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BACKGROUND: Functional neurological disorder (FND) is a common cause of neurological disability. Despite recent advances in pathophysiological understanding and treatments, application of this knowledge to clinical practice is variable and limited. OBJECTIVE: Our aim was to provide an expert overview of the state of affairs of FND practice across Europe, focusing on education and training, access to specialized care, reimbursement and disability policies, and academic and patient-led representation of people with FND. METHODS: We conducted a survey across Europe, featuring one expert per country. We asked experts to compare training and services for people with FND to those provided to people with multiple sclerosis (MS). RESULTS: Responses from 25 countries revealed that only five included FND as a mandatory part of neurological training, while teaching about MS was uniformly included. FND was part of final neurology examinations in 3/17 countries, unlike MS that was included in all 17. Seventeen countries reported neurologists with an interest in FND but the estimated mean ratio of FND-interested neurologists to MS neurologists was 1:20. FND coding varied, with psychiatric coding for FND impacting treatment access and disability benefits in the majority of countries. Twenty countries reported services refusing to see FND patients. Eight countries reported an FND special interest group or network; 11 reported patient-led organizations. CONCLUSIONS: FND is largely a marginal topic within European neurology training and there is limited access to specialized care and disability benefits for people with FND across Europe. We discuss how this issue can be addressed at an academic, healthcare and patient organization level.
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The progression of Parkinson's disease (PD) is associated with microstructural alterations in neural pathways, contributing to both motor and cognitive decline. However, conflicting findings have emerged due to the use of heterogeneous methods in small studies. Here we performed a large diffusion MRI study in PD, integrating data from 17 cohorts worldwide, to identify stage-specific profiles of white matter differences. Diffusion-weighted MRI data from 1654 participants diagnosed with PD (age: 20-89 years; 33% female) and 885 controls (age: 19-84 years; 47% female) were analyzed using the ENIGMA-DTI protocol to evaluate white matter microstructure. Skeletonized maps of fractional anisotropy (FA) and mean diffusivity (MD) were compared across Hoehn and Yahr (HY) disease groups and controls to reveal the profile of white matter alterations at different stages. We found an enhanced, more widespread pattern of microstructural alterations with each stage of PD, with eventually lower FA and higher MD in almost all regions of interest: Cohen's d effect sizes reached d = -1.01 for FA differences in the fornix at PD HY Stage 4/5. The early PD signature in HY stage 1 included higher FA and lower MD across the entire white matter skeleton, in a direction opposite to that typical of other neurodegenerative diseases. FA and MD were associated with motor and non-motor clinical dysfunction. While overridden by degenerative changes in the later stages of PD, early PD is associated with paradoxically higher FA and lower MD in PD, consistent with early compensatory changes associated with the disorder.
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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.
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Movimiento , Enfermedad de Parkinson , Masculino , Humanos , Femenino , Neurofisiología/educación , Electroencefalografía , ElectromiografíaRESUMEN
Patients with Parkinson's Disease or a tremor syndrome may present with additional functional movement disorders. The differential diagnosis is particularly difficult. In some cases, functional symptoms occur either before the manifestation of the organic disease or can emerge as an additional symptom after Parkinson's disease or tremor became apparent. In patients with Parkinson's disease the prevalence for additional functional symptoms is 7 %. In the case that patients with Parkinson's diseases have one side that is more severely affected, additional functional motor symptoms such as functional rest tremor also occur on that same, predominantly affected side. Functional gait disorders occur frequently. Clinically, patients appear notably slow in automatized, daily tasks. Their speech is more whispering than hypophonic, bradykinesia during finger tapping manifest without a decrement. The Dopamintransporterszintigraphy (123) I FP-CIT SPECT; DaTSCANTM) may be helpful to differentiate between functional Parkinsonism and Parkinson's disease. Functional tremor in patients with an organic tremor syndrome is diagnosed with the same distraction techniques as in solely functional tremor. This includes cognitive, motor, and suggestive distraction maneuvers. In some cases, additional neurophysiological investigations such as accelerometry are useful for the differential diagnosis. It is most important to identify patients with additional functional symptoms in non-functional movement disorders, because the therapeutic approach differs and a multi professional team is required to initiate effective treatment strategies.
