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Objective: Narcolepsy is a lifelong disease, manifesting with excessive daytime sleepiness and cataplexy, arising between childhood and young adulthood. The diagnosis is typically made after a long delay that burdens the disease severity. The aim of the project, promoted by the "Associazione Italiana Narcolettici e Ipersonni" is to develop Red Flags to detect symptoms for early referral, targeting non-sleep medicine specialists, general practitioners, and pediatricians.Materials and Methods: A multidisciplinary panel, including patients, public institutions, and representatives of national scientific societies of specialties possibly involved in the diagnostic process of suspected narcolepsy, was convened. The project was accomplished in three phases. Phase 1: Sleep experts shaped clinical pictures of narcolepsy in pediatric and adult patients. On the basis of these pictures, Red Flags were drafted. Phase 2: Representatives of the scientific societies and patients filled in a form to identify barriers to the diagnosis of narcolepsy. Phase 3: The panel produced suggestions for the implementation of Red Flags.Results: Red Flags were produced representing three clinical pictures of narcolepsy in pediatric patients ((1) usual sleep symptoms, (2) unusual sleep symptoms, (3) endocrinological signs) and two in adult patients ((1) usual sleep symptoms, (2) unusual sleep symptoms). Inadequate knowledge of symptoms at onset by medical doctors turned out to be the main reported barrier to diagnosis.Conclusions: This report will hopefully enhance knowledge and awareness of narcolepsy among non-specialists in sleep medicine in order to reduce the diagnostic delay that burdens patients in Italy. Similar initiatives could be promoted across Europe. [ABSTRACT FROM AUTHOR]

Epidemiological surveys on status epilepticus (SE) in adults in two Italian areas (Bologna and Lugo di Romagna) disclosed a major difference in 30-day case fatality (33% versus 7 %). Since suboptimal management was hypothesised in the first site, we compared the quality of treatment in the two cohorts and examined its contribution to prognosis. The Bologna and Lugo di Romagna cohorts of adults with incident SE were included. Patients with post-anoxic encephalopathy were excluded. Quality of treatment was independently classified by two experts. Clinical and treatment features were compared in the two sites. The contribution of variables collected to the 30-day case fatality was explored through multivariate logistic analysis in the whole group of patients. Fifty-seven patients were included. No differences were observed between Bologna and Lugo di Romagna either in clinical features or the time of management. The quality of global drug treatment significantly differed in disfavour of Bologna (p = 0.044). Independent predictors of a worse 30-day case fatality in the whole group of patients were the onset of SE in hospital (OR 9.67, p = 0.0095) and the poor global quality of treatment (partially correct versus correct OR 3.59, p = 0.55, and incorrect versus correct OR 21.09, p = 0.0084). By subgroup analysis, the site of onset factor encompasses the aetiologic background of patients. In addition to previously known prognostic factors, epidemiological comparison of mortality rates of SE between different regions must also consider the quality of treatment. [ABSTRACT FROM AUTHOR]

In 2003, the EFNS Task Force was set up for putting forth guidelines for the management of the Restless Legs Syndrome (RLS) and the Periodic Limb Movement Disorder (PLMD). After determining the objectives for management and the search strategy for primary and secondary RLS and for PLMD, a review of the scientific literature up to 2004 was performed for the drug classes and interventions employed in treatment (drugs acting on the adrenoreceptor, antiepileptic drugs, benzodiazepines/hypnotics, dopaminergic agents, opioids, other treatments). Previous guidelines were consulted. All trials were analysed according to class of evidence, and recommendations formed according to the 2004 EFNS criteria for rating. Dopaminergic agents came out as having the best evidence for efficacy in primary RLS. Reported adverse events were usually mild and reversible; augmentation was a feature with dopaminergic agents. No controlled trials were available for RLS in children and for RLS during pregnancy. The following level A recommendations can be offered: for primary RLS, cabergoline, gabapentin, pergolide, ropinirole, levodopa and rotigotine by transdermal delivery (the latter two for short-term use) are effective in relieving the symptoms. Transdermal oestradiol is ineffective for PLMD. [ABSTRACT FROM AUTHOR]

