A retrospective study comparing preoperative evaluations and postoperative outcomes in paediatric and adult patients undergoing surgical resection for refractory epilepsy

PurposeTo review and compare the preoperative characteristics and postsurgical outcomes in paediatric and adult patients who underwent surgical resections from 2001 to 2009.MethodsCombined data from noninvasive measures such as ictal semiology, interictal/ictal scalp EEGs, MRI and SPECT were utilised to identify the epileptogenic zones (EZ). When noninvasive investigations produced inconclusive or inconsistent findings, patients underwent intracranial EEG monitoring. Resective micro-surgical procedures were conducted according to the results of the anatomo-electro-clinical investigations and were carried out to remove the EZ. We then followed up 222 paediatric (≤18 years old) and 100 adult patients (≥19 years old) for 1–9 years postoperatively.ResultsThe mean age of seizure onset in paediatric group was significantly lower than that in adult group. 95 (43%) of the paediatric and 42 (42%) of the adult patients required long-term intracranial EEG recording. 54 (24.3%) of the paediatric and 62 (62%) of the adult patients were found to have temporal lobe epilepsy (TLE), while 149 (67.1%) of the paediatric and 37 (37.0%) of the adult patients had extra-temporal lobe epilepsy (ETLE) (p = 0.000). 19 (8.6%) of the paediatric patients and 1 (1%) adult patient had hemispheric lesions (p = 0.009). 148 (66.7%) of the paediatric and 61 (61.0%) of the adult patients were seizure-free during the follow-up period. 17 of 19 (89.5%) children who underwent hemispherectomy were seizure-free. In both paediatric and adult groups, the surgical outcome for patients with TLE was significantly better than that of patients with ETLE (p = 0.018 in children, p = 0.029 in adults). Both the location of EZs and seizure-free ratio were significantly different (p < 0.001) between the preadolescent (≤12 years old) and adolescent (13–18 years old) group. Hippocampal sclerosis was the most common pathologic finding in patients with TLE in both groups, and was followed by focal cortical dysplasia. In patients with TLE, the proportion of tumour was significantly higher in the paediatric than the adult group (25.9% vs. 10%, p = 0.021).ConclusionPaediatric patients with refractory seizures had more extratemporal or hemispheric resectable epileptogenic foci and fewer temporal foci than adults. Our study demonstrates that resective surgery is an effective and safe early intervention in strictly selected paediatric patients with refractory epilepsy.     

On the prognostic value of ictal EEG patterns in temporal lobe epilepsy surgery: A cohort study

PurposeTo investigate the prognostic value of ictal scalp EEG patterns in drug-resistant temporal lobe epilepsy associated with hippocampal sclerosis (MTLE-HS) prior to undergoing temporal lobectomy.MethodsScalp EEGs of the first seizure recorded during presurgical long-term video-EEG monitoring of 284 patients were reviewed. Patients were divided according to seizure laterality as either unilateral, when the EEG was restricted to one cerebral hemisphere for the entire seizure, or bilateral, when there was involvement of both hemispheres during the seizure. In patients with unilateral hippocampal sclerosis (HS), seizures were subdivided according to the side of initial ictal activity in relation to the side of the HS, as concordant, non-lateralising or contralateral. Postsurgical seizure outcome, according to Engel's classification, was verified at 1, 2, and 5 years after surgery.ResultsThere was no significant association between ictal EEG characteristics and postsurgical seizure outcome. An Engel I seizure outcome was observed in 87.1% of the patients with unilateral ictal EEGs and in 79.6% of those with bilateral ictal EEGs (p = 0.092).ConclusionAnalysis of the localisation, morphology, and lateralisation of ictal EEG patterns did not provide prognostic information regarding seizure-free status in patients with MTLE-HS undergoing temporal lobectomy.     

