Stroke

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Clinical characteristics of a large cohort of patients with narcolepsy candidate for pitolisant: a cross-sectional study from the Italian PASS Wakix® Cohort

Introduction Narcolepsy is a chronic and rare hypersomnia of central origin characterized by excessive daytime sleepiness and a complex array of symptoms as well as by several medical comorbidities. With growing pharmacological options, polytherapy may increase the possibility of a patient-centered management of narcolepsy symptoms. The aims of our study are to describe a large cohort of Italian patients with narcolepsy who were candidates for pitolisant treatment and to compare patients' subgroups based on current drug prescription (drug-naive patients in whom pitolisant was the first-choice treatment, switching to pitolisant from other monotherapy treatments, and adding on in polytherapy). Methods We conducted a cross-sectional survey based on Italian data from the inclusion visits of the Post Authorization Safety Study of pitolisant, a 5-year observational, multicenter, international study. Results One hundred ninety-one patients were enrolled (76.4% with narcolepsy type 1 and 23.6% with narcolepsy type 2). Most patients (63.4%) presented at least one comorbidity, mainly cardiovascular and psychiatric. Pitolisant was prescribed as an add-on treatment in 120/191 patients (62.8%), as switch from other therapies in 42/191 (22.0%), and as a first-line treatment in 29/191 (15.2%). Drug-naive patients presented more severe sleepiness, lower functional status, and a higher incidence of depressive symptoms. Conclusion Our study presents the picture of a large cohort of Italian patients with narcolepsy who were prescribed with pitolisant, suggesting that polytherapy is highly frequent to tailor a patient-centered approach

The spatial side of somatoparaphrenia: a case study

The perception of the bodily self in space is a composite cognitive function requiring a dynamic integrated brain mechanism. Somatoparaphrenia (SP), a delusional belief concerning the experienced disownership for the contralesional paralyzed arm, represents the disruption of such mechanism. In two experiments, we have investigated the alteration of limb disownership after spatial manipulations in a right-brain-damaged patient affected by chronic SP. In experiment 1 the patient's spatial attention was switched between the left and right sides of space. SP signs worsened when the patient was interviewed from the left compared to the right bedside. In the second experiment we showed the first systematic transient remission of SP using left caloric vestibular stimulation (CVS), a physiologic manipulation mainly acting on the spatial frame of reference. Taken together, these results shed further light on the spatial nuance of SP and on the importance of vestibular signals for the generation of a coherent body representation. Furthermore, our case study demonstrated the possibility of eliciting more severe SP signs if the patient is interviewed from the left bedside. Additionally, CVS applications may have an important impact on the rehabilitation of these symptoms

Assessing mood and cognitive functioning in acute stroke: clinical usability of a Visual Analogue Mood Scale (VAMS)

BACKGROUND: Patients suffering from stroke in the acute/post-acute phases often present with depressive mood — which negatively impacts on patients’ prognosis. However, psychometric evaluation of mood in acute stroke patients may be challenging due to cognitive deficits. Tools investigating emotional states via a vertical analogue line may overcome language/visuo-spatial disorders. This study thus aimed at (a) investigating the clinical usability of a Visual Analogue Mood Scale (VAMS) in acute stroke patients and (b) investigating the interplay between mood and cognition in this population. METHODS: Forty-one acute stroke patients were compared to 41 age-, education- and sex-matched healthy participants (HPs) on the VAMS and on cognitive measures (mental performance in acute stroke, MEPS). A control line bisection (LB) task was administered to control for potential visuo-spatial deficits in patients. RESULTS: Patients reported higher depression levels than HPs (lower VAMS scores); this between-group difference stayed significant when covarying for LB scores. MEPS scores discriminated patients from HPs; among cognitive measures, only the Clock drawing test (CDT) was positively associated with VAMS scores. Lesion side did not affect patients’ mood state; however, disease duration was inversely related to VAMS scores. DISCUSSION: The VAMS proved to be a suitable tool for assessing mood in acute stroke patients, as being independent from post-stroke cognitive sequelae. The CDT might represent an adequate measure of depression-induced, post-stroke cognitive efficiency decrease. Mood disorders might occur and thus should be adequately addressed also in post-acute phases — likely due to longer hospitalization times and regression of anosognosic features

Stroke territory and atherosclerosis in ischemic stroke patients with a history of migraine with aura

