Albert Christian Ludolph

Albert Christian Ludolph's AcademicInfluence.com Rankings

Mathematics

#6669

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Probability Theory

#63

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Mathematics

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Albert Christian Ludolph's Published Works

Number of citations in a given year to any of this author's works

Total number of citations to an author for the works they published in a given year. This highlights publication of the most important work(s) by the author

Published Works

Amyotrophic lateral sclerosis. (2012) (4801)
Guam amyotrophic lateral sclerosis-parkinsonism-dementia linked to a plant excitant neurotoxin. (1987) (878)
Stages of pTDP‐43 pathology in amyotrophic lateral sclerosis (2013) (747)
Haploinsufficiency of TBK1 causes familial ALS and fronto-temporal dementia (2015) (637)
Experimental and clinical neurotoxicology (1980) (615)
3-Nitropropionic Acid - Exogenous Animal Neurotoxin and Possible Human Striatal Toxin (1991) (465)
Energy metabolism in amyotrophic lateral sclerosis (2011) (438)
Genome-wide association analyses identify new risk variants and the genetic architecture of amyotrophic lateral sclerosis (2016) (424)
Point mutations of the p150 subunit of dynactin (DCTN1) gene in ALS (2004) (418)
Genome-wide Analyses Identify KIF5A as a Novel ALS Gene (2018) (414)
Amyotrophic lateral sclerosis—a model of corticofugal axonal spread (2013) (395)
Genome-wide association study identifies 19p13.3 (UNC13A) and 9p21.2 as susceptibility loci for sporadic amyotrophic lateral sclerosis (2009) (358)
A revision of the El Escorial criteria - 2015 (2015) (356)
LATHYRISM: EVIDENCE FOR ROLE OF THE NEUROEXCITATORY AMINOACID BOAA (1986) (347)
A yeast functional screen predicts new candidate ALS disease genes (2011) (327)
Frontal lobe function in amyotrophic lateral sclerosis: a neuropsychologic and positron emission tomography study (1992) (306)
Guidelines for preclinical animal research in ALS/MND: A consensus meeting (2010) (305)
Riluzole treatment, survival and diagnostic criteria in Parkinson plus disorders: The NNIPPS Study (2008) (305)
Prognosis for patients with amyotrophic lateral sclerosis: development and validation of a personalised prediction model (2018) (276)
Topography of cerebral atrophy in early Huntington’s disease: a voxel based morphometric MRI study (2004) (273)
Cognitive impairment in patients with multiple system atrophy and progressive supranuclear palsy. (2010) (244)
Axonal damage markers in cerebrospinal fluid are increased in ALS (2006) (234)
Davunetide in patients with progressive supranuclear palsy: a randomised, double-blind, placebo-controlled phase 2/3 trial (2014) (231)
TDP-43 is intercellularly transmitted across axon terminals (2015) (227)
Patients with elevated triglyceride and cholesterol serum levels have a prolonged survival in amyotrophic lateral sclerosis (2011) (224)
Sequential distribution of pTDP-43 pathology in behavioral variant frontotemporal dementia (bvFTD) (2014) (222)
Neurofilaments in the diagnosis of motoneuron diseases: a prospective study on 455 patients (2015) (222)
Dexpramipexole versus placebo for patients with amyotrophic lateral sclerosis (EMPOWER): a randomised, double-blind, phase 3 trial (2013) (214)
Comparison of longitudinal metabolite relaxation times in different regions of the human brain at 1.5 and 3 Tesla (2003) (206)
Diagnosis and treatment of bulbar symptoms in amyotrophic lateral sclerosis (2008) (204)
NEK1 variants confer susceptibility to amyotrophic lateral sclerosis (2016) (195)
Therapeutic vaccine for acute and chronic motor neuron diseases: Implications for amyotrophic lateral sclerosis (2003) (193)
FTDP‐17: An early‐onset phenotype with parkinsonism and epileptic seizures caused by a novel mutation (1999) (190)
TDP-43 pathology and neuronal loss in amyotrophic lateral sclerosis spinal cord (2014) (187)
Global brain atrophy and corticospinal tract alterations in ALS, as investigated by voxel‐based morphometry of 3‐D MRI (2005) (185)
Heterozygous R1101K mutation of the DCTN1 gene in a family with ALS and FTD (2005) (184)
Angiogenin variants in Parkinson disease and amyotrophic lateral sclerosis (2011) (184)
TDP-43 in cerebrospinal fluid of patients with frontotemporal lobar degeneration and amyotrophic lateral sclerosis. (2008) (181)
Cognitive function in bulbar– and spinal–onset amyotrophic lateral sclerosis (2005) (180)
High dose vitamin E therapy in amyotrophic lateral sclerosis as add-on therapy to riluzole: results of a placebo-controlled double-blind study (2005) (180)
Impaired DNA damage response signaling by FUS-NLS mutations leads to neurodegeneration and FUS aggregate formation (2018) (176)
Diffusion tensor imaging analysis of sequential spreading of disease in amyotrophic lateral sclerosis confirms patterns of TDP-43 pathology. (2014) (175)
Neurofilament levels as biomarkers in asymptomatic and symptomatic familial amyotrophic lateral sclerosis (2015) (174)
Inflammatory dysregulation of blood monocytes in Parkinson’s disease patients (2014) (174)
Increased Hypoxic Tolerance by Chemical Inhibition of Oxidative Phosphorylation: “Chemical Preconditioning” (1997) (172)
Toxic gain of function from mutant FUS protein is crucial to trigger cell autonomous motor neuron loss (2016) (171)
Riluzole in Huntington's disease: a 3‐year, randomized controlled study (2007) (169)
Tauopathies with parkinsonism: clinical spectrum, neuropathologic basis, biological markers, and treatment options (2009) (165)
Severity of Depressive Symptoms and Quality of Life in Patients with Amyotrophic Lateral Sclerosis (2005) (163)
Two German kindreds with familial amyotrophic lateral sclerosis due to TARDBP mutations. (2008) (158)
Dopamine transporter: involvement in selective dopaminergic neurotoxicity and degeneration (2004) (150)
X-linked bulbospinal neuronopathy: Kennedy disease. (2002) (149)
Mechanisms, models and biomarkers in amyotrophic lateral sclerosis (2013) (148)
A large-scale multicentre cerebral diffusion tensor imaging study in amyotrophic lateral sclerosis (2016) (148)
The RNA of the glutamate transporter EAAT2 is variably spliced in amyotrophic lateral sclerosis and normal individuals (1999) (143)
Apixaban for treatment of embolic stroke of undetermined source (ATTICUS randomized trial): Rationale and study design (2017) (139)
Nusinersen in adults with 5q spinal muscular atrophy: a non-interventional, multicentre, observational cohort study (2020) (139)
Neurofilament light chain in serum for the diagnosis of amyotrophic lateral sclerosis (2018) (139)
In vivo imaging of activated microglia using [11 C]PK11195 and positron emission tomography in patients after ischemic stroke (2000) (138)
Hot-spot KIF5A mutations cause familial ALS (2018) (136)
Phenotypic expression of the DYT1 mutation: A family with writer's cramp of juvenile onset (1998) (131)
Glial Fibrillary Acidic Protein in Serum is Increased in Alzheimer's Disease and Correlates with Cognitive Impairment. (2019) (129)
Cortical influences drive amyotrophic lateral sclerosis (2017) (127)
Age-dependent defects of alpha-synuclein oligomer uptake in microglia and monocytes (2016) (126)
Extracellular vesicle sorting of α-Synuclein is regulated by sumoylation (2015) (126)
Intrathecal application of neuroectodermally converted stem cells into a mouse model of ALS: limited intraparenchymal migration and survival narrows therapeutic effects (2007) (125)
Genetic Variants of the α-Synuclein Gene SNCA Are Associated with Multiple System Atrophy (2009) (122)
Systemic dysregulation of TDP-43 binding microRNAs in amyotrophic lateral sclerosis (2013) (122)
