Publications by authors named "Krismer F"

Huntington's disease (HD) is a progressive neurodegenerative disorder for which, until now, only symptomatic treatment has been available. Lately, there have been multiple ongoing clinical trials targeting therapeutic agents for preventing disease onset or slowing disease progression in HD. These studies are in constant need of reliable biomarkers for neurodegeneration in HD.

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Background And Objective: Non-motor symptoms frequently develop throughout the disease course of Parkinson's disease (PD), and pose affected individuals at risk of complications, more rapid disease progression and poorer quality of life. Addressing such symptom burden, the 2023 revised "Parkinson's disease" guideline of the German Society of Neurology aimed at providing evidence-based recommendations for managing PD non-motor symptoms, including autonomic failure, pain and sleep disturbances.

Methods: Key PICO (Patient, Intervention, Comparison, Outcome) questions were formulated by the steering committee and refined by the assigned authors.

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Article Synopsis
  • Multiple system atrophy (MSA) is a rare, progressive neurological disorder that usually starts in adulthood and features a variety of both motor and non-motor symptoms, often resembling other conditions like Parkinson's disease.
  • Research over the past five years has deepened understanding of MSA, particularly regarding the role of α-synuclein inclusions, which are linked to the disease's progression.
  • Updated diagnostic criteria in 2022 aim to improve early diagnosis, and while recent clinical trials show promise for new treatments, definitive evidence of neuroprotection is still lacking.
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Background: Subthalamic deep brain stimulation (STN-DBS) reduces antiparkinsonian medications in Parkinson's disease (PD) compared with the preoperative state. Longitudinal and comparative studies on this effect are lacking.

Objective: To compare longitudinal trajectories of antiparkinsonian medication in STN-DBS treated patients to non-surgically treated control patients.

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Article Synopsis
  • Multiple system atrophy (MSA) is a neurodegenerative disease that leads to symptoms like parkinsonism and ataxia, but its genetic causes are not well understood and treatment options are limited to supportive care.
  • A comprehensive study involving the whole genome sequencing of nearly 900 MSA patients and over 7,000 controls discovered four key genetic risk factors associated with the disease.
  • The research identified potential susceptibility genes and provided insights into how genetic variations influence gene expression in brain cells, offering a valuable resource for further studies on similar diseases.
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Background: A 4-item score based on ≥2 features out of orthostatic hypotension, overactive bladder, urinary retention and postural instability was previously shown to early distinguish the Parkinson-variant of multiple system atrophy (MSA-P) from Parkinson's disease (PD) with 78% sensitivity and 86% specificity.

Objectives: To replicate and improve the 4-item MSA-P score.

Methods: We retrospectively studied 161 patients with early parkinsonism [ie, ≤2 years disease duration or no postural instability, aged 64 (57; 68) years, 44% females] and a diagnosis of clinically established MSA-P (n = 38) or PD (n = 123) after ≥24 months follow-up.

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Article Synopsis
  • The study aimed to explore sex-related differences in the clinical presentation and progression of multiple system atrophy (MSA) using literature and a retrospective cohort analysis.
  • A total of 46 publications were reviewed, revealing comparable survival rates between men and women, though some suggested women might have an advantage due to less severe symptoms at onset.
  • Findings from a cohort analysis showed that women were more likely to experience depression and use specific medications, while men had higher occurrences of severe orthostatic hypotension and supine hypertension, emphasizing the need for sex consideration in MSA treatment and research.
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The synthetic tetrahydrocannabinol-analog nabilone improved non-motor symptoms (NMS) in Parkinson's disease (PD) patients in a placebo-controlled, double-blind, parallel-group, randomized withdrawal trial with enriched enrollment (NMS-Nab-study). This was a single-center open-label extension study to assess the long-term safety and efficacy of nabilone for NMS in PD. To be eligible for this study, patients had to be treatment responders during the previous NMS-Nab-trial and complete its double-blind phase without experiencing a drug-related serious/severe/moderate adverse event (AE).

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Background: Advanced imaging techniques have been studied for differential diagnosis between PD, MSA, and PSP.

Objectives: This study aims to validate the utility of individual voxel-based morphometry techniques for atypical parkinsonism in a blinded fashion.

Methods: Forty-eight healthy controls (HC) T1-WI were used to develop a referential dataset and fit a general linear model after segmentation into gray matter (GM) and white matter (WM) compartments.

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Objective: To determine the rates of brain atrophy progression in vivo in patients with multiple system atrophy (MSA).

Background: Surrogate biomarkers of disease progression are a major unmet need in MSA. Small-scale longitudinal studies in patients with MSA using magnetic resonance imaging (MRI) to assess progression of brain atrophy have produced inconsistent results.