We performed a 2-year population-based study on status epilepticus (SE) in adults in the rural area of Lugo di Romagna, northern Italy, to verify whether an area of low-level urbanization has a lower risk of occurrence of SE (as recently suggested), different clinical features and short-term prognosis than areas of high-level urbanization. We found crude and age- and sex-adjusted annual incidence rates of SE of 16.5/100 000 and 11.6/100 000, respectively. In patients under 60 years crude incidence was 2.9/100 000 and in the elderly (≥60 years) 38.6/100 000. Acute symptomatic SE accounted for 30% and a cerebrovascular pathology was the most frequently associated etiologic condition (60%). A history of seizures was reported in 41% of patients. The first therapeutic intervention was mainly benzodiazepines (lorazepam 46%; diazepam 33%). The 30-day case fatality was 7%. We observed that the adult population of an area with a low level of urbanization has the same risk for SE, clinical features and short-term prognosis as European urban areas. The only contrasting result is the 30-day case fatality of 7% against the 39% found in the other Italian study (Bologna), despite the similarity of the SE features in these two areas of the same region. We infer that the short-term prognosis of SE could also be considerably influenced by differences in health service organization (and hence management) possibly due to different levels of urbanization. [ABSTRACT FROM AUTHOR]

The aim of the study was to determine the clinical frequency and features of REM sleep behaviour disorder (RBD) in a large population of Parkinson’s disease (PD) patients using defined diagnostic criteria both for RBD and PD. Six trained neurologists used a semistructured questionnaire based on ICSD-R diagnostic criteria for RBD to evaluate 200 PD patients and their caregivers. Interobserver reliability for the diagnosis of RBD was “substantial” (Kappa 0.65). Five patients were excluded from the study because of an MMSE lower than 25. The demographic and PD clinical features were compared in the clinically defined RBD group and in those without RBD (NRBD). Then the RBD features during the last year were analysed in the affected group. Out of 195 patients, 66 fulfilled the ICSD-R criteria for RBD; 62 patients reported RBD during the last year (frequency 31.8%). RBD features: two or more episodes per week in 35.5%; upper limb movements in 87%; lower limb movements in 79%; vocalisations during events in 85%. RBD onset was before PD in 27% of patients; 69% of the RBD group had injured themselves or their caregivers during sleep. According to multivariate analysis, RBD was associated with male gender, age and PD duration. Brief training and the use of a semistructured questionnaire may help the neurologist in dealing with sleep disturbances in PD patients. The search for RBD symptoms in PD is highly recommended, especially in patients with a long disease duration, the risk of sleep-related injuries being high. [ABSTRACT FROM AUTHOR]

We assessed the sensitivity and the positive predictive value (PPV) of the ICD-9 codes in identifying ischemic strokes. The study involved the cross-sectional comparison between patients with an ischemic stroke diagnosis made by neurologists and patients with the 434 or 436 discharge codes. Sensitivity of the codes (all diagnostic levels and first level respectively) was 82% and 76%; PPV: 71% and 76%. The annual crude incidence of ischemic stroke was 2.62 per 1000 based on verified strokes and 3.03 per 1000 based on 434 or 436 coded medical records (at all diagnostic levels). Thirty-day case fatality ratio was 22.3% in verified strokes and 36.8% among patients diagnosed with codes 434 or 436 but without stroke (all levels). Our results disclosed inaccuracy in use of the ICD-9 codes in the diagnosis of ischemic stroke in the general hospital of Lugo di Romagna, Ravenna Province, Italy. The misdiagnosis of patients could be influenced by the degree of severity of clinical features. Epidemiological data and cost-analysis forecasts based only on the ICD-9 system must be considered with caution. [ABSTRACT FROM AUTHOR]

