Movement Disorders Clinical Practice最新文献

Background: Real-world clinical evidence is missing to understand the resorption characteristics of levodopa through duodenal and jejunal parts of the small intestine.

Objective: To characterize how different application sites of intestinal levodopa gel would impact on levodopa dosing and clinical outcomes.

Methods: This multicentre retrospective analysis investigated Parkinson's disease patients (n = 111) and their change in levodopa equivalent dosage when switching from oral treatment to intestinal continuous infusion therapy while stratifying for differences in percutaneous gastrojejunostomy (PEG-J) tube localizations. We analyzed data from patients treated with both levodopa-carbidopa (LCIG) and levodopa-carbidopa-entacapone (LECIG) intestinal gel.

Results: In dichotomic analysis, duodenal and jejunal tube positions showed similar levodopa equivalent dosages changes from baseline (P = 0.143). This was similar when subdividing patients for LCIG and LECIG treatment. In duodenal PEG-J positions, 44.4% of patients showed persistent motor fluctuations compared to 21.9% in jejunal placements (P = 0.026). In duodenal positions, fluctuations most often persisted when the PEG-J tube was placed proximally into the duodenum. In jejunal localizations, several patients displayed a satisfactory outcome from the primary intervention but experienced dislocation of the PEG-J tube to a duodenal position. This was associated with re-emergence of motor fluctuations in a majority of them.

Conclusions: Our real-world data suggest that LCIG and LECIG are absorbed similarly in both duodenal and jejunal portions of the small intestine. However, clinical data suggest, that jejunal positioning is critical to the stabilization of dopaminergic motor fluctuations.

Background: The new classification of progressive supranuclear palsy (PSP) subtypes necessitates identifying radiological biomarkers to support the clinical diagnosis.

Objective: The goals was to test if magnetic resonance imaging (MRI) morphometry, diffusion tensor imaging (DTI), susceptibility-weighted imaging (SWI), or [18F]fluorodeoxyglucose (^18FFDG)-positron emission tomography (PET) differentiates PSP subtypes from each other or Parkinson's disease (PD).

Methods: Midbrain/pons (M/P) area ratio, middle/superior cerebellar peduncle (MCP/SCP) width ratio, magnetic resonance parkinsonism indices (MRPI and MRPI2) and midbrain antero-posterior (AP) diameter were measured. Region of interest-based DTI, SWI, and ^18FFDG-PET analyses were performed.

Results: Four PSP subtypes (n = 85) and 24 PD were studied. MRI morphometry and DTI could differentiate PSP-Richardson syndrome (PSP-RS) from PSP-parkinsonism, PSP-postural instability, and PD (area under curve >0.7). SWI did not differentiate among PSP subtypes or PD. ^18FFDG-PET distinguished PSP from PD.

Conclusions: MRI morphometry and DTI differentiated PSP-RS from the other common PSP subtypes and PD and may be tested as a radiological marker of PSP-RS in larger studies.

Background: The MDS-UPDRS Parts IB and II are self-reported items providing a direct patient voice to the experiences of PD.

Objective: To determine the most sensitive combination of MDS-UPDRS Parts IB and II items that accurately predicted the clinically relevant target of dopaminergic therapy initiation.

Methods: Utilizing a longitudinal cohort of de novo non-treated PD patients, we applied item response theory (IRT) and survival analysis to assess the relationship between baseline patient-reported symptoms and the later initiation of dopaminergic therapy. The 20 MDS-UPDRS Parts IB and II items were analyzed for their relationship to PD severity (discrimination) and the amount of information they provided in this determination (information). These parameters were used to develop models of predictive accuracy for initiation of dopaminergic therapy.

Results: A six-item version showed a significantly higher C-index as compared to the full 20 item model (P = 0.001). This shortened version of the MDS-UPDRS contained only Part II items and provided a predictive accuracy for initiation of dopaminergic therapy better than the total combined scale score or any other combination.

Conclusions: A six-item "Baseline Outcome Voice" version of patient-reported MDS-UPDRS items significantly increases the sensitivity of predicting the key future clinical outcome of starting dopaminergic treatment in early PD. This study also demonstrates how IRT modeling can provide information useful to refining existing measures to identify the most sensitive combination of items honoring the voice of the patient in determining key clinically pertinent decisions. Further research is needed to validate these findings in underrepresented populations.

Background: KCNMA1-linked channelopathy is characterized by neurodevelopmental disorder, epileptic seizures and non-epileptic paroxysmal episodes.

Objectives: To describe the phenotype of paroxysmal non-epileptic episodes related to KCNMA1 pathogenic variants.

Methods: Videos of paroxysmal episodes were reviewed according to a standardized protocol by a group of movement disorders experts.

Results: Fourteen videos were reviewed (6 previously published patients and a new patient). The typical pattern of an episode was (i) facial changes including dyskinetic movements of tongue and jaws (ii) behavioral arrest (iii) loss of postural reflexes that could be associated with focal body stiffness, eventually leading to fall (iv) rapid recovery without post-ictal drowsiness. Attacks were brief, with a high daily frequency, occasionally triggered by emotion, and dramatically improved by psychostimulant therapy in three patients.

Conclusions: KCNMA1-related attacks are clearly distinguishable from paroxysmal dyskinesia, cataplexy or episodic ataxia indicating a unique phenomenological entity whose recognition will enhance accurate diagnosis and treatment.

Background: In Parkinson's Disease (PD), upper limb tremor during walking (TW) is observed and clinical observations suggest it may represent a variant of rest tremor. However, its neurophysiological characteristics remain unexplored.

Objectives: This study compared the neurophysiological features of TW with other PD tremors and tested whether TW arises from reduced ipsilateral arm swing.

Methods: Inertial measurement units were used to measure frequency and amplitude of tremors and arm swing during walking in 25 PD patients.

Results: TW shared a similar frequency with rest and re-emergent tremor (RET) but showed significantly greater amplitude. A positive correlation was observed between the amplitude and frequency of TW with those of rest and RET on the same side. TW distribution was unrelated to reduced arm swing during walking, suggesting TW is not due to decreased ipsilateral arm movement.

Conclusions: These findings suggest that walking may act as a provocation maneuver, triggering rest tremor.