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Background and Purpose: Patients with episodic ataxia type 2 (EA2) suffer from recurrent paroxysmal episodes of vertigo and oscillopsia. Pathophysiologically, altered neuronal excitability has been suspected. Vestibular excitability in 22 EA2 patients and 22 age-matched healthy participants was compared., Methods: Galvanic vestibular stimulation (GVS) was used to assess vestibular excitability by vestibular motion perception thresholds and mean postural sway velocity during various visual and proprioceptive conditions in the two groups. Control stimuli using sham and no GVS were established to identify the specificity of GVS-induced postural sway., Results: In the baseline condition, EA2 patients showed larger postural instability. However, motion perception thresholds and the increase in mean postural sway velocity during vestibular stimulation (stimulation ratio) did not differ between groups. Postural sway during suprathreshold GVS increased with the vestibular motion perception threshold in EA2 patients, in contrast to healthy participants., Conclusions: The larger postural unsteadiness of EA2 patients probably reflects their progressive cerebellar degeneration. It is not related to abnormal visual (Romberg's ratio) or proprioceptive control of stance. Postural unsteadiness during vestibular stimulation does not indicate altered vestibular excitability in EA2 patients. However, vestibular stimulation increasingly destabilized postural control of EA2 patients with higher motion perception thresholds when proprioceptive information was diminished. This conclusion, however, is restricted to the postural control of EA2 patients in the interval between the vestibulo-cerebellar episodes., (© 2024 The Author(s). European Journal of Neurology published by John Wiley & Sons Ltd on behalf of European Academy of Neurology.)

Background: X-linked dystonia-parkinsonism (XDP), a neurodegenerative movement disorder endemic to the Philippines, is primarily investigated in patients from Panay Island and the Greater Manila area. However, individuals residing in geographically distant regions may exhibit different clinical or genetic characteristics compared to those documented in earlier reports., Objective: The aim was to investigate the relationship of XDP clinical features in a Mindanao cohort with modifiers of age at onset (AAO) variability and utilization of a previously reported AAO model., Methods: We investigated clinical and genetic features in 27 XDP patients from southern Mindanao. In all patients, we genotyped the 4 polymorphisms linked to AAO., Results: The XDP-relevant hexanucleotide repeat number significantly correlated with AAO in the 27 patients and explained about 68% of AAO variability. There is no statistical difference between the predicted and actual AAO., Conclusion: The AAO model may provide reliable predictions by employing the effect of XDP genetic modifiers of AAO variability., (© 2024 The Author(s). Movement Disorders Clinical Practice published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.)

During composting of organic waste, nitrogen is lost through gaseous forms and ion leaching. Biochar has been shown to capture mineral nitrogen (N min : NH 4 + ) from compost, which we hypothesize reduces N 3 O formation. However, associating N - captured by biochar with the dynamics of N 2 O formation. However, associating N min captured by biochar with the dynamics of N 2 , N 2 O) emissions, N 4 , N 2 C-NMR spectroscopy) along composting. Biochar did not significantly reduce or increase GHG emissions and N min content in compost and biochar particles (sequential extractions), and biochar surface transformations (SEM-EDX and 13 C-NMR spectroscopy) along composting. Biochar did not significantly reduce or increase GHG emissions and N min content (mg kg -1 ) in compost. However, the final NO 3 - amount (g compost pile -1 ) in the Bc treatment was significantly higher (54%) compared to the Control, indicating lower NO 3 - -N kg 4 (44%, strongly retained). Although N + retention in biochar was not accompanied by lower N 3 O emissions, contradicting our hypothesis, we demonstrated the efficacy of biochar to recover N - content in biochar increased continuously during composting. In the final compost, the NO 3 - content extracted from biochar was 164 (37%, easily extractable), 80 (19%, moderately extractable), and 194 mg NO 3 - -N kg -1 (44%, strongly retained). Although N min retention in biochar was not accompanied by lower N 2 O emissions, contradicting our hypothesis, we demonstrated the efficacy of biochar to recover N min from organic waste without stimulating GHG emissions., (© 2024 The Author(s). Journal of Environmental Quality published by Wiley Periodicals LLC on behalf of American Society of Agronomy, Crop Science Society of America, and Soil Science Society of America.)