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Article Synopsis
  • - Multiple system atrophy (MSA) is a severe disease with varying motor and autonomic symptoms, and previous studies have linked certain clinical factors to reduced survival rates.
  • - Researchers analyzed 210 MSA patients over 17 years to create a survival risk model using clinical factors like age at symptom onset and early autonomic failure.
  • - They developed a nomogram to predict individual survival probabilities over 7 years, which showed good accuracy and could enhance patient counseling and treatment strategies.
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Objective: Emotional processing is a core feature of social interactions and has been well studied in patients with idiopathic Parkinson's disease (PD), albeit with contradictory.

Results: . However, these studies excluded patients with atypical parkinsonism, such as multiple system atrophy (MSA).

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Background: An absent dorsolateral nigral hyperintensity (DNH) is a common finding in patients with neurodegenerative parkinsonism at high or ultra-high field susceptibility-weighted magnetic resonance imaging (SWI).

Objective: Despite increasing use of high field magnetic resonance imaging (MRI) in specialized centers, these scanners are still frequently unavailable in primary care or outpatient facilities and underdeveloped or emerging countries. Therefore, the aim of the present study was to evaluate the diagnostic utility of DNH assessment at 1.

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Parkinson's disease (PD) is the second-most common neurodegenerative disorder with a long-term 60% cumulative prevalence of PD psychosis. Medical treatment is limited to few atypical antipsychotic drugs with low affinity to dopamine D2 receptors. In 2016, pimavanserin, a selective 5-HT2A inverse agonist/antagonist, was approved by the US Food and Drug Administration (FDA) as the only treatment for PD psychosis (PDP).

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Background: Real-time quaking-induced conversion (RT-QuIC) and protein misfolding cyclic amplification (PMCA) have been developed to detect minute amounts of amyloidogenic proteins via amplification techniques and have been used to detect misfolded α-synuclein (αSyn) aggregates in the cerebrospinal fluid (CSF) and other source materials of patients with Parkinson's Disease and other synucleinopathies.

Objectives: The aim of this systematic review and meta-analysis was to evaluate the diagnostic accuracy of αSyn seed amplification assays (αSyn-SAAs), including RT-QuIC and PMCA, using CSF as source material to differentiate synucleinopathies from controls.

Methods: The electronic MEDLINE database PubMed was searched for relevant articles published until June 30, 2022.

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Background: Sarcopenia is characterized by a progressive loss of muscle mass, strength, and function resulting in adverse health outcomes. Current assessment strategies are bothersome and means to simplify the diagnosis are an unmet medical need in Parkinson's disease (PD).

Objective: To evaluate temporal muscle thickness (TMT) obtained on routine cranial MRI as a surrogate marker of sarcopenia in PD patients.

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Background: Early identification of Parkinson's disease (PD) patients at risk for becoming functionally dependent is important for patient counseling. Several models describing the relationship between predictors and outcome have been reported, however, most of these require computer software for practical use.

Objective: Here we report the development of a risk nomogram allowing an approximate graphical computation of the risk of becoming functionally dependent in early PD.

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Introduction: Sarcopenia and Parkinson's disease are closely related diseases of the elderly population leading to progressive disability and nursing-dependent care.

Objective: The aim of this study was to estimate the prevalence of sarcopenia in PD patients with three different approaches: (1) the screening tool SARC-F, (2) EWGSOP-1 criteria, and (3) EWGSOP-2 criteria. Moreover, we aimed to evaluate the diagnostic accuracy of the screening tool SARC-F to detect sarcopenia according to the updated EWGSOP-2 criteria.

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Article Synopsis
  • The Global Multiple System Atrophy Registry (GLOMSAR), created in 2013, is an online platform where patients or caregivers can self-report information about multiple system atrophy (MSA).
  • The study aimed to analyze the demographics of GLOMSAR participants and gather data from a symptom-focused online questionnaire, with 33% of the 1083 participants completing it.
  • Findings revealed that many symptoms, like memory issues and visual hallucinations, are commonly reported by patients but often overlooked in traditional physician studies, emphasizing the importance of patient experiences in MSA research.
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Study Objectives: Sleep disorders, daytime sleepiness, and autonomic dysfunction are commonly reported among patients with multiple system atrophy and Parkinson disease (PD). We aimed to assess sleep and autonomic function in these patients to evaluate the relationships between sleep disorders, excessive daytime sleepiness, and autonomic function.

Methods: Twenty patients with multiple system atrophy (n = 7) and PD (n = 13) underwent clinical assessment including questionnaires for autonomic function and sleep.

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Background: The synthetic tetrahydrocannabinol analogue nabilone improved overall non-motor symptom (NMS) burden in Parkinson's disease (PD) patients in comparison to placebo.

Objectives: To characterize the effects of nabilone on different sleep outcomes in PD patients.

Methods: We performed a post-hoc analysis of the controlled, double-blind, enriched enrollment randomized withdrawal NMS-Nab study to assess the effects of nabilone on sleep outcomes in study participants who reported clinically-relevant sleep problems (MDS-UPDRS-1.

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