A Randomized, Double Blind, Placebo-Controlled Trial of Pioglitazone in Combination with Riluzole in Amyotrophic Lateral Sclerosis (2012) (122)
The Chemokine CXCL13 Is a Prognostic Marker in Clinically Isolated Syndrome (CIS) (2010) (121)
Alcohol withdrawal syndrome: mechanisms, manifestations, and management (2016) (120)
Multicenter evaluation of neurofilaments in early symptom onset amyotrophic lateral sclerosis (2018) (119)
Rasagiline alone and in combination with riluzole prolongs survival in an ALS mouse model (2004) (118)
Cardiac involvement in patients with Becker muscular dystrophy: new diagnostic and pathophysiological insights by a CMR approach (2008) (115)
Paroxysmal choreoathetosis/spasticity (DYT9) is caused by a GLUT1 defect (2011) (114)
Studies on the aetiology and pathogenesis of motor neuron diseases. 1. Lathyrism: clinical findings in established cases. (1987) (114)
Visualization of defective mitochondrial function in skeletal muscle fibers of patients with sporadic amyotrophic lateral sclerosis (1999) (113)
Occurrence and characterization of peripheral nerve involvement in neurofibromatosis type 2. (2002) (112)
To rise and to fall: functional connectivity in cognitively normal and cognitively impaired patients with Parkinson's disease (2015) (112)
Limited role of free TDP-43 as a diagnostic tool in neurodegenerative diseases (2014) (111)
The Edinburgh Cognitive and Behavioural Amyotrophic Lateral Sclerosis Screen: A cross-sectional comparison of established screening tools in a German-Swiss population (2015) (111)
Guidelines for the preclinical in vivo evaluation of pharmacological active drugs for ALS/MND: Report on the 142nd ENMC international workshop (2007) (108)
IgG Antibodies against Measles, Rubella, and Varicella Zoster Virus Predict Conversion to Multiple Sclerosis in Clinically Isolated Syndrome (2009) (107)
Genetic correlation between amyotrophic lateral sclerosis and schizophrenia (2017) (107)
Hypothalamic atrophy is related to body mass index and age at onset in amyotrophic lateral sclerosis (2017) (106)
Whole brain‐based analysis of regional white matter tract alterations in rare motor neuron diseases by diffusion tensor imaging (2010) (104)
Common and rare variant association analyses in amyotrophic lateral sclerosis identify 15 risk loci with distinct genetic architectures and neuron-specific biology (2021) (104)
NEK1 mutations in familial amyotrophic lateral sclerosis. (2016) (101)
NF‐κB activation in astrocytes drives a stage‐specific beneficial neuroimmunological response in ALS (2018) (98)
MOTORNEURONE DISEASE ON GUAM: POSSIBLE ROLE OF A FOOD NEUROTOXIN (1986) (98)
Peripheral monocytes are functionally altered and invade the CNS in ALS patients (2016) (97)
The El Escorial criteria: Strengths and weaknesses (2015) (97)
Effects of mitochondrial dysfunction on the immunological properties of microglia (2010) (97)
Amyotrophic lateral sclerosis: disease stage related changes of tau protein and S100 beta in cerebrospinal fluid and creatine kinase in serum (2003) (97)
Inhibition of energy metabolism by 3-nitropropionic acid activates ATP-sensitive potassium channels (1992) (96)
Current knowledge and recent insights into the genetic basis of amyotrophic lateral sclerosis (2018) (95)
Targeted Antioxidative and Neuroprotective Properties of the Dopamine Agonist Pramipexole and Its Nondopaminergic Enantiomer SND919CL2x [(+)2-Amino-4,5,6,7-tetrahydro-6-lpropylamino-benzathiazole Dihydrochloride] (2006) (95)
Preservation of diffusion tensor properties during spatial normalization by use of tensor imaging and fibre tracking on a normal brain database (2007) (95)
Motor neuron intrinsic and extrinsic mechanisms contribute to the pathogenesis of FUS-associated amyotrophic lateral sclerosis (2017) (94)
Human T-lymphotropic virus type I antibodies in the serum of patients with tropical spastic paraparesis in the Seychelles. (1987) (94)
Late-onset motoneuron disease caused by a functionally modified AMPA receptor subunit. (2005) (93)
Depression and quality of life in patients with amyotrophic lateral sclerosis. (2008) (92)
Protective Effects of Riluzole on Dopamine Neurons (2000) (92)
The chemokine CXCL13 in acute neuroborreliosis (2009) (88)
Antisense oligonucleotides in neurological disorders (2018) (87)
Different neuroinflammatory profile in amyotrophic lateral sclerosis and frontotemporal dementia is linked to the clinical phase (2018) (87)
Accelerated aging phenotype in mice with conditional deficiency for mitochondrial superoxide dismutase in the connective tissue (2011) (86)
4‐Aminopyridine Induced Activity Rescues Hypoexcitable Motor Neurons from Amyotrophic Lateral Sclerosis Patient‐Derived Induced Pluripotent Stem Cells (2016) (86)
The role of excitotoxicity in ALS – what is the evidence? (2000) (85)
Stages of granulovacuolar degeneration: their relation to Alzheimer’s disease and chronic stress response (2011) (85)
Glatiramer acetate has no impact on disease progression in ALS at 40 mg/day: A double- blind, randomized, multicentre, placebo-controlled trial (2009) (85)
Emotional responding in amyotrophic lateral sclerosis (2005) (85)
Two novel mutations in conserved codons indicate that CHCHD10 is a gene associated with motor neuron disease. (2014) (85)
White matter abnormalities in patients with treated hyperphenylalaninaemia: Magnetic resonance relaxometry and proton spectroscopy findings (1993) (83)
Serum microRNAs in patients with genetic amyotrophic lateral sclerosis and pre-manifest mutation carriers. (2014) (83)
Meta-analysis of vascular endothelial growth factor variations in amyotrophic lateral sclerosis: increased susceptibility in male carriers of the −2578AA genotype (2008) (82)
Roadmap and standard operating procedures for biobanking and discovery of neurochemical markers in ALS (2012) (82)
Longitudinal Effects of Noninvasive Positive-Pressure Ventilation in Patients with Amyotrophic Lateral Sclerosis (2003) (82)
Low stability of huntington muscle Mitochondria against Ca2+ in R6/2 mice (2006) (81)
Effect of High‐Caloric Nutrition on Survival in Amyotrophic Lateral Sclerosis (2019) (81)
Novel missense and truncating mutations in FUS/TLS in familial ALS (2010) (81)
Disease Severity and Progression in Progressive Supranuclear Palsy and Multiple System Atrophy: Validation of the NNIPPS – PARKINSON PLUS SCALE (2011) (80)
Multicenter validation of CSF neurofilaments as diagnostic biomarkers for ALS (2016) (80)
Decreased N-acetyl-aspartate/choline ratio and increased lactate in the frontal lobe of patients with Huntington's disease: a proton magnetic resonance spectroscopy study. (1997) (80)
Intrathecal administration of nusinersen in adolescent and adult SMA type 2 and 3 patients (2018) (80)
Four familial ALS pedigrees discordant for two SOD1 mutations: are all SOD1 mutations pathogenic? (2010) (79)
Chitotriosidase (CHIT1) is increased in microglia and macrophages in spinal cord of amyotrophic lateral sclerosis and cerebrospinal fluid levels correlate with disease severity and progression (2017) (78)
Cerebrospinal fluid biomarkers of neurodegeneration in chronic neurological diseases (2008) (77)
Alpha-, Beta-, and Gamma-synuclein Quantification in Cerebrospinal Fluid by Multiple Reaction Monitoring Reveals Increased Concentrations in Alzheimer′s and Creutzfeldt-Jakob Disease but No Alteration in Synucleinopathies* (2016) (77)
Amyotrophic lateral sclerosis and denervation alter sphingolipids and up-regulate glucosylceramide synthase (2015) (77)
Slow toxins, biologic markers, and long‐latency neurodegenerative disease in the western Pacific region (1991) (77)
A dynein mutation attenuates motor neuron degeneration in SOD1G93A mice (2006) (77)
