Clinical management and survival of patients with central nervous system hemangiopericytoma in the National Cancer Database

Purpose/objectivesHemangiopericytomas are rare central nervous system (CNS) tumors. We sought to investigate existing clinical management strategies and overall survival (OS) among patients with hemangiopericytomas of the CNS.Methods/materialsAll patients diagnosed with CNS hemangiopericytoma from 2004 to 2014 in the National Cancer Database were included. Clinical and treatment-related characteristics were analyzed for an association with OS following diagnosis using univariable and multivariable analyses.ResultsNine-hundred and eighty-one patients were included (0.22% of all CNS tumors). At diagnosis, 22 patients had spinal tumors (2%), 21 patients had multifocal tumors (2%) 28 had disseminated disease (3%), and the remainder were unifocal intracranial tumors. Patients either underwent surgical resection and radiation (48%), surgery alone (37%), radiation alone (6%), or biopsy alone (9%). Of patients with known extent of resection, 53% underwent gross total resection, and, of patients with known radiation modality, 15% received stereotactic radiosurgery. Among the total cohort, 3 and 10 year OS was 87% and 59%, respectively. On multivariable analysis, factors associated with inferior OS included age (HR = 1.05, p < 0.001), WHO grade (p < 0.001), multifocal disease (HR = 2.59, p = 0.04), disseminated disease (HR = 2.67, p = 0.01), and chemotherapy (HR = 2.66, p = 0.01). Patients receiving surgery alone or surgery and radiation demonstrated improved OS compared to biopsy alone (HR = 0.45, p = 0.01 and HR = 0.47, p = 0.02, respectively). However radiation utilization did not impact OS (p = 0.691).ConclusionsThe present data provide large-scale prognostic information from a contemporary cohort of patients with hemangiopericytoma and support an initial attempt at surgical extirpation. The benefits of ionizing radiation are likely limited to improved local control and neurologic function.     

Assessment of long-term cognitive dysfunction in older patients who undergo heart surgery

IntroductionOlder patients are more likely to have cognitive dysfunction, and a great proportion of patients undergone surgical procedures are older adults. Postoperative cognitive dysfunction (POCD) has been shown as a consistent complication after major surgical procedures such as heart surgery.AimTo determine the presence of long-term POCD in ≥65-year-old patients undergoing coronary artery bypass grafting and aortic valve replacement, and to establish related risk factors.MethodsWe prospectively and sequentially included 44 patients with coronary disease and aortic stenosis scheduled for heart surgery. Follow-up of all patients was standardized and a neurocognitive evaluation were performed preoperatively and at 1, 6 and 12 months after surgery.ResultsPatients experienced a significantly postoperative cognitive dysfunction (33.5%, 63.4% and 38.9% at 1, 6 and 12 months, respectively) from baseline (20.5%). Patient-associated aspects such as age (p < 0.01), history of smoking (p < 0.01), arterial hypertension (p = 0.022), diabetes mellitus (p = 0.024), heart failure (p = 0.036) and preoperative cognitive dysfunction (p < 0.01), and surgery-associated aspects such as EuroSCORE (p < 0.01) and operation time (p < 0.01) were identified as related risk factors.ConclusionsOlder patients who underwent heart surgery had long-term POCD. Both patient- and surgery-related risk factors were established as related risk factors. These findings suggest that the prevalence of cognitive dysfunction after cardiac surgery in older patients could be related to a possible progression to dementia. In addition, many of the risk factors identified may be modifiable but in practice, these patients are not attended to for their possible cognitive impairment.     

The executive profile of children with Benign Epilepsy of Childhood with Centrotemporal Spikes and Temporal Lobe Epilepsy

