Purpose
Persons with Huntington's disease (HD) have a high incidence of falls. Autonomic nervous system dysfunction has been reported even in early stages of this disease. To date, there has been no ...analysis of the relationship between heart rate variability (HRV) and falls in this patient population. The aim of the study reported here was to evaluate the relationship between HRV and falls in persons with HD.
Methods
Huntington's disease patients enrolled in a prospective study on fear of falling and falls were assessed using short-term HRV analyses and blood pressure measures in both the resting and standing states. Time–frequency domains and nonlinear parameters were calculated. Data on falls, the risk of falling (RoF) and disease-specific scales were collected at baseline and at the end of the 6-month follow-up.
Results
Of the 24 HD patients who were invited to participate in the study, 20 completed the baseline analysis and 18 completed the 6-month follow-up. At baseline, seven (35%) HD patients reported at least one fall (single fallers) and 13 (65%) reported ≥ 2 falls (recurrent fallers) in the previous 12 months. At baseline, recurrent fallers had lower RMSSD (root mean square of successive RR interval differences) in the resting state (RMSSD-resting), higher LF/HF (low/high frequency) ratio in both states and higher DFA-
α1
parameter (detrended fluctuation analyses over the short term) in both states. This association was similar at the 6-month follow-up for recurrent fallers, who showed lower RMSSD-resting and higher LF/HF ratio in the standing state (LF/HF-standing) than single fallers. Significant correlations were found between the number of falls, RMSSD-resting and LF/HF-standing. No differences were found between recurrent and single fallers for any blood pressure measures.
Conclusions
The observed HRV pattern is consistent with a higher sympathetic prevalence associated with a higher RoF. Reduced parasympathetic HRV values in this patient population predict being a recurrent faller at 6 months of follow-up, independently of orthostatic phenomena.
ABSTRACT
Background
Although the COVID‐19 pandemic is affecting a relatively small proportion of the global population, its effects have already reached everyone. The pandemic has the potential to ...differentially disadvantage chronically ill patients, including those with Parkinson's disease (PD). The first health care reaction has been to limit access to clinics and neurology wards to preserve fragile patients with PD from being infected. In some regions, the shortage of medical staff has also forced movement disorders neurologists to provide care for patients with COVID‐19.
Objective
To share the experience of various movement disorder neurologists operating in different world regions and provide a common approach to patients with PD, with a focus on those already on advanced therapies, which may serve as guidance in the current pandemic and for emergency situations that we may face in the future.
Conclusion
Most of us were unprepared to deal with this condition given that in many health care systems, telemedicine has been only marginally available or only limited to email or telephone contacts. In addition, to ensure sufficient access to intensive care unit beds, most elective procedures (including deep brain stimulation or the initiation of infusion therapies) have been postponed. We all hope there will soon be a time when we will return to more regular hospital schedules. However, we should consider this crisis as an opportunity to change our approach and encourage our hospitals and health care systems to facilitate the remote management of chronic neurological patients, including those with advanced PD.
Purpose
Despite the availability of the Unified Multiple System Atrophy (MSA) Rating Scale (UMSARS) for almost two decades, studies still use scales developed for Parkinson’s disease (PD) or ataxia ...(ATX). Our aim was to evaluate the use of UMSARS (part II, motor) compared to other motor rating scales in patients with MSA.
Methods
A Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA)-compliant literature search was conducted concerning studies of patients with MSA, reporting motor assessment with clinical rating scales, and focusing on the frequency of UMSARS use.
Results
We included 261 articles, of which 42.9% did not use UMSARS, but rather scales for PD (59.8%), ATX (24.1%), or both (14.3%). Although UMSARS use increased with time, misuse of PD and ATX scales persists, with no evidence of a decremental trend.
Conclusions
Although higher in observational studies, the misuse of PD and ATX-related scales in MSA patients persists in prospective (planned) trials. Reasons for that must be addressed.
Editorial on the Research Topic Pathophysiology of the Basal Ganglia and Movement Disorders: Gaining New Insights from Modeling and Experimentation, to Influence the Clinic The human brain is complex ...at every level, from the scale of single neurons to microcircuits and large neuronal networks. The basal ganglia together with the cerebellum and prefrontal cortical areas play a role in time processing, which is altered in movement disorders and affects both motor and cognitive performance (Avanzino et al.). The temporal structure function is a robust and simple to compute tool for the analysis of neuronal activity, which helps identifying random, oscillatory and non-linear behavior in the dynamics of single neurons.