We assessed the validity of an Italian language version of the Epworth sleepiness scale (ESS). The translated ESS was compared to the multiple sleep latency test (MSLT), considered the gold standard for the diagnosis of excessive daytime sleepiness (EDS). Within the context of a multicentric national study on narcolepsy (Gruppo Italiano Narcolessia Studio Epidemiologico Nazionale, GINSEN) involving 17 Italian sleep centres, we compared the two diagnostic tests on 91 prospectively recruited subjects with suspected EDS (34 with narcolepsy, 16 with obstructive sleep apnea syndrome, 19 with idiopathic hypersomnia, and 22 with other sleep, neurologic or psychiatric disorders). ESS scores were inversely correlated with mean sleep latency values, as measured with MSLT (rho = -0.31, p<0.01). ESS cut-off scores with best sensitivity and specificity were 12 and 17. For the 5-min MSLT cut-off, sensitivity was 87% and 47% respectively; specificity 39% and 74%. For the 8-min MSLT cut-off, sensitivity was 84% and 49%; specificity 50% and 88%. The Italian version of the ESS is an easy-to-use form useful for preliminary screening of daytime sleepiness level in specialist settings. [ABSTRACT FROM AUTHOR]

Background: Sleep disorders are highly prevalent in the general population and may be linked in a bidirectional fashion to stroke, which is one of the leading causes of morbidity and mortality. Aim: Four major scientific societies established a task force of experts in neurology, stroke, respiratory medicine, sleep medicine and methodology to critically evaluate the evidence regarding potential links and the impact of therapy. Materials and methods: Thirteen research questions were evaluated in a systematic literature search using a stepwise hierarchical approach: first, systematic reviews and meta‐analyses; second, primary studies post‐dating the systematic reviews/meta‐analyses. A total of 445 studies were evaluated and 88 were included. Statements were generated regarding current evidence and clinical practice. Results: Severe obstructive sleep apnoea (OSA) doubles the risk for incident stroke, especially in young to middle‐aged patients. Continuous positive airway pressure (CPAP) may reduce stroke risk, especially in treatment‐compliant patients. The prevalence of OSA is high in stroke patients and can be assessed by polygraphy. Severe OSA is a risk factor for recurrence of stroke and may be associated with stroke mortality, whilst CPAP may improve stroke outcome. It is not clear if insomnia increases stroke risk, whilst the pharmacotherapy of insomnia may increase it. Periodic limb movements in sleep (PLMS), but not restless limb syndrome (RLS), may be associated with an increased risk of stroke. Preliminary data suggest a high frequency of post‐stroke insomnia and RLS and their association with a less favourable stroke outcome, whilst treatment data are scarce. Discussion/Conclusion: Overall, the evidence base is best for OSA relationship with stroke and supports active diagnosis and therapy. Research gaps remain especially regarding insomnia and RLS/PLMS relationships with stroke. [ABSTRACT FROM AUTHOR]

Assesses the existence of a correlation between cognitive performances and the Epworth Sleepiness Scale in a group of patients with cirrhosis. Description of the patients studied; Percentage of patients with minimal hepatic encelophaty; Sensitivity of the frontal lobe-related cognitive functions to sleepiness. [ABSTRACT FROM AUTHOR]

Background: We describe the development and clinical validation of a patient self-administered tool assessing the quality of multiple sclerosis diagnosis disclosure. Method: A multiple sclerosis expert panel generated questionnaire items from the Doctor's Interpersonal Skills Questionnaire, literature review, and interviews with neurology inpatients. The resulting 19-item Comunicazione medico-paziente nella Sclerosi Multipla (COSM) was pilot tested/debriefed on seven patients with multiple sclerosis and administered to 80 patients newly diagnosed with multiple sclerosis. The resulting revised 20-item version (COSM-R) was debriefed on five patients with multiple sclerosis, field tested/debriefed on multiple sclerosis patients, and field tested on 105 patients newly diagnosed with multiple sclerosis participating in a clinical trial on an information aid. The hypothesized monofactorial structure of COSM-R section 2 was tested on the latter two groups. Results: The questionnaire was well accepted. Scaling assumptions were satisfactory in terms of score distributions, item-total correlations and internal consistency. Factor analysis confirmed section 2's monofactorial structure, which was also test-retest reliable (intraclass correlation coefficient [ICC] 0.73; 95% CI 0.54-0.85). Section 1 had only fair test-retest reliability (ICC 0.45; 95% CI 0.12-0.69), and three items had 8-21% missed responses. Conclusions: COSM-R is a brief, easy-to-interpret MS-specific questionnaire for use as a health care indicator. [ABSTRACT FROM AUTHOR]