Effects of seizure severity and seizure freedom on the health-related quality of life of an African population of people with epilepsy

PurposeThis study aimed at determining the effects of seizure severity and seizure freedom on health-related quality of life (HRQOL) of people with epilepsy (PWE) in the presence of perceived stigma in a sub-Saharan African culture.MethodsHealth-related quality of life was assessed using QOLIE-31 in 93 consecutive adults (56 males and 37 females) with epilepsy. They were stratified into seizure-free, low–moderate seizure severity, and high seizure severity groups based on the seizure type and the number of seizures in the previous 6 months. Other illness variables and sociodemographic variables were also obtained. A 3-item perceived stigma scale was administered. A modified QOLIE-31 (excluding the epilepsy-specific items) was given to 102 age- and sex-matched healthy controls.ResultsThere was moderate negative correlation between seizure severity and mean total HRQOL score as well as scores on the Seizure Worry (p = .000), Overall Quality of Life (p = .000), and Social Function (p = .001) subscales of QOLIE-31. Overall, the healthy control subjects had a higher mean HRQOL score compared with the PWE put together (71.0 + 11.1 vs 64.2 ± 13.6, p = .001). However, there was no difference in the mean HRQOL score between the seizure-free individuals and the healthy controls (p = .270). Seizure severity was associated with HRQOL independent of perceived stigma on a multiple regression analysis.ConclusionThis study provides evidence that seizure severity relates to health-related quality of life in an inverse, graded manner and independent of perceived stigma. Seizure-free people with epilepsy can have quality of life comparable with healthy individuals.     

Low resolution electromagnetic tomography analysis of ictal EEG patterns in mesial temporal lobe epilepsy with hippocampal sclerosis

ObjectiveTo investigate the difference in the spatial distribution of scalp initial ictal discharge (IID) patterns in mesial temporal lobe epilepsy with hippocampal sclerosis (HS–MTLE).MethodsScalp ictal EEG data in 22 seizure-free patients after temporal lobectomy with amygdalo-hippocampectomy were classified as follows: a regular 5–9 Hz rhythm with a restricted temporal/subtemporal distribution (type 1, 11 patients), or an irregular 2–5 Hz rhythm with a widespread fronto-temporal distribution (type 2, 11 patients). EEG data were fragmented into segments of 1.28 s, both at ictal onset and at baseline. The LORETA solution of three frequency bands was compared between ictal and baseline using statistical non-parametric mapping (p < 0.01).ResultsThe LORETA solution of 5–9 Hz in type 2 had wider cortical activity in the ipsilateral fronto-temporal area, compared to type 1 with activation of the ipsilateral focal mesial and lateral temporal regions. The LORETA solution of 10–13 Hz in both types showed increased activity in the fronto-temporal area, which was wider in type 2 than type 1. Increased cortical activity of <5 Hz was not observed in type 1, whereas increased cortical activity was observed in the bilateral anterior frontal area in type 2.ConclusionsThe cortical source distribution in HS–MTLE may depend on scalp IID frequency. The neural generators of 5–13 Hz may be important for the formation of the ictal onset zone in both ictal patterns.SignificanceSpatial distributions in HS–MTLE patients differ with scalp IID frequency.     

Vagus nerve stimulation: Outcome and predictors of seizure freedom in long-term follow-up

ObjectivesTo present long-term outcome and to identify predictors of seizure freedom after vagus nerve stimulation (VNS).MethodsAll patients who had undergone VNS implantation in the Epilepsy Centre Bethel were retrospectively reviewed. There were 144 patients who had undergone complete presurgical evaluation, including detailed clinical history, magnetic resonance imaging, and long-term video-EEG with ictal and interictal recordings. After implantation, all patients were examined at regular intervals of 4 weeks for 6–9 months. During this period the antiepileptic medication remained constant. All patients included in this study were followed up for a minimum of 2 years.ResultTen patients remained seizure-free for more than 1 year after VNS implantation (6.9%). Seizures improved in 89 patients (61.8%) but no changes were observed in 45 patients (31.3%). The following factors were significant in the univariate analysis: age at implantation, multifocal interictal epileptiform discharges, unilateral interictal epileptiform discharge, cortical dysgenesis, and psychomotor seizure. Stepwise multivariate analysis showed that unilateral interictal epileptiform discharges (IEDs), P = 0.014, HR = 0.112 (95% CIs, 0.019–0.642), cortical dysgenesis P = 0.007, HR = 0.065 (95% CIs, 0.009–0.481) and younger age at implantation P = 0.026, HR = 7.533 (95% CIs 1.28–44.50) were independent predictors of seizure freedom in the long-term follow-up.ConclusionVNS implantation may render patients with some forms of cortical dysgenesis (parietooccipital polymicrogyria, macrogyria) seizure-free. Patients with unilateral IEDs and earlier implantation achieved the most benefit from VNS.     

