity (MMA) events in seven different polysomnography (PSG) setups. Methods: Nineteen volunteers (13 females, 6 males, age 31.1 ± 12.9 years, 12 self-proclaimed bruxers) attended one-night PSG recording, supplemented with audio, video, and a separate frontal electroencephalography electrode set (FES). The same examiner scored the MMA events with seven different setups, with varying number of channels available: (1) one, (2) two, and (3) four EMG channels, (4) PSG without audio or video (PSG-N), (5) home PSG with FES and audio (FES-A), (6) PSG with audio (PSG-A), and (7) PSG with audio and video (PSG-AV). A subset (n = 10) of recordings was scored twice to determine intra-scorer reliability. MMA indices and accuracy of scoring the events in different setups were compared against PSG-AV. Results: The intra-class correlation coefficient (ICC) between PSG-AV and PSG-A was high (0.940, p 0.939 for all setups), with the exception of PSG-N (ICC = 0.764, p = 0.002). When comparing against the MMA events scored in PSG-AV, the sensitivity of MMA event recognition for PSG-A was 78.5% and specificity 95.5%, which were substantially higher than sensitivity (52.0%) and specificity (87.2%) of PSG-N. Conclusions: MMA event scoring accuracy with PSG-A or FES-A is almost comparable to PSG-AV. Since precise event recognition is essential for accurate MMA scoring, it is evident that one cannot rely exclusively on EMG.

Background Diagnostics of obstructive sleep apnea (OSA) is based on apnea-hypopnea index (AHI) determined as full-night average of occurred events. We investigate our hypothesis that intra-night variation in the frequency of obstructive events affects diagnostics and prognostics of OSA and should therefore be considered in clinical practice. Methods Polygraphic recordings of 1989 patients (mean follow-up 18.3 years) with suspected OSA were analyzed. Number and severity of individual obstructive events were calculated hourly for the first 6 h of sleep. OSA severity was determined based on the full-night AHI and AHI for the 2 h when the obstructive event frequency was highest (AHI2h). Hazard ratios for all-cause, cardiovascular, and non-cardiovascular mortalities were calculated for different OSA severity categories based on the full-night AHI and AHI2h. Results Frequency and duration of obstructive events varied hour-by-hour increasing towards morning. Using AHI2h led to a statistically significant rearrangement of patients between the OSA severity categories. The use of AHI2h for severity classification showed clearer relationship between the OSA severity and mortality than the full-night AHI. Conclusions Currently, the intra-night variation in frequency and severity of obstructive events is completely ignored by conventional, full-night AHI and considering this information could improve the diagnostics of OSA.

The aim of this neuropsychological study of a large cohort of patients with progressive myoclonus epilepsy type 1 (Unverricht-Lundborg disease, EPM1) was to characterize the cognitive function of EPM1 patients and to explore the association between the disability caused by the disease and cognitive performance.Sixty-eight genetically verified EPM1 patients homozygous for the expansion mutation in the CSTB gene (37 males and 31 females aged 35 ± 11) participated in a neuropsychological assessment of intellectual ability, verbal memory, and executive and psychomotor function. The clinical evaluation comprised administering (and video-recording) the unified myoclonus rating scale (UMRS) to assess the severity of each patient's myoclonus. Forty-six healthy volunteers (19 males and 27 females aged 32 ± 11) served as the control group for the neuropsychological tests.The cognitive performance of the EPM1 patient group was impaired. Verbal Intelligence Quotient (VIQ) was below the average range (VIQ 85) in 49% of the patients; further, Performance Intelligence Quotient (PIQ) was below average in 75% of the patients. The patients performed worse than the controls in both immediate and delayed story recall (p = 0.001); however, in the word list learning task, the patients performed only slightly worse than the controls. The one-hour delayed recall of the learned words was similar in both groups, and the percentage of retained words and story contents did not differ between the patients and controls. The patients were impaired in all of the executive function tests as well as in the psychomotor speed tests (p 0.001 for all). Also, the patients' simple psychomotor speed in the tapping task was significantly slowed in comparison to controls (p 0.001).The patients had impaired performance in the majority of the cognitive measures; they showed the highest level of impairment in all the executive function tests and in the psychomotor speed tests. The measures of these cognitive domains are timed-therefore, it is clear that severe myoclonus limits patients' performance. In contrast, verbal memory, especially delayed recall, was the least affected cognitive domain.