High-caloric food supplements in the treatment of amyotrophic lateral sclerosis: A prospective interventional study (2013) (77)
Mice with a mutation in the dynein heavy chain 1 gene display sensory neuropathy but lack motor neuron disease (2009) (77)
Brain responses to emotional stimuli in patients with amyotrophic lateral sclerosis (ALS) (2007) (76)
Poly‐GP in cerebrospinal fluid links C9orf72‐associated dipeptide repeat expression to the asymptomatic phase of ALS/FTD (2017) (76)
CSF filtration is an effective treatment of Guillain–Barré syndrome: A randomized clinical trial (2001) (76)
Adipose Tissue Distribution Predicts Survival in Amyotrophic Lateral Sclerosis (2013) (75)
Discovery and partial characterization of primate motor-system toxins. (1987) (75)
Alterations in the hypothalamic melanocortin pathway in amyotrophic lateral sclerosis. (2016) (74)
Grey-matter abnormalities in boys with Tourette syndrome: magnetic resonance imaging study using optimised voxel-based morphometry (2006) (73)
Association of Surgical Hematoma Evacuation vs Conservative Treatment With Functional Outcome in Patients With Cerebellar Intracerebral Hemorrhage. (2019) (73)
Percutaneous endoscopic gastrostomy in amyotrophic lateral sclerosis: a prospective observational study (2015) (72)
Increased Immune Activation by Pathologic α‐Synuclein in Parkinson's Disease (2019) (72)
PGC-1α is a male-specific disease modifier of human and experimental amyotrophic lateral sclerosis. (2013) (72)
Mutual exacerbation of peroxisome proliferator‐activated receptor γ coactivator 1α deregulation and α‐synuclein oligomerization (2014) (72)
The modulation of Amyotrophic Lateral Sclerosis risk by Ataxin-2 intermediate polyglutamine expansions is a specific effect (2012) (72)
Cortical Plasticity in Amyotrophic Lateral Sclerosis: Motor Imagery and Function (2007) (71)
Neurodegenerative Diseases: Neurobiology, Pathogenesis and Therapeutics (2005) (70)
Predicting behavioral variant frontotemporal dementia with pattern classification in multi-center structural MRI data (2017) (70)
Neurofilament heavy‐chain NfHSMI35 in cerebrospinal fluid supports the differential diagnosis of Parkinsonian syndromes (2006) (70)
Linking neuron and skin: Matrix metalloproteinases in amyotrophic lateral sclerosis (ALS) (2009) (69)
The use of P300-based BCIs in amyotrophic lateral sclerosis: from augmentative and alternative communication to cognitive assessment (2012) (68)
Association of Mutations in TBK1 With Sporadic and Familial Amyotrophic Lateral Sclerosis and Frontotemporal Dementia (2017) (67)
Formation and characterisation of neuromuscular junctions between hiPSC derived motoneurons and myotubes. (2015) (67)
CSF glial markers correlate with survival in amyotrophic lateral sclerosis (2010) (67)
Histopathological analysis of skeletal muscle in patients with Parkinson's disease and 'dropped head'/'bent spine' syndrome. (2009) (67)
Mitochondrial Oxidation in Rat Hippocampus Can Be Preconditioned by Selective Chemical Inhibition of Succinic Dehydrogenase (1996) (67)
Synapse loss in the prefrontal cortex is associated with cognitive decline in amyotrophic lateral sclerosis (2017) (67)
Blood GFAP as an emerging biomarker in brain and spinal cord disorders (2022) (67)
Imaging the pathoanatomy of amyotrophic lateral sclerosis in vivo: targeting a propagation-based biological marker (2017) (67)
Neurofilament as a blood marker for diagnosis and monitoring of primary progressive aphasias (2017) (67)
Multicentre quality control evaluation of different biomarker candidates for amyotrophic lateral sclerosis (2014) (66)
Chemical preconditioning: A cytoprotective strategy (1997) (66)
Neuroimaging of multimodal sensory stimulation in amyotrophic lateral sclerosis (2010) (66)
NF-κB Is Required for Smac Mimetic-Mediated Sensitization of Glioblastoma Cells for γ-Irradiation–Induced Apoptosis (2011) (65)
Life course body mass index and risk and prognosis of amyotrophic lateral sclerosis: results from the ALS registry Swabia (2017) (65)
Epidemiology of amyotrophic lateral sclerosis in Southern Germany (2017) (65)
Identification of novel Angiogenin (ANG) gene missense variants in German patients with amyotrophic lateral sclerosis (2009) (64)
Functional Connectivity Mapping in the Animal Model: Principles and Applications of Resting-State fMRI (2017) (64)
Quality of life in fatal disease: the flawed judgement of the social environment (2013) (64)
Functional connectivity changes resemble patterns of pTDP-43 pathology in amyotrophic lateral sclerosis (2016) (64)
TDP‐43 loss of function inhibits endosomal trafficking and alters trophic signaling in neurons (2016) (64)
FUS-mediated regulation of acetylcholine receptor transcription at neuromuscular junctions is compromised in amyotrophic lateral sclerosis (2019) (63)
Severe sensorimotor neuropathy after intake of highest dosages of vitamin B6 (2008) (63)
Comprehensive analysis of the mutation spectrum in 301 German ALS families (2018) (63)
Retinal single-layer analysis in Parkinsonian syndromes: an optical coherence tomography study (2013) (63)
Summary of cerebrospinal fluid routine parameters in neurodegenerative diseases (2010) (63)
MMP-2 and MMP-9 are elevated in spinal cord and skin in a mouse model of ALS (2010) (63)
Disturbed myelination in patients with treated hyperphenylalaninaemia: evaluation with magnetic resonance imaging (2005) (63)
Phosphoinositide 3-kinases upregulate system xc(-) via eukaryotic initiation factor 2α and activating transcription factor 4 - A pathway active in glioblastomas and epilepsy. (2014) (62)
De novo FUS mutations are the most frequent genetic cause in early-onset German ALS patients (2015) (62)
Alternative splicing of the glutamate transporter EAAT2 (GLT-1) (1998) (62)
Body Fat Distribution as a Risk Factor for Cerebrovascular Disease: An MRI-Based Body Fat Quantification Study (2013) (61)
Body weight is a robust predictor of clinical progression in Huntington disease (2017) (61)
Emotional adjustment in amyotrophic lateral sclerosis (ALS) (2012) (61)
Amyotrophic lateral sclerosis: a consensus viewpoint on designing and implementing a clinical trial (2004) (61)
Truncating mutations in FUS/TLS give rise to a more aggressive ALS‐phenotype than missense mutations: a clinico‐genetic study in Germany (2013) (61)
Disease-modifying and symptomatic treatment of amyotrophic lateral sclerosis (2017) (60)
Quantification of human body fat tissue percentage by MRI (2011) (60)
Serum neurofilament light chain in behavioral variant frontotemporal dementia (2018) (60)
Polymerase chain reaction and Southern blot-based analysis of the C9orf72 hexanucleotide repeat in different motor neuron diseases (2014) (59)
Antioxidant drugs block in vitro the neurotoxicity of CSF from patients with amyotrophic lateral sclerosis. (1996) (59)
Metabolic progression markers of neurodegeneration in the transgenic G93A‐SOD1 mouse model of amyotrophic lateral sclerosis (2007) (59)
CSF proteome analysis in clinically isolated syndrome (CIS): Candidate markers for conversion to definite multiple sclerosis (2009) (58)
Quantification of brain atrophy in patients with myotonic dystrophy and proximal myotonic myopathy: a controlled 3-dimensional magnetic resonance imaging study (2003) (58)
Dietary antioxidants and dementia in a population-based case-control study among older people in South Germany. (2012) (58)
Pathological TDP-43 changes in Betz cells differ from those in bulbar and spinal α-motoneurons in sporadic amyotrophic lateral sclerosis (2016) (58)
Differential diagnostic value of eye movement recording in PSP-parkinsonism, Richardson's syndrome, and idiopathic Parkinson's disease (2008) (57)
Management of therapeutic anticoagulation in patients with intracerebral haemorrhage and mechanical heart valves (2018) (57)
