Global Perceptions and Utilization of Clinical Neurophysiology in Movement Disorders.

BACKGROUND: Clinical neurophysiology (CNP) involves the use of neurophysiological techniques to make an accurate clinical diagnosis, to quantify the severity, and to measure the treatment response. Despite several studies showing CNP to be a useful diagnostic tool in Movement Disorders (MD), its more widespread utilization in clinical practice has been limited. OBJECTIVES: To better understand the current availability, global perceptions, and challenges for implementation of diagnostic CNP in the clinical practice of MD. METHODS: The International Parkinson and Movement Disorders Society (IPMDS) formed a Task Force on CNP. The Task Force distributed an online survey via email to all the members of the IPMDS between August 5 and 30, 2021. Descriptive statistics were used for analysis of the survey results. Some results are presented by IPMDS geographical sections namely PanAmerican (PAS), European (ES), African (AFR), Asian and Oceanian (AOS). RESULTS: Four hundred and ninety-one IPMDS members (52% males), from 196 countries, responded. The majority of responders from the AFR (65%) and PAS (63%) sections had no formal training in diagnostic CNP (40% for AOS and 37% for ES). The most commonly used techniques are electroencephalography (EEG) (72%) followed by surface EMG (71%). The majority of responders think that CNP is somewhat valuable or very valuable in the assessment of MD. All the sections identified "lack of training" as one of the biggest challenges for diagnostic CNP studies in MD. CONCLUSIONS: CNP is perceived to be a useful diagnostic tool in MD. Several challenges were identified that prevent widespread utilization of CNP in MD.

How to Do an Electrophysiological Study of Myoclonus.

SUMMARY: 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. This article aims to serve as a practical manual on how to do a myoclonus study. For this purpose, the authors combine their experience with available evidence. The authors provide detailed descriptions of recording poly-electromyography, combining electroencephalography and electromyography, Bereitschaftspotentials, somatosensory evoked potentials, and startle techniques. The authors discuss analysis considerations for these data and provide a simplified algorithm for their interpretation. Finally, the authors discuss some factors that they believe have hindered the broader use of these useful techniques.

Cortical mechanisms of sensory trick in cervical dystonia.

Patients with cervical dystonia (CD) often show an improvement in dystonic posture after sensory trick (ST), though the mechanisms underlying ST remain unclear. In this study, we aimed to investigate the effects of ST on cortical activity in patients with CD and to explore the contribution of motor and sensory components to ST mechanisms. To this purpose, we studied 15 CD patients with clinically effective ST, 17 without ST, and 14 healthy controls (HCs) who mimicked the ST. We used electroencephalographic (EEG) recordings and electromyography (EMG) data from bilateral sternocleidomastoid (SCM) muscles. We compared ST-related EEG spectral changes from sensorimotor and posterior parietal areas and EMG power changes between groups. To better understand the contribution of motor and sensory components to ST, we tested EEG and EMG correlates of three different conditions mimicking ST, the first without skin touch ("no touch" condition), the second without voluntary movements ("passive" condition), and finally without arm movements ("examiner touch" condition). Results showed ST-related alpha desynchronization in the sensorimotor cortex and theta desynchronization in the sensorimotor and posterior parietal cortex. Both spectral changes were more significant during maneuver execution in CD patients with ST than in CD patients without ST and HCs who mimicked the ST. Differently, the "no touch", "passive", or "examiner touch" conditions did not show significant differences in EEG or EMG changes determined by ST execution/mimicking between CD patients with or without ST. A higher desynchronization within alpha and theta bands in the sensorimotor and posterior parietal areas correlated with a more significant activity decrease in the contralateral SCM muscle, Findings from this study suggest that ST-related changes in the activity of sensorimotor and posterior parietal areas may restore dystonic posture and that both motor and sensory components contribute to the ST effect.

Diagnostic Neurophysiologic Biomarkers for Task-Specific Dystonia.