Detecting health disparities among Caucasians and African-Americans with epilepsy

ObjectiveThe aim of the study was to determine whether African-Americans and Caucasians who receive care at a tertiary epilepsy center can be distinguished on a variety of demographic, clinical, and psychosocial variables.MethodsWe surveyed 111 consecutive patients followed at a tertiary epilepsy center.ResultsOn univariate analysis, African-Americans had significantly more seizures (P = 0.03), lower scores on the Beliefs About Medicines Questionnaire—Specific (Necessity minus Concerns) (BMQ-S) (P = 0.01), and higher scores on the BMQ—General (BMQ-G) (P = 0.02). In binary logistic regression with race as the target variable, higher seizure frequency remained significantly associated with being African-American (P = 0.04). After ordinal regression with seizure frequency as the target variable, being African-American (P = 0.04) and higher BMQ-G scores (P = 0.02) remained significantly associated with increased seizure frequency.ConclusionCompared with Caucasians, African-Americans have higher seizure frequency and scores on the BMQ indicating a higher mistrust of medications. Aside from race, attitudes toward medications are also independently associated with seizure control.     

Seizure frequency in children with epilepsy: Factors influencing accuracy and parental awareness

RationaleThe objective of this study was to ascertain the accuracy of clinical reports to determine the seizure frequency in children diagnosed with epilepsy.MethodsWe reviewed the clinical record of 78 children (January–May of 2006) admitted to the EEG–video monitoring with epilepsy diagnosis. Clinical reports of parents and the files of EEG–video monitoring were reviewed to determine parents’ awareness for seizures.ResultsDuring video–EEG monitoring, 1244 were recorded on 78 children. Seizures were confirmed in 1095 of which 472 were correctly reported (38%) by parents whereas 623 remained under-reported (50%). Parents’ report thus had a sensitivity of 43%, positive predictive value of 76% to identify seizures. Based on the EEG–video monitoring, seizures were reported accurately in 22 (28%) and under-reported in 38 (49%) children. In the under-reported group, none of the seizures were recognized in 10 (13%), only a portion identified in 28 children. The parents’ report describing seizure frequency has limited value for young children (p = 0.01) and children with absence seizures (p = 0.03). However, clinical reports were accurate for the children with developmental delay (p < 0.06) or not being on any anticonvulsant drug (AED) therapy (p = 0.02).ConclusionOur results indicate that a significant number of seizures remain under-reported by parents of children with epilepsy. The current study underscores that the seizure frequency should be interpreted with caution for young children and children with absence seizures. Video–EEG recording has a complimentary role to the clinical observation for the accurate assessment of seizure frequency in children.     

Is elevated pre-ictal heart rate associated with secondary generalization in partial epilepsy?

BackgroundPeople with epilepsy are at risk for sudden unexpected death. Cardiac arrhythmia is one possible mechanism. We have studied seizure-related changes in cardiac rhythm.MethodsVideo-EEG and ECG from 38 patients with epileptic seizures during long-term monitoring for investigation of partial epilepsy with ictal impairment of consciousness were obtained. Seizures were classified as either complex partial or secondarily generalized. Inter-ictal, pre-ictal, ictal and post-ictal heart rate was calculated for the first recorded seizure.ResultsHeart rate during the pre-ictal period was higher (p = 0.016) in patients with secondarily generalized seizures (n = 11) compared to patients with complex partial seizures (n = 27). Heart rate was also elevated during and after generalized seizures (p < 0.015). Inter-ictal heart rate was not different in patients with secondary generalization compared to patients with partial seizures.ConclusionWe report elevated heart rate prior to partial seizure onset in those attacks which become secondarily generalized compared to seizures which remain localized. The finding may be relevant for the understanding of sudden death in epilepsy.     