Diffusion tensor imaging and tractwise fractional anisotropy statistics: quantitative analysis in white matter pathology (2007) (57)
Skin involvement in amyotrophic lateral sclerosis (1996) (56)
Functional Connectivity Within the Default Mode Network Is Associated With Saccadic Accuracy in Parkinson's Disease: A Resting-State fMRI and Videooculographic Study (2013) (56)
In vivo quantification of spinal and bulbar motor neuron degeneration in the G93A-SOD1 transgenic mouse model of ALS by T 2 relaxation time and apparent diffusion coefficient (2006) (56)
The relations between long-latency reflexes in hand muscles, somatosensory evoked potentials and transcranial stimulation of motor tracts. (1989) (56)
C9orf72 and UNC13A are shared risk loci for amyotrophic lateral sclerosis and frontotemporal dementia: A genome‐wide meta‐analysis (2014) (56)
A point mutation in the dynein heavy chain gene leads to striatal atrophy and compromises neurite outgrowth of striatal neurons. (2010) (56)
Neurofilament light chain as a blood biomarker to differentiate psychiatric disorders from behavioural variant frontotemporal dementia. (2019) (56)
Cerebral white matter alterations in idiopathic restless legs syndrome, as measured by diffusion tensor imaging (2008) (55)
Neuroanatomical patterns of cerebral white matter involvement in different motor neuron diseases as studied by diffusion tensor imaging analysis (2012) (55)
Amyotrophic lateral sclerosis: dash-like accumulation of phosphorylated TDP-43 in somatodendritic and axonal compartments of somatomotor neurons of the lower brainstem and spinal cord (2010) (55)
Diagnostic and prognostic significance of neurofilament light chain NF-L, but not progranulin and S100B, in the course of amyotrophic lateral sclerosis: Data from the German MND-net (2017) (55)
FUS Mislocalization and Vulnerability to DNA Damage in ALS Patients Derived hiPSCs and Aging Motoneurons (2016) (55)
Novel mutation in the ALS2 gene in juvenile amyotrophic lateral sclerosis (2005) (54)
Incidence and Geographical Variation of Amyotrophic Lateral Sclerosis (ALS) in Southern Germany – Completeness of the ALS Registry Swabia (2014) (54)
A large genome scan for rare CNVs in amyotrophic lateral sclerosis. (2010) (53)
Nusinersen for spinal muscular atrophy (2018) (53)
Stepwise acquirement of hallmark neuropathology in FUS-ALS iPSC models depends on mutation type and neuronal aging (2015) (53)
Biochemical markers in CSF of ALS patients. (2008) (52)
Joint genome-wide association study of progressive supranuclear palsy identifies novel susceptibility loci and genetic correlation to neurodegenerative diseases (2018) (52)
Proteome Analysis of Cerebrospinal Fluid in Amyotrophic Lateral Sclerosis (ALS) (2008) (52)
NIPA1 polyalanine repeat expansions are associated with amyotrophic lateral sclerosis. (2012) (52)
Alternative splicing of the 5′‐sequences of the mouse EAAT2 glutamate transporter and expression in a transgenic model for amyotrophic lateral sclerosis (2002) (50)
Proteome analysis reveals candidate markers of disease progression in amyotrophic lateral sclerosis (ALS) (2010) (50)
Imaging of activated microglia with PET and [11C]PK 11195 in corticobasal degeneration (2004) (49)
Altered perivascular fibroblast activity precedes ALS disease onset (2021) (48)
Laryngospasm: An underdiagnosed symptom of X-linked spinobulbar muscular atrophy (2005) (48)
Failure of neuronal ion exchange, not potentiated excitation, causes excitotoxicity after inhibition of oxidative phosphorylation (1995) (48)
Palliative care and circumstances of dying in German ALS patients using non‐invasive ventilation (2008) (48)
Chronic abuse of zolpidem. (1994) (48)
ROCK-ALS: Protocol for a Randomized, Placebo-Controlled, Double-Blind Phase IIa Trial of Safety, Tolerability and Efficacy of the Rho Kinase (ROCK) Inhibitor Fasudil in Amyotrophic Lateral Sclerosis (2019) (48)
Drug therapy for pain in amyotrophic lateral sclerosis or motor neuron disease. (2008) (47)
NEK1 loss-of-function mutation induces DNA damage accumulation in ALS patient-derived motoneurons. (2018) (47)
Age-dependent changes in MRI of motor brain stem nuclei in a mouse model of ALS (2004) (47)
Dynein mutations associated with hereditary motor neuropathies impair mitochondrial morphology and function with age (2013) (47)
Live and let die: existential decision processes in a fatal disease (2014) (47)
MRI-based functional neuroimaging in ALS: An update (2009) (46)
A post hoc analysis of subgroup outcomes and creatinine in the phase III clinical trial (EMPOWER) of dexpramipexole in ALS (2014) (46)
Are amygdalar volume alterations in children with Tourette syndrome due to ADHD comorbidity? (2008) (46)
Levodopa-induced striatal activation in Parkinson's disease: a functional MRI study. (2009) (46)
Tropical spastic paraparesis in the Seychelles Islands (1987) (46)
NMDA-antagonists reverse increased hypoxic tolerance by preceding chemical hypoxia (1996) (46)
Super-Resolution Microscopy Reveals Presynaptic Localization of the ALS/FTD Related Protein FUS in Hippocampal Neurons (2016) (46)
Eye movement impairments in Parkinson's disease: possible role of extradopaminergic mechanisms (2012) (46)
Neurologic diseases associated with use of plant components with toxic potential. (1993) (46)
Early diagnosis of cardiac involvement in idiopathic inflammatory myopathy by cardiac magnetic resonance tomography (2015) (46)
Optical coherence tomography does not support optic nerve involvement in amyotrophic lateral sclerosis (2013) (46)
Safety and efficacy of ozanezumab in patients with amyotrophic lateral sclerosis: a randomised, double-blind, placebo-controlled, phase 2 trial (2017) (45)
Heterozygous Tbk1 loss has opposing effects in early and late stages of ALS in mice (2019) (45)
On the decline and etiology of high‐incidence motor system disease in West Papua (southwest New Guinea) (2005) (44)
Signs of impaired selective attention in patients with amyotrophic lateral sclerosis (2008) (44)
Safety and efficacy of rasagiline as an add-on therapy to riluzole in patients with amyotrophic lateral sclerosis: a randomised, double-blind, parallel-group, placebo-controlled, phase 2 trial (2018) (44)
Generalized epilepsy with febrile seizures plus (2001) (44)
Pentoxifylline in ALS: a double-blind, randomized, multicenter, placebo-controlled trial. (2006) (43)
Imaging and neurochemical markers for diagnosis and disease progression in ALS (2001) (43)
The EAAT2 (GLT-1) gene in motor neuron disease: absence of mutations in amyotrophic lateral sclerosis and a point mutation in patients with hereditary spastic paraplegia (1998) (43)
Comparison of smooth pursuit eye movement deficits in multiple system atrophy and Parkinson’s disease (2009) (43)
Proteomics in cerebrospinal fluid and spinal cord suggests UCHL1, MAP2 and GPNMB as biomarkers and underpins importance of transcriptional pathways in amyotrophic lateral sclerosis (2019) (43)
Rare Variants in MME, Encoding Metalloprotease Neprilysin, Are Linked to Late-Onset Autosomal-Dominant Axonal Polyneuropathies. (2016) (43)
Soluble Beta-Amyloid Precursor Protein Is Related to Disease Progression in Amyotrophic Lateral Sclerosis (2011) (43)
Trial of Antisense Oligonucleotide Tofersen for SOD1 ALS. (2022) (42)
Superoxide dismutase 1 modulates expression of transferrin receptor (2006) (42)
Age and education-matched cut-off scores for the revised German/Swiss-German version of ECAS (2016) (41)
Eye Movement Deficits Are Consistent with a Staging Model of pTDP-43 Pathology in Amyotrophic Lateral Sclerosis (2015) (41)
Developmental and Functional Nature of Human iPSC Derived Motoneurons (2013) (40)
