Benign Idiopathic Myoclonus: A New Clinical Entity?

Background: Myoclonus is a brief shock-like, involuntary movement, which can be distinguished in physiologic, essential, epileptic, and symptomatic, according to its etiology. Physiologic myoclonus typically occurs in healthy people without disability or progression.

Objectives: We suggest a new nosological entity in the physiologic group: "benign idiopathic myoclonus.

Tremor after solid organ transplantation: Results from the TransplantLines Biobank and Cohort Study.

Background And Purpose: Tremor is a frequent complaint of solid organ transplant recipients. We report on the largest population investigated with clinical neurophysiological methods. Our aim is to objectively establish the tremor prevalence and syndrome in the largest population of solid organ transplant recipients.

Next move in movement disorders: neuroimaging protocols for hyperkinetic movement disorders.

Introduction: The Next Move in Movement Disorders (NEMO) study is an initiative aimed at advancing our understanding and the classification of hyperkinetic movement disorders, including tremor, myoclonus, dystonia, and myoclonus-dystonia. The study has two main objectives: (a) to develop a computer-aided tool for precise and consistent classification of these movement disorder phenotypes, and (b) to deepen our understanding of brain pathophysiology through advanced neuroimaging techniques. This protocol review details the neuroimaging data acquisition and preprocessing procedures employed by the NEMO team to achieve these goals.

Double lesion MRgFUS thalamotomy for essential tremor: 4.5-year outcomes and framework for assessing loss of efficacy and tremor progression.

Background: The essential tremor (ET) course to 54 months post-unilateral VIM/PSA magnetic resonance-guided focused ultrasound (MRgFUS) in the treated arm (TA) and non-treated arm (NTA) of 12 patients is reported.

Methods: Tremor severity was rated using Bain Findley spirography (BFS) scores in the TA and NTA. We divided follow-up into 'Early' (0-6 months) and 'Late' (6-54 months) phases, to minimise the effect of peri-lesion oedema resolution on the latter.

Testing for Alcohol Responsiveness in Familial Essential Tremor.

Background: Essential tremor (ET) is the most common movement disorder in adults and is considered to be highly heritable. A reduction of the tremor amplitude after alcohol consumption is reported in approximately half of the patients. In this study, we describe the alcohol response in our familial ET cohort by employing an alcohol responsivity test designed by Knudsen et al.

Lack of effect of deep brain stimulation on resting-state functional connectivity in essential tremor provides valuable insights.

This scientific commentary refers to 'Deep brain stimulation does not modulate resting-state functional connectivity in essential tremor', by Awad et al. (https://doi.org/10.

Clinical neurophysiology of functional motor disorders: IFCN Handbook Chapter.

Functional Motor Disorders are common and disabling. Clinical diagnosis has moved from one of exclusion of other causes for symptoms to one where positive clinical features on history and examination are used to make a "rule in" diagnosis wherever possible. Clinical neurophysiological assessments have developed increasing importance in assisting with this positive diagnosis, not being used simply to demonstrate normal sensory-motor pathways, but instead to demonstrate specific abnormalities that help to positively diagnose these disorders.

A Novel Accelerometry Method to Perioperatively Quantify Essential Tremor Based on Fahn-Tolosa-Marin Criteria.

The disease status, progression, and treatment effect of essential tremor (ET) patients are currently assessed with clinical scores, such as the Fahn-Tolosa-Marin Clinical Rating Scale for Tremor (FTM). The use of objective and rater-independent monitoring of tremors may improve clinical care for patients with ET. Therefore, the focus of this study is to develop an objective accelerometry-based method to quantify ET, based on FTM criteria.

Tremor, Daily Functioning, and Health-Related Quality of Life in Solid Organ Transplant Recipients.

Solid organ transplant recipients (SOTR) frequently report tremor. Data concerning tremor-related impairment and its potential impact on health-related quality of life (HRQoL) are lacking. This cross-sectional study assesses impact of tremor on activities of daily living and HRQoL using validated questionnaires among SOTR enrolled in the TransplantLines Biobank and Cohort Study.

Early Onset Dystonia: Complaints about Executive Functioning, Depression and Anxiety.

Early Onset Dystonia (EOD) is thought to result from basal ganglia dysfunction, structures also involved in non-motor functions, like regulation of behavior, mood and anxiety. Problems in these domains have been found in proxy-reports but not yet in self-reports of EOD patients. The main questions are whether proxy-reports differ from those of patients and how problems relate to everyday social functioning.

Tremor assessment scales before, during and after MRgFUS for essential tremor - results, recommendations and implications.

Background: neurosurgical interventions such as MR-guided focused ultrasound (MRgFUS) are increasingly deployed for treatment of essential tremor.

Objective: to make recommendations for monitoring treatment effects during and after MRgFUS based on our investigation of correlations between different scales of tremor severity.

Methods: twenty-five clinical assessments were collected from thirteen patients before and after unilateral MRgFUS sequential lesioning of the thalamus and posterior subthalamic area for alleviating essential tremor.

Altered brain connectivity in hyperkinetic movement disorders: A review of resting-state fMRI.

Background: Hyperkinetic movement disorders (HMD) manifest as abnormal and uncontrollable movements. Despite reported involvement of several neural circuits, exact connectivity profiles remain elusive.

Objectives: Providing a comprehensive literature review of resting-state brain connectivity alterations using resting-state fMRI (rs-fMRI).

Electrophysiological testing aids the diagnosis of tremor and myoclonus in clinically challenging patients.

Objective: We investigated how clinical neurophysiological testing can help distinguish tremor and myoclonus and their subtypes.