Background
Deep brain stimulation (DBS) of the subthalamic nucleus (STN) or internal globus pallidus (GPi) represents an effective and universally applied therapy for Parkinson's disease (PD) motor ...complications. However, certain procedure‐related problems and unrealistic patient expectations may detract specialists from indicating DBS more widely despite significant clinical effects.
Methods
This review provides a pragmatic educational summary of the most conflicting postoperative management issues in patients undergoing DBS for PD.
Results
DBS in PD has been associated with certain complications and post‐procedural management issues, which can complicate surgical outcome interpretation. Many PD patients consider DBS outcomes negative due to unfulfilled expectations, even when significant motor symptom improvement is achieved. Speech, gait, postural stability, and cognition may worsen after DBS and body weight may increase. Although DBS may induce impulse control disorders in some cases, in others, it may actually improve them when dopamine agonist dosage is reduced after surgery. However, apathy may also arise, especially when dopaminergic medication tapering is rapid. Gradual loss of response with time suggests disease progression, rather than the wearing off of DBS effects. Furthermore, implantable pulse generator expiration is considered a movement disorder emergency, as it may worsen parkinsonian symptoms or cause life‐threatening akinetic crises due to malignant DBS withdrawal syndrome.
Conclusion
Major unsolved issues occurring after DBS therapy preclude complete patient satisfaction. Multidisciplinary management at experienced centers, as well as careful and comprehensive delivery of information to patients, should contribute to make DBS outcome expectations more realistic and allow post procedural complications to be better accepted.
Purpose
The unified multiple system atrophy (MSA) rating scale (UMSARS) was developed almost 20 years ago as a clinical rating scale to capture multiple aspects of the disease. With its widespread ...use, the shortcomings of the UMSARS as a clinical outcome assessment (COA) have become increasingly apparent. We here summarize the shortcomings of the scale, confirm some of its limitations with data from the Natural History Study of the Synucleinopathies (NHSS), and suggest a framework to develop and validate an improved COA to be used in future clinical trials of disease-modifying drugs in patients with MSA.
Methods
Expert consensus assessment of the limitations of the UMSARS and recommendations for the development and validation of a novel COA for MSA. We used UMSARS data from the ongoing NHSS (ClinicalTrials.gov: NCT01799915) to showcase some of these limitations.
Results
The UMSARS in general, and specific items in particular, have limitations to detect change resulting in a ceiling effect. Some items have specific limitations including unclear anchoring descriptions, lack of correlation with disease severity, susceptibility to improve with symptomatic therapies (e.g., orthostatic hypotension, constipation, and bladder dysfunction), and redundancy, among others.
Conclusions
Because of the limitations of the UMSARS, developing and validating an improved COA is a priority. The time is right for academic MSA clinicians together with industry, professional societies, and patient advocacy groups to develop and validate a new COA.
Clinical case studies have long been recognized as a useful adjunct to problem-based learning and continuing professional development. Movement Disorders collects over 90 of the most memorable and ...challenging movement disorder cases from the worldís leading authorities in this specialty. Compelling vignettes covering the entire phenomenology of movement disorders are presented succinctly but descriptively to walk the reader through the diagnostic processómuch like being in the examining room with a master clinician. Each case follows a set format consisting of four sections: The Case; The Approach; The Lesson; Reference and Suggested Readings. Imaging findings and other illustrations amplify the discussion where pertinent. Movement Disorders features: * Collection of over 90 compelling cases covering standard movement disorders phenomenology * Cases are vividly described, well-illustrated, and authoritatively written with a section on ìlessons learnedî at the end of each vignette * Captures the ìhuman elementî in medicineófirst-person narratives simulate the experience of sitting at the elbow of a master clinician interviewing and examining the patient * Designed to help hone diagnostic skills and inform treatment decisions for the full spectrum of movement disorders * Vignette titles serve as a reference index for clinicians to easily search similar cases they may have encountered (i.e. An Elderly Man with Dystonia; Chorea in a Young Athlete)