RationaleBenign Epilepsy of Childhood with Centrotemporal Spikes (BECTS) and temporal lobe epilepsy (TLE) represent two distinct models of focal epilepsy of childhood. In both, there is evidence of executive dysfunction. The purpose of the present study was to identify particular deficits in the executive function that would distinguish children with BECTS from children with TLE.MethodsWe prospectively evaluated 19 consecutive children and adolescents with TLE with hippocampal sclerosis (HS) (57.9% male; mean 11.74 years [SD 2.05]; mean IQ 95.21 [SD 15.09]), 19 with BECTS (36.8% male; mean 10.95 years [SD 2.33]; mean IQ 107.40 [SD 16.01]), and 21 age and gender-matched controls (33.3% male; mean 11.86 years [SD 2.25]; mean IQ 108.67 [15.05]). All participants underwent a neuropsychological assessment with a comprehensive battery for executive and attentional functions. We used ANOVA and chi-square to evaluate differences on demographic aspects among groups (BECTS, TLE-HS, and control groups). Group comparisons on continuous variables were complemented by MANOVA and Bonferroni posthoc comparisons.ResultsPatients with BECTS had worse performance than controls in: Matching Familiar Figures Test, time (p = 0.001); Matching Familiar Figures Test, time × errors index (p < 0.001); Verbal Fluency for foods (p = 0.038); Trail Making Test, part B time (p = 0.030); Trail Making Test, part B number of errors (p = 0.030); and WCST, number of categories achieved (p = 0.043). Patients with BECTS had worse performance than patients with TLE-HS on Matching Familiar Figures Test, time (p = 0.004), and Matching Familiar Figures Test, time × errors index (p < 0.001). Patients with TLE-HS had worse performance than controls on the following tests: Verbal Fluency for foods (p = 0.004); Wisconsin Card Sorting Test, the number of categories achieved (p < 0.001); and Wisconsin Card Sorting Test, the number of perseverative errors (p = 0.028). Patients with TLE-HS had worse performance than patients with BECTS on Digit Backward (p = 0.002); and the Wisconsin Card Sorting Test, the number of perseverative errors (p < 0.001).ConclusionsPatients with TLE and BECTS present distinct cognitive profiles. Patients with TLE-HS had worse performance in mental flexibility, concept formation, and working memory compared to BECTS. Patients with BECTS had worse inhibitory control compared to children with TLE-HS. Both TLE-HS and BECTS had a higher number of errors on an inhibitory control test. However, patients with BECTS had a slower mental processing even when compared to patients with TLE-HS. Rehabilitation programs for children with epilepsy must include children with benign epilepsies and must take into account the epileptic syndrome and its particular neurocognitive phenotype.     

In search of optimal lithium levels and olanzapine doses in the long-term treatment of bipolar I disorder. A post-hoc analysis of the maintenance study by Tohen et al. 2005

PurposeThe aim of this study was to investigate whether lower lithium levels (LoLi) or olanzapine doses (LoOL) are risk factors for future mood episodes in patients with bipolar I disorder.MethodsA post-hoc analysis of the olanzapine-lithium-maintenance study [31] was performed using proportional hazards Cox regression models and marginal structural models (MSMs), adjusting for non-random assignments of dose during treatment.ResultsThe LoLi group (< 0.6 mmol/L) had a significantly increased risk of manic/mixed (hazard ratio [HR] = 1.96, p = 0.042), but not depressive (HR = 2.11, p = 0.272) episodes, compared to the combined medium (0.6–0.79 mmol/L) and high lithium level (≥ 0.8 mmol/L) groups. There was no significant difference in risk between the two higher lithium level groups (0.6-0.79 mmol/L; ≥ 0.8 mmol/L) for new manic/mixed (HR = 0.96, p = 0.893) or depressive (HR = 0.95, p = 0.922) episodes. The LoOL group (< 10 mg/day) showed a significantly increased risk of depressive (HR = 2.24, p = 0.025) episodes compared to the higher olanzapine (HiOL) dose group (HiOL: 10–20 mg/day), while there was no statistically significant difference in risk for manic/mixed episodes between the two groups (HR = 0.94, p = 0.895).ConclusionLithium levels ≥ 0.6 mmol/L and olanzapine doses ≥ 10 mg/day may be necessary for optimal protection against manic/mixed or depressive episodes, respectively in patients with bipolar I disorder.     

