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ObjectiveTo report clinical and electroneuromyographic (ENMG) characteristics of patients affected by severe COVID-19 infection, evaluated for muscular weakness.Materials and methodsENMGs performed for evaluation of diffuse weakness in patients who could not be discharged from semi-intensive care COVID unit because of difficulties in ventilation weaning were reviewed. Patients with severe COVID-19 infection who had undergone endotracheal intubation and able to co-operate were considered. ENMG protocol was focused on neurophysiological items that excluded or confirmed critical illness polyneuropathy (CIP), myopathy (CIM), or polyneuromyopathy (CIPM). Standardized clinical evaluation was performed using Medical Research Council (MRC) sum score.ResultsEight patients were included in the study. All presented known risk factors for intensive care unit-acquired weakness (ICU-AW), and none of them had history of underlying neuromuscular disorders. ENMG findings were normal in two patients, while only two patients had an altered MRC sum score (< 48). Neuromuscular involvement was diagnosed in 6/8 patients (75%): 2 had CIP, 1 had possible CIM, 1 had CIPM, while 1 patient, with clinically evident weakness but equivocal ENMG findings, was classified as ICU-AW. Finally, 1 patient was diagnosed with acute demyelinating neuropathy. Patients with neuromuscular involvement were those with longer intubation duration and higher levels of IL-6 at admission.ConclusionNeuromuscular complications are frequent in severe COVID-19 and cannot be excluded by MRC sum scores above 48. Standardized ENMG is helpful in guiding diagnosis when clinical evaluation is not reliable or possible. Elevated IL-6 at admission may be a predictor biomarker of ICU-AW in COVID-19.

Gustatory (GD) and olfactory (OD) dysfunctions are the most frequent neurological manifestations of COVID-19. We used mental imagery as an experimental psychological paradigm to access olfactory and gustatory brain representations in 80 Italian COVID-19 adult patients (68.75% reported both OD and GD). COVID-19 patients with OD + GD have a significantly and selectively decreased vividness of odor and taste imagery, indicating that COVID-19 has an effect on their chemosensory mental representations. OD + GD length and type influenced the status of mental chemosensory representations. OD + GD were become all COVID-19 negative at the time of testing. Data suggest that patients are not explicitly aware of long-term altered chemosensory processing. However, differences emerge when their chemosensory function is implicitly assessed using self-ratings. Among patients developing OD + GD, self-ratings of chemosensory function (taste, flavor) were significantly lower as compared to those who did not. At the level of mental representation, such differences can be further detected, in terms of a reduced ability to mentally activate an odor or taste mental image. Our study shows that COVID-19 infection not only frequently causes hyposmia and dysgeusia, but that may also alter the mental representations responsible for olfactory and gustatory perception.

Parkinson's disease (PD) is a neurodegenerative disorder that may sometimes be caused by deleterious genetic variants. Among them, RAB39B polymorphisms are known as rare causes of early‐onset PD associated with intellectual disability (Waisman's syndrome). Here we describe a 45‐year‐old white male affected by developmental delay, childhood onset intellectual disability, epilepsy, and PD who was treated with subthalamic deep brain stimulation and subcutaneous L‐DOPA infusion. Next Generation Sequencing analysis revealed a currently unknown pathogenic hemizygous sequence variant c.463C>T (NM_171998.4) in the RAB39B gene, confirmed also in the proband's mother, affected by late‐onset PD. This report expands the number of described RAB39B mutations in individuals with early‐ and late‐onset, X‐linked PD.

