Increased intake of fast acting carbohydrates in patients with Parkinson’s Disease

Background: Patients with Parkinson’s Disease (PD) regularly report an increased desire for food or beverages with high sugar content. Objective: To verify the hypothesis of an increase intake of fast-acting carbohydrates in PD patients. Methods: This study investigated the consumption of high sugar-content food products in 221 PD patients compared with 184 healthy controls using a self-administered questionnaire. Results: Male PD patients reported significant more often a high consumption of chocolate (p=0.005) and other sweets (p<0.001) when compared to healthy controls. Moreover, PD patients with a high intake of these products showed a significant longer disease duration (p=0.002). Conclusion: Our study confirmed changes in intake of fast acting carbohydrates derived from sweets in PD. Future studies should address the observed association with disease progression to understand underlying pathophysiological mechanisms leading to this behavioral change.

Heart Rate Variability during wake and sleep in Huntington’s Disease patients. An observational, cross-sectional, cohort study.

Introduction Autonomic dysfunction has been reported as one of non-motor manifestations of both pre-symptomatic and manifest Huntington’s Disease (HD). The aim of our study was to evaluate heart rate variability (HRV) during wake and sleep in a cohort of patients with manifest HD. Methods Thirty consecutive patients with manifest HD were enrolled, 14 men and 16 women, mean age 57.3±12.2 years. All patients underwent full-night attended video-polysomnography. HRV was analyzed during wake, NREM and REM sleep, in time and frequency domain. Results were compared with a control group of healthy volunteers matched for age and sex. Results During wake HD patients presented significantly higher mean heart rate than controls (72.4±9.6 vs 58.1±7.3 bpm; p<0.001). During NREM sleep, HD patients showed higher mean heart rate (65.6±11.1 vs 48.8±4.6 bpm; p<0.001) and greater Low Frequency (LF) component of HRV (52.9±22.6 vs 35.5±17.3 n.u.; p=0.004). During REM sleep, we observed lower standard deviation of the R-R interval (SDNN) in HD subjects (3.4±2.2 vs 3.7±1.3 ms; p=0.015). Conclusion Our results showed that HD patients have higher heart rate than controls, during wake and NREM, but not during REM sleep. Among HRV variability parameters, the most relevant difference regarded the LF component, which reflects, at least partially, the ortho-sympathetic output. Our results confirm the involvement of autonomic nervous system in HD and demonstrate that it is evident during both wake and sleep.

Review of available data for the efficacy and effectiveness of nabiximols oromucosal spray (Sativex) in multiple sclerosis patients with moderate to severe spasticity

Background: Sativex (USAN: nabiximols) oromucosal spray is indicated for treatment of multiple sclerosis (MS) patients with moderate to severe spasticity and inadequate response to other antispasticity medications who demonstrate clinically significant improvement during an initial trial of therapy. This narrative review investigated the efficacy and effectiveness of nabiximols oromucosal spray for moderate to severe MS spasticity by examining spasticity 010 Numerical Rating Scale (NRS) data from interventional and observational studies which featured a 4-week trial period as per the European Union approved label. Summary: Across both study types, clinically relevant and statistically significant reductions in mean MS spasticity 0–10 NRS scores were measured soon after treatment start and were maintained in the mid to long term in treatment responders. Initial responder rates (≥ 20% NRS improvement from baseline at week 4) ranged from 47.6% to 81.4%, tending lower in the randomized clinical trials setting. Clinically relevant responder rates (≥ 30% NRS improvement from baseline at week 12) were similar between study types (range 3041%) except for one outlier (74% in an observational study). Two open studies reported treatment continuation for ≥ 18 months in approximately half of patients who initiated treatment. In most longer-term studies, symptomatic improvement in MS spasticity was maintained at mean daily dosages of about 67 sprays/day. Safety was consistent with the known profile of nabiximols. Key messages: Experimental and observational studies of nabiximols oromucosal spray recorded similar findings. About half to two-thirds of MS patients who begin treatment will perceive initial symptomatic relief of spasticity within the 4-week trial period. About 40% of patients who initiate treatment will reach the ≥ 30% NRS improvement threshold at 3 months, comprising the majority of patients who continue long-term treatment. A trial of therapy with nabiximols is useful to identify patients most likely to gain longer-term improvement in spasticity symptoms and discontinue those with insufficient benefit.

Tit for tat: costly punishment in manifest Huntington’s disease

Objective: We aimed to investigate costly punishment in patients with HD. Background: Huntington’s disease (HD) is an autosomal dominant neurodegenerative disease with motor, cognitive, and psychiatric symptoms. As neuropsychiatric abnormalities often precede motor symptoms, we wanted to assess whether costly punishment is part of the neuropsychological profile of patients with HD. Methods: A total of 40 non demented subjects were prospectively enrolled in this study with a between-subject design comparing manifest HD patients (n=18) to healthy controls (HC; n=22). All participants performed eight rounds of a costly punishment task, in which money was shared unevenly in 5 rounds or in a fair manner in the remaining three rounds. Participants then had to decide whether they wanted to punish the trustee. Furthermore, all participants underwent neuropsychological background tasks. Results: HD patients performed worse in the neuropsychological background tests compared to HC (all p-values<0.05). Moreover, HD patients punished more often in fair (Wald x2=5.03, p=0.025) but not in unfair rounds (Wald x2=1.63, p=0.202). Conclusions: Our results demonstrate increased costly punishment during fair conditions in HD patients. Whether this behaviour is due to a lack of recognition of social norms, an impairment in top-down inhibition, or an effect of anti-dopaminergic medication remains unclear.

Cerebrospinal fluid levels of 5-hydroxyindoleacetic acid in Parkinson’s disease and atypical parkinsonian syndromes

Background: Various cerebrospinal fluid (CSF) biomarkers are studied in Parkinson’s disease (PD) and atypical parkinsonian syndromes (APS). Several studies found reduced 5-hydroxyindoleacetic acid (5-HIAA), the main serotonin metabolite, in PD. There is little evidence regarding its levels in APS. Methods: We measured 5-HIAA in the CSF of 90 PD patients, 16 MSA patients, 26 progressive supranuclear palsy (PSP) patients, 11 corticobasal degeneration (CBD) patients, and 31 controls. We also compared the values in depressed and non-depressed patients. Results: There was a statistically significant difference in CSF 5-HIAA in PD and MSA compared to the control group (median in PD 15.8 µg/l, in MSA 13.6 µg/l vs. 24.3 µg/l in controls; P=0.0008 in PD, P=0.006 in MSA). There was no statistically significant difference in CSF 5-HIAA in PSP and CBD compared to the control group (median in PSP 22.7 µg/l, in CBD 18.7 µg/l vs. 24.3 µg/l in controls; P= 1 in both PSP and CBD). CSF 5-HIAA levels were lower in PD patients with depression compared to PD patients without depression (median 8.34 vs. 18.48, P<0.0001). Conclusions: CSF 5-HIAA is decreased in PD and MSA. The CSF 5-HIAA levels in PSP and CBS did not differ from those of the control group. There was a tendency toward lower CSF 5-HIAA in MSA than in PD, however, the results did not reach statistical significance. These results may be explained by more severe damage of the serotonergic system in synucleinopathies (PD, MSA) than in tauopathies (PSP, CBS).