Movement Disorders Clinical Practice最新文献

Background: Limited evidence exists regarding the meaningfulness of symptoms experienced in early Parkinson's disease (PD).

Objectives: To identify the most bothersome symptoms experienced by people with early PD, leveraging data from the Parkinson's Disease Patient Report of Problems (PD-PROP) questionnaire within the Fox Insight Study.

Methods: Individuals with a self-reported diagnosis of PD completed the PD-PROP questionnaire, reporting up to five most bothersome symptoms. Symptom types and frequencies were derived through a combination of human expertise and machine learning.

Results: Participants (N = 8536) were 0.9 years since diagnosis, predominantly white (96%), male (53.3%), and with an average age of 64.6 years. Top most bothersome motor symptoms were tremor (55.9%) and gait issues (36.7%). Top most bothersome non-motor symptoms were pain/discomfort (33.1%) and physical fatigue (27.5%).

Conclusions: This study underscores the complexity of early PD symptomatology. Future consideration of diverse patient experiences is needed to improve therapeutic and outcome measurement strategies.

Background: Nowadays, cognitive impairment has been characterized as one of the most vital clinical symptoms in progressive supranuclear palsy (PSP).

Objectives: Based on a relatively large cohort, we aimed to show the cognitive deterioration in different PSP subtypes during 1-year follow-up and investigate potential contributors for disease prognosis.

Methods: One hundred seventeen patients from Progressive Supranuclear Palsy Neuroimage Initiative (PSPNI) cohort underwent neuropsychological tests and 1-year follow-up, with 73 diagnosed as PSP-Richardson syndrome (PSP-RS) and 44 as PSP-non-RS. Patients were divided into normal cognition (PSP-NC), mild cognitive impairment (PSP-MCI), and PSP-dementia. Cognitive impairment and progression rates were compared between PSP-RS and PSP-non-RS, and determinants for MCI conversion to dementia were calculated by multiple cox regression.

Results: At baseline, 30.8% of PSP patients were diagnosed as dementia, 53.0% as MCI, and only 16.2% as NC. Compared to PSP-non-RS, PSP-RS suffered more from motor symptoms and cognitive impairment. During follow-up, PSP-RS also exhibited faster disease progression in Mini-Mental State Examination and visuospatial function, with cognitive deterioration in attention and executive function, but retained in language and memory subdomains. Twenty-seven of 62 PSP-MCI patients converted to dementia during follow-up, with the diagnosis of RS subtype as the most significant contributor to conversion (hazard ration = 2.993, 95% confidence interval = 1.451, 5.232, P = 0.009).

Conclusions: Patients with PSP-RS showed more severe cognitive impairment and faster decline longitudinally than patients with PSP-non-RS. Additionally, the diagnosis of RS subtype appears to be the most contributed factor for MCI conversion to dementia within just 1-year follow-up period.

Background: While previous imaging studies have generally shown normal striatal dopamine transporter (DAT) binding in essential tremor (ET), emerging evidence suggests a partial dopaminergic mechanism in this condition and an epidemiological link between ET and Parkinson's disease (PD). This link seems particularly meaningful in ET patients with additional neurological signs, such as slowness of movements, rigidity, or rest tremor (ET+).

Objectives: To investigate the potential dopaminergic pathophysiology of ET+ and to compare it to PD.

Methods: Fourty-three ET+ patients, 115 PD patients and 40 healthy controls were studied using [¹²³I]FP-CIT SPECT imaging and clinical examinations. A median follow-up of 3.0 years was carried out to confirm the diagnoses. ET+ patients underwent an extended follow-up with a median of 7.7 years (range 4.3-9.8 years). Region-specific binding ratios of striatal DAT binding were compared among the groups and correlated with the MDS-UPDRS motor scores.

Results: Bradykinesia scores were negatively associated with posterior putamen DAT binding in both the ET+ and PD groups, with the strongest correlation observed in finger tapping (F = 11.1, β = -0.10, 95%CI -0.16 to -0.04, P = 0.001). In ET+ patients, kinetic tremor asymmetry correlated with posterior putamen DAT binding asymmetry (r = 0.33, P = 0.043), indicating a relationship between more severe tremor and subtle contralateral DAT loss.