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Enfermedad de Parkinson , Temblor , Humanos , Diagnóstico Diferencial , Trastornos Neurológicos de la Marcha/fisiopatología , Trastornos Neurológicos de la Marcha/diagnóstico , Trastornos Neurológicos de la Marcha/etiología , Enfermedad de Parkinson/diagnóstico , Enfermedad de Parkinson/complicaciones , Enfermedad de Parkinson/fisiopatología , Síndrome , Temblor/diagnóstico , Temblor/fisiopatología , Temblor/etiología , Temblor/terapiaRESUMEN
BACKGROUND AND PURPOSE: Quantification of neurofilament light chain protein in serum (sNfL) enables the neuro-axonal damage in peripheral blood to be reliably assessed and monitored. There is a long-standing debate whether essential tremor represents a 'benign' tremor syndrome or whether it is linked to neurodegeneration. This study aims to investigate sNfL concentrations in essential tremor compared to healthy controls (cross-sectionally and longitudinally) and to assess whether sNfL is associated with motor and nonmotor markers of disease progression. METHODS: Data of patients with essential tremor from our prospective registry on movement disorders (PROMOVE) were retrospectively analysed. Age-, sex- and body-mass-index-matched healthy controls were recruited from an ongoing community-dwelling aging cohort. sNfL was quantified by an ultra-sensitive single molecule array (Simoa). All participants underwent detailed clinical examination at baseline and after approximately 5 years of follow-up. RESULTS: Thirty-seven patients with clinically diagnosed essential tremor were included and 37 controls. The essential tremor group showed significantly higher sNfL levels compared to healthy controls at baseline and follow-up. sNfL levels increased over time in both groups, and the slope of sNfL increase was similar in the essential tremor and healthy control groups. Comparing patients with a disease duration under 5 years to those with a longer disease duration, the former group had a significantly greater increase of sNfL over time, which strongly correlated to worsening of tremor and cognition. CONCLUSION: Our findings indicate that neurodegeneration, possibly happening at an early disease stage, might play a role in the pathophysiology of essential tremor.
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Temblor Esencial , Esclerosis Múltiple , Humanos , Biomarcadores , Estudios Retrospectivos , Temblor , Filamentos Intermedios , Proteínas de NeurofilamentosRESUMEN
BACKGROUND: Increasing evidence points to a pathophysiological role for the cerebellum in Parkinson's disease (PD). However, regional cerebellar changes associated with motor and non-motor functioning remain to be elucidated. OBJECTIVE: To quantify cross-sectional regional cerebellar lobule volumes using three dimensional T1-weighted anatomical brain magnetic resonance imaging from the global ENIGMA-PD working group. METHODS: Cerebellar parcellation was performed using a deep learning-based approach from 2487 people with PD and 1212 age and sex-matched controls across 22 sites. Linear mixed effects models compared total and regional cerebellar volume in people with PD at each Hoehn and Yahr (HY) disease stage, to an age- and sex- matched control group. Associations with motor symptom severity and Montreal Cognitive Assessment scores were investigated. RESULTS: Overall, people with PD had a regionally smaller posterior lobe (dmax = -0.15). HY stage-specific analyses revealed a larger anterior lobule V bilaterally (dmax = 0.28) in people with PD in HY stage 1 compared to controls. In contrast, smaller bilateral lobule VII volume in the posterior lobe was observed in HY stages 3, 4, and 5 (dmax = -0.76), which was incrementally lower with higher disease stage. Within PD, cognitively impaired individuals had lower total cerebellar volume compared to cognitively normal individuals (d = -0.17). CONCLUSIONS: We provide evidence of a dissociation between anterior "motor" lobe and posterior "non-motor" lobe cerebellar regions in PD. Whereas less severe stages of the disease are associated with larger motor lobe regions, more severe stages of the disease are marked by smaller non-motor regions. © 2023 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.
