Corrigendum to "Transverse extension of partons in the proton probed in the sea-quark range by measuring the DVCS cross section" [Phys. Lett. B 793 (2019) 188]

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The Challenging Clinical Management of Patients with Cranial Dural Arteriovenous Fistula and Secondary Parkinson's Syndrome: Pathophysiology and Treatment Options

Cranial dural arteriovenous fistula (cDAVF) may rarely lead to parkinsonism and rapid cognitive decline. Dysfunction of the extrapyramidal system and the thalamus, due to venous congestion of the Galenic system with subsequent parenchymal edema, is likely to represent an important pathophysiological mechanism. Here, we report a case of a 57-year-old man with a cDAVF of the straight sinus (Borden type III; DES-Zurich bridging vein shunt [BVS] type with direct, exclusive, and strained leptomeningeal venous drainage [LVD]) and subsequent edema of both thalami, the internal capsule, the hippocampi, the pallidum, and the mesencephalon. Several attempts at venous embolization were unsuccessful, and the neurological condition of the patient further deteriorated with progressive parkinsonism and intermittent episodes of loss of consciousness (KPS 30). A suboccipital mini-craniotomy was performed and the culminal vein was disconnected from the medial tentorial sinus, achieving an immediate fistula occlusion. Three-month follow-up MRI revealed complete regression of the edema. Clinically, parkinsonism remitted completely, allowing for tapering of dopaminergic medication. His cognition markedly improved in further course. The purpose of this report is to highlight the importance of rapid and complete cDAVF occlusion to reverse venous hypertension and prevent progressive clinical impairment. The review of the literature underlines the high morbidity and mortality of these patients. Microsurgical disconnection of the fistula plays an important role in the management of these patients and, surprisingly, has not been reported so far

Remitting narcolepsy? Longitudinal observations in a hypocretin-deficient cohort

Study Objective Narcolepsy type 1 (NT1) is considered a chronic, incurable disease. Excessive daytime sleepiness (EDS) is typically the most troublesome symptom, and more difficult to control by pharmacologic treatment than cataplexy. Although many NT1 patients are monitored by regular follow-ups, the purported relentless persistence of EDS has rarely been the object of longitudinal studies. Methods Retrospective analysis of 26 well-defined hypocretin-deficient NT1 patients who underwent longitudinal assessments of Epworth sleepiness scale (ESS) scores under stable pharmacotherapy. We present detailed case reports of four patients with unusual spontaneous improvement. Results Over a mean observation period of 5 years, changes in ESS scores between first and last examination were ≤4 points in 19 patients (73%). Three patients deteriorated by 5 points, four patients ameliorated by 7-11 points. Among the latter, subjective sleepiness resolved in all four patients, and three of them continued showing ESS scores 8 minutes), one of whom did not fall asleep during maintenance of wakefulness test. Multiple linear regression analysis identified higher cerebrospinal fluid (CSF) hypocretin level (p < 0.001) and absence of fragmented nighttime sleep (p = 0.001) as independent associates of EDS improvement. Conclusions The longitudinal course of NT1-related sleepiness is not invariably stable, but included spontaneous deterioration or improvement in 27%. Spontaneous improvement can persist after treatment discontinuation and resemble remission. Milder hypocretin deficiency and good nighttime sleep may predict a more favorable disease course

Remitting narcolepsy? Longitudinal observations in a hypocretin-deficient cohort

Abstract Study Objective Narcolepsy type 1 (NT1) is considered a chronic, incurable disease. Excessive daytime sleepiness (EDS) is typically the most troublesome symptom, and more difficult to control by pharmacologic treatment than cataplexy. Although many NT1 patients are monitored by regular follow-ups, the purported relentless persistence of EDS has rarely been the object of longitudinal studies. Methods Retrospective analysis of 26 well-defined hypocretin-deficient NT1 patients who underwent longitudinal assessments of Epworth sleepiness scale (ESS) scores under stable pharmacotherapy. We present detailed case reports of four patients with unusual spontaneous improvement. Results Over a mean observation period of 5 years, changes in ESS scores between first and last examination were ≤4 points in 19 patients (73%). Three patients deteriorated by 5 points, four patients ameliorated by 7-11 points. Among the latter, subjective sleepiness resolved in all four patients, and three of them continued showing ESS scores 8 minutes), one of whom did not fall asleep during maintenance of wakefulness test. Multiple linear regression analysis identified higher cerebrospinal fluid (CSF) hypocretin level (p < 0.001) and absence of fragmented nighttime sleep (p = 0.001) as independent associates of EDS improvement. Conclusions The longitudinal course of NT1-related sleepiness is not invariably stable, but included spontaneous deterioration or improvement in 27%. Spontaneous improvement can persist after treatment discontinuation and resemble remission. Milder hypocretin deficiency and good nighttime sleep may predict a more favorable disease course