Background and purpose: Some epilepsy syndromes (sleep‐related epilepsies, SREs) have a strong link with sleep. Comorbid sleep disorders are common in patients with SRE and can exert a negative impact on seizure control and quality of life. Our purpose was to define the standard procedures for the diagnostic pathway of patients with possible SRE (scenario 1) and the general management of patients with SRE and comorbidity with sleep disorders (scenario 2). Methods: The project was conducted under the auspices of the European Academy of Neurology, the European Sleep Research Society and the International League Against Epilepsy Europe. The framework entailed the following phases: conception of the clinical scenarios; literature review; statements regarding the standard procedures. For the literature search a stepwise approach starting from systematic reviews to primary studies was applied. Published studies were identified from the National Library of Medicine's MEDLINE database and Cochrane Library. Results: Scenario 1: Despite a low quality of evidence, recommendations on anamnestic evaluation and tools for capturing the event at home or in the laboratory are provided for specific SREs. Scenario 2: Early diagnosis and treatment of sleep disorders (especially respiratory disorders) in patients with SRE are likely to be beneficial for seizure control. Conclusions: Definitive procedures for evaluating patients with SRE are lacking. Advice is provided that could be of help for standardizing and improving the diagnostic approach of specific SREs. The importance of identifying and treating specific sleep disorders for the management and outcome of patients with SRE is underlined. [ABSTRACT FROM AUTHOR]

Abstract: Objective: We investigated the prevalence of nocturnal smoking (NS) in patients with RLS. Methods: One hundred RLS patients living in Emilia-Romagna (Northern Italy) and 100 matched controls, randomly selected from the general population, underwent interviews for the presence of nocturnal smoking and for obsessive-compulsive traits, depression, excessive daytime sleepiness (EDS) and subjective sleep quality. Results: NS was more prevalent in RLS patients than controls (lifetime prevalence: 12% vs. 2%, P =0.012). Patients with NS had more frequently Sleep-Related Eating Disorders (SRED) than patients without NS (83.3% vs. 26.1%, P =0.0002). Pathological and borderline Maudsley Obsessive-Compulsive Inventory (MOCI) values as well as pathological values at the Beck Depression Inventory (BDI) increased from controls to RLS patients without NS to RLS patients with NS (P =0.005 and P =0.01, respectively). Conclusions: We demonstrate an increased prevalence of NS in patients with RLS, in many cases associated with increased SRED. NS may be associated with psychopathological traits in RLS and may be relevant in the management of RLS patients. [Copyright &y& Elsevier]