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Enfermedad de Parkinson , Humanos , Enfermedad de Parkinson/complicaciones , Estudios Transversales , Imagen por Resonancia Magnética , Cerebelo , EncéfaloRESUMEN
IMPORTANCE: The Purdue Pegboard Test (PPT) is widely used as a measure of manual dexterity. Declining manual dexterity may predict cognitive decline among elderly people, but normative data for this population are scarce. OBJECTIVE: To identify demographic and clinical predictors of PPT results in normal middle-aged and elderly Austrian people and to provide norms stratified by significant determinants. DESIGN: A prospective, community-based cohort study using baseline data of participants from two study panels (1991-1994 and 1999-2003). SETTING: Monocentric study Participants: 1,355 healthy, randomly selected, community-dwelling people ages 40 to 79 yr. METHOD: Extensive clinical examination, including completion of the PPT. OUTCOMES AND MEASURES: The number of pegs placed within a 30-s time limit on four subtests: using the right hand, left hand, both hands, and assembly (within 60 s), respectively. Demographic outcomes were the highest grade achieved. RESULTS: For all four subtests, increasing age (ßs = -0.400 to -0.118, SEs = 0.006 to 0.019, p < .001) and male sex (ßs = -1.440 to -0.807, SEs = 0.107 to 0.325, p < .001) was related to worse test results. Among vascular risk factors, diabetes (ßs = -1.577 to -0.419, SEs = 0.165 to 0.503, p < .001) was related to worse test results but explained only a small portion (0.7%-1.1%) of the variability in PPT performance. CONCLUSIONS AND RELEVANCE: We provide age- and sex-specific norms of the PPT for a middle-aged and elderly population. The data represent useful reference values when assessing manual dexterity in older age groups. What This Article Adds: Advancing age and male sex relate to worse performance on the PPT in a community-dwelling cohort without signs and symptoms of neurological disease. Vascular risk factors explain only very little of the variance of test results in our population. Our study adds to the limited age- and sex-specific norms of the PPT among middle-aged and older people.
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Mano , Estado de Salud , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Austria , Estudios de Cohortes , Destreza Motora , Estudios Prospectivos , AdultoRESUMEN
BACKGROUND AND OBJECTIVES: Perivascular spaces (PVS) are emerging markers of cerebral small vessel disease (CSVD), but research on their determinants has been hampered by conflicting results from small single studies using heterogeneous rating methods. In this study, we therefore aimed to identify determinants of PVS burden in a pooled analysis of multiple cohort studies using 1 harmonized PVS rating method. METHODS: Individuals from 10 population-based cohort studies with adult participants from the Uniform Neuro-Imaging of Virchow-Robin Spaces Enlargement consortium and the UK Biobank were included. On MRI scans, we counted PVS in 4 brain regions (mesencephalon, hippocampus, basal ganglia, and centrum semiovale) according to a uniform and validated rating protocol, both manually and automated using a deep learning algorithm. As potential determinants, we considered demographics, cardiovascular risk factors, APOE genotypes, and other imaging markers of CSVD. Negative binomial regression models were used to examine the association between these determinants and PVS counts. RESULTS: In total, 39,976 individuals were included (age range 20-96 years). The average count of PVS in the 4 regions increased from the age 20 years (0-1 PVS) to 90 years (2-7 PVS). Men had more mesencephalic PVS (OR [95% CI] = 1.13 [1.08-1.18] compared with women), but less hippocampal PVS (0.82 [0.81-0.83]). Higher blood pressure, particularly diastolic pressure, was associated with more PVS in all regions (ORs between 1.04-1.05). Hippocampal PVS showed higher counts with higher high-density lipoprotein cholesterol levels (1.02 [1.01-1.02]), glucose levels (1.02 [1.01-1.03]), and APOE ε4-alleles (1.02 [1.01-1.04]). Furthermore, white matter hyperintensity volume and presence of lacunes were associated with PVS in multiple regions, but most strongly with the basal ganglia (1.13 [1.12-1.14] and 1.10 [1.09-1.12], respectively). DISCUSSION: Various factors are associated with the burden of PVS, in part regionally specific, which points toward a multifactorial origin beyond what can be expected from PVS-related risk factor profiles. This study highlights the power of collaborative efforts in population neuroimaging research.