Background: The suggested association between severe obstructive sleep apnea (OSA) and risk of Alzheimer’s disease (AD) needs further study. Only few recent reports exist on associations between brain amyloid-β (Aβ) burden and severe OSA in middle-aged patients. Objective: Examine the possible presence of cortical Aβ accumulation in middle-aged patients with severe OSA. Methods: We performed detailed multimodal neuroimaging in 19 cognitive intact patients (mean 44.2 years) with severe OSA (Apnea-Hypopnea Index >30 h–1). Known etiological factors for possible Aβ accumulation were used as exclusion criteria. Aβ uptake was studied with [11C]-PiB-PET, glucose metabolism with [18F]-FDG-PET, and structural imaging with 3.0T MRI. Results: When analyzed individually, in [11C]-PiB-PET a substantial number (∼32%) of the patients exhibited statistically significant evidence of increased cortical Aβ uptake based on elevated regional Z-score values, mostly seen bilaterally in the precuneus and posterior cingulum regions. Cortical glucose hypometabolism in [18F]-FDG-PET was seen in two patients. MRI did not show structural changes suggestive of AD-related pathology. Conclusion: Increased [11C]-PiB uptake was seen in middle-aged cognitively intact patients with severe OSA. These findings are similar to those described in cognitive unimpaired older OSA patients. The changes in cortical Aβ uptake suggest that severe OSA itself may predispose to alterations related to AD already in middle-age. Aβ clearance may be compromised without simultaneous evidence of metabolic or structural alterations. The results emphasize the importance of early diagnostics and proper treatment of severe OSA in cognitively intact middle-aged subjects, possibly diminishing the individual risk for later cognitive dysfunction.

Current diagnostics of sleep apnea relies on the time-consuming manual analysis of complex sleep registrations, which is impractical for routine screening in hospitalized patients with a high probability for sleep apnea, e.g. those experiencing acute stroke or transient ischemic attacks (TIA). To overcome this shortcoming, we aimed to develop a convolutional neural network (CNN) capable of estimating the severity of sleep apnea in acute stroke and TIA patients based solely on the nocturnal oxygen saturation (SpO

BACKGROUND: Obesity is one of the most prominent risk factors for obstructive sleep apnea (OSA). Weight loss decreases the number of shorter respiratory events (

Unverricht-Lundborg disease (EPM1) is associated with progressive functional and anatomic changes in the thalamus and motor cortex. The neurophysiological mechanisms behind the impaired thalamocortical system were studied through short-term adaptation of the motor cortex to transcranial magnetic stimulation (TMS) via repetition suppression (RS) phenomenon. RS is considered to be related to neural processing of external stimuli. We hypothesized that this neural processing is progressively impaired in EPM1 from adolescence to adulthood. Eight adult patients with EPM1 (age: 40 ± 13 yr), six adolescent patients with EPM1 (age: 16 ± 1 yr), and ten adult controls (age: 35 ± 12 yr) were studied using navigated TMS and RS study protocol including trains of four repeated stimuli with intertrain interval of 20 s and interstimulus interval of 1 s. Changes in RS were investigated from adolescence to adulthood in EPM1 by comparing with adult controls. In controls, the RS was seen as 50–55% reduction in motor response amplitudes to TMS after the first stimulus. RS was mild or missing in EPM1. RS from first to second stimulus within the stimulus trains was significantly stronger in adolescent patients than in adult patients ( P = 0.046). Abnormal RS correlated with the myoclonus severity of the patients. In agreement with our hypothesis, neural processing of external stimuli is progressively impaired in EPM1 possibly due to anatomically impaired thalamocortical system or inhibitory tonus preventing sufficient adaptive reactiveness to stimuli. Our results suggest that RS abnormality might be used as a biomarker in the therapeutic trials for myoclonus. NEW & NOTEWORTHY Unverricht-Lundborg disease (EPM1) is associated with impaired thalamocortical function, which we studied in 8 adult and 6 adolescent patients and in 10 adult controls through repetition suppression (RS) of the motor cortex. We hypothesized that neural processing is progressively impaired in EPM1 from adolescence to adulthood. RS was normal in controls, whereas it was mild or missing in EPM1. Stronger RS was seen in adolescent patients than in adult patients correlating with the myoclonus severity.