Cortical excitability varies upon ictal onset patterns in neocortical epilepsy: A cortico-cortical evoked potential study

ObjectiveTo better understand pathological neuronal excitation in epilepsy by comparing cortico-cortical evoked potential (CCEP) responses in regions with different ictal onset patterns: focal paroxysmal fast (PF) and repetitive spiking (RS).MethodsFourteen patients undergoing invasive monitoring (six patients with PF and eight with RS) were studied with CCEPs. A repetitive 1 Hz bipolar electrical stimulus was applied to both the ictal onset region (iCCEP) and to a control region (nCCEP) and CCEPs were recorded from the surrounding electrodes. The two groups were compared by subtracting the amplitude of nCCEP from that of iCCEP (CCEP_{ictal?control}) at each stimulus intensity, and then normalizing the amplitudes of iCCEP at maximum stimulus intensity by dividing by nCCEP (CCEP_{ictal/control}).ResultsThe CCEP response to stimulation in the ictal onset region was significantly larger than to control stimulation for both ictal patterns (paroxysmal fast: P = 0.02, repetitive spiking: P < 0.01), with repetitive spiking group amplitudes higher than the paroxysmal fast group (CCEP_{ictal?control}: P < 0.01 and CCEP_{ictal/control}: P = 0.04).ConclusionsPro-epileptic excitability is more accentuated in regions showing an ictal repetitive spiking pattern than a paroxysmal fast pattern.SignificanceThese findings confirm in a new way that cortical excitability varies depending on the ictal onset pattern.     

The effect of vitamin B supplementation on homocysteine metabolism and clinical state of patients with chronic epilepsy treated with carbamazepine and valproic acid

PurposeTo investigate the influence of vitamin B supplementation on the plasma total homocysteine (p-tHcy), serum folate (s-FA), serum B12 (s-B12), and clinical state of patients with chronic epilepsy.MethodsBeck Depression Inventory (BDI) scores and p-tHcy, s-B12, and s-FA levels were assessed at baseline, after 1 year of supplementation (G1), and before and after 1 year of VPA or CBZ therapy (G2).ResultsEighty-one patients participated in the study: 51 patients with chronic epilepsy (G1) treated with carbamazepine (CBZ) or valproic acid (VPA), and 30 patients with newly diagnosed epilepsy (G2). At baseline, mean p-tHcy level was significantly higher in G1 than G2 (p = 0.0001) with no significant differences in s-FA or s-B12 levels. p-tHcy level significantly decreased in CBZ-treated G1 patients (p = 0.00002) after 1 year of supplementation and increased in G2 after 1 year of anti-epileptic drug (AED) therapy without supplementation. BDI scores in G1 decreased significantly after 1 year of supplementation (p = 0.0001) and increased significantly in VPA-treated G2 patients after 1 year of AED therapy (p = 0.02). The number of hyperhomocysteinemic patients significantly decreased in G1 after vitamin B supplementation (p = 0.01) and increased in G2 (p = 0.002). We also observed improved BDI scores and reduced seizure frequency in patients with chronic epilepsy.ConclusionsThese data support the hypothesis that AEDs play a major role in hyperhomocysteinemia development in patients with epilepsy. Adding folate and vitamin B12 to AED therapy is a safe and inexpensive way to reduce the risk of hyperhomocysteinemia.     

Electrode location and clinical outcome in hippocampal electrical stimulation for mesial temporal lobe epilepsy