Differential pattern of brain‐specific CSF proteins tau and amyloid‐beta in Parkinsonian syndromes (2010) (40)
Brain metabolites in definite amyotrophic lateral sclerosis. A longitudinal proton magnetic resonance spectroscopy study. (2007) (40)
Intersubject variability in the analysis of diffusion tensor images at the group level: fractional anisotropy mapping and fiber tracking techniques. (2009) (40)
Safety and efficacy of tilavonemab in progressive supranuclear palsy: a phase 2, randomised, placebo-controlled trial (2021) (40)
Review: Evidence-based drug treatment in amyotrophic lateral sclerosis and upcoming clinical trials (2009) (40)
Directional colour encoding of the human thalamus by diffusion tensor imaging (2008) (40)
Cognitive phenotypes of sequential staging in amyotrophic lateral sclerosis (2018) (40)
The association between alterations of eye movement control and cerebral intrinsic functional connectivity in Parkinson’s disease (2016) (39)
Status epilepticus: Clinical characteristics and EEG patterns associated with and without MRI diffusion restriction in 69 patients (2016) (39)
Adenosine receptor up‐regulation: initiated upon preconditioning but not upheld (2000) (39)
Amygdala size in amyotrophic lateral sclerosis without dementia: an in vivo study using MRI volumetry (2006) (39)
Genomic organization of the human excitatory amino acid transporter gene GLT‐1 (1997) (39)
Adipokines, C-reactive protein and Amyotrophic Lateral Sclerosis – results from a population- based ALS registry in Germany (2017) (38)
TDP‐43 in amyotrophic lateral sclerosis – is it a prion disease? (2015) (38)
GFAP in early multiple sclerosis: A biomarker for inflammation (2017) (38)
Reversible induction of TDP-43 granules in cortical neurons after traumatic injury (2018) (38)
Serotonin 2B receptor slows disease progression and prevents degeneration of spinal cord mononuclear phagocytes in amyotrophic lateral sclerosis (2016) (38)
Transient Global Amnesia. Evidence against vascular ischemic etiology from diffusion weighted imaging. (2002) (37)
Therapeutic decisions in ALS patients: cross-cultural differences and clinical implications (2018) (37)
The glial glutamate transporter complementary DNA in patients with amyotrophic lateral sclerosis (1996) (37)
Differential diagnosis of peripheral facial nerve palsy: a retrospective clinical, MRI and CSF-based study (2019) (37)
Phenotypes and malignancy risk of different FUS mutations in genetic amyotrophic lateral sclerosis (2019) (37)
Neurofilament light chain in serum of adolescent and adult SMA patients under treatment with nusinersen (2019) (37)
Retinal involvement in amyotrophic lateral sclerosis: a study with optical coherence tomography and diffusion tensor imaging (2016) (37)
Predicting primary progressive aphasias with support vector machine approaches in structural MRI data (2017) (36)
July 2017 ENCALS statement on edaravone (2017) (36)
Functional deficits of central sensory and motor pathways in patients with cervical spinal stenosis: a study of SEPs and EMG responses to non-invasive brain stimulation. (1989) (36)
Whole brain-based computerized neuroimaging in ALS and other motor neuron disorders (2008) (36)
The dynactin p150 subunit: cell biology studies of sequence changes found in ALS/MND and Parkinsonian Syndromes (2013) (36)
The advanced glycation end-product N ɛ-(carboxymethyl)lysine level is elevated in cerebrospinal fluid of patients with amyotrophic lateral sclerosis (2004) (36)
Toxic models of upper motor neuron disease (1996) (36)
Prognostic factors in ALS: a comparison between Germany and China (2019) (36)
No evidence of association of FLJ10986 and ITPR2 with ALS in a large German cohort (2011) (35)
Metabolite profiling in identifying metabolic biomarkers in older people with late-onset type 2 diabetes mellitus (2017) (35)
Increase of cellular hypoxic tolerance by erythromycin and other antibiotics. (1999) (35)
ALS-causing mutations differentially affect PGC-1α expression and function in the brain vs. peripheral tissues (2017) (35)
NADH in the pyramidal cell layer of hippocampal regions CA1 and CA3 upon selective inhibition and uncoupling of oxidative phosphorylation (1996) (35)
Neurofilaments and tau in CSF in an infant with SMA type 1 treated with nusinersen (2019) (35)
Atrophy in the Thalamus But Not Cerebellum Is Specific for C9orf72 FTD and ALS Patients – An Atlas-Based Volumetric MRI Study (2018) (35)
Tropical spastic paraparesis: HTLV-I antibodies in patients from the Seychelles. (1987) (35)
Safety and Effectiveness of Long-term Intravenous Administration of Edaravone for Treatment of Patients With Amyotrophic Lateral Sclerosis. (2022) (35)
Neuroimaging of motor neuron diseases (2012) (35)
Studies on the etiology and pathogenesis of motor neuron diseases. II. (1988) (34)
Fast progressive lower motor neuron disease is an ALS variant: A two-centre tract of interest-based MRI data analysis (2017) (34)
Provision of assistive technology devices among people with ALS in Germany: a platform-case management approach (2018) (34)
Age-related brain parenchymal fraction is significantly decreased in young multiple sclerosis patients: a quantitative MRI study (2003) (34)
Impaired activation of ALS monocytes by exosomes (2017) (34)
Intact protein analysis of ubiquitin in cerebrospinal fluid by multiple reaction monitoring reveals differences in Alzheimer's disease and frontotemporal lobar degeneration. (2014) (33)
Targeted Mass Spectrometry Suggests Beta-Synuclein as Synaptic Blood Marker in Alzheimer's Disease. (2020) (33)
Neurochemical markers in CSF of adolescent and adult SMA patients undergoing nusinersen treatment (2019) (33)
Possible gender-dependent association of vascular endothelial growth factor (VEGF) gene and ALS (2006) (33)
Neurological outcome in 22 treated adolescents with hyperphenylalaninemia (1992) (32)
Rate of change in early Huntington's disease: A clinicometric analysis (2012) (32)
Mutual exacerbation of PGC-1α deregulation and α-synuclein oligomerization (2015) (32)
Frontotemporal cognitive function in X-linked spinal and bulbar muscular atrophy (SBMA): a controlled neuropsychological study of 20 patients (2009) (32)
Patterns of Eye Movement Impairment Correlate with Regional Brain Atrophy in Neurodegenerative Parkinsonism (2017) (32)
Comparison of Sirtuin 3 Levels in ALS and Huntington’s Disease—Differential Effects in Human Tissue Samples vs. Transgenic Mouse Models (2017) (31)
The relation between functional deficits, motor and sensory conduction times and MRI findings in syringomyelia. (1992) (31)
Fumaric Acid Esters Stimulate Astrocytic VEGF Expression through HIF-1α and Nrf2 (2013) (31)
Are Human Neurodegenerative Disorders Linked to Environmental Chemicals with Excitotoxic Properties? a (1992) (31)
Lathyrism and western Pacific amyotrophic lateral sclerosis: etiology of short and long latency motor system disorders. (1991) (31)
CHCHD10 mutations p.R15L and p.G66V cause motoneuron disease by haploinsufficiency (2018) (31)
Guam ALS-PDC: possible causes. (1993) (31)
MR-Pathologic Comparison of the Upper Spinal Cord in Different Motor Neuron Diseases (2005) (31)
Investigation of common, low-frequency and rare genome-wide variation in anorexia nervosa (2017) (30)
Splicing of the glutamate transporter EAAT2: a candidate gene of amyotrophic lateral sclerosis (1998) (30)
CSF Free Light Chains as a Marker of Intrathecal Immunoglobulin Synthesis in Multiple Sclerosis: A Blood-CSF Barrier Related Evaluation in a Large Cohort (2019) (30)
Cerebrospinal fluid erythropoietin (EPO) in amyotrophic lateral sclerosis (2007) (30)
Deficits in verbal fluency in presymptomatic C9orf72 mutation gene carriers—a developmental disorder (2020) (30)