Obstructive sleep apnea (OSA) increases cardiovascular morbidity and mortality. Little is known on acute peri-apneic hemodynamic alterations due to apneas. We assessed these rapid changes and how duration of apnea might contribute to them. Eight patients with severe OSA were studied with polysomnography including continuous blood pressure monitoring. Peri-apneic hemodynamic alterations, heart rate, blood pressure, stroke volume, cardiac output and peripheral resistance, were assessed in short (20s) and long (27s) apneas. Systolic and diastolic blood pressure along with heart rate elevated significantly in both apneas. These changes occurred within first 10 beats immediately after apnea. In contrast to short apneas long apneas caused sudden increase of 0.7l in cardiac output. Acute and pronounced peri-apneic hemodynamic alterations were seen during both short and long apneas. These described rapid hemodynamic changes might escape autoregulatory mechanisms of several organs, thus making OSA patients vulnerable to acute cardiovascular events.

Purpose Myoclonus in progressive myoclonus epilepsy type 1 (EPM1) patients shows marked variability, which presents a substantial challenge in devising treatment and conducting clinical trials. Consequently, fast and objective myoclonus quantification methods are needed. Methods Ten video-recorded unified myoclonus rating scale (UMRS) myoclonus with action tests were performed on EPM1 patients who were selected for the development and testing of the automatic myoclonus quantification method. Human pose and body movement analyses of the videos were used to identify body keypoints and further analyze movement smoothness and speed. The automatic myoclonus rating scale (ARMS) was developed. It included the jerk count during movement score and the log dimensionless jerk (LDLJ) score to evaluate changes in the smoothness of movement. Results The scores obtained with the automatic analyses showed moderate to strong significant correlation with the UMRS myoclonus with action scores. The jerk count of the primary keypoints and the LDLJ scores were effective in the evaluation of the myoclonic jerks during hand movements. They also correlated moderately to strongly with the total UMRS test panel scores (r2 = 0,77, P = 0,009 for the jerk count score and r2 = 0,88, P = 0,001 for the LDLJ score). The automatic analyses was weaker in quantification of the neck, trunk, and leg myoclonus. Conclusion Automatic quantification of myoclonic jerks using human pose and body movement analysis of patients’ videos is feasible and was found to be quite consistent with the accepted clinical gold standard quantification method. Based on the results of this study, the automatic analytical method should be further developed and validated to improve myoclonus severity follow-up for EPM1 patients.

Purpose In obstructive sleep apnea (OSA), severity of individual obstruction events is connected to increased mortality rate and it can be significantly different in patients with similar apnea-hypopnea index (AHI). However, possible gender-dependent variation in severity of obstruction events in different OSA categories is unknown. We investigated whether the severity of obstruction events differs between genders with similar AHI and if this difference varies between OSA categories. Methods Polygraphic recordings of 2057 consecutive patients with suspected OSA were reanalyzed and those with AHI ≥5/h were included for further analysis (nmale = 893 and nfemale = 197). Statistical significance of differences in AHI, apnea index, hypopnea index, oxygen desaturation index, obstruction severity parameter, and severity of individual obstruction events between genders were evaluated using the Mann-Whitney U (MWU) test as well as the general linear model (GLM) univariate analysis adjusted for age, BMI, smoking, daytime sleepiness, snoring, and heart failure. Results Apneas were 16.9 and 19.6% longer (MWU p ≤ 0.015, GLM p ≤ 0.036) and desaturation areas were 15.4 and 23.7% larger (MWU p ≤ 0.024, GLM p ≤ 0.053) in males compared to females with moderate and severe OSA, respectively. In contrast, hypopneas were 9.1% shorter (MWU p = 0.001, GLM p ≤ 0.001) and desaturation areas were 6.0% smaller (MWU p = 0.114, GLM p = 0.025) in men with mild OSA. The apnea index was 433.3 and 313.1% higher (MWU p ≤ 0.001, GLM p ≤ 0.043) and the hypopnea index was 12.2 and 17.8% lower (MWU p ≤ 0.001, GLM p = 0.002, p = 0.083) in males with mild and moderate OSA, respectively. Conclusion As severity of individual obstruction events was significantly different in males and females, the overall severity of OSA may not be similar despite the similarity in AHI.

