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Additional file 3. SPIRIT Checklist for Trials.

Additional file 2: Supplementary Table 1. Parent Study schedule of enrolment, interventions, and assessments. Supplementary Table 2. Extension Phase schedule of enrolment, interventions, and assessments.

Additional file 1: Supplementary Material I. Safety Parameters. Supplementary Material II. Data Collection.

Objective: To compare the efficacy of the Sémont maneuver (SM) with the new "SémontPLUS maneuver" (SM+) in patients with posterior canal BPPV canalolithiasis (pcBPPVcan). Methods and Patients: In a prospective trinational (Germany, Italy, and Belgium) randomized trial, patients with pcBPPVcan were randomly assigned to SM or SM+; SM+ means overextension of the head by 60+° below earth horizontal line during the movement of the patient toward the affected side. The first maneuver was done by the physician, and the subsequent maneuvers by the patients 9 times/day on their own. Each morning the patient documented whether vertigo could be induced. The primary endpoints were: "How long (in days) does it take until no attacks can be induced?" and "What is the efficacy of a single SM/SM+?" Results: In the 194 patients analyzed (96 SM, 98 SM+), it took 2 days (median, range 1-21 days, mean 3.6 days) for recovery with SM and 1 day (median, range 1-8 days, mean 1.8 days) with SM+ (p = 0.001, Mann-Whitney U-test). There was no difference in the second primary endpoint (chi2-test, p = 0.39). Interpretation: This prospective trial shows that SM+ is more effective than SM when repeated therapeutic maneuvers are performed but not when a single maneuver is performed. It also supports the hypothesis of the biophysical model: overextension of the head during step 2 brings the clot of otoconia beyond the vertex of the canal, which increases the effectivity. Classification of Evidence: This study provides Class I evidence that SM+ is superior to SM for multiple treatment maneuvers of pcBPPVcan.

BACKGROUND AND PURPOSE: The diagnosis of cerebral venous and sinus thrombosis (CVST) as a rare but important cause of stroke is challenging. We aimed to investigate the diagnostic value of multidetector-row CT angiography (MDCTA) as a fast and cost-effective imaging tool in diagnosing CVST. MATERIALS AND METHODS: Nineteen patients who presented with clinical symptoms of a possible CVST were included. All patients had received both MDCTA and MR imaging with venous MR-angiography. Three blinded readers were asked to identify the cerebral sinuses and veins in MDCTA and to evaluate the presence of CVST in MDCTA. Consensus reading with interpretation of the MR imaging served to establish the definite diagnosis. RESULTS: The consensus reading revealed CVST in 10 of the 19 patients. With MDCTA, the venous sinuses could be identified in 99.2% and the cerebral veins in 87.6% of cases. The sensitivity and specificity of MDCTA for the diagnosis of CVST were 100%. CONCLUSION: Our study demonstrates that MDCTA provides excellent sensitivity and specificity for the diagnosis of CVST. Further studies are needed to evaluate the diagnostic potential of MDCTA in specific subsets of the general entity of CVST such as cortical venous thrombosis, thrombosis of the cavernous sinus, and thrombosis of the internal cerebral veins.

Objective: to delineate the full phenotypic spectrum, discriminative features, piloting longitudinal progression data, and sample size calculations of replication factor complex subunit 1 (RFC1) repeat expansions, recently identified as causing cerebellar ataxia, neuropathy, vestibular areflexia syndrome (CANVAS). Methods: multimodal RFC1 repeat screening (PCR, Southern blot, whole-exome/genome sequencing-based approaches) combined with cross-sectional and longitudinal deep phenotyping in (1) cross-European cohort A (70 families) with ≥2 features of CANVAS or ataxia with chronic cough (ACC) and (2) Turkish cohort B (105 families) with unselected late-onset ataxia. Results: prevalence of RFC1 disease was 67% in cohort A, 14% in unselected cohort B, 68% in clinical CANVAS, and 100% in ACC. RFC1 disease was also identified in Western and Eastern Asian individuals and even by whole-exome sequencing. Visual compensation, sensory symptoms, and cough were strong positive discriminative predictors (>90%) against RFC1-negative patients. The phenotype across 70 RFC1-positive patients was mostly multisystemic (69%), including dysautonomia (62%) and bradykinesia (28%) (overlap with cerebellar-type multiple system atrophy [MSA-C]), postural instability (49%), slow vertical saccades (17%), and chorea or dystonia (11%). Ataxia progression was ≈1.3 Scale for the Assessment and Rating of Ataxia points per year (32 cross-sectional, 17 longitudinal assessments, follow-up ≤9 years [mean 3.1 years]) but also included early falls, variable nonlinear phases of MSA-C-like progression (SARA points 2.5-5.5 per year), and premature death. Treatment trials require 330 (1-year trial) and 132 (2-year trial) patients in total to detect 50% reduced progression. Conclusions: RFC1 disease is frequent and occurs across continents, with CANVAS and ACC as highly diagnostic phenotypes yet as variable, overlapping clusters along a continuous multisystemic disease spectrum, including MSA-C-overlap. Our natural history data help to inform future RFC1 treatment trials., European Union (EU); Horizon 2020; Research and Innovation Program; BMBF; E-Rare-3 network; PREPARE; DFG; EJP-RD network; PROSPAX; Solve-RD; University of Tubingen Medical Faculty; Clinician Scientist Program; Medical Research Council; Fondazione CARIPLO; ZonMW; Hersenstichting; Gossweiler Foundation; uniQure; Radboud University Medical Centre; Suna and İnan Kıraç Foundation; Koç University School of Medicine