Correlation between cognitive function and the association fibers in patients with Alzheimer’s disease using diffusion tensor imaging

White matter (WM) changes, along with well-characterized cortical abnormalities, occur in patients with Alzheimer’s disease (AD). We investigated the integrity of WM tracts within association fibers by the use of fractional anisotropy (FA), and the relationship between FA values and cognitive function in patients with AD. Neuropsychological examination and conventional MRI, as well as diffusion tensor imaging, (DTI) were conducted on 12 patients with mild to moderate AD and 18 cognitively healthy volunteers. DTI was performed to measure FA in the bilateral inferior fronto-occipital fasciculus (IFOF) and the superior longitudinal fasciculus (SLF). Mini-Mental State Examination (MMSE) scores and Montreal Cognitive Assessment (MoCA) values were used to evaluate cognitive function and the Clinical Dementia Rating (CDR) scale was used as a staging tool for dementia severity. FA measures were analyzed and correlated with neuropsychological data. No patient showed any WM tract abnormality on either T1-weighted or T2-weighted MRI. However, the FA values in the bilateral IFOF and SLF and the MoCA scores in patients with AD were significantly decreased (p < 0.05) compared to the controls. Furthermore, the decreased FA values in the SLF were positively correlated with cognitive function (MMSE scores – right: r = 0.672, p = 0.033, left: r = 0.919, p < 0.01; MoCA values – right: r = 0.747, p = 0.013, left: r = 0.679, p = 0.031). Our findings confirmed that the loss of integrity of microstructural WM connectivity has a role in the cognitive decline of patients with AD. The data also suggest that the FA values of the SLF may be used as a clinical marker of cognitive function.     

Assessment of sleep satisfaction in patients with dementia due to Alzheimer’s disease

Sleep length and architecture are potential markers of progressive cognitive impairment, while neuropsychiatric symptoms and APOE4− haplotypes have been associated with more sleep complaints in patients with dementia due to Alzheimer’s disease (AD). In this cross-sectional study, we sought to investigate which factors might be related to sleep satisfaction in patients with AD. A total of 217 consecutive patients with AD were assessed for demographic features, neuropsychiatric symptoms, cognitive decline, functional impairment for activities of daily living, caregiver burden, APOE haplotypes, self-reported sleep satisfaction and length of sleep. Statistical comparisons were conducted with significance at p < 0.05. Concerning sleep complaints, 179 patients (82.5%) reported satisfactory sleep, while 38 (17.5%) were unsatisfied, with no relation to age, sex, APOE haplotypes, obesity, education, marital status, alcohol consumption or smoking found. Length of sleep (p = 0.011) and behavioural symptoms (p = 0.009) had significant associations with sleep satisfaction. Length of sleep was positively correlated with apathy (p = 0.014) and scores on the Clock Drawing Test (p = 0.015), and inversely correlated with anxiety (p = 0.015) and independence for instrumental activities of daily living (p = 0.003). Patients who were treated with memantine (p = 0.02) or anti-psychotics (p < 0.01) had longer duration of sleep. In conclusion, behavioural symptoms had strong associations with sleep satisfaction, which is highly correlated with length of sleep in patients with AD. Functional independence, apathy, anxiety, use of memantine or anti-psychotics, and scores on the Clock Drawing Test were significantly associated with length of sleep in this sample.     

Validación argentino-chilena de la versión en español del test Addenbrooke's Cognitive Examination III para el diagnóstico de demencia

BackgroundThe Addenbrooke's Cognitive Examination III (ACE-III), an adaptation of the ACE cognitive screening test, has been demonstrated to have high sensitivity and specificity in detecting cognitive impairment in patients with dementia and other neurological and psychiatric disorders. Although the Spanish-language version of the ACE-III has already been validated in Spain, it is yet to be validated in Latin America. The aim of this study was to validate the ACE-III test in an Argentinean and Chilean population.MethodsACE-III was administered to 70 patients with Alzheimer disease, 31 patients with behavioural variant frontotemporal dementia, and a control group of 139 healthy volunteers. Participants were recruited at centres in both countries.ResultsThe Spanish-language version of ACE-III was found to have good internal consistency (Cronbach's alpha = 0.87). We found significant differences in total ACE-III scores between patients with Alzheimer disease and controls (p  <  .05) and between patients with Alzheimer disease and bvFTD (p  <  .05). With a cut-off point of 86, 98.6% of AD patients, 83.9% of behavioural variant frontotemporal dementia patients, and 84.2% of controls were correctly classified.ConclusionsThis study shows that the Spanish-language version of ACE-III continues to be an effective tool for detecting cognitive dysfunction in patients with dementia.     