Several studies have shown that, in spite of the fact that motor symptoms manifest late in the course of Alzheimer's disease (AD), neuropathological progression in the motor cortex parallels that in other brain areas generally considered more specific targets of the neurodegenerative process. It has been suggested that motor cortex excitability is enhanced in AD from the early stages, and that this is related to disease's severity and progression. To investigate the neurophysiological hallmarks of motor cortex functionality in early AD we combined transcranial magnetic stimulation (TMS) with electroencephalography (EEG). We demonstrated that in mild AD the sensorimotor system is hyperexcitable, despite the lack of clinically evident motor manifestations. This phenomenon causes a stronger response to stimulation in a specific time window, possibly due to locally acting reinforcing circuits, while network activity and connectivity is reduced. These changes could be interpreted as a compensatory mechanism allowing for the preservation of sensorimotor programming and execution over a long period of time, regardless of the disease's progression. Hum Brain Mapp 37:2083-2096, 2016. © 2016 Wiley Periodicals, Inc.

Positional obstructive sleep apnea (OSA) is common among OSA patients. In severe OSA, the obstruction events are longer in supine compared to nonsupine positions. Corresponding scientific information on mild and moderate OSA is lacking. We studied whether individual obstruction and desaturation event severity is increased in supine position in all OSA severity categories and whether the severity of individual events is linked to OSA severity categories. Polygraphic recordings of 2026 patients were retrospectively analyzed. The individual apnea, and hypopnea durations and desaturation event depth, duration, and area of 526 included patients were compared between supine and nonsupine positions in different OSA severity categories. Apnea events were 6.3%, 12.5%, and 11.1% longer (p<0.001) in supine compared to nonsupine position in mild, moderate, and severe OSA categories, respectively. In moderate and severe OSA categories desaturation areas were 5.7% and 25.5% larger (p<0.001) in supine position. In both positions the individual event severity was elevated along increasing OSA severity category (p<0.05). Supine position elevates apnea duration in all and desaturation area in moderate and severe OSA severity categories. This might be more hazardous for supine OSA patients and therefore, estimation of clinical severity of OSA should incorporate also information about individual event characteristics besides AHI.

Prevention of the recurrence of major depression and its residual symptoms requires effective treatment. Our aim was to study the effects of bifrontal active rTMS controlled by sham rTMS in treatment-resistant unipolar major depressive disorder (MDD).Thirty-seven patients with treatment-resistant MDD were randomized into two groups. One group received a total of 30 sessions of active bifrontal rTMS (10 Hz rTMS on left dorsolateral prefrontal cortex (DLPFC) and 1 Hz rTMS on right DLPFC) and the other group received bilateral sham rTMS on five days a week for six weeks.Depressive symptoms significantly improved in both the groups, but without a significant group difference. Furthermore, patients with psychotic depression improved similarly to those with moderate or severe depression.The results of present study indicate a large sham effect of stimulation treatment. The intensive structured treatment protocol may explain the positive outcome in both the groups. It is important to recognize, appreciate, and utilize placebo effects as a significant means of rehabilitation in psychiatric care.

Objective: In obstructive sleep apnea (OSA), breathing cessations are often followed by arousals, leading to sleep fragmentation and thus impaired sleep quality. Arousals and fragmented sleep are also related to detrimental cardiovascular events. The key index for OSA diagnosis (i.e. the apnea-hypopnea index) attributes equal diagnostic value to apneas and hypopneas, despite the fact that the associated arousals and desaturations may be very different. Thus, considering the severity of the consequences of apneas and hypopneas could enhance the estimation of OSA severity. In this study, we investigate whether the probability and duration of apnea- and hypopnea-related arousals differ and whether the differences in desaturation severity following apneas and hypopneas are dependent on sleep stage. Approach: Polysomnographic recordings of 348 consecutive OSA patients were included for analysis. The severity of arousals and desaturations associated with hypopneas within different sleep stages was compared to that of arousals and desaturations associated with apneas. In addition, the probability of arousals related to apneas and hypopneas was evaluated within OSA severity categories. Main results: Apneas caused arousals less frequently than hypopneas in N1, N2, and N3 sleep in all OSA severity categories. However, the arousals caused by apneas were longer (p < 0.001) and the desaturations related to apneas were more severe (p < 0.001) than those related to hypopneas in N1, N2, and rapid eye movement sleep even after adjustment for respiratory event durations. Significance: Desaturations and arousals related to apneas are more severe than those related to hypopneas. Therefore, apneas followed by arousal or desaturation should have a different diagnostic value than hypopneas when assessing OSA severity and related risk for cardiovascular consequences.