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Enfermedades de los Pequeños Vasos Cerebrales , Sistema Glinfático , Masculino , Adulto , Humanos , Femenino , Adulto Joven , Persona de Mediana Edad , Anciano , Anciano de 80 o más Años , Encéfalo/diagnóstico por imagen , Encéfalo/irrigación sanguínea , Estudios de Cohortes , Imagen por Resonancia Magnética/métodos , Neuroimagen , Enfermedades de los Pequeños Vasos Cerebrales/diagnóstico por imagen , Enfermedades de los Pequeños Vasos Cerebrales/epidemiología , Enfermedades de los Pequeños Vasos Cerebrales/complicacionesRESUMEN
Background: Functional (un-)coupling (task-related change of functional connectivity) between different sites of the brain is a mechanism of general importance for cognitive processes. In Alzheimer's disease (AD), prior research identified diminished cortical connectivity as a hallmark of the disease. However, little is known about the relation between the amount of functional (un-)coupling and cognitive performance and decline in AD. Method: Cognitive performance (based on CERAD-Plus scores) and electroencephalogram (EEG)-based functional (un-)coupling measures (connectivity changes from rest to a Face-Name-Encoding task) were assessed in 135 AD patients (age: M = 73.8 years; SD = 9.0). Of these, 68 patients (M = 73.9 years; SD = 8.9) participated in a follow-up assessment of their cognitive performance 1.5 years later. Results: The amounts of functional (un-)coupling in left anterior-posterior and homotopic interhemispheric connections in beta1-band were related to cognitive performance at baseline (ß = .340; p < .001; ß = .274; P = .001, respectively). For both markers, a higher amount of functional coupling was associated with better cognitive performance. Both markers also were significant predictors for cognitive decline. However, while patients with greater functional coupling in left anterior-posterior connections declined less in cognitive performance (ß = .329; P = .035) those with greater functional coupling in interhemispheric connections declined more (ß = -.402; P = .010). Conclusion: These findings suggest an important role of functional coupling mechanisms in left anterior-posterior and interhemispheric connections in AD. Especially the complex relationship with cognitive decline in AD patients might be an interesting aspect for future studies.
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Enfermedad de Alzheimer , Disfunción Cognitiva , Humanos , Anciano , Imagen por Resonancia Magnética , Electroencefalografía/métodos , Encéfalo , Progresión de la EnfermedadRESUMEN
Language impairments, hallmarks of speech/language variant progressive supranuclear palsy, also occur in Richardson's syndrome (PSP-RS). Impaired communication may interfere with daily activities. Therefore, assessment of language functions is crucial. It is uncertain whether the Aachen Aphasia Test (AAT) is practicable in PSP-RS, behavioral variant frontotemporal dementia (bvFTD) and Alzheimer's dementia (AD) and language deficits differ in these disorders. 28 PSP-RS, 24 AD, and 24 bvFTD patients were investigated using the AAT and the CERAD-Plus battery. 16-25% of all patients failed in AAT subtests for various reasons. The AAT syndrome algorithm diagnosed amnestic aphasia in 5 (23%) PSP-RS, 7 (36%) bvFTD and 6 (30%) AD patients, Broca aphasia in 1 PSP-RS and 1 bvFTD patient, Wernicke aphasia in 1 bvFTD and 3 (15%) AD patients. However, aphasic symptoms resembled non-fluent primary progressive aphasia in 14 PSP-RS patients. In up to 46% of PSP-RS patients, 61% of bvFTD and 64% of AD patients significant impairments were found in the AAT subtests spontaneous speech, written language, naming, language repetition, language comprehension and the Token subtest. The CERAD-Plus subtest semantic fluency revealed significant impairment in 81% of PSP-RS, 61% of bvFTD, 44% of AD patients, the phonemic fluency subtest in 31, 40 and 31%, respectively. In contrast to bvFTD and AD, severity of language impairment did not correlate with cognitive decline in PSP-RS. In summary, the patterns of aphasia differ between the diagnoses. Local frontal language networks might be impaired in PSP-RS, whereas in AD and bvFTD, more widespread neuropathology might underly language impairment.