Predictors and patterns of insomnia symptoms in OSA before and after PAP therapy

ObjectiveIdentify factors that predict improvement versus persistence of insomnia symptoms following treatment of obstructive sleep apnea (OSA) with positive airway pressure (PAP) therapy.MethodsArchival data from 68 PAP-treated sleep apnea patients aged 25–83 were analyzed using nonparametric tests and stepwise regression to assess the relationships between insomnia symptoms, multiple OSA variables, and PAP use over time.ResultsPretreatment insomnia symptom severity (ISS; b = −0.72, p < 0.001), PAP average use (b = −0.01, p = 0.01) and respiratory disturbance index (RDI; b = −0.02, p = 0.03) predict change in insomnia following PAP therapy. Forty-five percent (24/53) of the subjects with moderate to severe insomnia at pretreatment reported no/mild symptoms after PAP therapy and were considered improved. Improved subjects had lower pretreatment ISS (p < 0.001), higher RDI (p = 0.01), and higher average PAP use (p < 0.035) than subjects with persistent insomnia. Number of medications and comorbidities were similar between improved and persistent groups. New onset of insomnia symptoms occurred in 13% (2/15) of the patients with no/mild pretreatment insomnia.ConclusionsAlthough ISS declines following PAP treatment, 55% of OSA patients have persistent moderate to severe symptoms despite treatment. More severe OSA is linked to higher likelihood of insomnia improvement and the effect of PAP therapy on insomnia may be mediated by OSA severity. Persistent insomnia is unrelated to medication use or comorbidities and may represent an independent, self-sustaining disorder requiring targeted intervention.     

Association of early-onset dementia with activities of daily living (ADL) in middle-aged adults with intellectual disabilities: The caregiver's perspective

Few studies have investigated in detail which factors influence activities of daily living (ADL) in adults with intellectual disabilities (ID) comorbid with/without dementia conditions. The objective of the present study was to describe the relation between early onset dementia conditions and progressive loss of ADL capabilities and to examine the influence of dementia conditions and other possible factors toward ADL scores in adults with ID. This study was part of the “Healthy Aging Initiatives for Persons with an Intellectual Disability in Taiwan: A Social Ecological Approach” project. We analyzed data from 459 adults aged 45 years or older with an ID regarding their early onset symptoms of dementia and their ADL profile based on the perspective of the primary caregivers. Results show that a significant negative correlation was found between dementia score and ADL score in a Pearson's correlation test (r = −0.28, p < 0.001). The multiple linear regression model reported that factors of male gender (β = 4.187, p < 0.05), marital status (β = 4.79, p < 0.05), education level (primary: β = 5.544, p < 0.05; junior high or more: β = 8.147, p < 0.01), Down's syndrome (β = −9.290, p < 0.05), severe or profound disability level (β = −6.725, p < 0.05; β = −15.773, p < 0.001), comorbid condition (β = −4.853, p < 0.05) and dementia conditions (β = −9.245, p < 0.001) were variables that were able to significantly predict the ADL score (R² = 0.241) after controlling for age. Disability level and comorbidity can explain 10% of the ADL score variation, whereas dementia conditions can only explain 3% of the ADL score variation in the study. The present study highlights that future studies should scrutinize in detail the reasons for the low explanatory power of dementia for ADL, particularly in examining the appropriateness of the measurement scales for dementia and ADL in aging adults with ID.     

When is electrical cortical stimulation more likely to produce afterdischarges?