Obstructive sleep apnea (OSA) is a highly prevalent disease with severe health consequences. The severity of OSA is estimated with apnea-hypopnea-index (AHI). OSA is often treated with continuous positive airway pressure (CPAP). The aim of the current work was to create a numerical simulator showing benefits of different levels of usage of CPAP treatment. 226 male OSA patients were evaluated. CPAP treatment was simulated in 5 min intervals starting from the beginning of the night and continuing until the end. The cutoff point where AHI reached normal level of

Objective: To report the increasing frequency with which temporal anteroinferior encephalocele is a cause of adult temporal lobe epilepsy, to illustrate the clinical and imaging characteristics of this condition, and to report its surgical treatment in a series of 23 adult patients. Methods: Epilepsy patients diagnosed with temporal anteroinferior encephalocele from January 2006 to December 2013 in a national epilepsy reference center were included in this noninterventional study. Results: Twenty-three epilepsy patients (14 female, mean age 43.8 years) were diagnosed with temporal anteroinferior encephalocele in our institute. Thirteen patients had ≥2 encephaloceles; 7 cases presented bilaterally. The estimated frequency of this condition was 0.3% among MRI examinations performed due to newly diagnosed epilepsy (n = 6) and 1.9% among drug-resistant patients referred to our center (n = 17). Nine patients with local encephalocele disconnection (n = 4) or anterior temporal lobectomy and amygdalohippocampectomy (n = 5) have become seizure-free (Engel 1) for a mean 2.8 years (range 3 months–6.2 years) of follow-up. Three patients with local encephalocele disconnection were almost seizure-free or exhibited worthwhile improvement. Histologically, all 12 surgical patients had gliosis at the base of the encephalocele; some had cortical laminar disorganization (n = 5) or mild hippocampal degeneration (n = 1). Conclusions: The possibility of a temporal encephalocele should be considered when interpreting MRI examinations of patients with medically intractable focal epilepsy. These patients can significantly benefit from unitemporal epilepsy surgery, even in cases with bilateral encephaloceles.

Severity of obstructive sleep apnea (OSA) is estimated based on respiratory events per hour [i.e., apnea-hypopnea index (AHI)]. The aim of this study was to investigate effects of weight change on the severity of respiratory events. Respiratory event severity, including duration and morphology, was estimated by determining parameters quantifying obstruction and desaturation event lengths and areas, respectively. Respiratory events of 54 OSA patients treated with dietary intervention were evaluated at baseline and after 5-year follow-up in subgroups with different levels of weight change. AHI, oxygen desaturation index (ODI) and obstruction event severities decreased during weight loss. In lower level weight loss, the decrease was milder in obstruction severity than in AHI and ODI, indicating that the decrease in the number of events is more focused on less severe events. In weight gain groups, parameters incorporating obstruction event severity, AHI and ODI increased, although increase was greater in parameters incorporating obstruction event severity. The number and severity of respiratory events were modulated differently by the level of weight change. AHI misses this change in the severity of respiratory events. Therefore, parameters incorporating information on the respiratory event severities may bring additional information on the health effects obtained with dietary treatment of OSA.

Objective: This Finnish nationwide study aimed to refine the clinical phenotype variability and to identify factors that could explain the extensive variability in the clinical severity of the symptoms observed among patients with Unverricht-Lundborg disease (progressive myoclonus epilepsy type 1 [EPM1]) homozygous for the dodecamer expansion mutation in the cystatin B (CSTB) gene. Methods: The study population consisted of 66 (33 men and 33 women) patients with genetically confirmed EPM1 homozygous for the CSTB expansion mutation for whom the sizes of the expanded alleles were determined. The clinical evaluation included videorecorded Unified Myoclonus Rating Scale and retrospectively collected medical history. The navigated transcranial magnetic stimulation test was used to determine motor threshold (MT) and silent period (SP) of the motor cortex. Results: An earlier age at onset for EPM1 and longer disease duration were associated with more severe action myoclonus, lower performance IQ, increased MT, and prolonged SP. The number of dodecamer repeats in CSTB alleles varied between 38 and 77. On average, the size of the longer expanded alleles of patients was independently associated with MT, but exerted only a modulating effect on age at onset, myoclonus severity, and SP. Conclusions: As a group, earlier disease onset and longer duration are associated with more severe phenotype. Even though the vast majority of patients with EPM1 have a uniform genetic mutation, the actual size of the longer CSTB expansion mutation allele is likely to have a modulating effect on the age at disease onset, myoclonus severity, and cortical neurophysiology.