Methods: We retrospectively analysed clinical and neurophysiological data from patients who had undergone polymyography (EMG + accelerometry) to diagnose suspected tremor or myoclonus. We show a systematic approach, which includes contraction pattern, rhythm regularity, burst duration and evidence of cortical drive.

Next move in movement disorders (NEMO): developing a computer-aided classification tool for hyperkinetic movement disorders.

Introduction: Our aim is to develop a novel approach to hyperkinetic movement disorder classification, that combines clinical information, electromyography, accelerometry and video in a computer-aided classification tool. We see this as the next step towards rapid and accurate phenotype classification, the cornerstone of both the diagnostic and treatment process.

Methods And Analysis: The Next Move in Movement Disorders (NEMO) study is a cross-sectional study at Expertise Centre Movement Disorders Groningen, University Medical Centre Groningen.

The diagnostic value of clinical neurophysiology in hyperkinetic movement disorders: A systematic review.

Introduction: To guide the neurologist and neurophysiologist with interpretation and implementation of clinical neurophysiological examinations, we aim to provide a systematic review on evidence of electrophysiological features used to differentiate between hyperkinetic movement disorders.

Methods: A PRISMA systematic search and QUADAS quality evaluation has been performed in PubMed to identify diagnostic test accuracy studies comparing electromyography and accelerometer features. We included papers focusing on tremor, dystonia, myoclonus, chorea, tics and ataxia and their functional variant.

Locked-In Syndrome after Traumatic Basilar Artery Entrapment within a Clivus Fracture: A Case Report and Review of the Literature.

We report the case of a 58-year-old male with a rare vascular complication after traumatic head injury: entrapment of the basilar artery into a fracture of the clivus, ultimately leading to a locked-in syndrome due to brainstem infarction. Review of the literature revealed 19 earlier published cases of basilar artery entrapment within traumatic longitudinal clival fractures. In the majority of these patients there is an unfavorable neurological outcome.

The chronnectome as a model for Charcot's 'dynamic lesion' in functional movement disorders.

This exploratory study set out to investigate dynamic functional connectivity (dFC) in patients with jerky and tremulous functional movement disorders (JT-FMD). The focus in this work is on dynamic brain states, which represent distinct dFC patterns that reoccur in time and across subjects. Resting-state fMRI data were collected from 17 patients with JT-FMD and 17 healthy controls (HC).

Systematic clinical approach for diagnosing upper limb tremor.

Tremor is the most common movement disorder worldwide, but diagnosis is challenging. In 2018, the task force on tremor of the International Parkinson and Movement Disorder Society published a consensus statement that proposes a tremor classification along two independent axes: a clinical tremor syndrome and its underlying aetiology. In line with this statement, we here propose a stepwise diagnostic approach that leads to the correct clinical and aetiological classification of upper limb tremor.

Myoclonus-dystonia: Distinctive motor and non-motor phenotype from other dystonia syndromes.

Background: Myoclonus-dystonia (M-D) due to a pathogenic variant of SGCE is an autosomal dominant inherited movement disorder. Apart from motor symptoms, psychiatric disorders are highly prevalent in patients with M-D. Previous studies suggest, but never tested directly, that the type of psychiatric disorder differs between dystonia syndromes, probably related to disease specific pathology.

Which disease features run in essential tremor families? A systematic review.

Essential tremor is a common and highly heritable movement disorder. It is largely unknown, however, to what extent family members share overlapping symptoms. Such knowledge would be useful, as it may lead to the definition of familial essential tremor phenotypes, which will aid the ongoing search for genotypes.

Similar association between objective and subjective symptoms in functional and organic tremor.

Background: A previous study reported a dramatic mismatch in objectively detected and self-reported tremor duration in patients with functional tremor. As these findings have an important and widespread impact in both clinical care and research, we conducted a validation study with a longer study duration and a larger sample of patients.

Methods: Fourteen patients with functional tremor and 19 with organic tremor completed a 30 day study period.

Wavelet coherence analysis: A new approach to distinguish organic and functional tremor types.

Objective: To distinguish tremor subtypes using wavelet coherence analysis (WCA). WCA enables to detect variations in coherence and phase difference between two signals over time and might be especially useful in distinguishing functional from organic tremor.

Methods: In this pilot study, polymyography recordings were studied retrospectively of 26 Parkinsonian (PT), 26 functional (FT), 26 essential (ET), and 20 enhanced physiological (EPT) tremor patients.

How typical are 'typical' tremor characteristics? Sensitivity and specificity of five tremor phenomena.

Introduction: Distinguishing between different tremor disorders can be challenging. Some tremor disorders are thought to have typical tremor characteristics: the current study aims to provide sensitivity and specificity for five 'typical' tremor phenomena.

Methods: Retrospectively, we examined 210 tremor patients referred for electrophysiological recordings between January 2008 and January 2014.

Bilateral cerebellar activation in unilaterally challenged essential tremor.

Background: Essential tremor (ET) is one of the most common hyperkinetic movement disorders. Previous research into the pathophysiology of ET suggested underlying cerebellar abnormalities.

Objective: In this study, we added electromyography as an index of tremor intensity to functional Magnetic Resonance Imaging (EMG-fMRI) to study a group of ET patients selected according to strict criteria to achieve maximal homogeneity.

Cerebellar Atrophy in Cortical Myoclonic Tremor and Not in Hereditary Essential Tremor-a Voxel-Based Morphometry Study.

Essential tremor (ET) presumably has a cerebellar origin. Imaging studies showed various cerebellar and also cortical structural changes. A number of pathology studies indicated cerebellar Purkinje cell pathology.