Background: The newly discovered intronic repeat expansions in the genes encoding replication factor C subunit 1 (RFC1) and fibroblast growth factor 14 (FGF14) frequently cause late-onset cerebellar ataxia., Objectives: To investigate the presence of RFC1 and FGF14 pathogenic repeat expansions in Serbian patients with adult-onset cerebellar ataxia., Methods: The study included 167 unrelated patients with sporadic or familial cerebellar ataxia. The RFC1 repeat expansion analysis was performed by duplex PCR and Sanger sequencing, while the FGF14 repeat expansion was tested for by long-range PCR, repeat-primed PCR, and Sanger sequencing., Results: We identified pathogenic repeat expansions in RFC1 in seven patients (7/167; 4.2%) with late-onset sporadic ataxia with neuropathy and chronic cough. Two patients also had bilateral vestibulopathy. Repeat expansions in FGF14 were found in nine unrelated patients (9/167; 5.4%) with ataxia, less than half of whom presented with neuropathy and two-thirds with global brain atrophy. Tremor and episodic features were the most frequent additional characteristics in carriers of uninterrupted FGF14 repeat expansions. Among the 122 sporadic cases, 12 (9.8%) carried an expansion in either RFC1 or FGF14, comparable to 4/45 (8.9%) among the patients with a positive family history., Conclusions: Pathogenic repeat expansions in RFC1 and FGF14 are relatively frequent causes of adult-onset cerebellar ataxia, especially among sporadic patients, indicating that family history should not be considered when prioritizing ataxia patients for testing of RFC1 or FGF14 repeat expansions., (© 2024 The Authors. Movement Disorders Clinical Practice published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.)

Background: X-linked dystonia-parkinsonism (XDP) is a rare movement disorder characterized by profound neurodegeneration in the basal ganglia. The molecular consequences and the bioenergetic state of affected individuals remain largely unexplored., Objectives: To investigate the bioenergetic state in male patients with XDP and female carriers using 31 phosphorus magnetic resonance spectroscopy imaging and to correlate these findings with clinical manifestations., Methods: We examined the levels of high-energy phosphorus-containing metabolites (HEP) in the basal ganglia and cerebellum of five male patients with XDP, 10 asymptomatic female heterozygous carriers, and 10 SVA-insertion-free controls., Results: HEP levels were reduced in the basal ganglia of patients with XDP (PwXDP) compared to controls, but increased in the cerebellum of both male patients and female carriers., Conclusions: Our findings suggest a potential compensatory mechanism in the cerebellum of female carriers regardless of sex. Our study highlights alterations in HEP levels in PwXDP patients and female carriers., (© 2024 International Parkinson and Movement Disorder Society.)

Ecological sanitation combined with thermophilic composting is a viable option to transform human excreta into a stabilized, pathogen-free, and nutrient-rich fertilizer. In combination with suitable bulking materials such as sawdust and straw, and additives such as biochar, this could also be a suitable waste management strategy for reducing greenhouse gas (GHG) emissions. In this study, we conducted a 143-days thermophilic composting of human excreta or cattle manure together with teff straw, organic waste, and biochar to investigate the effect that biochar has on GHG (CO 2 , N 2 O, and CH 4 ) and NH 3 emissions. The composting was performed in wooden boxes (1.5 × 1.5 × 1.4 m 3 ), GHG were measured by using a portable FTIR gas analyzer and NH 3 was sampled as ammonium in an H 2 SO 4 trap. We found that the addition of biochar significantly reduced CH 4 emissions by 91% in the cattle manure compost, and N 2 O emissions by 56%-57% in both humanure and cattle manure composts. Overall, non-CO 2 GHG emissions were reduced by 51%-71%. In contrast, we did not observe a significant biochar effect on CO 2 and NH 3 emissions. Previous data already showed that it is possible to sanitize human fecal material when using this composting method. Our results suggest that thermophilic composting with biochar addition is a safe and cost-effective waste management practice for producing a nutrient-rich fertilizer from human excreta, while reducing GHG emissions at the same time., (© 2023 The Authors. Journal of Environmental Quality published by Wiley Periodicals LLC on behalf of American Society of Agronomy, Crop Science Society of America, and Soil Science Society of America.)

The application of livestock slurry in soils can lead to nitrogen (N) losses through ammonia (NH 3 ) emission or nitrate (NO 3 ) leaching. Oxidized biochar has great potential to mitigate N losses due to its strong adsorption capacity; however, the effects of oxidized biochar in different soils treated with slurry are currently unclear. Here, we investigated the effect of untreated and oxidized biochar (applied at a rate of 50 kg C m - slurry) on reducing N losses in a laboratory experiment with three different soils (loamy sand, sandy loam, loam) amended with cattle slurry at an application rate of 73 kg N ha -3 slurry) on reducing N losses in a laboratory experiment with three different soils (loamy sand, sandy loam, loam) amended with cattle slurry at an application rate of 73 kg N ha -1 . Oxidized biochar reduced NH 3 emissions by 64-75% in all soils, whereas untreated biochar reduced NH 3 emissions by 61% only in the loamy sand. Oxidized biochar significantly reduced the NO 3 - retention in this organic C-poor soil. We conclude that oxidized biochar can reduce N losses, both in the form of NH 3 - concentration in the same soil compared with the slurry-only treatment at the end of the experiment, indicating a significant increase in NO 3 - retention in this organic C-poor soil. We conclude that oxidized biochar can reduce N losses, both in the form of NH 3 emission and NO 3 - leaching, from cattle slurry applied to soil, particularly in soil with soil organic carbon content <1% and pH <5 (i.e., oxidized biochar can serve as a means for improving the quality of marginal and acidic soils)., (© 2022 The Authors. Journal of Environmental Quality published by Wiley Periodicals LLC on behalf of American Society of Agronomy, Crop Science Society of America, and Soil Science Society of America.)