PurposeTo study the clinical outcome in hippocampal deep brain stimulation (DBS) for the treatment of patients with refractory mesial temporal lobe epilepsy (MTLE) according to the electrode location.MethodsEight MTLE patients implanted in the hippocampus and stimulated with high-frequency DBS were included in this study. Five underwent invasive recordings with depth electrodes to localize ictal onset zone prior to chronic DBS. Position of the active contacts of the electrode was calculated on postoperative imaging. The distances to the ictal onset zone were measured as well as atlas-based hippocampus structures impacted by stimulation were identified. Both were correlated with seizure frequency reduction.ResultsThe distances between active electrode location and estimated ictal onset zone were 11 ± 4.3 or 9.1 ± 2.3 mm for patients with a >50% or <50% reduction in seizure frequency. In patients (N = 6) showing a >50% seizure frequency reduction, 100% had the active contacts located <3 mm from the subiculum (p < 0.05). The 2 non-responders patients were stimulated on contacts located >3 mm to the subiculum.ConclusionDecrease of epileptogenic activity induced by hippocampal DBS in refractory MTLE: (1) seems not directly associated with the vicinity of active electrode to the ictal focus determined by invasive recordings; (2) might be obtained through the neuromodulation of the subiculum.     

Cerebrospinal fluid tau as a marker of neuronal damage after epileptic seizure

PurposeWhether repeated brief seizures can cause neuronal damage is controversial. Cerebrospinal fluid (CSF) total tau (T-tau) and phosphorylated tau (P-tau) measurements have been suggested for the diagnosis of Alzheimer's disease, and T-tau may also be a marker of axonal damage and neuronal degeneration. We studied T-tau and P-tau levels and P-tau/T-tau ratio in CSF after epileptic seizures in order to determine whether they are increased after seizures.MethodsA total of 54 patients with tonic–clonic or partial secondarily generalized seizures due to various etiologies were studied and CSF obtained within 48 h after the seizure.ResultsThere were no statistical differences in the levels of T-tau (p = 0.09, ANOVA) or P-tau (p = 0.60) between different etiologic groups or controls. No patients with epilepsy of unknown origin had abnormal CSF T-tau whereas 11 patients with acute or remote symptomatic seizures had abnormal T-tau levels and the P-tau/T-tau ratio showed significant differences between the groups and controls (p = 0.003).ConclusionsEpileptic seizures with unknown etiology did not increase CSF tau levels. Abnormal tau levels were associated with either acute or remote symptomatic seizures with known etiology. The presence of elevated CSF tau increases the probability of symptomatic cause in a patient with a seizure.     

Circadian patterns of generalized tonic-clonic evolutions in pediatric epilepsy patients

ObjectiveTo investigate the sleep/wake, day/night, and 24-h periodicity of pediatric evolution to generalized tonic–clonic seizures (GTC).MethodsCharts of 407 consecutive patients aged 0–21 years undergoing continuous video-EEG monitoring for epilepsy were reviewed for the presence of GTC evolution. Seizures were characterized according to 2001 ILAE terminology. Charts were reviewed for EEG seizure localization, MRI lesion, and for seizure occurrence in 3-h time blocks, out of sleep or wakefulness, and during the day (6 AM–6 PM) or night. Analysis was done with binomial testing. Regression models were fitted using generalized estimating equations with patients as the cluster level variable.Results71 patients (32 girls, mean age 12.63 ± 5.3 years) had 223 seizures with GTC evolution. Sleep/wake seizure distribution predicted tonic–clonic evolution better than time of day, with more occurring during sleep (p < 0.001). Tonic–clonic evolution occurred most frequently between 12–3 AM and 6–9 AM (p < 0.05). Patients with generalized EEG onset had more tonic–clonic evolution between 9 AM and 12 PM (p < 0.05). Patients with extratemporal focal seizures were more likely to evolve during sleep (p < 0.001); this pattern was not found in patients with temporal or generalized seizure onset on EEG. Patients without MRI lesions were more likely to evolve between 12 AM and 3 AM (p < 0.05), in the sleeping state (p < 0.001), and at night (p < 0.05). Logistic regression revealed that sleep and older patient age were the most important predictors of GTC evolution.ConclusionGTC evolution occurs most frequently out of sleep and in older patients. Our results may assist in seizure prediction, individualized treatment patterns, and potentially complication and SUDEP prevention.     