Background: Task-specific dystonia (TSD) is a challenging clinical diagnosis with no objective diagnostic biomarkers. Objective: The objective of this study was to test 2 neurophysiologic variables using transcranial magnetic stimulation as potential diagnostic biomarkers for TSD. Methods: We tested (1) cortical silent period (CSP) and (2) dorsal inferior parietal lobule-motor cortex (dIPL-M1) physiologic connectivity in 9 patients with the writer's cramp form of TSD and 12 healthy volunteers on 2 separate sessions. Results: CSP was significantly prolonged (P < 0.0001) in TSD and could classify TSD with high sensitivity and specificity with areas under the receiver operating characteristic curve (AUCs) = 0.94 and 0.90, respectively, for 2 separate sessions with an intraclass correlation = 0.79. dIPL-M1 interaction was notable for significant motor cortical inhibition in TSD compared with facilitation in healthy subjects (P < 0.0001) and could classify TSD with high sensitivity and specificity with AUCs = 0.96 and 0.86, respectively. Conclusion: CSP and dIPL-M1 physiologic connectivity can classify TSD with high sensitivity, specificity, reproducibility, and reliability.

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. While common structural MRI techniques have been previously used for this purpose, they can be biased by neurodegeneration-driven increases in extracellular CSF-like water. In a cross-sectional study, we analyzed diffusion tensor imaging (DTI) data collected from a cohort of 13 SCA7 patients and 14 healthy volunteers using: 1) a diffusion tensor-based image registration technique, and 2) a dual-compartment DTI model to control for the potential increase in extracellular CSF-like water. These methodologies allowed us to assess both volumetric and microstructural abnormalities in both white and gray matter brain-wide in SCA7 patients for the first time. To measure tissue volume, we performed diffusion tensor-based morphometry (DTBM) using the tensor-based registration. To assess tissue microstructure, we computed the parenchymal mean diffusivity (pMD) and parenchymal fractional anisotropy (pFA) using the dual compartment model. This model also enabled us to estimate the parenchymal volume fraction (pVF), a measure of parenchymal tissue volume within a given voxel. While DTBM and pVF revealed tissue loss primarily in the brainstem, cerebellum, thalamus, and major motor white matter tracts in patients (p < 0.05, FWE corrected; Hedge's g > 1), pMD and pFA detected microstructural abnormalities in virtually all tissues brain-wide (p < 0.05, FWE corrected; Hedge's g > 1). The Scale for the Assessment and Rating of Ataxia trended towards correlation with cerebellar pVF (r = -0.66, p = 0.104, FDR corrected) and global white matter pFA (r = -0.64, p = 0.104, FDR corrected). These results advance our understanding of neurodegeneration in living SCA7 patients by providing the first voxel-wise characterization of white matter volume loss and gray matter microstructural abnormalities. Moving forward, this comprehensive approach could be applied to characterize the full spatiotemporal pattern of neurodegeneration in SCA7, and potentially develop an accurate imaging biomarker of disease progression.

Myoclonus: An Electrophysiological Diagnosis.

BACKGROUND: Many different movement disorders have similar "jerk-like" phenomenology and can be misconstrued as myoclonus. Different types of myoclonus also share similar phenomenological characteristics that can be difficult to distinguish solely based on clinical exam. However, they have distinctive physiologic characteristics that can help refine categorization of jerk-like movements. OBJECTIVES: In this review, we briefly summarize the clinical, physiologic, and pathophysiologic characteristics of different types of myoclonus. The methodology and technical considerations for the electrophysiologic assessment of jerk-like movements are reviewed. A simplistic pragmatic approach for the classification of myoclonus and other jerk-like movements based on objective electrophysiologic characteristics is proposed. CONCLUSIONS: Clinical neurophysiology is an underutilized tool in the diagnosis and treatment of movement disorders. Various jerk-like movements have distinguishing physiologic characteristics, differentiated in the milliseconds range, which is beyond human capacity. We argue that the categorization of movement disorders as myoclonus can be refined based on objective physiology that can have important prognostic and therapeutic implications.

The role of the inferior parietal lobule in writer's cramp.