ObjectiveTo study when afterdischarges (ADs) are more likely to occur during cortical stimulation.MethodsWe examined 6250 electrical stimulation trials in 13 patients with subdural electrodes, studying whether AD occurrence during a trial was influenced by electrode pair stimulated or AD occurrence during the previous trial. In total 545 electrodes were stimulated, 119 frontal (pre-perirolandic), 289 perirolandic, 36 parietal (post-perirolandic), 95 temporal, and 6 occipital.ResultsWhen the same electrode pair was stimulated as the prior trial, 19% produced ADs compared to 5% of trials when a different electrodes pair was stimulated (p < 0.0001). When trials showed ADs, and the next trial stimulated the same electrode pair, ADs occurred in 46% of cases, compared to 13% of trials following trials without ADs (p < 0.0001). AD probability decreased with increased inter-trial interval length only when the prior trial was at the same electrode pair and had produced an AD (p = 0.001). AD probability increased with stimulation duration, whether the trial followed a trial with (p < 0.001) or without (p < 0.0001) an AD.ConclusionsADs were more likely to occur when an electrode pair showed ADs and was stimulated again, especially when stimulating after short inter-trial intervals or for longer duration.SignificanceWhen ADs occur, waiting about a minute before resuming stimulation might lessen the likelihood of AD recurrence.     

Meta-analysis of the endogenous N200 latency event-related potential subcomponent in patients with Alzheimer’s disease and mild cognitive impairment

ObjectivesThe N200 latency subcomponent has the potential to be an accurate neurophysiological marker of the cognitive deterioration seen in Alzheimer’s disease (AD) and mild cognitive impairment (MCI).MethodsStandard mean difference (SMD) estimates of the N200 latency subcomponent were compared in three treatment groups: patients with AD, patients with MCI, and an unrelated elderly control group.ResultsPatients with AD had significantly prolonged N200 latencies compared to the control group, pooled SMD: 0.866 (95% CI: 0.517 to 1.214, z = 4.87, p < 0.001). Patients with MCI had significantly prolonged N200 latencies compared to the control group, pooled SMD: 0.578 (95% CI: 0.213 to 0.943, z = 3.31, p = 0.002). When comparing patients with AD and MCI the N200 latencies were similar, pooled SMD: 0.096 (95% CI: −0.261 to 0.453, z = 0.53, p = 0.598).ConclusionThe abnormalities present in the N200 latency subcomponent validate previous research that N200 latency is an informative indicator of information-processing deterioration in patients with cognitive impairment.SignificanceClinically, measurements of N200 latency can be used as a risk assessment of elderly patients that may be progressing to mild cognitive impairment and/or Alzheimer’s disease.     

Leptomeningeal failure in patients with breast cancer receiving stereotactic radiosurgery for brain metastases

PurposePrior studies suggest a high incidence of leptomeningeal failure (LMF) in breast cancer metastatic to brain. This study examines breast cancer-specific variables affecting development of LMF and survival after Gamma-Knife Radiosurgery (GKS).MethodsBetween 2000–2010, 149 (breast) and 658 other-histology patients were treated with GKS. Hormone/HER2, age, local/distant brain failure, prior craniotomy, and prior whole-brain radiotherapy (WBRT) were assessed. Median follow-up was 54 months (range, 0–106). Serial MRI determined local and distant-brain failure and LMF. Statistical analysis with categorical/continuous data comparisons were done with Fisher’s-exact, Wilcoxon rank-sum, log-rank tests, and Cox-Proportional Hazard models.ResultsOf 149 patients, 21 (14%) developed LMF (median time of 11.9 months). None of the following predicted for LMF: Her2-status (HR = 0.49, p = 0.16), hormone-receptor status (HR = 1.15, p = 0.79), prior craniotomy (HR = 1.58, p = 0.42), prior WBRT (HR = 1.36, p = 0.55). Non-significant factors between patients that did (n = 21) and did not (n = 106) develop LMF included neurologic death (p = 0.34) and median survival (8.6 vs 14.2 months, respectively). Breast patients who had distant-failure after GKS (65/149; 43.6%) were more likely to later develop LMF (HR 4.2, p = 0.005); including 15/65 (23%) patients who had distant-failure and developed LMF. Median time-to-death for patients experiencing LMF was 6.1 months (IQR 3.4–7.8) from onset of LMF. Median survival from LMF to death was much longer in breast (6.1 months) than in other (1.7 months) histologiesConclusionBreast cancer patients had a longer survival after diagnosis of LMF versus other histologies. Neither ER/PR/HER2 status, nor prior surgery or prior WBRT predicted for development of LMF in breast patients.     