An observational study on quality of life and preferences to sustain life in locked-in state (2019) (29)
Vacuolization correlates with spin–spin relaxation time in motor brainstem nuclei and behavioural tests in the transgenic G93A‐SOD1 mouse model of ALS (2007) (29)
The epidemiology of CuZn-SOD mutations in Germany: a study of 217 families (2010) (29)
Transient Global Amnesia (2002) (29)
Differential regulation of 5′ splice variants of the glutamate transporter EAAT2 in an in vivo model of chemical hypoxia induced by 3‐nitropropionic acid (2003) (29)
Proton MRS in Kennedy disease: Absolute metabolite and macromolecular concentrations (2002) (29)
Clinical features and differential diagnosis of flail arm syndrome (2016) (29)
HEK-293 cells expressing the human dopamine transporter are susceptible to low concentrations of 1-methyl-4-phenylpyridine (MPP+) via impairment of energy metabolism (1999) (29)
A new MRI rating scale for progressive supranuclear palsy and multiple system atrophy: validity and reliability (2011) (28)
Autism-associated SHANK3 mutations impair maturation of neuromuscular junctions and striated muscles (2020) (28)
Retinoic acid worsens ATG10-dependent autophagy impairment in TBK1-mutant hiPSC-derived motoneurons through SQSTM1/p62 accumulation (2019) (28)
Problems and pitfalls in the diagnosis of ALS (1999) (28)
Corticoefferent pathways in pure lower motor neuron disease: a diffusion tensor imaging study (2016) (28)
The concept and diagnostic criteria of primary lateral sclerosis (2017) (28)
Ex post facto assessment of diffusion tensor imaging metrics from different MRI protocols: Preparing for multicentre studies in ALS (2015) (28)
Neuroprotective function of cellular prion protein in a mouse model of amyotrophic lateral sclerosis. (2010) (28)
Brain metabolites in definite amyotrophic lateral sclerosis (2007) (28)
Ribosomal transcription is regulated by PGC-1alpha and disturbed in Huntington’s disease (2017) (27)
LRRK2 contributes to monocyte dysregulation in Parkinson’s disease (2016) (27)
A biochemical marker panel in MRI-proven hyperacute ischemic stroke-a prospective study (2012) (27)
Evaluating multicenter DTI data in Huntington's disease on site specific effects: An ex post facto approach☆ (2013) (27)
α-synuclein interacts with SOD1 and promotes its oligomerization (2015) (27)
Telomere shortening leads to earlier age of onset in ALS mice (2016) (27)
Erythropoietin in Cerebrospinal Fluid: Age-related Reference Values and Relevance in Neurological Disease (2007) (27)
Acute ethanol administration results in a protective cytokine and neuroinflammatory profile in traumatic brain injury (2017) (27)
Proteomic studies in the discovery of cerebrospinal fluid biomarkers for amyotrophic lateral sclerosis (2017) (27)
Proton magnetic resonance spectroscopy in Kennedy syndrome. (1999) (27)
Common and rare variant association analyses in amyotrophic lateral sclerosis identify 15 risk loci with distinct genetic architectures and neuron-specific biology (2021) (26)
Is spinal epidural lipomatosis an MRI‐based diagnosis with clinical implications? A retrospective analysis (2007) (26)
Additional resources and the default mode network: Evidence of increased connectivity and decreased white matter integrity in amyotrophic lateral sclerosis (2014) (26)
The SETX missense variation spectrum as evaluated in patients with ALS4-like motor neuron diseases (2013) (26)
Implementation of a population-based epidemiological rare disease registry: study protocol of the amyotrophic lateral sclerosis (ALS) - registry Swabia (2013) (26)
Beta-synuclein in cerebrospinal fluid as an early diagnostic marker of Alzheimer’s disease (2020) (26)
N-isopropyl-p-123I-amphetamine single photon emission computer tomography in motor neuron disease. (1989) (26)
Phenotypic differences of amyotrophic lateral sclerosis (ALS) in China and Germany (2018) (26)
C9orf72 and UNC13A are shared risk loci for ALS and FTD: A genome-wide meta-analysis (2014) (26)
Dysregulation of a novel miR-1825/TBCB/TUBA4A pathway in sporadic and familial ALS (2018) (25)
Effect of high-caloric nutrition on serum neurofilament light chain levels in amyotrophic lateral sclerosis (2020) (25)
Only subtle cognitive deficits in non–bulbar amyotrophic lateral sclerosis patients (2006) (25)
H63D polymorphism in HFE is not associated with amyotrophic lateral sclerosis (2013) (25)
Images in cardiovascular medicine. Cardiomyopathy in a Duchenne muscular dystrophy carrier and her diseased son: similar pattern revealed by cardiovascular MRI. (2010) (25)
Corticoefferent pathology distribution in amyotrophic lateral sclerosis: in vivo evidence from a meta-analysis of diffusion tensor imaging data (2018) (25)
The combined use of Brain Computer Interface and Eye-Tracking technology for cognitive assessment in Amyotrophic Lateral Sclerosis (2011) (25)
CuZn-Superoxide Dismutase in D90A Heterozygotes from Recessive and Dominant ALS Pedigrees (2002) (25)
Exome array analysis of rare and low frequency variants in amyotrophic lateral sclerosis (2019) (25)
Can lesions to the motor cortex induce amyotrophic lateral sclerosis? (2014) (25)
HEK-293 cells expressing the human dopamine transporter are susceptible to low concentrations of 1-methyl-4-phenylpyridine (MPP+) via impairment of energy metabolism. (1999) (25)
Concentrations of beta-amyloid precursor protein processing products in cerebrospinal fluid of patients with amyotrophic lateral sclerosis and frontotemporal lobar degeneration (2009) (25)
Profilin 1 with the amyotrophic lateral sclerosis associated mutation T109M displays unaltered actin binding and does not affect the actin cytoskeleton (2015) (25)
Identical patterns of cortico-efferent tract involvement in primary lateral sclerosis and amyotrophic lateral sclerosis: A tract of interest-based MRI study (2018) (25)
White matter abnormalities in phenylketonuria: results of magnetic resonance measurements. (1994) (24)
Progranulin as a candidate biomarker for therapeutic trial in patients with ALS and FTLD (2016) (24)
In Vivo Protein Complementation Demonstrates Presynaptic α-Synuclein Oligomerization and Age-Dependent Accumulation of 8-16-mer Oligomer Species. (2019) (24)
Connective tissue disorders in dissections of the carotid or vertebral arteries (2008) (24)
Candidate biomarkers of chronic inflammatory demyelinating polyneuropathy (CIDP): Proteome analysis of cerebrospinal fluid (2009) (24)
A clinical neurophysiologic study of tropical spastic paraparesis (1988) (24)
Mutations in cytoplasmic dynein lead to a Huntington's disease-like defect in energy metabolism of brown and white adipose tissues. (2011) (24)
Camptocormia in Parkinson’s Disease: A Review of the Literature (2011) (24)
Quantitative muscle ultrasound in neuromuscular disorders using the parameters ‘intensity’, ‘entropy’, and ‘fractal dimension’ (2009) (24)
STAT6 mediates the effect of ethanol on neuroinflammatory response in TBI (2019) (24)
Impaired RNA splicing of 5′-regulatory sequences of the astroglial glutamate transporter EAAT2 in human astrocytoma (2001) (24)
Screening for CHCHD10 mutations in a large cohort of sporadic ALS patients: no evidence for pathogenicity of the p.P34S variant. (2016) (24)
Analysis of the KIFAP3 gene in amyotrophic lateral sclerosis: a multicenter survival study (2014) (24)
CSF Concentrations of cAMP and cGMP Are Lower in Patients with Creutzfeldt-Jakob Disease but Not Parkinson's Disease and Amyotrophic Lateral Sclerosis (2012) (24)
5′‐Heterogeneity of the human excitatory amino acid transporter cDNA EAAT2 (GLT‐1) (1998) (24)
Cardiovascular magnetic resonance imaging (CMR) reveals characteristic pattern of myocardial damage in patients with mitochondrial myopathy (2012) (24)
