Movement Disorders Clinical Practice最新文献

Background: The Powassan virus is a rare neurotropic, tick-borne arbovirus associated with meningoencephalitis. Despite the virus's known predilection for the basal ganglia, there are no reports detailing the spectrum of movement disorders in children with Powassan meningoencephalitis.

Cases: We present 3 cases of pediatric Powassan encephalitis, highlighting the diverse and evolving movement disorders associated with this disease. We observed subcortical myoclonus and progressive generalized dystonia (patient 1), transient dyskinesias and refractory focal dystonia (patient 2), and generalized dystonia evolving into chorea and lingual dyskinesias (patient 3). One patient exhibited multifocal vasculitis on magnetic resonance imaging angiography, a novel finding.

Conclusions: Movement disorders were a primary source of the morbidity experienced by pediatric Powassan encephalitis patients throughout their disease course, underscoring the importance of regular monitoring and adaptable treatment strategies in this condition. Larger, prospective studies are necessary to fully delineate the spectrum of associated movement disorders in this rare and severe disease.

Background: Although performance rating scales, spiral drawing, water pouring, and accelerometry are commonly used to assess tremor severity, the extent to which their results correlate with impairment in activities of daily living (ADL) remains unclear.

Objective: The aim was to identify the most effective predictors of ADL in essential tremor (ET).

Methods: Forty ET patients were examined using The Essential Tremor Rating Assessment Scale (TETRAS), spiral drawing, volume of water spilled, and accelerometric tremor power. Root-mean-square error, R², and F-test were calculated for models predicting TETRAS ADL subscore.

Results: TETRAS Performance Subscale explained the variability in TETRAS ADL with an R² value of 0.686. Models incorporating spiral rating and accelerometric tremor power (R² = 0.731) and water spillage volume (R² = 0.756) were not statistically superior.

Conclusions: TETRAS performance subscore predicted nearly 70% ADL impairment in ET patients. Incorporating the spiral rating, accelerometric tremor power, and water pouring test did not enhance ADL estimation.

Background: Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) is widely employed for cervical dystonia (CD) evaluation.

Objective: To assess the inter-rater reliability of the severity subscale of the original and revised TWSTRS using video recordings.

Methods: Three raters, a PhD student with a nursing degree, a physiotherapist specialized in CD, and a neurologist-in-training independently rated all videos. The inter-rater reliability was assessed with the intra-class correlation coefficient (ICC).

Results: The total severity score of both tools demonstrated a good inter-rater reliability (ICC = 0.87 to 0.88). The inter-rater reliability of individual sub-items varied from poor (ICC = 0.29) to excellent (ICC = 0.9).

Conclusions: The total severity score of both TWSTRS showed good inter-rater reliability in a multidisciplinary team, indicating their applicability for online patients' assessment. We recommend using the total subscale for outcome comparison. Furthermore, there is a need for more accurate definitions of duration factor and shoulder elevation.

Background: Imbalance is the most commonly reported side effect following focused ultrasound (FUS) thalamotomy for essential tremor (ET). It remains unknown which patients are more likely to develop imbalance following FUS treatment.

Objective: To identify preoperative and treatment-related sonication parameters that are predictive of imbalance following FUS treatment.

Methods: We retrospectively collected demographic data, preoperative Fahn-Tolosa-Marin Clinical Rating Scale for Tremor (FTM) scores and FUS treatment parameters in patients undergoing FUS thalamotomy for treatment of ET. The presence of imbalance was evaluated at several discrete time-points with up to 4 years of follow-up. Multiple machine learning classifiers were built and evaluated, aiming to maximize accuracy while minimizing feature set.

Results: Of the 297 patients identified, the presence of imbalance peaked at 1 week following operation at 79%. This declined rapidly with 29% reporting imbalance at 3 months, and only 15% at 4 years. At 1 week, total preoperative FTM scores and Maximum Energy delivered in FUS could predict the presence of imbalance at 92.8% accuracy. At 3 months, the total preoperative FTM scores and maximum power delivered could predict the presence of imbalance with 90.6% accuracy. Post-operative lesion size and extent into thalamic nuclei, internal capsule, and subthalamic regions were identified as likely key underlying drivers of these predictors.