Impact of sleeping position on central sleep apnea/Cheyne–Stokes respiration in patients with heart failure

BackgroundThe present study determines the influence of sleeping position on central sleep apnea (CSA) in patients with heart failure (HF).MethodsThe apnea/hypopnea index (AHI) during different body positions while asleep was examined by cardiorespiratory polygraphy in 71 patients with HF (ejection fraction <45%).ResultsTwenty-five of the patients having predominantly CSA (central apnea index ?10/h) with a lower obstructive apnea index (<5/h) were assigned to groups with positional (lateral to supine ratio of AHI <50%, n = 12) or non-positional (ratio ?50%, n = 13) CSA. In the non-positional group the BNP level was higher, the ejection fraction was lower and the trans-tricuspid pressure gradient was higher than in the positional group. Multiple regression analysis revealed more advanced age (p = 0.006), log₁₀ BNP (p = 0.017) and lung-to-finger circulation time (p = 0.020) as independent factors of the degree of positional CSA. Intensive treatment for HF changed CSA from non-positional to positional in all eight patients tested. Single night of positional therapy reduced CSA (p < 0.05) and BNP level (p = 0.07) in seven positional patients.ConclusionAs cardiac dysfunction progresses, severity of CSA also increases and positional CSA becomes position-independent. Positional therapy could decrease CSA, thereby having a valuable effect on HF.     

Diaphragm ultrasound in the diagnosis of respiratory dysfunction in patients with amyotrophic lateral sclerosis

ObjectiveIn this study, we aimed to select the best diaphragm ultrasonography (DUS) parameter as an alternative index for the diagnosis of lung function impairment in amyotrophic lateral sclerosis (ALS).MethodsTwenty-nine patients with ALS and 15 healthy subjects were enrolled in the study. DUS, lung function tests, phrenic nerve conduction study and arterial blood gas analysis were performed.ResultsPatients with respiratory dysfunction had a significantly lower level of ΔTmax than those without (P = 0.039). Significant correlations (P < 0.05) were found between forced vital capacity (FVC) and Tdi-ins (r = 0.665, P < 0.0001) and ΔTmax (r = ?0.748, P < 0.0001) and Δins-exp (r = 0.627, P < 0.0001) and ΔTdi (r = 0.485, P < 0.0001). Receiver Operating Curves analysis demonstrated that ΔTmax (AUC = 0.76, P = 0.044) had a better overall accuracy for detection of respiratory dysfunction compared with Tdi-ins (AUC = 0.27, P = 0.067), Δins-exp (AUC = 0.312, P = 0.139), and ΔTdi (AUC = 0.38, P = 0.359).ConclusionΔTmax is the most valuable DUS index in the diagnosis of diaphragmatic dysfunction.SignificanceDUS can provide functional and structural information of diaphragm and help to diagnose diaphragmatic dysfunction in ALS.     

CYP46A1 variants influence Alzheimer's disease risk and brain cholesterol metabolism

BackgroundCholesterol 24S-hydroxylase (CYP46) catalyzes the conversion of cholesterol to 24S-hydroxycholesterol, the primary cerebral cholesterol elimination product. Only few gene variations in CYP46 gene (CYP46A1) have been investigated for their relevance as genetic risk factors of Alzheimer's disease (AD) and results are contradictory.MethodsWe performed a gene variability screening in CYP46A1 and investigated the effect of gene variants on the risk of AD and on CSF levels of cholesterol and 24S-hydroxycholesterol.ResultsTwo of the identified 16 SNPs in CYP46A1 influenced AD risk in our study (rs7157609: p = 0.016; rs4900442: p = 0.019). The interaction term of both SNPs was also associated with an increased risk of AD (p = 0.006). Haplotypes including both SNPs were calculated and haplotype G–C was identified to influence the risk of AD (p = 0.005). AD patients and non-demented controls, who were carriers of the G–C haplotype, presented with reduced CSF levels of 24S-hydroxycholesterol (p = 0.001) and cholesterol (p < 0.001).ConclusionOur results suggest that CYP46A1 gene variations might act as risk factor for AD via an influence on brain cholesterol metabolism.     