Humans have a distinguishing ability for fine motor control that is subserved by a highly evolved cortico-motor neuronal network. The acquisition of a particular motor skill involves a long series of practice movements, trial and error, adjustment and refinement. At the cortical level, this acquisition begins in the parieto-temporal sensory regions and is subsequently consolidated and stratified in the premotor-motor cortex. Task-specific dystonia can be viewed as a corruption or loss of motor control confined to a single motor skill. Using a multimodal experimental approach combining neuroimaging and non-invasive brain stimulation, we explored interactions between the principal nodes of the fine motor control network in patients with writer's cramp and healthy matched controls. Patients and healthy volunteers underwent clinical assessment, diffusion-weighted MRI for tractography, and functional MRI during a finger tapping task. Activation maps from the task-functional MRI scans were used for target selection and neuro-navigation of the transcranial magnetic stimulation. Single- and double-pulse TMS evaluation included measurement of the input-output recruitment curve, cortical silent period, and amplitude of the motor evoked potentials conditioned by cortico-cortical interactions between premotor ventral (PMv)-motor cortex (M1), anterior inferior parietal lobule (aIPL)-M1, and dorsal inferior parietal lobule (dIPL)-M1 before and after inducing a long term depression-like plastic change to dIPL node with continuous theta-burst transcranial magnetic stimulation in a randomized, sham-controlled design. Baseline dIPL-M1 and aIPL-M1 cortico-cortical interactions were facilitatory and inhibitory, respectively, in healthy volunteers, whereas the interactions were converse and significantly different in writer's cramp. Baseline PMv-M1 interactions were inhibitory and similar between the groups. The dIPL-PMv resting state functional connectivity was increased in patients compared to controls, but no differences in structural connectivity between the nodes were observed. Cortical silent period was significantly prolonged in writer's cramp. Making a long term depression-like plastic change to dIPL node transformed the aIPL-M1 interaction to inhibitory (similar to healthy volunteers) and cancelled the PMv-M1 inhibition only in the writer's cramp group. These findings suggest that the parietal multimodal sensory association region could have an aberrant downstream influence on the fine motor control network in writer's cramp, which could be artificially restored to its normal function.

How to do an electrophysiological study of tremor.

The electrophysiological characterization of hand tremors is a useful method to complement the history and physical exam of tremor patients. Our article describes the methodology (recording, processing and interpretation) used in a diagnostic/phenotypic hand tremor study conducted in our lab at the Human Motor Control Section of the National Institute of Neurological Disorders and Stroke (NINDS), at the National Institutes of Health. The necessary equipment includes two one-axis accelerometers and four-channel electromyography (EMG). The hand tremor is recorded at rest, posture with and without weight loading, and during movement (kinetic). The recorded signals are analyzed in the time and frequency domains. The characterization of the dominant frequencies in the accelerometers and their relationship with the EMG frequencies are essential for the differential diagnosis of different tremor syndromes. We describe the electrophysiological characteristics of several tremor syndromes such as enhanced physiological tremor, essential tremor, Parkinson tremor, pharmacological-induced tremor, orthostatic tremor, and functional (psychogenic) tremor. Simplified guidance for adoption of tremor studies as a clinical tool in a movement disorders subspecialty clinic is provided.

A novel exaggerated "spino-bulbo-spinal like" reflex of lower brainstem origin.

BACKGROUND: Many different oligosynaptic reflexes are known to originate in the lower brainstem which share phenomenological and neurophysiological similarities. OBJECTIVE: To evaluate and discuss the differences and aberrancies among these reflexes, which are hard to discern clinically using neurophysiological investigations with the help of a case report. METHODS: We describe the clinical and neurophysiological assessment of a young man who had a childhood history of opsoclonus-myoclonus syndrome with residual mild ataxia and myoclonic jerks in the distal extremities presenting with subacute onset total body jerks sensitive to sound and touch (in a limited dermatomal distribution), refractory to medications. RESULTS: Based on clinical characteristics and insights gained from neurophysiological testing we could identify a novel reflex of caudal brainstem origin. CONCLUSIONS: The reflex described is likely an exaggerated normal reflex, likely triggered by a dolichoectatic vertebral arterial compression and shares characteristics of different reflexes known to originate in caudal brainstem, which subserve distinctive roles in human postural control.

