Multimodal neuroimaging of fatigability development.

Fatigability refers to the inability of the neuromuscular system to generate enough force to produce movements to meet task challenges. Fatigability has a central and a peripheral component linked via the neuromuscular system, but how these two components interact as fatigue develops lacks a complete understanding. The effects of fatigability are experienced in healthy humans but also accompany various disorders, often exacerbating their symptoms.

Patterns of brain activity in choice or instructed go and no-go tasks.

The goal of this study was to investigate the decision making process for choosing what movements to make. We used electroencephalography (EEG) to investigate patterns of the contingent negative variation (CNV) associated with free-choice decisions to move or abstain, comparing them to conditions where actions were commanded. Our primary hypothesis was that choice tasks would differ significantly from each other and exhibit EEG patterns akin to their command-driven counterparts after the decisions were made, at least, in the 50 ms block of time prior to movement.

A randomized feasibility trial of medium chain triglyceride-supplemented ketogenic diet in people with Parkinson's disease.

Background: A ketogenic diet (KD) may benefit people with neurodegenerative disorders marked by mitochondrial depolarization/insufficiency, including Parkinson's disease (PD).

Objective: Evaluate whether a KD supplemented by medium chain triglyceride (MCT-KD) oil is feasible and acceptable for PD patients. Furthermore, we explored the effects of MCT-KD on blood ketone levels, metabolic parameters, levodopa absorption, mobility, nonmotor symptoms, simple motor and cognitive tests, autonomic function, and resting-state electroencephalography (rsEEG).

Deep phenotyping of post-infectious myalgic encephalomyelitis/chronic fatigue syndrome.

Post-infectious myalgic encephalomyelitis/chronic fatigue syndrome (PI-ME/CFS) is a disabling disorder, yet the clinical phenotype is poorly defined, the pathophysiology is unknown, and no disease-modifying treatments are available. We used rigorous criteria to recruit PI-ME/CFS participants with matched controls to conduct deep phenotyping. Among the many physical and cognitive complaints, one defining feature of PI-ME/CFS was an alteration of effort preference, rather than physical or central fatigue, due to dysfunction of integrative brain regions potentially associated with central catechol pathway dysregulation, with consequences on autonomic functioning and physical conditioning.

Objective response to ethanol in essential tremor: results from a standardized ethanol challenge study.

Background And Objectives: Ethanol has been reported to improve tremor severity in approximately two thirds of patients with essential tremor (ET), but the accuracy of that proportion is not certain and the mechanism of action is unknown. The goal of this study was to investigate alcohol response on tremor by applying an a priori objective response definition and subsequently to describe the responder rate to a standardized ethanol dose in a cohort of 85 ET patients. A secondary analysis evaluated other tremor and nontremor features, including demographics, tremor intensity, breath alcohol concentration, nontremor effects of alcohol, self-reported responder status to ethanol, and prior ethanol exposure.

Electrophysiological Characterization of a Variant with Tremor Phenotype.

Background: The concept of a myopathy with associated tremor ("myogenic tremor") in humans has been previously described for specific (Myosin-Binding Protein C) variants. Here we report for the first time an individual with tremor who was found to have a de-novo likely pathogenic variant in Myosin Heavy Chain 7 (MYH7

Unlabelled: We provide a detailed electrophysiological characterization of the tremor syndrome in a human individual with a myopathy and this pathogenic MYH7 variant to provide further insight in the phenotypic spectrum and pathomechanism of myogenic tremors in skeletal sarcomeric myopathies.

Methods: Electromyographic recordings were obtained from facial muscles, as well as bilateral upper and lower extremities.

How to Do an Electrophysiological Study of Myoclonus.

Diagnosing and characterizing myoclonus can be challenging. Many authors agree on the need to complement the clinical findings with an electrophysiological study to characterize the movements. Besides helping to rule out other movements that may look like myoclonus, electrophysiology can help localize the source of the movement.

Continuous Monitoring of Essential Tremor: Standards and Challenges.

Background: The value of continuous symptom monitoring in people with essential tremor is uncertain.

Objectives: To determine the relationship between tremor amplitude measured with wearable inertial sensors and clinician- and patient-rated measures.

Methods: For 14 days, patients (1) wore inertial sensors on both wrists, (2) self-rated their tremor using a diary, (3) drew spirals, and (4) completed an activities of daily living scale once daily.

Identifying transcranial magnetic stimulation induced EEG signatures of different neuronal elements in primary motor cortex.

Objective: To investigate the neuronal elements involved in the activation of corticospinal neurons in the primary motor cortex (M1).

Methods: We studied 10 healthy subjects. Cortical evoked potentials with different components induced by monophasic transcranial magnetic stimulation (TMS) in anterior-posterior and posterior-anterior currents recorded with electroencephalography (EEG) were analyzed.

Myoclonus generators in sialidosis.

Objective: Sialidosis is an inborn error of metabolism. There is evidence that the myoclonic movements observed in this disorder have a cortical origin, but this mechanism does not fully explain the bilaterally synchronous myoclonus activity frequently observed in many patients. We present evidence of a subcortical basis for synchronous myoclonic phenomena.