Obstructive sleep apnea (OSA) increases the risk of TIA and ischemic stroke, and it also worsens strokes rehabilitation outcomes. However, screening of OSA is not routinely performed in TIA or acute stroke. The aims were (i) to study the prevalence and severity of OSA with polygraphy (PG) in patients suffering from first TIA or acute ischemic stroke at university hospital acute stroke unit, and (ii) to evaluate the usability of fast forehead EEG electrode set for portable sleep studies in stroke units when full polysomnography (PSG) is not available. Fifty-four patients with TIA or mild to moderate ischemic stroke were evaluated. The patients (33M, 21F, mean age 66.3±10.1y; BMI 28.3±5.0 kg/m 2 ) were recruited from neurological ward at Kuopio University Hospital by experienced neurologists. All patients underwent an acute (1-4 days after TIA or stroke) in-hospital PG supplemented with a forehead EEG electrode set, previously shown to serve a reliable and simple solution to measure sleep. Based on standard PG data, OSA was diagnosed in 69% of the TIA/stroke patients (AHI›5). The prevalence of OSA was significantly higher in males (82%) than in females (48%). In 55% of male patients, OSA severity was at least moderate (AHI›15). The forehead EEG electrode set was easy to apply, and it provided high quality overnight recordings. Overall, PG combined with forehead EEG seemed to be well-suited for acute stroke unit settings. The vast majority of patients with first acute episode of TIA or ischemic stroke had evidence of undiagnosed OSA, thus urging for routine screening for OSA in stroke units. This is possible to perform accurately with portable PSG devices and simple forehead EEG electrode sets.

Summary Currently, definite diagnosis of sleep bruxism requires polysomnography. However, it is restrictedly available, and too cumbersome and expensive for the purpose. The aim of this study was to introduce an ambulatory electrode set and evaluate its feasibility for more cost-effective diagnostics of sleep bruxism. Six self-assessed bruxers (one male, five females; aged 21–58 years) and six healthy controls (four males, two females, aged 21–25 years) underwent a standard polysomnographic study and a concurrent study with the ambulatory electrode set. Bruxism events, cortical arousals and sleep stages were scored for the two montages separately in a random order, and obtained sleep parameters were compared. In addition, the significance of video recording and sleep stage scoring for the diagnostic accuracy of ambulatory electrode set was determined. Ambulatory electrode set yielded similar diagnoses as standard polysomnography in all subjects. However, compared with standard polysomnography the median (interquartile range) tonic bruxism event index was significantly higher in the control group [+0.38 (+0.08 to +0.56) events per hour, P = 0.046], and the phasic bruxism event index was significantly lower in the bruxer group [−0.44 (−1.30 to +0.07) events per hour, P = 0.046]. Exclusion of video recording and both video recording and sleep stage scoring from analysis increased overestimation of the tonic bruxism event index in the control group +0.86 (+0.42 to +1.03) and +1.19 (+0.55 to +1.39) events per hour, P = 0.046 and P = 0.028, respectively], resulting in one misdiagnosed control subject. To conclude, ambulatory electrode set is a sensitive method for ambulatory diagnostics of sleep bruxism, and video recording and sleep stage scoring help reaching the highest specificity of sleep bruxism diagnostics.

