SUMMAR Y In the absence of a golden standard for the diagnosis of narcolepsy, the clinical spectrum of disorder remains controversial. The aims of this study were (1) to determine frequency and characteristics of sleep-wake symptoms in patients with narcolepsy with cataplexy, (2) to compare clinical characteristics with results of ancillary tests, and (3) to identify factors that discriminate narcolepsy from other conditions with excessive daytime sleepiness (EDS). We prospectively studied 57 narcoleptics with cataplexy, 56 patients with non-narcoleptic hypersomnia (H), and 40 normal controls (No). Based on suggested and published criteria, we differentiated between narcoleptics with definite cataplexy (N) and narcoleptics without definite cataplexy (possible cataplexy, NpC). Assessment consisted of questionnaires [all patients and controls, including the Ullanlinna Narcolepsy Score (UNS)], polysomnography (all patients), multiple sleep latency test (MSLT) and human leukocyte antigen typing (in most narcoleptics). A new narcolepsy score based on five questions was developed. Data were compared with those of 12 hypocretindeficient narcoleptics (N-hd). There were significant differences between N and NpC (including mean sleep latency on MSLT), but none between N and N-hd. A score of sleep propensity during active situations (SPAS) and the frequency of sleep paralysis/ hallucinations at sleep onset, dreams of flying, and history of sleep shouting discriminated N from H and No (P < 0.001). Cataplexy-like symptoms in H (18%) and No (8%) could be discriminated from Ôtrue' cataplexy in N on the basis of topography of motor effects, triggering emotions and triggering situations (P < 0.001). Our narcolepsy score had a similar sensitivity (96% versus 98%) but a higher specificity (98% versus 56%) than the UNS. Analysis of co-occurring symptoms in narcolepsy revealed two symptom complexes: EDS, cataplexy, automatic behaviors; and sleep paralysis, hallucinations, parasomnias. Low/undetectable cerebrospinal fluid hypocretin-1 levels and a history of definite cataplexy identify similar subgroups of narcoleptics. Specific questions on severity of EDS (SPAS score) and characteristics of cataplexy allow the recognition of subgroups of narcoleptics and their differentiation from non-narcoleptic EDS patients, including those reporting cataplexy-like episodes. The existence of co-occurring symptoms supports the hypothesis of a distinct pathophysiology of single narcoleptic symptoms.k e y w o r d s cataplexy, diagnosis, Epworth sleepiness score, excessive daytime sleepiness, hypocretin-1, multiple sleep latency test, narcolepsy, parasomnias, spectrum

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Background Learning and learning-related neuroplasticity in motor cortex are potential mechanisms mediating recovery of movement abilities after stroke. These mechanisms depend on dopaminergic projections from midbrain that may encode reward information. Likewise, therapist experience confirms the role of feedback/reward for training efficacy after stroke. Objective To test the hypothesis that rehabilitative training can be enhanced by adding performance feedback and monetary rewards. Methods This multicentric, assessor-blinded, randomized controlled trial used the ArmeoSenso virtual reality rehabilitation system to train 37 first-ever subacute stroke patients in arm-reaching to moving targets. The rewarded group (n = 19) trained with performance feedback (gameplay) and contingent monetary reward. The control group (n = 18) used the same system without monetary reward and with graphically minimized performance feedback. Primary outcome was the change in the two-dimensional reaching space until the end of the intervention period. Secondary clinical assessments were performed at baseline, after 3 weeks of training (15 1-hour sessions), and at 3 month follow-up. Duration and intensity of the interventions as well as concomitant therapy were comparable between groups. Results The two-dimensional reaching space showed an overall improvement but no difference between groups. The rewarded group, however, showed significantly greater improvements from baseline in secondary outcomes assessing arm activity (Box and Block Test at post-training: 6.03±2.95, P = .046 and 3 months: 9.66±3.11, P = .003; Wolf Motor Function Test [Score] at 3 months: .63±.22, P = .007) and arm impairment (Fugl-Meyer Upper Extremity at 3 months: 8.22±3.11, P = .011). Conclusions Although neutral in its primary outcome, the trial signals a potential facilitating effect of reward on training-mediated improvement of arm paresis. Trial registration ClinicalTrials.gov (ID: NCT02257125).

Introduction: Narcolepsy is a chronic neurological sleep disorder, the diagnosis of which is based upon clinical evaluation and additional paraclinical assessments. Awareness of narcolepsy is low and screening tools needed. The Epworth Sleepiness Scale (ESS), the Ullanlinna Narcolepsy Scale (UNS), and more recently the Swiss Narcolepsy Scale (SNS) were used as screening tools for narcolepsy. The aims of the present study are (1) to assess the value of the SNS in a new series of patients with narcolepsy with cataplexy (NC), and (2) to compare its sensitivity and specificity with the ESS and UNS. Patients and Methods: We prospectively assessed by questionnaire patients with NC (n ¼ 80, all with assessment of cerebrospinal fluid (CSF) hypocretin-1 levels) and patients with excessive daytime sleepiness (EDS) of other origin (n ¼ 111) in Zurich and Leiden. Causes of EDS included idiopathic hypersomnia (n ¼ 12), behaviorally induced sleep insufficiency syndrome (n ¼ 32), restless legs syndrome (n ¼ 7), sleep-disordered breathing (n ¼ 22), and hypersomnia due to medical disorders (n ¼ 9). Diagnoses were made according to the International Classification of Sleep Disorders (third edition). Cutoff values of UNS and SNS for diagnosis of narcolepsy were those suggested in the literature. Results: For the diagnosis of narcolepsy, the following sensitivities and specificities were found: SNS (89% and 88%), UNS (100% and 62%), and ESS (91% and 54%). For the diagnosis of narcolepsy with low or non-detectable CSF hypocretin-1, the sensitivities and specificities were: SNS (93% and 88%), UNS (100% and 62%), and ESS (93% and 54%). Discussion: In conclusion, the SNS is accurate and superior to the ESS and the UNS for the screening/diagnosis of NC and allows, in addition, the identification of hypocretin-1 deficient patients.