Interferon-beta and disability progression in relapsing-remitting multiple sclerosis

ObjectiveTo assess the impact of interferon (IFN)-beta treatment on the progression of unremitting disability in IFN-beta treated and untreated relapsing-remitting (RR) patients with multiple sclerosis (MS) using prospective cohort study.MethodsA cohort of 419 RRMS (236 IFN-beta-treated and 183 untreated) patients was followed for up to 7 years. Cox proportional hazards regression models adjusted for the number of relapses in the last year before first visit was used to assess the differences between the two groups for the three end points: secondary progression (SP), and sustained Expanded Disability Status Scale (EDSS) score 4 and 6. Time from disease onset was used as survival time variable.ResultsThe IFN-beta-treated group showed a highly significant reduction (hazard ratio [HR], 0.34, 95% confidence interval [CI] 0.19–0.61, p < 0.001) in the risk of SP when compared with untreated patients. There were significant differences in favor of the IFN-beta-treated group for the end point EDSS score of 4 (HR = 0.45, 95%CI 0.28–0.73, p = 0.001) and EDSS score of 6 (HR = 0.34, 95%CI 0.16–0.75, p = 0.007).ConclusionThis observational study further supports the notion that IFN-beta could have potential beneficial effect on disease progression in RRMS.     

Sleep and cardiometabolic function in obese adolescent girls with polycystic ovary syndrome

ObjectiveTo compare the polysomnography findings and cardiometabolic function among adolescent girls with polycystic ovary syndrome (PCOS) and matched female and male controls.MethodRetrospective chart review of electronic medical records of 28 girls with PCOS (age: 16.8 ± 1.9 years, body mass index (BMI) Z-score 2.4 ± 0.4), 28 control females (age: 17.1 ± 1.8, BMI Z-score 2.4 ± 0.3) and 28 control males (age: 16.6 ± 1.6, BMI Z-score 2.5 ± 0.5) in a tertiary care centre.ResultsThe prevalence of obstructive sleep apnoea (OSA) was higher in girls with PCOS compared to control females (16/28 (57%) vs. 4/28(14.3%), p < 0.01); however, it was comparable to that of the control males (16/28(57%) vs. 21/28(75%), p = 0.4). Girls with PCOS had a significantly higher prevalence of insulin resistance compared to control females and control males (20/28 (71.4%) vs. 9/22 (41.0%) (p = 0.04) vs. 8/23 (34.8%) (p = 0.01). Among girls with PCOS, those with OSA had significantly higher proportions of metabolic syndrome (MetS) (9/16 (56.3%) vs. 1/12 (8.3%) p = 0.03), higher insulin resistance (14/16 (87.5%) vs. 6/12 (50%), p = 0.04), elevated daytime systolic blood pressure (128.4 ± 12.8 vs. 115.6 ± 11.4, p < 0.01), lower high-density lipoprotein (HDL) (38.6 ± 8.7 vs. 49 ± 10.9, p = 0.01) and elevated triglycerides (TG) (149.7 ± 87.7 vs. 93.3 ± 25.8, p = 0.03) compared to those without OSA.ConclusionsWe report a higher prevalence of OSA and metabolic dysfunction in a selected group of obese girls with PCOS referred with sleep-related complaints compared to BMI-matched control girls without PCOS. We also report higher prevalence of cardiometabolic dysfunction in girls with PCOS and OSA compared to girls with PCOS without OSA.     

Retinal nerve fiber thickness is reduced in sleep apnea syndrome

ObjectiveTo investigate the prevalence of glaucoma, visual field abnormalities, as well as changes in retinal nerve fiber layer in patients with obstructive sleep apnea syndrome (OSAS).MethodsIn this cross-sectional study, 51 patients with OSAS were included. Based on apnea hypopnea index (AHI), there were 26, 6 and 19 cases of severe (AHI ⩾ 30), moderate (15 ⩽ AHI < 30), and mild (5 ⩽ AHI < 15) OSAS, respectively. The control group was matched for age, sex and body mass index. Prevalence of glaucoma and ocular hypertension as well as the following values were assessed and compared between two groups: best-corrected visual acuity, intraocular pressure, central corneal thickness (CCT), cup:disk ratio, mean deviation (MD), pattern standard deviation, and retinal nerve fiber layer (RNFL) parameters using glaucoma diagnosis measurement (GDx).ResultsSeven eyes (6.7%) had intraocular pressure (IOP) > 21 mm Hg; of these, four eyes (3.9%) had glaucoma. No significant difference was detected in CCT between the two groups. IOP was significantly higher in the OSAS group before (p < 0.001) and after (p < 0.001) correcting for CCT. There was a significant difference between groups in MD and most GDx parameters including DISK (temporal–superior–nasal–inferior–temporal) average (p = 0.002), superior average (p = 0.05) and nerve fiber indicator (NFI) (p = 0.03), where those in the patient group showed lower values. There was a significant positive correlation between AHI and both IOP and NFI.ConclusionsOSAS patients had a higher prevalence of glaucoma and ocular hypertension. OSAS patients also had higher IOP, worse visual field indices, and lower RNFL parameters compared with the control group.     

