A Clinical Applicable Smartwatch Application for Measuring Hyperkinetic Movement Disorder Severity

Measuring the severity of hyperkinetic movement disorders like tremor and myoclonus is challenging. Although many accelerometers are available to quantify movements, the vast majority lacks real-time analysis and an interface that makes it possible to real-time adjust therapy like deep brain stimulation (DBS). Here, we developed a smartwatch / smartphone application that is capable of real-time analysing movement disorder severity. Movement analysis was realised by integrating acceleration values, to velocity and subsequently to distance. Measured distances were compared with a validated accelerometer already applied for quantifying movement disorders. Further validation was done by quantitative assessment of simulated movement disorders in 10 healthy volunteers. Finally, the approach was tested in two patients treated with DBS to quantify the effect of different DBS settings on myoclonus and tremor severity, respectively. The distance measured with the application had a 96% accuracy. This was non-inferior (p = 0.76) compared to accelerometers already clinically applied. Furthermore, (simulated) movement disorder severity could be classified correctly in 93% of the cases. Finally, the method was capable of distinguishing effective from non-effective DBS parameters in two patients. In summary, with our approach we realised an instantaneous and reliable estimation of the severity of movement disorders which can assist in real time titrating therapy like DBS.</p

Cruise report hydro acoustic survey for blue whiting (Micromesistius poutassou) with R.V. Tridens, 17 March - 04 April 2008

This is the report of the Dutch part of the international North East Atlantic hydro acoustic survey for blue whiting. The survey is coordinated by ICES and has been executed annually. The purpose of the survey is to estimate the blue whiting stock of the North East Atlantic. The ICES uses this estimation is as a “tuning index” to assess the North East Atlantic blue whiting stock. The applied method was echo integration. By sailing transects over the survey area, the total acoustic cross-section can be calculated by surface area sampled. Trawling identified species composition of localized schools. The length composition of each species was determined. Blue whiting was examined on age and fecundity from which a split up stock structure was mad

Natural course of Myoclonus-Dystonia in adulthood: stable motor signs but increased psychiatry

Myoclonus‐dystonia (M‐D) is a rare hyperkinetic movement disorder characterized by upper body–predominant myoclonus and dystonia.1 A large proportion of cases are caused by autosomal‐dominant inherited mutations in the SGCE gene. In addition to the motor manifestations, psychiatric disorders are frequently reported.2 Several studies have suggested that they may form a primary component of the M‐D phenotype.3, 4 This study represents the first long‐term follow‐up study of both motor and psychiatric symptomatology in adults with M‐D (SGCE mutation), providing further insights into the natural history of M‐D and enabling more prognostic information

Functional Jerky Movements

Functional jerky movements (or functional myoclonus) are commonly seen in patients with functional movement disorder. Positive features both from history and clinical examination are important for the diagnosis. However, due to their heterogeneous and paroxysmal nature a home-made video-recording of the jerky movements can be essential to make the diagnose by a movement disorder specialist. Supportive clinical clues include abrupt symptom onset often triggered by a physical event, whereas entrainment and distractibility are supportive signs during physical examination. Localization of the jerky movements, especially proximal localization, is supportive, and can also be helpful in distinguishing functional jerks from tics and myoclonus, which are the most important differential diagnostic considerations. Additional neurophysiological tests include polymyography and electroencephalography-electromyography (EEG-EMG) co-registration in order to demonstrate a readiness potential (RP). Management includes disease education and specialized physical therapy

New developments in diagnostics and treatment of adult-onset focal dystonia

Purpose of reviewThe aim of this review is to showcase the recent developments in the field of diagnosis and treatment of adult-onset focal dystonia.Recent findingsAccurate phenotyping of focal dystonia is essential in the process of finding an underlying cause, including acquired, genetic, and idiopathic causes. Motor symptoms as well as the associated nonmotor symptoms and their detrimental impact on quality of life have received increased interest over the last years. The diagnostic process is complicated by the steadily increasing numbers of newly discovered genes associated with dystonia. Recent efforts have been aimed at further developing recommendations and algorithms to aid in diagnosis and in navigating the use of diagnostic tools. In terms of treatment, research on DBS is advancing towards a better understanding of the most effective stimulation locations within the globus pallidus. Moreover, with the introduction of the LFP-recording devices, the search continues for an accurate electrophysiological biomarker for dystonia.SummaryAccurate phenotyping and (sub)classification of patients with dystonia is important for improving diagnosis, subsequent treatment effect and population-based study outcomes in research. Medical practitioners should be attentive to the presence of nonmotor symptoms in dystonia.</p