Neuropharmacology and Neurotoxicology (1996) (24)
Parkinson’s disease-like midbrain hyperechogenicity is frequent in amyotrophic lateral sclerosis (2013) (23)
Safety, tolerability, and preliminary efficacy of an IGF-1 mimetic in patients with spinal and bulbar muscular atrophy: a randomised, placebo-controlled trial (2018) (23)
Association of Serum Retinol-Binding Protein 4 Concentration With Risk for and Prognosis of Amyotrophic Lateral Sclerosis (2018) (23)
White matter abnormalities in phenylketonuria: results of magnetic resonance measurements (1994) (23)
Spastin related hereditary spastic paraplegia with dysplastic corpus callosum (2005) (23)
Safety and efficacy of immunoadsorption versus plasma exchange in steroid-refractory relapse of multiple sclerosis and clinically isolated syndrome: A randomised, parallel-group, controlled trial (2019) (23)
Endothelial damage, vascular bagging and remodeling of the microvascular bed in human microangiopathy with deep white matter lesions (2018) (23)
Different CSF protein profiles in amyotrophic lateral sclerosis and frontotemporal dementia with C9orf72 hexanucleotide repeat expansion (2020) (23)
Comparison of CSF and serum neurofilament light and heavy chain as differential diagnostic biomarkers for ALS (2021) (23)
Body fat distribution in Parkinson's disease: An MRI-based body fat quantification study. (2016) (23)
Identification of a potential non-coding RNA biomarker signature for amyotrophic lateral sclerosis (2020) (23)
Story of the ALS-FTD continuum retold: rather two distinct entities (2018) (22)
Perception of Emotional Facial Expressions in Amyotrophic Lateral Sclerosis (ALS) at Behavioural and Brain Metabolic Level (2016) (22)
Increase of ATP levels by glutamate antagonists is unrelated to neuroprotection (1994) (22)
Hyperconnective and hypoconnective cortical and subcortical functional networks in multiple system atrophy. (2018) (22)
Differential RNA cleavage and polyadenylation of the glutamate transporter EAAT2 in the human brain. (2000) (22)
Increase of ATP levels by glutamate antagonists is unrelated to neuroprotection (1994) (22)
Limited effects of glatiramer acetate in the high-copy number hSOD1-G93A mouse model of ALS (2007) (22)
Meningeal derived cerebrospinal fluid proteins in different forms of dementia: is a meningopathy involved in normal pressure hydrocephalus? (2004) (22)
Modified serpinA1 as risk marker for Parkinson’s disease dementia: Analysis of baseline data (2016) (22)
The metabolic and endocrine characteristics in spinal and bulbar muscular atrophy (2018) (22)
FDG-PET underscores the key role of the thalamus in frontotemporal lobar degeneration caused by C9ORF72 mutations (2019) (22)
An eye-tracker controlled cognitive battery: overcoming verbal-motor limitations in ALS (2017) (21)
Diffusion Tensor Magnetic Resonance Imaging of the Brain in APP Transgenic Mice: A Cohort Study (2013) (21)
Functional and morphological deficits in late‐treated patients with homocystinuria: a clinical, electrophysiologic and MRI study (1991) (21)
Efficient processing of Alzheimer's disease amyloid-Beta peptides by neuroectodermally converted mesenchymal stem cells. (2010) (21)
Lack of association of apolipoprotein E epsilon 4 allele with bulbar-onset motor neuron disease. (1997) (21)
Intrinsic functional connectivity alterations in progressive supranuclear palsy: Differential effects in frontal cortex, motor, and midbrain networks (2017) (21)
Micronutrients supplementation and nutritional status in cognitively impaired elderly persons: a two-month open label pilot study (2013) (21)
Fully Automated Atlas-Based MR Imaging Volumetry in Huntington Disease, Compared with Manual Volumetry (2011) (21)
Eye-tracking controlled cognitive function tests in patients with amyotrophic lateral sclerosis: a controlled proof-of-principle study (2015) (21)
Association of NIPA1 repeat expansions with amyotrophic lateral sclerosis in a large international cohort (2019) (21)
Cardiomyopathy in motor neuron diseases (2006) (21)
Stability effects on results of diffusion tensor imaging analysis by reduction of the number of gradient directions due to motion artifacts: an application to presymptomatic Huntington’s disease (2011) (21)
Do eye movement impairments in patients with small vessel cerebrovascular disease depend on lesion load or on cognitive deficits? A video-oculographic and MRI study (2014) (20)
New methods in fMRI analysis (2002) (20)
Calcineurin A and calbindin immunoreactivity in the spinal cord of G93A superoxide dismutase transgenic mice (2001) (20)
Two novel mutations in conserved codons indicate that CHCHD10 is a gene associated with motor neuron disease (2014) (20)
Efficacy and safety of high‐dose cabergoline in Parkinson's disease (2006) (20)
In vitro toxicological investigations of isoxazolinone amino acids of Lathyrus sativus. (1995) (20)
Medical decisions are independent of cognitive impairment in amyotrophic lateral sclerosis (2016) (20)
Functional MR Imaging in Patients with Carotid Artery Stenosis before and after Revascularization (2010) (19)
Lack of association of apolipoprotein E ϵ4 allele with bulbar‐onset motor neuron disease (1997) (19)
Oral levosimendan in amyotrophic lateral sclerosis: a phase II multicentre, randomised, double-blind, placebo-controlled trial (2019) (19)
Intrinsic Functional Connectivity Networks in Healthy Elderly Subjects: A Multiparametric Approach with Structural Connectivity Analysis (2014) (19)
Neuropathology of Partial PGC-1α Deficiency Recapitulates Features of Mitochondrial Encephalopathies but Not of Neurodegenerative Diseases (2013) (19)
Cerebrospinal fluid markers of idiopathic intracranial hypertension: Is the renin-angiotensinogen system involved? (2011) (19)
High rate of constitutional chromosomal rearrangements in apparently sporadic ALS (2003) (19)
Clinical Trials in Spinal and Bulbar Muscular Atrophy—Past, Present, and Future (2016) (19)
Brain-Specific Cytoskeletal Damage Markers in Cerebrospinal Fluid: Is There a Common Pattern between Amyotrophic Lateral Sclerosis and Primary Progressive Multiple Sclerosis? (2015) (19)
Acute DWI Reductions In Patients After Single Epileptic Seizures - More Common Than Assumed (2018) (19)
Genome‐wide survey of copy number variants finds MAPT duplications in progressive supranuclear palsy (2019) (19)
Benzodiazepine receptors and cerebral blood flow in partial epilepsy (2004) (19)
Functional reorganization during cognitive function tasks in patients with amyotrophic lateral sclerosis (2018) (18)
A first approach to a neuropsychological screening tool using eye-tracking for bedside cognitive testing based on the Edinburgh Cognitive and Behavioural ALS Screen (2017) (18)
Early diagnosis of rhinocerebral mucormycosis by cerebrospinal fluid analysis and determination of 16s rRNA gene sequence (2007) (18)
An eye-tracking controlled neuropsychological battery for cognitive assessment in neurological diseases (2017) (18)
Low dietary protein content alleviates motor symptoms in mice with mutant dynactin/dynein-mediated neurodegeneration. (2015) (18)
Serum Vitamin D Concentrations and Cognitive Function in a Population-Based Study among Older Adults in South Germany. (2015) (18)
Neurodevelopmental and neurodegenerative diseases - is there a pathophysiological link? Attention-deficit/hyperactivity disorder and amyotrophic lateral sclerosis as examples. (2008) (18)
Primary progressive aphasia accompanied by environmental sound agnosia: A neuropsychological, MRI and PET study (2006) (18)
Pattern of paresis in ALS is consistent with the physiology of the corticomotoneuronal projections to different muscle groups (2020) (18)
Ifosfamide encephalopathy presenting with asterixis (2002) (18)