Background Age is a risk factor of obstructive sleep apnea (OSA). It has been shown that OSA progresses over time, although conflicting results have been reported. However, the effect of age on the severity of OSA and individual obstruction events has not been investigated within different OSA severity categories by taking the most prominent confounding factors (i.e., body mass index, gender, smoking, daytime sleepiness, snoring, hypertension, heart failure, and proportion of supine sleep) into account. Methods Polygraphic data of 1090 patients with apnea–hypopnea index (AHI) ≥5 were retrospectively reanalyzed. The effect of age on the severity of OSA and obstruction events was investigated in general, within different OSA severity categories, and in different age groups (age, final draft, peerReviewed

The sensorimotor cortical system undergoes structural and functional changes across its lifespan. Some of these changes are physiological and parallel the normal aging process, while others might represent pathophysiological mechanisms underlying neurodegenerative disorders. In the last years, the study of possible age-related modifications in brain sensorimotor functional characteristics has been the focus of several research projects. Here we have used the transcranial magnetic stimulation (TMS)-electroencephalography (EEG) navigated co-registration to investigate the influence of physiological aging on the excitability and connectivity of the human sensorimotor cortical system. To this end, we compared the TMS-evoked EEG potentials (TEPs) collected after stimulating the dominant primary motor cortex (M1) in healthy young subjects (mean age 24.5 years) with those collected in healthy older adults (mean age 67.6 years). We have shown that, after stimulation of the left motor cortex, TEPs are significantly affected by physiological aging. This phenomenon has a clear spatio-temporal specificity and we speculate that normal aging per se leads to some changes in the excitability of specific cortical neural assemblies whereas other alterations could reflect compensatory mechanisms to such changes., final draft, peerReviewed

It was recently demonstrated that the characteristics of EEG rhythms preceding a transcranial magnetic stimulation (TMS) of the motor cortex (M1) influence the motor-evoked potential (MEP) amplitude with a peculiar pattern, thus reflecting the M1 functional state. As physiological aging is related to a decrease in motor performance and changes in excitability and connectivity strength within cerebral sensorimotor circuits, we aimed to explore whether aging affects EEG-MEP interactions. Using MRI-navigated TMS and multichannel EEG, we compared the EEG-MEP interactions observed in healthy aged subjects with those observed in young volunteers. We divided the MEPs amplitude into two different subgroups consisting of "high" and "low" MEPs, based on the 50th percentile of their amplitude distribution. Then we analysed the characteristics of the pre-stimulus EEG from M1 and correlated areas separately for the "high" and "low" MEPs, comparing the two conditions. In both young and old subjects, significantly larger MEPs were evoked when the stimulated M1 was coupled in the beta-2 band with the homolateral prefrontal cortex. Conversely, only in young participants was the MEP size modulated when the M1 and homolateral parieto-occipital cortices were coupled in the delta band. The elderly didn't show this kind of pattern. Importantly, this coupling was significantly higher in elderly brains than in young brains, both for high and low MEPs. Our results suggest an age-related significant influence of time-varying coupling of spatially patterned EEG rhythms on motor cortex excitability in response to TMS.

Weight loss is an effective treatment for obstructive sleep apnea (OSA). The mechanisms of how weight loss affects nocturnal breathing are not fully understood. The severity of OSA is currently estimated by the number of respiratory events per hour of sleep (i.e. apnea-hypopnea-index, AHI). AHI neglects duration and morphology of individual respiratory events, which describe the severity of individual events. In the current paper, we investigate the novel Adjusted-AHI parameter (incorporating individual event severity) and AHI after weight loss in relation to sleeping position. It was hypothesised that there are positional differences in individual event severity changes during weight loss. Altogether, 32 successful (> 5% of weight) and 34 unsuccessful weight loss patients at baseline and after 1 year follow-up were analysed. The results revealed that individual respiratory event severity was reduced differently in supine and non-supine positions during weight loss. During weight loss, AHI was reduced by 54% (p = 0.004) and 74% (p < 0.001), while Adjusted-AHI was reduced by 14% (p = 0.454) and 48% (p = 0.003) in supine and non-supine positions, respectively. In conclusion, the severity of individual respiratory events decreased more in the non-supine position. The novel Adjusted-AHI parameter takes these changes into account and might therefore contribute additional information to the planning of treatment of OSA patients.