Objective predictors of 'early tolerance' to ventral intermediate nucleus of thalamus deep brain stimulation in essential tremor patients.

OBJECTIVE: To identify pre-operative clinical and computerized spiral analysis characteristics that may help ascertain which patients with Essential Tremor (ET) will exhibit 'early tolerance' to ventral intermediate nucleus of thalamus (Vim) deep brain stimulation (DBS). METHODS: Identification of comparative characteristics of defined cases of 'early tolerance' versus patients with sustained satisfactory response treated with Vim DBS surgery for medically-refractory ET, based on retrospective chart review by a clinician blinded to the findings of computerized spiral analysis. RESULTS: Statistically significant differences in two spiral analysis indices, SWVI and DoS, were found in the dominant upper limbs of patients who developed 'early tolerance', whereas the clinical characteristics were not significantly different. CONCLUSION: Objective measurements of upper limb kinematics using graphonomic tests like spiral analysis should be considered in the pre-operative evaluation for DBS, especially in the setting of moderate-severe predominantly action and proximal postural tremors. SIGNIFICANCE: Ours is the first investigation looking into the pre-operative clinical and objective physiologic characteristics of the patients who develop 'early tolerance' to Vim DBS for the treatment of essential tremor. The study has significant implications for pre-operative evaluation and potential surgical target selection for the treatment of tremors.

Evidence already exists for motor system reorganization in CRPS.

Complex regional pain syndrome (CRPS) is a disabling condition that is usually preceded by trauma or surgical procedure. Involvement of the motor system is a well-known phenomenon in CRPS, though the pathophysiologic mechanisms of motor system affliction in CRPS are poorly understood. Graded motor imagery (GMI) has been proposed to be one of the therapeutic interventions to help improve pain and other disabling symptoms associated with CRPS, though the benefits noted are modest and inconsistent. The neurophysiological mechanisms implicated in motor imagery are intended to target the aberrant prefrontal and sensorimotor integration areas, which may potentially help restore the aberrant cortical plasticity in CRPS. Detailed well-controlled experiments using insights from the existing body of literature on motor system reorganization in CRPS are required to better understand this complicated disorder. Attempts to gain pathophysiologic insights about complicated disorders like CRPS based on case reports with poorly performed and uncontrolled interventions are misguided.

Le syndrome douleureux régional complexe est une affection invalidante habituellement précédée d'un traumatisme ou d'une intervention chirurgicale. L'implication du système moteur dans le SDRC est un phénomène bien connu, malgré le fait que les mécanismes pathophysiologiques qui l'affectent soient mal compris. L'imagerie motrice progressive (IMP) a été proposée en tant que l'une des interventions théraeutiques pouvant aider à améliorer la douleur et d'autres symptômes invalidants associés au SDRC, bien que les effets bénéfiques observés soient modestes et contradictoires. Les mécanismes neurophysiologiques impliqués dans l'imagerie motrice sont destinés à cibler l'aire préfrontale et l'aire d'intégration sensorimotrice anormales qui peuvent potentiellement aider à rétablir la plasticité corticale dans le SDRC. Des expériences approfondies bien contrôlées fondées sur les connaissances que l'on retrouve dans la littérature existante en ce qui concerne la réorganisation du système moteur dans le SDRC sont nécessaires afin de mieux comprendre ce trouble compliqué. Les tentatives pour améliorer les connaissances pathophsyiologiques concernant des troubles compliqués comme le SDRC qui sont fondées sur des études de cas et des interventions effectuées de manière médiocre et non contrôlées sont malavisées.

Cervical angina: an overlooked source of noncardiac chest pain.

Cervical angina has been widely reported as a cause of chest pain but remains underrecognized. This series demonstrates the varied clinical presentation of patients with cervical angina, the delay in diagnosis, and the extensive cardiac examinations patients with this condition typically undergo prior to a definitive diagnosis. Recognition of this condition in patients with acute chest pain requires a high index of suspicion and an awareness of the common presenting features and clinical findings of cervical angina.