Introduction: The mechanisms subtending the increased stroke risk in migraine with aura (MA) are not fully understood. Our study aims to evaluate if the clinical profile in stroke patients with MA differentiates from those without MA. Methods: We retrieved the prospective registered electronic clinical dossiers of adult patients younger than 60 years with acute ischemic stroke admitted in four hospitals between January 2016 and June 2022. Patients were classified by the history of MA (MA+ and MA-). Results: We identified 851 stroke patients (59 MA+, 6.9%). Compared to MA-, MA+ patients were characterized by younger age (44.0 ± 10.6 vs 50.1 ± 8.2 years), female sex (59.3% vs 29.0%), and affected by cryptogenic (OR 2.594 95% CI 1.483-4.537), and cerebellar stroke (OR 3.218 95% CI 1.657-6.250; p ≤ 0.001 for all comparisons). After adjusting for age and sex, MA+ patients presented less frequently hypertension (OR 0.349 95% CI 0.167-0.470; p=0.005) and dyslipidemia (OR 0.523 95% CI 0.280-0.974; p = 0.041). After adjusting also for risk factors, the MA+ group had less frequently symptomatic large vessel stenosis (OR 0.126 95% CI 0.017-0,924; p = 0.042) and clinical atherosclerosis (OR 0.103 95% CI 0.014-0.761; p = 0.026), while intima-media thickness did not differ (p = 0.395). Discussion: Cryptogenic and cerebellar stroke and fewer vascular risk factors and clinical atherosclerosis seem to characterize stroke patients with MA

Polysomnographic features differentiating disorder of arousals from sleep-related hypermotor epilepsy.

OBJECTIVE The differential diagnosis between sleep-related hypermotor epilepsy (SHE) and disorders of arousal (DOA) may be challenging. We analyzed the stage and the relative time of occurrence of parasomnic and epileptic events to test their potential diagnostic accuracy as criteria to discriminate SHE from DOA. METHODS Video-polysomnography recordings of 89 patients with a definite diagnosis of DOA (59) or SHE (30) were reviewed to define major or minor events and to analyze their stage and relative time of occurrence. The "event distribution index" was defined on the basis of the occurrence of events during the first versus the second part of sleep period time. A group analysis was performed between DOA and SHE patients to identify candidate predictors and to quantify their discriminative performance. RESULTS The total number of motor events (i.e. major and minor) was significantly lower in DOA (3.2 ± 2.4) than in SHE patients (6.9 ± 8.3; p = 0.03). Episodes occurred mostly during N3 and N2 in DOA and SHE patients, respectively. The occurrence of at least one major event outside N3 was highly suggestive for SHE (p = 2*e-13; accuracy = 0.898, sensitivity = 0.793, specificity = 0.949). The occurrence of at least one minor event during N3 was highly suggestive for DOA (p = 4*e-5; accuracy = 0.73, sensitivity = 0.733, specificity = 0.723). The "event distribution index" was statistically higher in DOA for total (p = 0.012) and major events (p = 0.0026). CONCLUSION The stage and the relative time of occurrence of minor and major motor manifestations represent useful criteria to discriminate DOA from SHE episodes

Assessment of self-reported and objective daytime sleepiness in adult-onset myotonic dystrophy type 1

Study objectives: Excessive daytime sleepiness (EDS) in myotonic dystrophy type 1 is mostly of central origin but it may coexist with sleep-related breathing disorders. However, there is no consensus on the sleep protocols to be used, assessments vary, and only a minority of patients are regularly tested or are on treatment for EDS. Our study presents data on self-reported and objective EDS in adult-onset myotonic dystrophy type 1. Methods: Sixty-three patients with adult-onset DM1 were subjected to EDS-sleep assessments (polysomnography, Multiple Sleep Latency Test, Epworth Sleepiness Scale). Correlation coefficients were computed to assess the relationship between sleep and sleepiness test results, fatigue, and quality of life. Results: 33% and 48% of patients had EDS based, respectively, on the Epworth Sleepiness Scale and the Multiple Sleep Latency Test, with a low concordance between these tests (k = 0.19). Thirteen patients (20%) displayed 2 or more sleep-onset rapid eye movement periods on Multiple Sleep Latency Test. Patients having EDS by Multiple Sleep Latency Test had a shorter disease duration (P < .05), higher total sleep time and sleep efficiency and lower wake after sleep onset on polysomnography. Patients with self-reported EDS reported significantly higher fatigue score compared with patients without EDS (P < .05). No other difference was found in demographic, clinical, and respiratory features. Conclusions: EDS test results are contradictory, making treatment options difficult. Combining quantitative tests and self-reported scales may facilitate physicians in planning EDS care with patients and families. Citation: Sansone VA, Proserpio P, Mauro L, et al. Assessment of self-reported and objective daytime sleepiness in adult-onset myotonic dystrophy type 1. J Clin Sleep Med. 2021;17(12):2383-2391