Publications by authors named "Petra Baum"

Article Synopsis
  • A study was conducted at 11 ALS centers in Germany from October 2021 to February 2024 to evaluate the frequency of pathogenic gene variants in ALS patients and their transition to an expanded access program for tofersen treatment.
  • Out of 1935 patients screened, 48.8% chose to be informed about genetic variants related to tofersen, revealing that 1.8% had (likely) pathogenic variants, 0.9% had other pathogenic variants, and 7.0% showed hexanucleotide repeat expansion.
  • The transition to tofersen treatment from genetic testing averaged 94 days, with a notable 74.0% of patients with certain variants opting for the therapy, highlighting the importance of comprehensive
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Objective: Robotic arms are innovative assistive devices for ALS patients with progressive motor deficits of arms and hands. The objective was to explore the patients´ expectations towards a robotic arm system and to assess the actual experiences after the provision of the device.

Methods: A prospective observational study was conducted at 9 ALS centers in Germany.

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Article Synopsis
  • The study aimed to evaluate the relationship between serum neurofilament light chain (sNfL) levels and different clinical forms of amyotrophic lateral sclerosis (ALS) in nearly 3000 patients across Germany and Austria.
  • Results indicated that sNfL levels varied significantly across ALS phenotypes, with higher levels associated with faster disease progression and bulbar onset, while primary lateral sclerosis (PLS) and some other phenotypes showed lower levels.
  • The findings suggest that understanding these phenotypes is crucial for interpreting sNfL results and could influence clinical trials and practice in ALS management.
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Article Synopsis
  • The study examined the long-term efficacy and safety of nusinersen in adults with 5q-associated spinal muscular atrophy (SMA) over a period of 38 months, utilizing a large cohort from Germany, Switzerland, and Austria.
  • Overall, significant improvements were noted in various motor performance measures (HFMSE, RULM, and 6MWT) at multiple time points compared to baseline, indicating ongoing benefits from the treatment.
  • No new safety concerns were found, reinforcing the idea that nusinersen remains a viable therapy for adults with SMA over extended periods.
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Background: In sepsis-associated critical illness neuromyopathy (CIPNM) serial electrical stimulation of motor nerves induces a short-lived temporary recovery of compound muscle action potentials (CMAPs) termed facilitation phenomenon (FP). This technique is different from other stimulation techniques published. The identification of FP suggests a major functional component in acute CIPNM.

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Background: Nonconvulsive seizures (NCS) and nonconvulsive status epilepticus (NCSE) are frequently observed in human patients. Diagnosis of NCS and NCSE only can be achieved by the use of electroencephalography (EEG). Electroencephalographic monitoring is rare in veterinary medicine and consequently there is limited data on frequency of NCS and NCSE.

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Background: Causal therapies are not yet available for most neuromuscular diseases. Additionally, data on the use of complementary or alternative therapies (CAM) in patients groups with a variety of different neuromuscular diseases are rare. This retrospective cross-sectional study aims to record the frequency of use and satisfaction of conventional therapies and complementary or alternative medicine (CAM) in patients with neuromuscular disorders in order to compare them afterwards.

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Introduction: Post-stroke depressive symptoms (PSDS) are common and relevant for patient outcome, but their complex pathophysiology is ill understood. It likely involves social, psychological and biological factors. Lesion location is a readily available information in stroke patients, but it is unclear if the neurobiological substrates of PSDS are spatially localized.

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Background And Purpose: The objective was to assess the performance of serum neurofilament light chain (sNfL) in amyotrophic lateral sclerosis (ALS) in a wide range of disease courses, in terms of progression, duration and tracheostomy invasive ventilation (TIV).

Methods: A prospective cross-sectional study at 12 ALS centers in Germany was performed. sNfL concentrations were age adjusted using sNfL Z scores expressing the number of standard deviations from the mean of a control reference database and correlated to ALS duration and ALS progression rate (ALS-PR), defined by the decline of the ALS Functional Rating Scale.

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Background: Depressive symptoms are a common stroke sequela, yet their neurobiological substrates are still unclear. We sought to determine if they are associated with specific lesion locations.

Methods: In a prospective observational study, 270 patients with stroke were tested twice with the Hospital Anxiety and Depression Scale around day 6 and again 6 months poststroke and voxel-based lesion behavior mapping was performed.

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Objective: Critical Illness Neuromyopathy (CIPNM) is a complication in sepsis patients with still enigmatic disease mechanisms. We investigated a novel electrical stimulation method to better define neuromuscular dysfunction in patients with CIPNM.

Methods: We studied 18 sepsis CIPNM patients on intensive care units, 13 at an early and 5 at a later disease stage, 7 sepsis control, and 8 neuropathy control patients.

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Objective: Remote self-assessment of the revised amyotrophic lateral sclerosis functional rating scale (ALSFRS-R) using digital data capture was investigated for its feasibility as an add-on to ALSFRS-R assessments during multidisciplinary clinic visits.