Background: Apart from the underlying cardiovascular (CV) risk associated with migraine, both triptans and ergotamines can induce vasoconstriction and potentially increase the risk of serious ischemic events. Because of the low frequency of such events in eligible patients, randomized controlled trials are not exhaustive to assess the drug-related CV risk. Observational studies are, therefore, an essential source of information to clarify this matter of concern. Aim: The aim of this study was to systematically review the available published observational studies investigating the risk of serious CV events in triptan or ergotamine users, as compared to unexposed migraineur controls. Methods: We systematically searched MEDLINE and EMBASE electronic databases for cohort or case-control studies up to December 1, 2013. Studies retrieved from CDSR, DARE and HTA databases of the Cochrane Library were used for snowballing. Studies investigating the risk of any CV outcome in patients with a migraine diagnosis and exposed to triptans or ergotamines were considered for inclusion. Selection of studies, data extraction, and risk of bias assessment were conducted independently by two reviewers. Pooled odds ratios (ORs) with 95% confidence interval (95% CI) were computed using a random-effects model for studies and outcomes judged eligible for quantitative data synthesis. Results: From a total of 3370 citations retrieved, after duplicate removal and screening, only four studies met the inclusion criteria (three nested case-control analyses and one retrospective cohort study). These studies investigated the risk of different CV outcomes associated with either the recency or the intensity of exposure to the studied drugs. As for the intensity of use, the pooled OR of serious ischemic events was 2.28 (95% CI 1.18-4.41; I2=0%) for ergotamine use (two studies), whereas for triptans (three studies) it was 0.86 (95% CI 0.52-1.43; l2=24.5%). Recent use of ergotamines was not significantly associated with any CV outcome (only one available study). Two studies investigated the risk of stroke related to recent triptan use: the first study reported an OR of 0.90 (0.64-1.26), and the second one suggested an increased risk of 2.51 (1.10-5.71). In this case, because of the high degree of heterogeneity, results were not pooled. Conclusions: To date, few comparative observational studies have investigated the CV safety of migraine-specific drugs in clinical practice. Evidence gathered here suggests that intense consumption of ergotamines may be associated with an increased risk of serious ischemic complications. As for triptans, available studies do not suggest strong CV safety issues, although no firm conclusions can be drawn. In particular, evidence on stroke risk is conflicting. However, if an increase of the absolute stroke risk in recently exposed patients does actually exist, it must be small. Overall, residual uncontrolled confounding factors reduce the confidence in the risk estimates collected from the included studies. Further investigations are needed to better define the risk for rare but serious CV events related to triptan and ergotamine use fortreatment of migraine. [ABSTRACT FROM AUTHOR]

Introduction: Sleep assessment in suspected sleep related violence (SRV) and sleep-related abnormal sexual behaviour (SRASB) cases is mandatory. When such behaviours are suspected or purported to have caused a criminal offence (i.e. assault, attempted murder, murder, sexual assaults) sleep experts asked to provide report/testimony relied on previous forensic cases as a valuable source of information. We aimed at analyzing reported forensic cases of SRV and SRASB to investigate if they provided information about a minimal set of medical-legal key elements. Materials and methods: Systematic review was performed searching MEDLINE and PSYCHINFO databases from January 1980 through December 2012. One expert in legal medicine and two experts in sleep medicine reviewed all reports to assess whether they provided information about 15 key elements grouped in four categories: (1) legal issues (charge, defense, verdict); (2) defendant and victim characteristics (sex, age, relationship); (3) circumstantial factors (timing of the event, proximity, psychophysical condition of the defendant at the time of the event); and (4) forensic evaluation (clinical sleep assessment, polysomnography (PSG), PSG findings, other medical evaluations). Results: Out of 624 retrieved references we included 35 reports (19 SRV and 16 SRASB), ranging from single case reports to case series up to summaries of legal cases. The number of provided key elements ranged from 4 to 14. The most frequently reported were the defendant’s sex and the relation with the victim (97%), while the less frequent were PSG (37%) and PSG findings (29%). In average, legal issues were provided in 84% of reports, defendant and victim characteristics in 74%, circumstantial factors in 77%, and forensic evaluation in 47%. Conclusion: Criminal trials involving SRV and SRASB are rare. In this light, exhaustive reporting is essential. This is the first attempt to analyze the information provided by published SRV/SRASB forensic cases. We disclosed that a minimal set of medical-legal key elements was never provided. While information about legal issue is frequently provided, the lack of key elements concerning defendant and victim characteristics, circumstantial factors and especially forensic evaluation weakens the forensic relevance and the scientific interest of many reports. Improving the quality of reports of forensic cases of SRV/SRABS would provide essential information for sleep medicine experts called for forensic evaluation and a homogeneous body of data for scientific research. [Copyright &y& Elsevier]