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Enfermedad de Alzheimer , Afasia , Demencia Frontotemporal , Trastornos del Desarrollo del Lenguaje , Parálisis Supranuclear Progresiva , Enfermedad de Alzheimer/complicaciones , Enfermedad de Alzheimer/diagnóstico , Afasia/etiología , Demencia Frontotemporal/complicaciones , Humanos , Pruebas Neuropsicológicas , Parálisis Supranuclear Progresiva/complicaciones , Parálisis Supranuclear Progresiva/diagnósticoRESUMEN
Functional tremor is the most common functional movement disorder. It can be diagnosed with clinically definite certainty at the bedside by ascertaining its inconsistent (distractibility, frequency variability) and incongruent features (entrainment, ballistic suppression), requiring no additional neurological investigations except, in selected cases, those serving to elevate the diagnostic category to laboratory supported using accelerometry and surface electromyography. In the background of excessive attention to the affected body part and abnormal beliefs and expectations, functional correlates include the impairment of emotion processing, sense of agency, and abnormal connectivity between limbic and motor regions. While the treatment options remain under-studied, promising interventions in physiotherapy, cognitive behavioral therapy, and other psychotherapies are under evaluation to assessing their efficacy in attenuating this important source of neurological disability. This article is part of the Special Issue "Tremor" edited by Daniel D. Truong, Mark Hallett, and Aasef Shaikh.
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Acelerometría , Temblor , Electromiografía , Emociones , Humanos , Examen Neurológico , Temblor/diagnóstico , Temblor/terapiaRESUMEN
BACKGROUND: Generation of functional tremor relies on the structures involved in the control of voluntary movements. The clinical diagnosis is based on the presence of "positive signs", which are expression of cognitive and motor distractibility and reflect functional tremor dependence on explicit motor control. In patients who manifest less distractibility, habitual (implicit) control may be of greater significance. Habitual behaviours are inflexible and difficult to eradicate. OBJECTIVES: To investigate if motor and cognitive distractibility predicts response to treatment with repetitive transcranial magnetic stimulation. METHODS: 21 patients with functional tremor underwent 5-day repeated sessions of continuous theta burst stimulation over primary motor cortex. A battery of tests to provoke positive signs was performed during accelerometry recordings and the total functional tremor accelerometry score was calculated for each patient. Response to treatment was measured as change in tremor amplitude, expressed as total power of the spectra between 1 and 30 Hz. RESULTS: On the group level, cTBS significantly changed postural tremor amplitude (Z = -1.9; p = 0.05), with the median decrease of 40%, IQR (-90-(+24)). There was a positive correlation between the functional tremor accelerometry score and reduction of postural tremor amplitude with treatment (rs = -0.75, p < 10-3). Responders had higher functional tremor accelerometry scores compared to non-responders (p = 0.001). The total functional tremor accelerometry score was a significant predictor of treatment response (OR = 2.8, p = 0.03; 95% CI 1.1; 7.2). CONCLUSIONS: Patients who are more distractible are better candidates for treatment with transcranial magnetic stimulation. The likely explanation is the between-subjects differences on the reliance of functional tremor generation on explicit vs. implicit motor network.
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Temblor Esencial , Corteza Motora , Potenciales Evocados Motores/fisiología , Humanos , Estimulación Magnética Transcraneal , Temblor/terapiaRESUMEN
The various forms of tremor are now classified in two axes: clinical characteristics (axis 1) and etiology (axis 2). Electrophysiology is an extension of the clinical exam. Electrophysiologic tests are diagnostic of physiologic tremor, primary orthostatic tremor, and functional tremor, but they are valuable in the clinical characterization of all forms of tremor. Electrophysiology will likely play an increasing role in axis 1 tremor classification because many features of tremor are not reliably assessed by clinical examination alone. In particular, electrophysiology may be needed to distinguish tremor from tremor mimics, assess tremor frequency, assess tremor rhythmicity or regularity, distinguish mechanical-reflex oscillation from central neurogenic oscillation, determine if tremors in different body parts, muscles, or brain regions are strongly correlated, document tremor suppression or entrainment by voluntary movements of contralateral body parts, and document the effects of voluntary movement on rest tremor. In addition, electrophysiologic brain mapping has been crucial in our understanding of tremor pathophysiology. The electrophysiologic methods of tremor analysis are reviewed in the context of physiologic tremor and pathologic tremors, with a focus on clinical characterization and pathophysiology. Electrophysiology is instrumental in elucidating tremor mechanisms, and the pathophysiology of the different forms of tremor is summarized in this review.