Predictors of response to group cognitive-behavioral therapy in the treatment of obsessive-compulsive disorder

PurposeTo identify the presence of factors associated with treatment outcome in patients under group cognitive-behavioral therapy (GCBT) for obsessive-compulsive disorder (OCD).Subjects and methodsThis study evaluated 181 patients with OCD that attended a 12-session weekly GCBT program. Response criteria were: ≥35% reduction in Y-BOCS scores and global improvement score of the Clinical Global Impression (CGI) ≤ 2 at post-treatment evaluation. Sociodemographic data, OCD characteristics, and treatment data were studied.ResultsIn the bivariate analysis, the following variables showed statistical significance (p < 0.20) to enter the regression model: being woman (p = 0.074), greater insight (p = 0.017) and better quality of life (QOL) in all domains before treatment (p = 0.053), overall severity of disease according to the CGI (p = 0.007), number of associated comorbidities (p = 0.063), social phobia (p = 0.044), and dysthymia (p = 0.072). In the final regression model, these variables were associated with response to GCBT: female gender (p = 0.021); WHOQOL-BREF psychological domain (p = 0.011); insight (p = 0.042); and global improvement score of the CGI severity-scale before therapy (p = 0.045).ConclusionSpecial attention should be paid to patients with poor insight, increasing the cognitive aspects of the therapy in an attempt to modify the rigidity and fixity of their beliefs. In addition, male patients should be more observed, since they showed lower chance of response to GCBT when compared to women. Patients with more severe global symptoms (CGI) are poorer responders to GCBT, which indicates that not only obsessive-compulsive symptoms (OCS) should be evaluated, since other symptoms, such as depression and anxiety, may affect the treatment; therefore, an attempt to reduce these symptoms, prior to the treatment of OCD, should be considered as an option in some cases.     

Efficacy and tolerability of the first antiepileptic drug in children with newly diagnosed idiopathic epilepsy

PurposeLimited data are available for the effectiveness of the antiepileptic drugs in children in daily clinical practice. The aim of this study was to investigate the efficacy and tolerability of the first prescribed old and new antiepileptic drugs in children with newly diagnosed idiopathic epilepsy during a 12-month period.MethodA total of 289 children (141 females and 148 males) who received phenobarbital (n = 33), valproate (n = 142), carbamazepine (n = 42), oxcarbazepine (n = 38), or levetiracetam (n = 34) as the first-line treatment, were enrolled in the study. Seizure control and the occurrence of adverse events were assessed during a treatment period of 12 months.ResultsOverall, 245 (84.8%) patients remained seizure-free during the study period. The rate of seizure control did not differ significantly between the drug groups (p = 0.099). Forty-four (15.2%) patients including 1 (3.0%) treated with phenobarbital, 22 (15.5%) with valproate, 7 (16.7%) with carbamazepine, 10 (26.3%) with oxcarbazepine, and 4 (11.8%) with levetiracetam had treatment failure. There was no significant difference between seizure-free and failure groups in terms of age, gender, seizure type, and drugs used. Overall, 80 (27.7%) patients had adverse events, of those the most common ones were behavioral problems, nausea and/or vomiting, weight gain, and learning difficulties. The reasons for treatment failures were lack of seizure control in 29 (10.0%) patients and intolerable adverse events in 15 (5.2%) patients.ConclusionIt appears that old (phenobarbital, valproate and carbamazepine) and new antiepileptic drugs (oxcarbazepine and levetiracetam) have similar efficacy and tolerability profiles.Institutional ethic number is 28.3.2013/14.     

PLM detection by actigraphy compared to polysomnography: a validation and comparison of two actigraphs