Methods: From August 2017 to December 2021, at 12 ALS centers in Germany, an observational study on remote assessment of the ALSFRS-R was performed. In addition to the assessment of ALSFRS-R during clinic visits, patients were offered a digital self-assessment of the ALSFRS-R - either on a computer or on a mobile application ("ALS-App").

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Motor-assisted movement exercisers (MME) are devices that assist with physical therapy in domestic settings for people living with ALS. This observational cross-sectional study assesses the subjective experience of the therapy and analyzes users' likelihood of recommending treatment with MME. The study was implemented in ten ALS centers between February 2019 and October 2020, and was coordinated by the research platform Ambulanzpartner.

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Background: A treatment-induced drop in HbA1c has been suggested to be a risk factor for TIND.

Methods: From 60 included patients with severe diabetes mellitus (HbA1c over 8.5) only 21 patients adhered to the study protocol over 1 year with a battery of autonomic nervous system tests scheduled before and after starting antidiabetic treatment.

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Recent studies have shown an association between iron homeostasis, obesity and diabetes. In this work, we investigated the differences in the metabolic status and inflammation in liver, pancreas and visceral adipose tissue of leptin receptor-deficient mice dependent on high iron concentration diet. 3-month-old male BKS-Leprdb/db/JOrlRj () mice were divided into two groups, which were fed with different diets containing high iron (29 g/kg, = 57) or standard iron (0.

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The pathogenesis of diabetic neuropathy is complex, and various pathogenic pathways have been proposed. A better understanding of the pathophysiology is warranted for developing novel therapeutic strategies. Here, we summarize recent evidence from experiments using animal models of type 1 and type 2 diabetes showing that low-grade intraneural inflammation is a facet of diabetic neuropathy.

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Treatment-induced neuropathy in diabetes (TIND) is defined by the occurrence of an acute neuropathy within 8 weeks of an abrupt decrease in glycated hemoglobin-A1c (HbA1c). The underlying pathogenic mechanisms are still incompletely understood with only one mouse model being explored to date. The aim of this study was to further explore the hypothesis that an abrupt insulin-induced fall in HbA1c may be the prime causal factor of developing TIND.

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Background And Purpose: Poststroke depression is a common stroke sequel, yet its neurobiological substrates are still unclear. We sought to determine whether specific lesion locations are associated with depressive symptoms after stroke.

Methods: In a prospective study, 270 patients with first ever stroke were repeatedly tested with the depression subscale of the Hospital Anxiety and Depression Scale within the first 4 weeks and 6 months after stroke.

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Muscle pain as a common symptom in daily practice frequently occurs as a non-specific accompanying symptom in multiple internal and neurological diseases. Primarily inflammatory or autoimmune muscular diseases are causing muscle pain. However, a number of non-inflammatory causes of pain can also be considered for differential diagnosis.

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Cartilage oligomeric matrix protein (COMP)-Angiopoietin-1 is a potent angiopoietin-1 (Ang-1) variant that possesses therapeutic potential in angiogenesis and vascular endothelial dysfunction. Noteworthy, we have shown that COMP-Ang-1 improves hyperglycemia and neuroregeneration in / mice. However, the mechanism of the antidiabetic effect of COMP-Ang-1 is completely unknown.

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Peripheral diabetic neuropathy (PDN) is one of the most common complications of diabetes mellitus. Previous studies showed an association between dietary iron load and inflammation in the development of PDN in a rat model of type 1 diabetes (T1D). Here we investigated the role of iron and neural inflammation in development of PDN in a animal model of obesity and type 2 diabetes (T2D).

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Introduction: Here, we investigated inflammatory signs of peripheral nerves in leptin-deficient obese ob/ob mice and the modulating effects of the exogenous iron load.

Methods: Ob/ob and ob/+ control mice were fed with high, standard, or low iron diet for four months.

Results: We found intraepidermal nerve fiber degeneration in foot skin and low-grade neuropathic abnormalities including mildly slowed motor and compound sensory nerve conduction velocities and low-grade macrophage and T-cell infiltration without overt neuropathology in sciatic nerves of all ob/ob mice.

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Objective: The procurement of assistive technology devices (ATD) is an essential component of managed care in ALS. The objective was to analyze the standards of care for ATD and to identify challenges in the provision process.

Methods: A cohort study design was used.

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Purpose: To develop specific diagnostic ultrasound (US) models for hereditary motor and sensory neuropathies (HMSN) in patients with primarily demyelinating or axonal polyneuropathies (PNP) according to standard nerve conduction studies (NCS) criteria.

Methods: Single-centre, examiner-blinded cross-sectional study in acquired PNP (consecutive recruitment strategy) and HMSN patients (convenience sample). Allocation into demyelinating or axonal phenotype via easily applicable NCS criteria.

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