New developments in diagnostics and treatment of adult-onset focal dystonia

Purpose of reviewThe aim of this review is to showcase the recent developments in the field of diagnosis and treatment of adult-onset focal dystonia.Recent findingsAccurate phenotyping of focal dystonia is essential in the process of finding an underlying cause, including acquired, genetic, and idiopathic causes. Motor symptoms as well as the associated nonmotor symptoms and their detrimental impact on quality of life have received increased interest over the last years. The diagnostic process is complicated by the steadily increasing numbers of newly discovered genes associated with dystonia. Recent efforts have been aimed at further developing recommendations and algorithms to aid in diagnosis and in navigating the use of diagnostic tools. In terms of treatment, research on DBS is advancing towards a better understanding of the most effective stimulation locations within the globus pallidus. Moreover, with the introduction of the LFP-recording devices, the search continues for an accurate electrophysiological biomarker for dystonia.SummaryAccurate phenotyping and (sub)classification of patients with dystonia is important for improving diagnosis, subsequent treatment effect and population-based study outcomes in research. Medical practitioners should be attentive to the presence of nonmotor symptoms in dystonia.</p

New developments in diagnostics and treatment of adult-onset focal dystonia

Purpose of reviewThe aim of this review is to showcase the recent developments in the field of diagnosis and treatment of adult-onset focal dystonia.Recent findingsAccurate phenotyping of focal dystonia is essential in the process of finding an underlying cause, including acquired, genetic, and idiopathic causes. Motor symptoms as well as the associated nonmotor symptoms and their detrimental impact on quality of life have received increased interest over the last years. The diagnostic process is complicated by the steadily increasing numbers of newly discovered genes associated with dystonia. Recent efforts have been aimed at further developing recommendations and algorithms to aid in diagnosis and in navigating the use of diagnostic tools. In terms of treatment, research on DBS is advancing towards a better understanding of the most effective stimulation locations within the globus pallidus. Moreover, with the introduction of the LFP-recording devices, the search continues for an accurate electrophysiological biomarker for dystonia.SummaryAccurate phenotyping and (sub)classification of patients with dystonia is important for improving diagnosis, subsequent treatment effect and population-based study outcomes in research. Medical practitioners should be attentive to the presence of nonmotor symptoms in dystonia.</p

New developments in diagnostics and treatment of adult-onset focal dystonia

Purpose of reviewThe aim of this review is to showcase the recent developments in the field of diagnosis and treatment of adult-onset focal dystonia.Recent findingsAccurate phenotyping of focal dystonia is essential in the process of finding an underlying cause, including acquired, genetic, and idiopathic causes. Motor symptoms as well as the associated nonmotor symptoms and their detrimental impact on quality of life have received increased interest over the last years. The diagnostic process is complicated by the steadily increasing numbers of newly discovered genes associated with dystonia. Recent efforts have been aimed at further developing recommendations and algorithms to aid in diagnosis and in navigating the use of diagnostic tools. In terms of treatment, research on DBS is advancing towards a better understanding of the most effective stimulation locations within the globus pallidus. Moreover, with the introduction of the LFP-recording devices, the search continues for an accurate electrophysiological biomarker for dystonia.SummaryAccurate phenotyping and (sub)classification of patients with dystonia is important for improving diagnosis, subsequent treatment effect and population-based study outcomes in research. Medical practitioners should be attentive to the presence of nonmotor symptoms in dystonia.</p

The auditory startle response in relation to outcome in functional movement disorders

Background: The auditory startle reflex (ASR) is enlarged in patients with functional movement disorders (FMD). Objectives: To study whether the ASR relates to symptom reduction in FMD patients, who participated in a placebo controlled double blind treatment trial with Botulinum Neurotoxin (BoNT). Methods: Response to treatment in the BoNT study was assessed using the Clinical Global Impression - Improvement scale (CGI-I). The electromyography (EMG) muscle activity of 7 muscles following 110 dB tones was measured in 14 FMD patients before and after one-year treatment and compared to 11 matched controls. The early and a late (behaviorally affected) component of the ASR and the sympathetic skin response (SSR) were assessed. Results: 10 of 14 patients (71.4%) showed symptom improvement, which was believed to be mainly caused by placebo effects. The early total response probability of the ASR at baseline tended to be larger in patients compared to controls (p = 0.08), but normalized at follow-up (p = 0.84). The late total response probability was larger in patients vs. controls at baseline (p < 0.05), a trend that still was present at follow-up (p = 0.08). The SSR was higher in patients vs. controls at baseline (p < 0.01), and normalized at follow-up (p = 0.71). Conclusions: On a group level 71.4% of the patients showed clinical symptom improvement after treatment. The early part of the ASR, most likely reflecting anxiety and hyperarousal, normalized in line with the clinical improvement. Interestingly, the augmented late component of the ASR remained enlarged suggesting persistent altered behavioral processing in functional patients despite motor improvement