Conclusions: A machine learning model based on preoperative tremor scores and maximum energy/power delivered predicted the development of short-term imbalance and long-term imbalance following FUS thalamotomy.

Background: We hypothesized that many neurologists underestimate patients' willingness to self-administer injectable Parkinson's disease (PD) medication.

Objective: To evaluate patient and physician perceptions contributing to underutilization of PD medications for acute OFF episodes.

Analytic method: Data were collected using an online survey including n = 4063 PD patients experiencing OFF episodes and n = 200 neurologists.

Results: 89% of patients were willing to self-inject rescue therapies to treat acute OFF episodes. After reviewing patient survey data, 54% of general neurologists and 37% of movement disorder specialist (MDS) demonstrated a change in perceptions about patients' willingness to use self-injected therapies (P < 0.05). 37% of general neurologists and 21% of MDS indicated a greater likelihood of prescribing these treatments (P < 0.05).

Conclusions: Most patients suffering from OFF episodes would be willing to self-inject to abort their symptoms. Neurologists underestimate this patient acceptance. Understanding patient attitudes and further education about rescue therapies is likely to increase use of these therapies.

Background: Huntington's Disease is a rare neurodegenerative disorder in which appropriate medication management is essential. While many medications are prescribed based on expert knowledge, overviews of actual medication use in HD are sparse.

Objectives: We provide a detailed overview of medication use and associated indications across HD disease stages, considering sex and regional differences.

Methods: Data from the largest observational HD study, ENROLL-HD, were used. We created HD-related medication and indication classes to identify medication trends in manifest, premanifest and control subjects. We studied medication use in adult, childhood- and adolescent-onset HD, incorporating disease stage (including phenoconverters), sex and regional differences.

Results: In 8546 manifest HD patients, 84.6% used medication (any type), with the average number of medications per user rising from 2.5 in premanifest HD to 5.2 in end stage disease. Antipsychotics (29.2%), SSRIs (27.5%) and painkillers (21.8%) were most often used. Medication use varied with disease progression. Several differences were observed between the sexes, and notably between Europe and Northern America as well. Medication use increased after phenoconversion (from 64.8% to 70.6%, P < 0.05), with the largest difference in antipsychotic use (4.4%-7.8%, P < 0.05). Medication patterns were different in childhood-onset HD, with no use of painkillers, less use of anti-chorea and antidepressant drugs, and more for aggression and irritability.

Conclusions: Medication use in HD increases with disease progression, with varying types of medications prescribed based on disease stage, sex, and region of living. Recognizing these medication trends is vital for further personalized HD management.

Background: Little is known about factors modulating pain and pain-related functional impairment in isolated cervical dystonia (CD).

Objective: The aim was to assess the prevalence and interrelationship between pain-modulating factors and pain-related determinants of functional impairment and quality of life in CD.

Methods: We analyzed pain-aggravating and pain-relieving external factors, the degree of pain-related functional impact on routine activities, and the relationship between these and pain severity, using cross-sectional data collected using the Pain in Dystonia Scale (PIDS) from 85 participants with CD. Pairwise correlation analyses and age- and sex-adjusted linear regression models estimated the relationship between pain-modulating factors and pain severity, and the impact of pain severity, dystonia severity, and psychiatric symptoms on pain-related functional impairment and disease-specific quality of life (measured using the Craniocervical Dystonia Questionnaire-24).

Results: Stress and prolonged fixed position were the most frequent and impacting pain triggers, with women reporting larger impact. The average impact of pain-relieving factors was lower than that of pain triggers. Physical exercise and social gatherings were the most impacted activities by pain in CD. The intensity of external modulating factors was a predictor of pain severity. Severity of pain, CD, and psychiatric symptoms independently predicted pain-related functional impairment, whereas quality of life was predicted by pain severity, pain-related functional impairment, and psychiatric symptom severity, but not dystonia severity.

Conclusion: The PIDS provides insight into external modulation and functional impact of pain in CD. The pattern of external modulation of pain in CD is in line with a multifactorial modulation and complex physiology.