Nitrogen (N) losses during fertilization with livestock slurry, mainly in the form of ammonia (NH 3 ), can cause environmental problems and reduce fertilizer efficiency. Leonardite, which is characterized by oxygen-rich functional groups and low pH, has been found to decrease losses of slurry N. However, leonardite, as a byproduct of open-cast lignite mining, is not a renewable resource. The objective of this study was to modify biochar by chemical surface oxidation in order to find a sustainable but similarly effective substitute for leonardite. Biochar was produced from spruce sawdust in a pyrolysis oven at a maximum temperature of 610 °C. Then the biochar was oxidized using the Fenton reaction, with a ratio of Fe 2+ /H 2 O 2 of 1:1,000, as a source of highly reactive HO· radicals to introduce oxygen-rich functional groups to the biochar surface. The ammonium (NH 4 + ) adsorption capacity of biochar, oxidized biochar, and leonardite was tested in ammonium sulfate [(NH 4 ) 2 SO 4 ] solution, pH-adjusted (NH 4 ) 2 SO 4 adsorption of 1.4 mg N g 4 + adsorption of 1.4 mg N g -1 in (NH 4 ) 2 adsorption of 0.8 mg N g 4 solution, whereas oxidized biochar had the highest reversible NH 4 + adsorption of 0.8 mg N g -1 . In the pH-adjusted ammonium solution, all materials reduced NH 3 emissions by ≥90%, and oxidized biochar reduced NH 3 emissions by 99.99%. In contrast, leonardite reduced NH 3 in cattle slurry compared with non-oxidized biochar, indicating the great potential of oxidized biochar for reducing N losses during slurry application.3 emissions from 22 to 67% compared with non-oxidized biochar. In conclusion, biochar oxidized by means of the Fenton reaction greatly decreased NH 3 emissions by increased adsorption of NH 4 + in cattle slurry compared with non-oxidized biochar, indicating the great potential of oxidized biochar for reducing N losses during slurry application., (© 2022 The Authors. Journal of Environmental Quality published by Wiley Periodicals LLC on behalf of American Society of Agronomy, Crop Science Society of America, and Soil Science Society of America.)

Ecological sanitation via thermophilic composting could be a promising solution to the lack of sanitation and limited access to fertilizers, particularly in developing countries. Here, we conducted a 185-d thermophilic composting experiment with human excreta, and separately with cattle manure, mixed with kitchen scraps, teff [Eragrostis tef (Zuccagni) Trotter] straw, sawdust, and biochar (BC) by using an appropriate-technology approach. We followed the dynamics of the most important macronutrients (N, P, K), temperature, moisture, pH, electrical conductivity, cation exchange capacity, as well as content of organic matter, organic C, Ca, Mg, and micronutrients throughout the process. Low N (<47%), P (<9%), K (<11%), Ca (<18%), and Mg (<21%) losses and the temperature profile indicated a well-functioning thermophilic composting process. Compost temperature was >60 °C for 7, 6, 5, and 8 consecutive days for treatments containing human excreta, human excreta amended with BC, cattle manure, and cattle manure amended with BC, respectively, suggesting a final compost product free of pathogens. The compost mixture with cattle manure and BC reached a significantly higher temperature than the same variant without BC, with a maximum value of 65.9 °C on Day 6. For all treatments, final germination index values >100% indicated compost maturity and the absence of phytotoxic substances. Biochar addition reduced losses of organic matter (18-23%), C (33-42%), and N (49-100%) and decreased the amount of extractable NO 3 - (32-36%) in the final compost. The tested ecological sanitation concept via thermophilic composting is thus a promising strategy to improve access to cheap fertilizer by safe and sustainable sanitation and waste management., (© 2021 The Authors. Journal of Environmental Quality © 2021 American Society of Agronomy, Crop Science Society of America, and Soil Science Society of America.)

Competing Interests: The study was supported by the German Research Foundation (FOR2488), the German Research Foundation via the Clinician Scientist School Lübeck (DFG‐GEPRIS 413535489), and the Damp Foundation. The authors do not declare any conflicts of interest or competing interests.