Thick-film technologies, such as screen printing, are becoming more popular in the manufacturing of bioelectrodes due to their simplicity, low cost, high reproducibility, and efficiency in large-scale production. Since electroencephalography (EEG) is a method for measuring potential differences in the microvolt scale, it is important to minimize all electromagnetic interferences. However, protecting screen-printed electrodes and their conduction traces from electromagnetic interferences has not been adequately investigated. We hypothesized that interference pick-up could be effectively reduced by an optimized silver or graphite shielded construction. The interference pick-up of the electrodes was technically evaluated in an electromagnetic compatibility laboratory and with EEG recordings of healthy volunteers. The grounding layer significantly (p

There is a need for an easy-to-use EEG electrode set allowing that EEG could be recorded immediately when an unconscious patient arrives at the emergency room. In addition, easy-to-use EEG electrode sets could be useful in many situations in intensive care unit, e.g. , for long-term monitoring of patients with status epilepticus. In this study, we developed a novel disposable screen-printed EEG electrode set, called here BrainStatus , which consists of 16 hydrogel-coated electrodes (12 recording, 2 reference and 2 ground electrodes) and evaluated its performance by signal quality measurements and EEG recordings in two patients. The quality of EEG signal was excellent even without abrading of skin and comparable to EEG recorded with conventional cup electrodes. The clinical referral question could be answered in both cases based solely on recordings with BrainStatus. BrainStatus was demonstrated to allow MRI and CT imaging without any signs of significant imaging artifacts. We consider that the introduced screen-printed EEG electrode set may be a solution for EEG registrations when the conventional 10–20 electrode setup is not available or feasible. However, further studies with a larger number of patients are warranted to clarify the true diagnostic accuracy and applicability of this approach.

Are words with form variations in the stem (e.g. foot ~ feet) perceptually processed in the same way as those without (e.g. table)? The majority of psycholinguistic literature concerning (ir)regular morphology concentrates on the processing or storage of inflected forms (e.g., feet vs. tables or thought vs. walked) in relation to their base forms. This article discusses the recognition of base forms of words in relation to their paradigmatic complexity (stem allomorphy). Do words like foot have some benefits in their recognition because of stem allomorphy, or do they rather cause an inhibition effect because of competition in form variation? Or could it be that there is no activation of the allomorph feet when we process the word foot, and thus no influence on the base form? With these and related questions in mind, we investigated the temporal dynamics of processing Finnish monomorphemic nouns with rich stem allomorphy versus nouns with no variation in stem by conducting an event-related potential (ERP) ...

Presently, the severity of obstructive sleep apnea (OSA) is estimated based on the apnea-hypopnea index (AHI). Unfortunately, AHI does not provide information on the severity of individual obstruction events. Previously, the severity of individual obstruction events has been suggested to be related to the outcome of the disease. In this study, we incorporate this information into AHI and test whether this novel approach would aid in discriminating patients with the highest risk. We hypothesize that the introduced adjusted AHI parameter provides a valuable supplement to AHI in the diagnosis of the severity of OSA. This hypothesis was tested by means of retrospective follow-up (mean ± sd follow-up time 198.2 ± 24.7 months) of 1,068 men originally referred to night polygraphy due to suspected OSA. After exclusion of the 264 patients using CPAP, the remaining 804 patients were divided into normal (AHI

Obstructive sleep apnea (OSA) is linked to an increased mortality rate. However, the severity of individual obstruction events is rarely considered quantitatively in clinical practice. We hypothesized that OSA with especially severe obstruction events would predispose a patient to greater health risks than OSA with a similar apnea-hypopnea index (AHI), but lower severity of individual events. This hypothesis was tested in a follow-up (198.2 ± 24.7 months) of a population of 1068 men referred for ambulatory polygraphic recording due to suspected OSA. The recordings were analysed according to the guidelines of the American Academy of Sleep Medicine. Furthermore, a novel obstruction severity parameter was determined; this was defined as the product of duration of the individual obstruction event and area of the related desaturation event. Patients treated with continuous positive airway pressure (CPAP) were omitted. We identified 125 deceased patients from our original population and for 113 of these a matching alive patient with similar AHI, age, body mass index (BMI), smoking habits and follow-up time could be found. The deceased patients with severe OSA (based on conventional AHI) showed higher obstruction severity values than their AHI-matched alive controls. Based on the multivariate logistic regression analysis, obstruction severity was the only parameter which was related statistically significantly to mortality in the severe OSA category. Furthermore, 59% of all deceased patients and 83% of those who had severe OSA displayed higher obstruction severity than the AHI-matched alive counterparts. To conclude, the obstruction severity parameter provided valuable prognostic information supplementing AHI. The obstruction severity parameter might improve recognition of the patients with the highest risk.