ObjectiveTo compare periodic leg movement (PLM) counts obtained with polysomnography (PSG) to those obtained from actigraphy with two devices (Actiwatch and PAM-RL).MethodsTwenty-four patients underwent full night actigraphy with Actiwatch from both legs and simultaneous PSG. Out of these patients, 10 had additional actigraphy with PAM-RL. Bilateral and unilateral PLM indices (PLMI) for both actigraphs were calculated for time in bed and compared to polysomnographic PLMI. Additionally, a comparison between the two different actigraphs was performed.ResultsPLMI obtained with Actiwatch were significantly lower than those obtained with PSG (21.2 ± 25.6/h versus 34.4 ± 30.7/h; p < 0.001), whereas the PLMI from PAM-RL were significantly higher than in PSG (63.6 ± 39.3/h versus 37.0 ± 33.5/h; p = 0.009). In direct comparison, Actiwatch gave significantly lower PLMI than the PAM-RL (p = 0.005). The correlations between Actiwatch and PSG (rho = 0.835, p < 0.001), PAM-RL and PSG (rho = 0.939, p < 0.001), and Actiwatch and PAM-RL (rho = 0.915, p < 0.001) were significant. Unilateral actigraphy compared to standard PSG gave less consistent findings. When comparing different settings of the PAM-RL, manual threshold setting resulted in PLMI that were no longer different from PSG (p = 0.074), in contrast to the default threshold setting.ConclusionsThe Actiwatch underestimated and the PAM-RL overestimated PLMI compared to PSG. Whereas PLMI obtained with two actigraphs and PSG were highly correlated, they differed in mean values. Therefore, PSG, actigraphy and also the different actigraphs cannot be interchanged in longitudinal studies, and actigraphy should not be used for diagnostic decision making based on PLM indices. The best approximation to PSG PLMI was achieved by using manual threshold setting with the PAM-RL.     

Health-related quality of life in Portuguese patients with narcolepsy

ObjectiveTo evaluate the perception of health-related quality of life (HRQoL) in Portuguese patients with narcolepsy, and to compare the results to normative data.MethodsFifty-one narcoleptic adults (26M, 25F), aged between 18 and 80 years (mean = 43.35, SD = 15.32), were included in the final analysis of a multicentric cross-sectional study. The Medical Outcome Study – 36 Item Short-Form Survey (SF-36) was used to assess quality of life, and the Beck Depression Inventory (BDI) was used for self-assessment of depression.ResultsSeveral HRQoL domains were significantly lower than National surveys, except physical function and bodily pain (p between 0.000 and 0.006). SF-36 presented the lowest score in vitality (39.93). Deterioration was significantly higher in role physical (p = 0.006), vitality (p = 0.011), and mental health (p = 0.008) in women, and in physical function (p = 0.003) and bodily pain (p = 0.045) in elderly subjects. Those with higher literacy had better physical function (p = 0.046).ConclusionHRQoL is significantly deteriorated in narcoleptics, affecting all dimensions (except physical function and bodily pain) when compared with the general Portuguese population. The results are consistent with studies of narcolepsy in other countries in demonstrating the profound impact of this disorder on quality of life.     

Restless legs syndrome, sleep impairment, and fatigue in chronic obstructive pulmonary disease

ObjectiveTo investigate the frequency of factors associated with restless legs syndrome (RLS) in patients with chronic obstructive pulmonary disease (COPD).MethodsRLS diagnosis was investigated (International RLS Study Group, IRLSSG) and severity was assessed (IRLS rating scale) in 104 consecutive COPD patients (age 69.1 ± 8). Other measures were dyspnea severity (Modified Medical Research Council, MMRC), sleep quality (Pittsburgh Sleep Quality Index, PSQI), daytime somnolence (Epworth Sleepiness Scale, ESS), depressive symptoms (Beck Depression Inventory, BDI-II), and fatigue (Fatigue Severity Scale, FSS). Laboratory values included hemoglobin, ferritin, creatinine, and fibrinogen.ResultsThirty-two patients (30.8%) were diagnosed with RLS (65.6% women), which was moderate/severe (IRLS >11) in 26 (81.3%). RLS symptoms started after age 40 in most patients (93.3%). RLS patients had poorer sleep quality (PSQI >5 = 59.6%; p = 0.002), worse fatigue (FSS >27 = 51%; p = 0.005), and more depressive symptoms (BDI-II >10 = 14.4%; p = 0.005). Patients with RLS also presented more severe dyspnea (p = 0.009) and lower creatinine levels (p = 0.005). Overall, fatigue severity was correlated with older age (p = 0.001); level of dyspnea was positively correlated with PSQI and FSS (p < 0.005) and negatively correlated with ferritin (p = 0.03) and creatinine (p = 0.005), and PSQI scores correlated positively with FSS (p < 0.005) and negatively with ferritin (p = 0.005) and creatinine (p = 0.02). Quality of sleep was independently predicted by dyspnea severity and creatinine and fatigue by age and depression.ConclusionRLS is common in COPD. Patients with RLS have low creatinine, poorer quality of sleep, and more fatigue and depressive symptoms. RLS symptom severity is correlated to lower ferritin and severity of dyspnea.     