Objective: Primary objective of this study was to find a statistical link between the most worldwide comorbidities affecting the elderly population (hypertension, diabetes, osteoarthrosis, osteoporosis and depression) and recurrent episodes of BPPV. Secondary objective was defining possible "groups of risk" for people suffering recurrent positional vertigo related to the presence of a well documented comorbidity. Methods: This was an observational, cross-sectional, multicenter, spontaneous, non-pharmacological study. The data of 1092 patients suffering BPPV evaluated in 11 different Departments of Otolaryngology, Otoneurology and Neurology, referring Centers for positional vertigo evaluation, were retrospectively collected. Results: Regarding evaluated comorbidities (hypertension, diabetes, osteoarthrosis, osteoporosis and depression), data analysis showed the presence of at least one comorbid disorder in 216 subjects (19.8%) and 2 or more in 408 subjects (37.4%). Moreover there was a statistical significant difference between the number of comorbidities and the number of recurrences, otherwise said as comorbidity disorders increased the number of relapses increased too. Conclusion: The presence of a systemic disease may worsen the status of the posterior labyrinth causing a more frequent otolith detachment. This condition increases the risk for patients suffering BPPV to have recurrent episodes, even if correctly managed by repositioning maneuvers. The combination of two or more of aforementioned comorbidities further increases the risk of relapsing BPPV, worsened by the presence of osteoporosis. On the basis of this results it was possible to define "groups of risk" useful for predicting BPPV recurrence in patients with one or more comorbidity. © 2013.

We thought it might be useful to update our readers, authors and reviewers on some new developments in the Journal of Neurology (JoN). As many of you know the JoN has a long history and in recent times it has been linked to the European Neurological Society (ENS). Efforts continue to focus on not only further development of the JoN but also our position with respect to other neurological journals. As such, we thought this might be a useful time to rethink some aspects of the JoN to try and improve on its already impressive achievements to date. This is becoming more of an issue as the popularity of the journal increases with more than 1,500 submissions in 2012 compared to 917 in 2009. This has also brought about a higher rejection rate for manuscripts, 82 % in 2012 compared to 66 % in 2009, but also has necessitated a faster editorial turnaround time for making a final decision on a paper which has dropped from 60 days in 2009 to 23 days in 2012. In order to try and improve on this, we have decided to provide some further guidelines to help authors and reviewers. For those wishing to submit papers, we have decided that the article should be relevant to clinical neurology and, as such, manuscripts on experimental studies in animal models of disease will not be considered by JoN. In addition, the clinical studies should in some way move the field forward, be clinically relevant and well designed, i.e., preferably prospective randomized controlled trials; underpowered, anecdotal studies will have less chance of being published. Further, Review Articles on clinical relevant topics are very welcome and highly appreciated by the readership. We are also keen to get papers through the review process as fast as possible and publish them in a timely and attractive fashion. We are therefore asking referees to review the paper within 14 days (not 21 as it is at present) and in cases where the paper needs to be revised, whether it also needs to be re-reviewed. We are also asking authors to ensure that their paper is no longer than 4,000 words with a maximum of five figures or tables and 40 references. We are also hoping to make the titles of the papers shorter and more precise, and restructure the contents page to make it more obvious what category of sub-speciality each paper falls within in every issue of the Journal. In terms of new additions to the JoN, we have decided to have a new section called ‘‘Neurological Progress’’ and this in part will replace the ‘‘Medical Progress Section’’ that we have been featuring for the last two years. This will contain short reviews on topics that have either been the subject of several papers within the JoN in recent times or been the subject of a recent workshop or meeting. These articles will be invited and we are hopeful that they will be published very quickly so that our readers will be informed of new developments and ideas in the world of neurology. The section ‘‘Techniques in Clinical Science’’ will be enhanced; the new editor responsible for this section will be Massimo Filippi. R. A. Barker (&) Cambridge Centre for Brain Repair, University of Cambridge, The E.D. Adrian Building, Forvie Site, Robinson Way, Cambridge CB2 2PY, UK e-mail: rab46@cam.ac.uk