Background: Sialorrhea is a troublesome symptom in a variety of neurological diseases. Recently, local injection of botulinum toxin into the salivary glands was approved for treatment of sialorrhea, and injection guidance by anatomical landmarks was suggested., Objective: To compare the accuracy of ultrasound versus previously proposed anatomical landmarks for localizing the salivary glands., Methods: In a cross-sectional study in 21 adults, landmark positions (LM) of the parotid gland (PG) and the submandibular gland (SG) were identified following published recommendations. The ultrasound position (US) was defined as the position representing the maximum gland thickness. The distance between positions, gland thickness, and optimal injection depth were recorded by US., Results: Gland thickness differed significantly between LM and US positions (PG, 4 vs. 17.8 mm; P < 0.001; SG, 3.5 vs. 13.6 mm; P < 0.001). The spatial deviation between the recommended LM and identified US positions in the horizontal plane was 21 mm to the posterior direction for the PG and 19.6 mm for the SG. The deviation in vertical orientation was small for both glands; however, there was a positive correlation between the distance from the SG to the mandibular bone with age. The optimal injection depth measured by US was 11.8 mm for the PG and 13.6 mm for the SG. This showed to be positively correlated with the body mass index., Conclusions: The position of the salivary glands differs from proposed landmarks and depends on the individual age and body weight; therefore, we recommend ultrasound guidance for injection., (© 2019 International Parkinson and Movement Disorder Society.)

The progressive neurodegenerative process in Parkinson's disease (PD) is not restricted to dopaminergic midbrain neurons but involves the entire nervous system. In this review, we outline established treatment options at different disease stages and address new therapeutic approaches. These include, based on recent advances in the understanding of the pathophysiology of PD, genetic and disease-modifying approaches to reduce abnormal accumulation and aggregation of alpha-synuclein (aSYN), mitochondrial dysfunction, and dysfunction of lysosomal proteins. Moreover, we highlight clinical trials to reduce neuroinflammation and increase neurorestoration., (© 2019 The Authors Clinical Pharmacology & Therapeutics © 2019 American Society for Clinical Pharmacology and Therapeutics.)

The execution of coordinated hand movements requires complex interactions between premotor and primary motor areas in the two hemispheres. The supplementary motor area (SMA) is involved in movement preparation and bimanual coordination. How the SMA controls bimanual coordination remains unclear, although there is evidence suggesting that the SMA could modulate interhemispheric interactions. With a delayed-response task, we investigated interhemispheric interactions underlying normal movement preparation and the role of the SMA in these interactions during the delay period of unimanual or bimanual hand movements. We used functional MRI and transcranial magnetic stimulation in 22 healthy volunteers (HVs), and then in two models of SMA dysfunction: (a) in the same group of HVs after transient disruption of the right SMA proper by continuous transcranial magnetic theta-burst stimulation; (b) in a group of 22 patients with congenital mirror movements (CMM), whose inability to produce asymmetric hand movements is associated with SMA dysfunction. In HVs, interhemispheric connectivity during the delay period was modulated according to whether or not hand coordination was required for the forthcoming movement. In HVs following SMA disruption and in CMM patients, interhemispheric connectivity was modified during the delay period and the interhemispheric inhibition was decreased. Using two models of SMA dysfunction, we showed that the SMA modulates interhemispheric interactions during movement preparation. This unveils a new role for the SMA and highlights its importance in coordinated movement preparation., (© 2019 Wiley Periodicals, Inc.)

Understanding environmental and physiological controls of the variations in δ(13) C of CO(2) respired (δ(13) C(R)) from different compartments of an ecosystem is important for separation of CO(2) fluxes and to assess coupling between assimilation and respiration. In a wheat field, over 3 days we characterised the temporal dynamics of δ(13) C(R) from shoots and roots, from the soil and from the whole agroecosystem. To evaluate the basis of potential variations in δ(13) C(R), we also measured δ(13) C in different organic matter pools, as well as meteorological and gas exchange parameters. We observed strong diel variations up to ca. 6% in shoot, root and soil δ(13) C(R), but not in δ(13) C of the putative organic substrates for respiration, which varied by not more than ca. 1% within 24 h. Whole ecosystem-respired CO(2) was least depleted in (13) C in the afternoon and most negative in the early morning. We assume that temporally variable respiratory carbon isotope fractionation and changes in fluxes through metabolic pathways, rather than photosynthetic carbon isotope fractionation, governs the δ(13) C of respired CO(2) at the diel scale, and thus provides insights into the metabolic processes related to respiration under field conditions., (© 2010 German Botanical Society and The Royal Botanical Society of the Netherlands.)