A5028251660- Amyotrophic Lateral Sclerosis Research
- Mitochondrial Function and Pathology
- Neurogenetic and Muscular Disorders Research
- Parkinson's Disease Mechanisms and Treatments
- Genetic Neurodegenerative Diseases
- Metabolism and Genetic Disorders
- Alzheimer's disease research and treatments
- Neuroscience and Neuropharmacology Research
- Hereditary Neurological Disorders
- ATP Synthase and ATPases Research
- Intracerebral and Subarachnoid Hemorrhage Research
- Neurological disorders and treatments
- Long-Term Effects of COVID-19
- Peripheral Neuropathies and Disorders
- Advanced Neuroimaging Techniques and Applications
- Neuroinflammation and Neurodegeneration Mechanisms
- Glycogen Storage Diseases and Myoclonus
- Cholinesterase and Neurodegenerative Diseases
- Dementia and Cognitive Impairment Research
- Prion Diseases and Protein Misfolding
- Epilepsy research and treatment
- Advanced MRI Techniques and Applications
- Botulinum Toxin and Related Neurological Disorders
- Muscle Physiology and Disorders
- Muscle activation and electromyography studies
University Hospital Magdeburg
2013-2025
Otto-von-Guericke University Magdeburg
2016-2025
Klinikum Magdeburg
1998-2025
German Center for Neurodegenerative Diseases
2015-2024
Center for Behavioral Brain Sciences
2020-2024
John Wiley & Sons (United States)
2023
Hudson Institute
2023
Liechtenstein Institute
2023
Cognitive Neuroimaging Lab
2023
University of Bonn
2001-2022
Mitochondrial respiratory chain complexes I and III have been shown to produce superoxide but the exact contribution localization of individual sites remained unclear. We approached this question investigating effects oxygen, substrates, inhibitors, NAD+/NADH redox couple on H2O2 production isolated mitochondria from rat human brain. Although brain in presence glutamate+malate alone do generate only small amounts (0.04 ± 0.02 nmol H2O2/min/mg), a substantial is observed after addition...
Evidence for symptomatic convergence of schizophrenia and N-methyl-D-aspartate glutamate receptor (NMDA-R) encephalitis highlights the need an assessment antibody prevalence specificity distinct disease mechanisms in patients with a diagnosis among glutamatergic pathophysiologic abnormalities psychiatric disorders.To compare NMDA-R antibodies (DSM-IV criteria) those other diagnoses to determine whether subtypes characterize overlap distinction from encephalitis.Serum 459 admitted acute...
Hereditary spastic paraplegias (HSPs) are genetically driven disorders with the hallmark of progressive gait disturbance. To investigate phenotypic spectrum, prognostic factors, and genotype-specific differences, we analyzed baseline data from a continuous, prospective cohort.We recruited 608 HSP cases 519 families mostly German origin. Clinical severity was assessed by Spastic Paraplegia Rating Scale. Complicating symptoms were recorded standardized inventory.Family history indicated...
Myasthenic crisis (MC) and disease exacerbation in myasthenia gravis (MG) are associated with significant lethality continue to impose a high burden on affected patients. Therefore, we sought determine potential predictors for MC as well identify factors affecting outcome.
Amyotrophic lateral sclerosis is a neurodegenerative disease affecting the anterior horn cells of spinal cord and cortical motor neurons. Previous findings have suggested specific impairment mitochondrial function in skeletal muscle at least limited number patients. Applying flavoprotein/NAD(P)H autofluorescence imaging saponin-permeabilized fibres, we detected heterogeneous distribution respiratory chain defect among individual fibres biopsies patients (11 out 17) with sporadic amyotrophic...
Mitochondria are cellular organelles crucial for energy supply and calcium homeostasis in neuronal cells, their dysfunction causes seizure activity some rare human epilepsies. To directly test whether mitochondrial respiratory chain enzymes abnormal the most common form of chronic epilepsy, temporal lobe epilepsy (TLE), living brain specimens from 57 epileptic patients 2 nonepileptic controls were investigated. In TLE with a hippocampal focus, we demonstrated specific deficiency complex I...