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Temblor Esencial , Temblor , Encéfalo , Mapeo Encefálico/efectos adversos , Temblor Esencial/diagnóstico , HumanosRESUMEN
BACKGROUND: Brain structure abnormalities throughout the course of Parkinson's disease have yet to be fully elucidated. OBJECTIVE: Using a multicenter approach and harmonized analysis methods, we aimed to shed light on Parkinson's disease stage-specific profiles of pathology, as suggested by in vivo neuroimaging. METHODS: Individual brain MRI and clinical data from 2357 Parkinson's disease patients and 1182 healthy controls were collected from 19 sources. We analyzed regional cortical thickness, cortical surface area, and subcortical volume using mixed-effects models. Patients grouped according to Hoehn and Yahr stage were compared with age- and sex-matched controls. Within the patient sample, we investigated associations with Montreal Cognitive Assessment score. RESULTS: Overall, patients showed a thinner cortex in 38 of 68 regions compared with controls (dmax = -0.20, dmin = -0.09). The bilateral putamen (dleft = -0.14, dright = -0.14) and left amygdala (d = -0.13) were smaller in patients, whereas the left thalamus was larger (d = 0.13). Analysis of staging demonstrated an initial presentation of thinner occipital, parietal, and temporal cortices, extending toward rostrally located cortical regions with increased disease severity. From stage 2 and onward, the bilateral putamen and amygdala were consistently smaller with larger differences denoting each increment. Poorer cognition was associated with widespread cortical thinning and lower volumes of core limbic structures. CONCLUSIONS: Our findings offer robust and novel imaging signatures that are generally incremental across but in certain regions specific to disease stages. Our findings highlight the importance of adequately powered multicenter collaborations. © 2021 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.
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Enfermedad de Parkinson , Encéfalo/diagnóstico por imagen , Encéfalo/patología , Humanos , Imagen por Resonancia Magnética , Neuroimagen , Enfermedad de Parkinson/complicaciones , Tálamo/patologíaRESUMEN
Progressive supranuclear palsy (PSP) and corticobasal syndrome (CBS) progress relentlessly and lead to a need for care. Caregiving is often burdensome. Little is known about the course of caregiver burden (CB) in PSP and CBS patients. Longitudinal analysis of CB in family members caring for PSP and CBS patients. Single-center longitudinal pilot study in 68 newly diagnosed patients with probable PSP and CBS (52 Richardson's syndrome; 1 progressive gait freezing of PSP; 15 CBS). Demographic, educational, occupational parameters, family status, motor functions (UPDRSIII, Hoehn and Yahr Score, Tinetti) and neuropsychological performance (CERAD Plus, Frontal Assessment Battery) were assessed, as well as behavioral and neuropsychiatric impairments (Frontal Behavioral Inventory, Neuropsychiatric Inventory), activities of daily living (ADL) and caregiver burden using the Caregiver Strain Index (CSI), in most patients also the Zarit Burden Interview (ZBI). Patients were followed up every 6 months for up to 2 years. Caregivers reported mild to moderate CB at baseline, which increased by 25-30% in 2 years and was significantly greater in PSP than in CBS. Risk for mental health problems increased over time, especially in female caregivers (depression). Important patient-related factors were apathy, aspontaneity, depression, irritability, disorganization, poor judgment, impairment of language, impairments in ADL, a high educational level of the patient and close family relationship. Behavioral symptoms and impaired ADL are the main patient-related factors of CB in PSP and CBS. CB can be severe and needs to be assessed repeatedly from the time of diagnosis to provide comprehensive support.