Aims: The aim of this study is to assess the sleep quality and daytime sleepiness improvement in chronic migraineurs after 6 months of a 2:1 KD (ketogenic diet) and LGID (low-glycemic-index diet). Methods: Twenty-six patients underwent 2:1 KD (11 patients) and LGID (15 patients). PSQI (Pittsburgh sleep quality index) and ESS (Epworth sleepiness scale) were administered at the baseline and the 3-month and 6-month follow-up. MIDAS (Migraine Disability Assessment), HIT-6 (Headache Impact Test 6), migraine frequency (migraine days per month), migraine intensity, BMI (Body Mass Index), FM (Fat Mass), and FFM (Fat-Free Mass) were also assessed. Results: PSQI (F1.544, 38.606 = 7.250; p = 0.004), ESS (F1.988, 49.708 = 9.938; p < 0.001), HIT-6 (F1.432, 35.805 = 12.693; p < 0.001), migraine frequency (F1.522, 38.041 = 23.070; p < 0.001), migraine intensity (F1.949, 48.721 = 18.798; p < 0.001), BMI (F1.274, 31.857 = 38.191; p < 0.001), and FM (F1.245, 31.134 = 45.487; p < 0.001) improved significantly. The MIDAS (F1.005, 25.121 = 3.037; p = 0.093) and the FMM (F1.311, 32.784 = 1.741; p = 0.197) did not improve significantly. The ESS (p = 0.712) and PSQI (p = 0.776) data at 3-month and 6-month follow-ups did not differ significantly, as well as for migraine frequency, migraine intensity, BMI, FM, and HIT-6. A mild correlation emerged between the mean FM and mean ESS reduction during the 6 months (r = 0.497, p = 0.010). Conclusions: Six months of LGID and 2:1 KD can improve sleep quality and daytime sleepiness in patients with chronic migraine. The effectiveness on migraine, sleep quality, and daytime sleepiness does not differ significantly between the 3-month and 6-month follow-up periods.

In the context of neurodegenerative disorders, cognitive decline is frequently reported in older population. Recently, numerous metabolic pathways have been implicated in neurodegeneration, including signaling disruption of insulin and other glucose-regulating hormones. In fact, Alzheimer’s disease has now been considered as “type-3 diabetes”. In this review, we tried to clarify the role of sleep impairment as the third major player in the complex relationship between metabolic and neurodegenerative diseases. Altered sleep may trigger or perpetuate these vicious mechanisms, leading to the development of both dementia and type 2 diabetes mellitus. Finally, we analyzed these reciprocal interactions considering the emerging role of the gut microbiota in modulating the same processes. Conditions of dysbiosis have been linked to circadian rhythm disruption, metabolic alterations, and release of neurotoxic products, all contributing to neurodegeneration. In a future prospective, gut microbiota could provide a major contribution in explaining the tangled relationship between sleep disorders, dementia and diabetes.

We describe a case of a young male patient with narcolepsy type 1 (NT1), who developed generalized cataplexy attacks during sexual intercourses, on which we have obtained a satisfactory control with pitolisant. Orgasmolepsy is an uncommon feature of NT1 that has been poorly described in the literature. The prevalence of this condition is unclear, as it is reasonably underreported by patients for embarrassment and not well investigated by physicians. Pitolisant is a novel treatment for narcolepsy, effective on excessive daytime sleepiness and cataplexy by modulating the histaminergic system. Real-world data collection on pitolisant efficacy and safety is still ongoing. However, pitolisant effectiveness on orgasmolepsy in NT1 has no precedent in the literature. Orgasmolepsy and other sexual disturbances should be actively searched in narcoleptic patients and, if present, may guide clinicians to prefer pitolisant or sodium oxybate, avoiding antidepressants for their possible sexual side effects.

The following prospective study has the aim of evaluating the efficiency of the F22 MAD (mandibular advancement device), a new oral device for the treatment of OSAS (Obstructive Sleep Apnea Syndrome) and snoring. Methods: AHI (apnea-hypopnea index), ODI (Oxygen Desaturation Index), snoring percentage, time spent in the supine position, and Epworth Sleepiness Scale score were evaluated in 19 patients with snoring, mild to moderate OSAS, or severe OSAS who declined CPAP (Continuous Positive Air Pressure) treatment, before and after the application of the F22 MAD. Results: The median value of AHI varied from 15.6 ± 10.7 to 5.7 ± 5.7; the median value of ODI varied from 13.4 ± 8.8 to 6.2 ± 5.2; the median value of the percentage of snoring varied from 30.7 ± 7 to 7.5 ± 10.8, except for the patient who has severe OSAS who increased their value. The value obtained by the self-completion of the ESS questionnaire (Epworth Sleepiness Scale) underwent a statistically significant variation, while clinically significant for 13\\19 patients who obtained a reduction of the value >/= of 2 points. Conclusions: It is possible to conclude that the F22 MAD is effective in the treatment of patients with mild and moderate OSAS or simple snoring, reducing the polysomnographic outcomes with statistically and clinically significant results in terms of reduction of AHI, ODI and percentage of snoring.