To evaluate basal ganglia changes along the amyotrophic lateral sclerosis (ALS)-ALS-frontotemporal dementia (FTD) continuum using multiple, complementary imaging techniques.Sixty-seven C9orf72-negative patients with ALS and 39 healthy controls were included in a cross-sectional quantitative MRI study. Seven met criteria for comorbid behavioral variant FTD (ALS-FTD), 18 Strong cognitive and/or impairment (ALS-Plus), 42 had no (ALS-Nci). Volumetric, shape, density analyses performed thalamus,...
Abstract Patients with COVID-19 can have a variety of neurological symptoms, but the active involvement central nervous system (CNS) in remains unclear. While routine cerebrospinal fluid (CSF) analyses patients manifestations generally show no or only mild inflammation, more detailed data on inflammatory mediators CSF are scarce. We studied response paired and serum samples ( n = 38). herpes simplex virus encephalitis (HSVE, 10) non-inflammatory, non-neurodegenerative diseases 28) served as...
Abstract Mitochondrial function is a key determinant of both excitability and viability neurons. Here, we demonstrate seizure‐dependent changes in mitochondrial oxidative phosphorylation the epileptic rat hippocampus. The intense pathological neuronal activity pilocarpine‐treated rats exhibiting spontaneous seizures resulted selective decline activities NADH–CoQ oxidoreductase (complex I respiratory chain) cytochrome c oxidase IV CA3 CA1 hippocampal pyramidal subfields. In line with these...
Summary: Purpose: For the antiepileptic drug (AED) topiramate (TPM), neuroprotective effects have been reported in models of focal cerebral ischemia and experimental status epilepticus, but putative mechanism action has remained elusive. Methods: We studied TPM on mitochondrial function pilocarpine rat model chronic epilepsy isolated mitochondria from brain. Results: treatment epilepticus at doses ranging 20 to 100 mg/kg considerably improved survival rats CA1 CA3 pyramidal cell a...
Introduction : In this study we compare the ultrasound features in median nerve patients with different types of Charcot–Marie–Tooth (CMT) disease and hereditary neuropathies liability to pressure palsies (HNPP) as a typical entrapment neuropathy. Methods Median conduction studies were performed CMT1A ( n = 12), MFN2‐associated CMT2A 7), CMTX 5), HNPP controls 28). Results cross‐sectional area (CSA) was significantly increased CMT1A, whereas, axonal CMT2A, fascicle diameter (FD) enlarged....
Purpose To investigate the whole-brain landscape of iron-related abnormalities in amyotrophic lateral sclerosis (ALS) by using vivo MRI technique quantitative susceptibility mapping (QSM). Materials and Methods For this prospective study, 28 patients with ALS (mean age, 61 years; age range, 43–77 18 men [mean years] 10 women 47–74 years]) recruited between January 17, 2014, September 4, 2015, 39 matched control subjects 39–77 24 62 15 59 39–73 were examined structural 3.0-T techniques. Group...
Mitochondrial oxidative phosphorylation (OXPHOS) and cellular workload are tightly balanced by the key regulator, calcium (Ca2+). Current models assume that cytosolic Ca2+ regulates mitochondrial uptake precedes activation of matrix dehydrogenases, thereby matching OXPHOS substrate supply to ATP demand. Surprisingly, knockout (KO) uniporter (MCU) in mice results only minimal phenotypic changes does not alter OXPHOS. This implies adaptive dehydrogenases intramitochondrial cannot be exclusive...
Objective Myasthenia gravis (MG) is the most common autoimmune disorder affecting neuromuscular junction. However, evidence shaping treatment decisions, particularly for treatment-refractory cases, sparse. Both rituximab and eculizumab may be considered as therapeutic options refractory MG after insufficient symptom control by standard immunosuppressive therapies. Methods In this retrospective observational study, we included 57 rituximab-treated 20 eculizumab-treated patients with to...