Restless legs syndrome in end-stage renal disease: Clinical characteristics and associated comorbidities

BackgroundDespite being frequently described in patients with end-stage renal disease (ESRD), clinical characteristics and comorbidities in association with restless legs syndrome (RLS) are still to be confirmed.ObjectivesThe aim of this study was to investigate clinical factors associated with RLS in ESRD patients in hemodialysis.MethodsThis is a cross-sectional study of 400 patients on hemodialysis, evaluating RLS, clinical features and other sleep abnormalities.ResultsOut of 400, 86 patients presented RLS (21.5%; mean age 48.8 ± 13.8 y), being more frequent in females (p < 0.005). Forty-eight individuals (12% mean age 50.7 ± 13.1 y) had moderate/severe RLS, 14 reported symptoms prior to hemodialysis, 13 described family history of RLS, and eight described symptoms as disturbing during dialysis. RLS cases showed lower hemoglobin (p < 0.005), poorer quality of sleep (Pittsburgh Sleep Quality Index >5, p = 0.002), higher scores on the Beck Depression Inventory Scale (p < 0.005), greater scores on the Charlson Comorbidity Index (p = 0.01) and the Epworth Sleepiness Scale (p = 0.001) and higher risk of obstructive sleep apnea (OSA; Berlin questionnaire, p = 0.01). Hypertension was more frequent in cases with moderate/severe RLS (p = 0.01) and remained after controlling for the risk of OSA (p = 0.02).ConclusionIn ESRD patients in hemodialysis, RLS is present in 21.5%; 16% report symptoms prior to hemodialysis and a family history of RLS. Symptoms are disturbing during hemodialysis in 9% of cases. RLS is associated with lower hemoglobin, worse sleep quality, excessive daytime sleepiness, depressive symptoms and higher risk of OSA. Hypertension is associated with moderate/severe RLS.     

Severity of depressive symptomatology and functional impairment in children and adolescents with temporal lobe epilepsy

ObjectiveDepression is a frequent psychiatric disorder in children with temporal lobe epilepsy (TLE). However, severity of depressive symptoms (DS) is frequently neglected in these patients. This study aimed to determine severity of DS and global functioning by using quantitative measures and to establish their correlation with patients’ demographics and clinical variables.Methods31 children (mean age of 11.8 ± 2.3 years) with TLE were assessed with K-SADS-PL for axis I DSM-IV diagnosis. Severity of DS was measured by Children Depression Rating Scale-Revised – CDRS-R. Global functional impairment was evaluated with Child Global Assessment Scale-CGAS.Results25 patients (56% boys; 12 ± 2.3 years) had current DS, moderate or severe in 84% according to CDRS-R T-Score. Severity of DS was not correlated with age (p = 0.377), gender (p = 0.132), seizure control (p = 0.936), age of onset (p = 0.731), duration of epilepsy (p = 0.602) and the presence of hippocampal sclerosis (p = 0.614). Patients had moderate to major functional impairment measured by CGAS (48.7 ± 8.8), being adolescents more impaired than children (p = 0.03). Impairment of global functioning was not associated with epilepsy variables (p > 0.05).ConclusionChildren with TLE had moderate to severe DS early in the course of their disease with a relevant impact on their global functional activities, especially considering adolescents. Epilepsy severity seems not to be correlated to the severity of DS, contradicting the idea of a cause–consequence relationship. More systematic research is needed to better understand the association of depressive disorders in children and adolescents with TLE.