Physiology of Tremor Reduction by Putting the Hands Together in Essential Tremor.

Background: Essential tremor is a common movement disorder, characterized by 4-12 Hz tremor of the hands and arms that can affect many activities of daily living. It has been reported by patients that when performing tasks bimanually their tremor is reduced, but why this happens is unknown.

Objectives: We measured patients' tremors in different conditions when performed with 1 hand and 2 hands to observe if bimanual task performance changes the characteristics of the tremor.

KCNN2 Mutation in Pediatric Tremor Myoclonus Dystonia Syndrome with Electrophysiological Evaluation.

Background: Here we combine clinical, electrophysiological, and genetic findings to phenotype an unusual childhood movement disorder in a patient with a rare form of KCNN2 mutation.

Case Report: A 10-year-old male presented with a clinical syndrome of tremor and myoclonus. Electrophysiology demonstrated muscle activity indicative of myoclonus dystonia, an observation that was not appreciated clinically.

In vivo assessment of neurodegeneration in Spinocerebellar Ataxia type 7.

Spinocerebellar Ataxia type 7 (SCA7) is a neurodegenerative disease characterized by progressive cerebellar ataxia and retinal degeneration. Increasing loss of visual function complicates the use of clinical scales to track the progression of motor symptoms, hampering our ability to develop accurate biomarkers of disease progression, and thus test the efficacy of potential treatments. We aimed to identify imaging measures of neurodegeneration, which may more accurately reflect SCA7 severity and progression.

Errata to "Quantifying Tremor in Essential Tremor Using Inertial Sensors Validation of an Algorithm".

[This corrects the article DOI: 10.1109/JTEHM.2020.

Quantifying Tremor in Essential Tremor Using Inertial Sensors-Validation of an Algorithm.

Assessment of essential tremor is often done by a trained clinician who observes the limbs during different postures and actions and subsequently rates the tremor. While this method has been shown to be reliable, the inter- and intra-rater reliability and need for training can make the use of this method for symptom progression difficult. Many limitations of clinical rating scales can potentially be overcome by using inertial sensors, but to date many algorithms designed to quantify tremor have key limitations.

Electrophysiological Evidence for Functional (Psychogenic) Essential Palatal Tremor.

Background: There is little published work describing the electrophysiological characteristics of essential palatal tremor, a condition now believed by many to be a functional (psychogenic) movement disorder.

Case Report: Here we combine electroencephalography and electromyography with time-locked video recordings to document two cases of essential palatal tremor in which a definitive diagnosis is achieved using these electrophysiological tools.

Discussion: We believe that sharing how these objective tools can be used to diagnose a functional movement disorder, as well as providing more published evidence to support the functional origin of essential palatal myoclonus, will help to diagnose this condition in the future.

BacAv, a new free online platform for clinical back-averaging.

Objective: The back-average technique is very useful to study the relation between the activity in the cortex and the muscles. It has two main clinical applications, Bereitschaftspotential (BP) recording and myoclonus studies. The BP is a slow wave negativity originating in the supplementary motor cortex and premotor cortex that precedes voluntary movements.

Tremoroton, a new free online platform for tremor analysis.

Objective: The electrophysiological classification of tremors can be a key element in the diagnosis and can facilitate treatment of a patient with tremor; however, the ability to conduct electrophysiological studies of tremor is not widely available. The purpose of this study was to develop and validate a free online platform for tremor analysis.

Methods: An online platform for tremor analysis was developed using "R" language; called "Tremoroton".

Dissociation, shame, complex PTSD, child maltreatment and intimate relationship self-concept in dissociative disorder, chronic PTSD and mixed psychiatric groups.

Background: Whilst a growing body of research has examined dissociation and other psychiatric symptoms in severe dissociative disorders (DDs), there has been no systematic examination of shame and sense of self in relationships in DDs. Chronic child abuse often associated with severe DDs, like dissociative identity disorder, is likely to heighten shame and relationship concerns. This study investigated complex posttraumatic stress disorder (PTSD), borderline and Schneiderian symptoms, dissociation, shame, child abuse, and various markers of self in relationships (e.

Corticospinal excitability underlying digit force planning for grasping in humans.

Control of digit forces for grasping relies on sensorimotor memory gained from prior experience with the same or similar objects and on online sensory feedback. However, little is known about neural mechanisms underlying digit force planning. We addressed this question by quantifying the temporal evolution of corticospinal excitability (CSE) using single-pulse transcranial magnetic stimulation (TMS) during two reach-to-grasp tasks.

Dissociated effects of anticipating smoking versus monetary reward in the caudate as a function of smoking abstinence.

Background: Theories of addiction suggest that chronic smoking may be associated with both hypersensitivity to smoking and related cues and hyposensitivity to alternative reinforcers. However, neural responses to smoking and nonsmoking rewards are rarely evaluated within the same paradigm, leaving the extent to which both processes operate simultaneously uncertain. Behavioral evidence and theoretical models suggest that dysregulated reward processing may be more pronounced during deprivation from nicotine, but neuroimaging evidence on the effects of deprivation on reward processing is limited.