Onset of Postural Instability in Parkinson's Disease Depends on Age rather than Disease Duration

BACKGROUND: Postural instability and falls are considered a major factor of impaired quality of life in patients with advanced Parkinson's disease (PD). The knowledge of the time at which postural instability occurs will help to provide the evidence required to introduce fall-prevention strategies at the right time in PD. OBJECTIVE: To investigate whether postural instability of patients with different age at disease onset is associated with age or with disease duration of PD. METHODS: Patients diagnosed with sporadic PD between 1991 and 2017 and postural instability (according to the International Parkinson and Movement Disorder Society Unified Parkinson's Disease Rating Scale (MDS-UPDRS) part III, item 3.12 postural instability) were included, with strict inclusion criteria including regular follow-ups, agreement on data use, and exclusion of comorbidities affecting the free stand. RESULTS: Applying these strict inclusion criteria, we included 106 patients. Those younger than 50 years at PD onset took significantly longer to develop postural instability (n = 23 patients, median: 18.4 years) compared with patients with later onset of PD (50-70 years, n = 66, median: 14.2 years, p 70 years, n = 17, median: 5.7 years, p < 0.001, Kruskal-Wallis test followed by Dunn's multiple comparisons test). There was no association between total MDS-UPDRS III (as a measure of motor symptom severity) at onset of postural instability. CONCLUSIONS: In PD, postural instability is primarily associated with the age of the patient and not with disease duration

Novel rat model of weight drop-induced closed diffuse traumatic brain injury compatible with electrophysiological recordings of vigilance states

Traumatic brain injury (TBI) is a major cause of persistent disabilities such as sleep-wake disorders (SWD). However, rodent studies of SWD after TBI are scarce due to lack of appropriate TBI models reproducing acceleration-deceleration forces and compatible with electroencephalography/myography (EEG/EMG)-based recordings of vigilance states. We therefore adapted Marmarou's impact acceleration model to allow for compatibility with EEG-headset implantation. Following implantation of EEG/EMG electrodes, we induced closed TBI by a frontal, angular hit with a weight-drop device (44 rats, weight 2500g, fall height 25cm). Subsequently, we tested our model's usefulness for long-term studies on a behavioral, electrophysiological and histological level. Neurological, motor and memory deficits were assessed with the neurological severity score, open field, and novel object recognition tests, respectively. EEG/EMG recordings were performed in both SHAM (n=7) and TBI (n=7) rats before and 1, 7 and 28 days after trauma to evaluate sleep-wake proportions and posttraumatic implant stability. Histological assessments included hematoxylin and eosin staining for parenchymal damage and hemorrhage and amyloid precursor protein staining for diffuse axonal damage. All rats survived TBI without major neurological or motor deficits. Memory function was impaired after TBI at weeks 1, 2, 3 and recovered at week 4. EEG implants were stable for at least 1 month and enabled qualitative and quantitative sleep analyses. Histological assessments revealed no major bleedings or necrosis but intense diffuse axonal damage following TBI. In conclusion, this approach fulfills major preconditions for experimental TBI models and offers a possibility to electrophysiologically study behavioral states before and after trauma

Development of a Textile Integrated, Two-State Controlled Tremor Suppression Orthosis for the Wrist

Tremor is one of the most common movement disorders with the highest prevalence in the upper limb. Apart from medication or surgery, the mechanical suppression of the involuntary movement with an orthosis can be used as alternative treatment. Here we propose a controlled energy dissipating suppression orthosis using a mechanical brake. For this approach, we focused on improved wearability with voluntary movement preservation and ergonomics while providing tremor suppression. The novelty of this orthosis is the decentralization of the tremor suppression mechanism and the integration of textiles in the orthosis structure. We performed computational and test bench simulations of a controlled two-state brake with a 1D human model to optimize the brake duration and timing. The objective was to optimize the trade-off between tremor suppression and voluntary movement suppression. The textile-integrated prototype, with the optimized parameter, was validated in a proof-of-concept case study with a tremor-affected person performing activities of daily living. With the optimized parameters, we achieved a tremor suppression of 78.8%, 66.5%, and 40.8% for the simulation, test bench, and case study, respectively as measured by the change in power spectral density (PSD) at the tremor frequency peak. While minimizing the voluntary movement suppression in the simulation and test bench by introducing the trajectory distance as new validation method (23.7% and 31.2%), no voluntary movements suppression was measured in the case study using PSD analysis. Our new orthosis has the potential to become a daily wearable device that can improve the quality of life for tremor-affected people.ISSN:2576-320