We determined the cerebrospinal fluid (CSF) and serum neuron-specific enolase (NSE) concentrations in 19 patients with acute benign headache. All patients had normal neurological examination, CSF and head computed tomography scan. The final diagnoses were: primary thunderclap headache ( n = 7), primary exertional headache ( n = 3), primary cough headache ( n = 1), migraine without aura ( n = 4), headache unspecified ( n = 2), probable infrequent episodic tension-type headache ( n = 1), headache attributed to hypertensive crisis without hypertensive encephalopathy ( n = 1). A group of 108 healthy subjects served as controls. CSF NSE concentration was 14.16 ng/ml [95% confidence interval (CI) 11.86, 16.47)] in the headache sample (controls 17.19 ng/ml, 95% CI 16.23, 18.15). Serum NSE concentration was 7.50 ng/ml (95% CI 5.20, 9.80) in the headache sample (controls 8.45 ng/ml, 95% CI 7.67, 9.23). CSF/serum ratio was 2.81 (95% CI 2.21, 3.40) in the headache sample (controls 2.23, 95% CI 2.03, 2.42). Acute benign headache is not associated with neuronal damage as estimated by means of CSF and serum NSE concentration. [ABSTRACT FROM AUTHOR]

Sleep-related eating disorder (SRED) is characterized by recurrent arousals from sleep associated with compulsive ingestion of food. No controlled therapeutic trials are available for SRED. We assessed the safety, tolerability and efficacy of pramipexole, a dopamine D3-receptor agonist, in the treatment of SRED. Eleven consecutive patients with SRED in the absence of concurrent daytime eating disorders underwent actigraphic recording and subjective sleep diary evaluation for a week before and every week for 2 weeks of treatment with pramipexole 0.18–0.36 mg or placebo, administered in a double-blind crossover randomized sequence. The primary outcomes of the trial were actigraphic measures of night sleep parameters (sleep efficiency, motor activity mean and median, number and duration of wake episodes), secondary outcomes were the number of good sleep nights/weekly, number and duration of nocturnal awakenings/night, and visual analogue preference score. Pramipexole was well tolerated without any patient withdrawing from the study. Pramipexole reduced night-time activity median (P = 0.02) and increased the number of nights of good sleep/week (P = 0.02). All other measurements remained unaffected. Pramipexole at low doses was well tolerated, improving some measures of sleep quality and reducing median night activity in SRED. Further studies with higher dosages and for longer time-periods are warranted. [ABSTRACT FROM AUTHOR]

The aim of this study was to determine cerebrospinal fluid (CSF) and serum neuron-specific enolase (NSE) concentrations in a normal population and to analyse their relationship with sex and age. The sample was recruited among patients undergoing spinal anaesthesia, without neurological diseases. NSE was determined by means of immunometric assay. One hundred and eight patients (68 men) were recruited. CSF-NSE concentration was (mean ± SD) 17.3 ± 4.6 ng/ml (men 17.4 ± 4.2, women 17.0 ± 5.2,P = 0.62); serum concentration was 8.7 ± 3.9 ng/ml (men 8.9 ± 3.9, women 8.3 ± 4.0,P = 0.06). The mean CSF/serum NSE ratio was 2.3 ± 0.8 (men 2.2 ± 0.8, women 2.4 ± 0.9,P = 0.22). In both sexes, simple regression analysis showed not significantly increasing concentrations with advancing age for both CSF and serum NSE. Serum and CSF concentrations did not correlate in both sexes. In our study, CSF-NSE was twice the serum concentration; both were not influenced by sex or age. Serum and CSF-NSE values vary widely among different studies on normal populations because of different determination methods; therefore, each laboratory should obtain its own reference values. Finally, serum NSE should be used with caution as an indicator of CSF concentration as no correlation could be demonstrated between them in our study. [ABSTRACT FROM AUTHOR]