IgM-monoclonal gammopathy neuropathy and tremor: a first epidemiologic case control study

IntroductionSmall case series suggest tremor occurs frequently in IgM-monoclonal gammopathy of undetermined significance (IgM-MGUS) neuropathy. Epidemiologic study to confirm this association is lacking. Whether the neuropathy or another remote IgM-effect is causal remains unsettled.Materials and methodsAn IgM-MGUS neuropathy case cohort (n = 207) was compared to age, gender, and neuropathy impairment score (NIS) matched, other-cause neuropathy controls (n = 414). Tremor details were extracted from structured neurologic evaluation. All patients underwent nerve conductions.ResultsTremor occurrence was significantly higher in IgM-MGUS case cohort (29%) than in control cohort (9.2%) (p = 0.001). In IgM-MGUS cases, tremor was associated with worse NIS (p = 0.025) and demyelinating nerve conductions (p = 0.020), but 11 of 60 (18%) IgM-MGUS cases with tremor had axonal neuropathy. In other-cause neuropathy controls, tremor was associated with axonal nerve conductions (p = 0.03) but not with NIS severity (p = 0.57). Tremor occurrence associated with older age in controls, (p = 0.004) but not in IgM-MGUS cases (p = 0.272). Most IgM-MGUS tremor cases (49/60) had a postural-kinetic tremor, 8 had rest tremor, 3 had mixed rest-action. Alternative causes of tremor was identified in 42% of IgM-MGUS cases, the most common type is inherited essential tremor 6/60 (p = 0.04).ConclusionsThis first epidemiologic case-control study validates association between IgM-MGUS neuropathy and tremor. Among IgM-MGUS neuropathy cases, severity as well as type of neuropathy (demyelinating over axonal) correlated with tremor occurrence. IgM-MGUS paraproteinemia may increase tremor expression in persons recognized with common other risk factors for tremor.     

Emergent EEG in the emergency department in patients with altered mental states

ObjectiveTo evaluate whether EEG performed within 30 min of referral by an ED physician helps establish diagnosis and/or changes management and in which clinical setting.MethodsSingle-center prospective cohort intervention study 1 day/week, of sequentially referred adult patients with clinical seizures or altered mental status (AMS). Standard EEGs were performed by an EEG technician using a commercially available cap, interpreted by an epileptologist, immediately reported to the ED physician and a utility survey completed. Quality and interpretation of 20 min EEGs was compared to pre-specified 5 min segments of each EEG using the kappa coefficient.ResultsOver 1 year, 82 patients underwent ED EEG. Tonic clonic seizure activity had occurred in 33%. Mean time for EEG setup was 13.1 ± 6.2 min. EEG assisted the diagnosis in 51%, changed ED management in 4% and would be ordered again if EEG was available in 46%. Positive utility of EEG was significantly associated with toxicologic, psychiatric and endocrine/metabolic causes of AMS vs. other causes (p < 0.001) and sudden onset AMS (p = 0.007). Independent predictors of whether ED EEG would be ordered if available were witnessed seizures (p = 0.01), no prior head trauma (p = 0.001) and survey respondent being a physician assistant (vs. MD) (p = 0.02). The 5 (vs. 20) min EEG presented good agreement on waveform shape/amplitude (kappa = 0.78), artifact (kappa = 0.75) and interpretation categories (all kappa levels ?0.70).ConclusionsRapid availability of standard full-montage EEG in the ED is feasible and helps establish a diagnosis in about half of AMS patients, but rarely changes management. An abbreviated 5 min full-montage EEG presents adequate reliability which may improve use in the ED.SignificanceSpecific presentations of AMS offer the best diagnostic benefit for EEG in the ED.