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Parálisis Supranuclear Progresiva , Actividades Cotidianas , Cuidadores , Femenino , Humanos , Proyectos Piloto , SíndromeRESUMEN
BACKGROUND: An objective evaluation of tremor severity is necessary to document the course of disease, the efficacy of treatment, or interventions in clinical trials. Most available objective quantification devices are complex, immobile, or not validated. NEW METHOD: We used the TREMITAS-System that comprises a pen-shaped sensor for tremor quantification. The Power of Main Peak and the Total Power were used as surrogate markers for tremor amplitude. Tremor severity was assessed by the TREMITAS-System and relevant subscores of the MDS-UPDRS and TETRAS rating scales in 14 patients with Parkinson's disease (PD) and 16 patients with Essential tremor (ET) off and on therapy. We compared tremor amplitudes assessed during wearable and hand-held constellations. RESULTS: We found significant correlations between tremor amplitudes captured by TREM and tremor severity assessed by the MDS-UPDRS in PD (r = 0.638-0.779) and the TETRAS in ET (r = 0.597-0. 704) off and on therapy. The TREMITAS-System captured the L-Dopa-induced improvement of tremor in PD patients (p = 0.027). Tremor amplitudes did not differ between the handheld and wearable constellation (p > 0.05). COMPARISON WITH EXISTING METHODS: We confirm the results of previous studies using inertial based sensors that tremor severity and drug-induced changes of tremor severity can be quantified using inertial based sensors. The assessment of tremor amplitudes was not influenced by using a handheld or wearable constellation. CONCLUSIONS: The TREMITAS-System can be used to quantify rest tremor in PD and postural tremor in ET and is capable of detecting clinically relevant changes in tremor in clinical and research settings.
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Background: Cervical dystonia is the most common form of focal dystonia. The frequency and pattern of degenerative changes of the cervical spine in patients with cervical dystonia and their relation to clinical symptoms remain unclear as no direct comparison to healthy controls has been performed yet. Here, we used magnetic resonance imaging (MRI) to investigate (1) whether structural abnormalities of the cervical spine are more common in patients with cervical dystonia compared to age-matched healthy controls, (2) if there are clinical predictors for abnormalities on MRI, and (3) to calculate the inter-rater reliability of the respective radiological scales. Methods: Twenty-five consecutive patients with cervical dystonia and 20 age-matched healthy controls were included in the study. MRI scans of the cervical spine were analyzed separately by three experienced raters blinded to clinical information, applying different MRI rating scales. Structural abnormalities were compared between groups for upper, middle, and lower cervical spine segments. The associations between scores differentiating both groups and clinical parameters were assessed in dystonia patients. Additionally, inter-rater reliability of the MRI scales was calculated. Results: Comparing structural abnormalities, we found minor differences in the middle cervical spine, indicated by a higher MRI total score in patients but no significant correlation between clinical parameters and MRI changes. Inter-rater reliability was satisfying for most of the MRI rating scales. Conclusion: Our results do not provide evidence for a role of MRI of the cervical spine in the routine work-up of patients with cervical dystonia in the absence of specific clinical signs or symptoms.
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OBJECTIVE: We investigated R2* relaxation rates as a marker of iron content in the substantia nigra in patients with common tremor disorders and explored their diagnostic properties. METHODS: Mean nigral R2* rates were measured in 40 patients with tremor-dominant Parkinson's disease (PD), 15 with tremor in dystonia, 25 with essential tremor, and 25 healthy controls. RESULTS: Tremor-dominant PD patients had significantly higher nigral R2* values (34.1 ± 5.7) than those with tremor in dystonia (30.0 ± 3.9), essential tremor (30.6 ± 4.8), and controls (30.0 ± 2.8). An R2* threshold of 31.15 separated tremor-dominant PD from controls with a sensitivity and specificity of 67.5% and 72%. The sensitivity and specificity for discrimination between PD and non-PD tremor patients was 67.5% and 60%. CONCLUSION: Iron content in the substantia nigra is significantly higher in tremor-dominant PD than in tremor in dystonia, essential tremor, and controls. Because of the considerable overlap, nigral R2* cannot be suggested as a useful diagnostic tool. © 2018 The Authors. Movement Disorders published by Wiley Periodicals, Inc. on behalf of International Parkinson and Movement Disorder Society.