Conclusions: In ET+, subtle increases in bradykinesia scores correlate with striatal dopaminergic dysfunction, while kinetic tremor asymmetry is associated with hemispheric DAT binding asymmetry. These findings support the concept of partial dopaminergic involvement in the pathophysiology of ET+.

Background: Gastrointestinal dysfunction (GID) accompanies any phase of Parkinson's disease (PD), underlying differential clinical-pathological trajectories.

Objective: To investigate associations between GID and peripheral immune or neurodegeneration-related markers in PD.

Methods: One-hundred-and-fourteen patients (n = 55 de novo, DN; n = 59 middle-advanced, MA) completed the Gastrointestinal Dysfunction Scale for PD (GIDS-PD), and other motor and non-motor scales; paired measurement of amyloid-β42, amyloid-β42β/β40, total-tau, phosphorylated-181-tau, total α-synuclein CSF levels, albumin ratio, and peripheral blood cell count were collected. Group and correlation analyses were performed.

Results: MA patients had greater GID than DN. GIDS-PD scores directly correlated with MDS-UPDRS-III and non-motor scores in both groups, although more in DN. GIDS-PD scores were directly associated with α-synuclein and inversely with lymphocytes only in DN; conversely, they were positively associated with tau proteins and albumin ratio, and negatively with amyloid-β-peptides in both groups.

Conclusions: The burden of GID increases along the PD course with associated stage-specific clinical-biological patterns.

Background: Parkinson's disease (PD) is the fastest-growing neurological disorder globally. Defining features include tremor, muscular rigidity, bradykinesia, and postural instability, which in combination with nonmotor symptoms such as cognitive impairment and orthostatic hypotension increase the risk of falls. Along with low bone mineral density, fracture risk is high in PD.

Objectives: The aims were to identify and appraise clinical practice guidelines, consensus statements, and treatment algorithms containing recommendations for bone health in people with PD (PwP).

Methods: We systematically searched 4 electroninc databases (MEDLINE, Embase, Emcare, and Web of Science) (n = 78), in addition to the websites of organizations, societies, and professional bodies focused on PD or osteoporosis (n = 28), up to April 22, 2024.

Results: After duplicate removal, screening, and full-text review, 6 records were included. Included records were appraised using the AGREE II (Appraisal of Guidelines for Research and Evaluation) tool. All records recognized bone health as a concern in PD, yet recommendations for fracture-risk screening were inconsistent. Two of six records grouped PD under the broad category of neurological diseases. The acceptability and tolerance of anti-osteoporosis medications in PwP was discussed only in 1 record, which incorporated national osteoporosis guidelines into a PD-specific treatment algorithm.

Conclusions: This review highlights that despite the documented high fracture rates of PwP, health professionals do not always have adequate resources to support them when considering how to manage osteoporosis. Osteoporosis screening and management needs to be incorporated into PD treatment guidelines, and equally providing specific recommendations for PwP related to bone health in national osteoporosis guidelines should be a priority given the high burden of fracture in the patient population.

Background: The role played by sympathetic and parasympathetic autonomic branches in patients with Parkinson's disease carrying variants in the GBA1 gene (GBA-PD) is still elusive.

Objectives: To characterize cardiovascular autonomic function in GBA-PD and I-PD patients with early and mid-stage disease.

Methods: These assessments were performed: cardiovascular autonomic tests, analysis of heart rate and blood pressure variability, cardiac noradrenergic imaging. The frequency and severity of autonomic symptoms were comparatively assessed with the SCOPA-AUT questionnaire.

Results: Compared to the I-PD cohort, GBA-PD patients displayed an increased burden of autonomic symptoms. Autonomic tests revealed worse parasympathetic scores in GBA-PD while sympathetic indexes and the degree of cardiac sympathetic binding were comparable in the two groups. Heart rate variability indexes also revealed lower vagal modulation in the GBA-PD group.

Conclusions: The cardiovascular autonomic profile in GBA PD is characterized by a prominent cardiovagal dysfunction compared to I-PD.