Two novel mutations in conserved codons indicate that CHCHD 10 is a gene associated with motor neuron disease (2014) (18)
Chronic ergotamine abuse: evidence of functional impairment of long ascending spinal tracts. (1988) (18)
Identifying ischemic stroke associated with cancer: a multiple model derived from a case–control analysis (2017) (18)
Atrophy and structural covariance of the cholinergic basal forebrain in primary progressive aphasia (2016) (17)
Serum creatine kinase and creatinine in adult spinal muscular atrophy under nusinersen treatment (2021) (17)
Two-Point Magnitude MRI for Rapid Mapping of Brown Adipose Tissue and Its Application to the R6/2 Mouse Model of Huntington Disease (2014) (17)
Cerebellar atrophy following acute 0intoxication with phenytoin (1989) (17)
Superoxide dismutase mutations of familial amyotrophic lateral sclerosis and the oxidative inactivation of calcineurin (2001) (17)
Elevated mRNA-levels of distinct mitochondrial and plasma membrane Ca2+ transporters in individual hypoglossal motor neurons of endstage SOD1 transgenic mice (2014) (17)
Frontal Corpus Callosum Alterations in Progressive Supranuclear Palsy but Not in Parkinson's Disease (2014) (17)
Heparin for prophylaxis of venous thromboembolism in intracerebral haemorrhage (2019) (17)
Adipose Tissue Distribution in Patients with Alzheimer's Disease: A Whole Body MRI Case-Control Study. (2015) (17)
Characteristics in Non-Vitamin K Antagonist Oral Anticoagulant-Related Intracerebral Hemorrhage. (2019) (17)
Neuroprotective effect of acute ethanol intoxication in TBI is associated to the hierarchical modulation of early transcriptional responses (2018) (17)
Novel ETFDH mutation and imaging findings in an adult with glutaric aciduria type II (2014) (17)
A complex form of hereditary spastic paraplegia in three siblings due to somatic mosaicism for a novel SPAST mutation in the mother (2014) (16)
A mutation in the dynein heavy chain gene compensates for energy deficit of mutant SOD1 mice and increases potentially neuroprotective IGF-1 (2011) (16)
De novo mutations in SOD1 are a cause of ALS (2021) (16)
Generalized epilepsy with febrile seizures plus: Further heterogeneity in a large family (2001) (16)
In vivo histopathological staging in C9orf72-associated ALS: A tract of interest DTI study (2020) (16)
Cortico-efferent tract involvement in primary lateral sclerosis and amyotrophic lateral sclerosis: A two-centre tract of interest-based DTI analysis (2018) (16)
Excitotoxicity, energy metabolism and neurodegeneration (1993) (16)
The advanced glycation end-product N epsilon-(carboxymethyl)lysine level is elevated in cerebrospinal fluid of patients with amyotrophic lateral sclerosis. (2004) (16)
A novel presenilin1 mutation (Q223R) associated with early onset Alzheimer’s disease, dysarthria and spastic paraparesis and decreased Abeta levels in CSF (2010) (16)
Mechanisms of Disease: Motoneuron Disease Aggravated by Transgenic Expression of a Functionally Modified AMPA Receptor Subunit (2005) (16)
Stage-dependent remodeling of projections to motor cortex in ALS mouse model revealed by a new variant retrograde-AAV9 (2018) (16)
A Score Based on NfL and Glial Markers May Differentiate Between Relapsing–Remitting and Progressive MS Course (2020) (16)
Inflammatory dysregulation of blood monocytes in Parkinson's disease patients (2014) (16)
Nuclear shrinkage in live mouse hippocampal slices (2001) (16)
Complementary Image Analysis of Diffusion Tensor Imaging and 3‐Dimensional T1‐Weighted Imaging: White Matter Analysis in Amyotrophic Lateral Sclerosis (2011) (16)
The Combination of Dopa-Responsive Parkinsonian Syndrome and Motor Neuron Disease (2009) (15)
The Economic Costs of Progressive Supranuclear Palsy and Multiple System Atrophy in France, Germany and the United Kingdom (2011) (15)
Amyotrophic lateral sclerosis and glutamate. (1998) (15)
Short-term and long-term effects of immunoadsorption in refractory chronic inflammatory demyelinating polyneuropathy: a prospective study in 17 patients (2018) (15)
A biallelic mutation links MYORG to autosomal-recessive primary familial brain calcification. (2019) (15)
Fabry Disease With Concomitant Lewy Body Disease (2019) (15)
Quantifying progression in primary progressive aphasia with structural neuroimaging (2021) (15)
The emerging picture of ALS: a multisystem, not only a "motor neuron disease. (2017) (15)
Identification of candidate genes for sporadic amyotrophic lateral sclerosis by array comparative genomic hybridization (2009) (15)
Cardiac Findings in Amyotrophic Lateral Sclerosis: A Magnetic Resonance Imaging Study (2017) (15)
Matrix metalloproteinases—A conceptional alternative for disease-modifying strategies in ALS/MND? (2006) (15)
Pathological laughing and crying in amyotrophic lateral sclerosis is related to frontal cortex function (2016) (15)
Cognitive assessment in Amyotrophic Lateral Sclerosis by means of P300-Brain Computer Interface: a preliminary study (2016) (15)
CSF SerpinA1 in Creutzfeldt–Jakob disease and frontotemporal lobar degeneration (2020) (15)
Neurotransplantation in mice: The concorde-like position ensures minimal cell leakage and widespread distribution of cells transplanted into the cisterna magna (2008) (15)
Neuroectodermally converted human mesenchymal stromal cells provide cytoprotective effects on neural stem cells and inhibit their glial differentiation. (2010) (15)
Perimenopause and Postmenopause – Diagnosis and Interventions. Guideline of the DGGG and OEGGG (S3-Level, AWMF Registry Number 015-062, September 2020) Peri- und Postmenopause – Diagnostik und Interventionen. Leitlinie der DGGG und OEGGG (S3-Level, AWMF-Registernummer 015-062, September 2020) (2021) (15)
In vivo assessment of retinal vessel pathology in amyotrophic lateral sclerosis (2018) (14)
Routine Cerebrospinal Fluid (CSF) Parameters in Patients With Spinal Muscular Atrophy (SMA) Treated With Nusinersen (2019) (14)
Intensified testing for attention-deficit hyperactivity disorder (ADHD) in girls should reduce depression and smoking in adult females and the prevalence of ADHD in the longterm. (2009) (14)
Seizures associated with antibodies against cell surface antigens are acute symptomatic and not indicative of epilepsy: insights from long-term data (2020) (14)
Studies of the etiology and pathogenesis of motor neuron diseases: III. Magnetic cortical stimulation in patients with lathyrism (1993) (14)
Chemical Hypoxia Facilitates Alternative Splicing of EAAT2 in Presymptomatic APP23 Transgenic Mice (2008) (14)
Longitudinal Diffusion Tensor Imaging Resembles Patterns of Pathology Progression in Behavioral Variant Frontotemporal Dementia (bvFTD) (2018) (14)
Experience matters: neurologists’ perspectives on ALS patients’ well-being (2017) (14)
Cerebral Microstructural Alterations after Radiation Therapy in High-Grade Glioma: A Diffusion Tensor Imaging-Based Study (2017) (14)
Glial fibrillary acidic protein as blood biomarker for differential diagnosis and severity of major depressive disorder. (2021) (14)
Cytoplasmic FUS triggers early behavioral alterations linked to cortical neuronal hyperactivity and inhibitory synaptic defects (2021) (14)
Hypercapnia is a Possible Determinant of the Function of the Blood-Cerebrospinal Fluid Barrier in Amyotrophic Lateral Sclerosis (2010) (14)
Longitudinal diffusion tensor magnetic resonance imaging analysis at the cohort level reveals disturbed cortical and callosal microstructure with spared corticospinal tract in the TDP-43G298S ALS mouse model (2019) (13)
Necrosome‐positive granulovacuolar degeneration is associated with TDP‐43 pathological lesions in the hippocampus of ALS/FTLD cases (2020) (13)
Complicated hereditary spastic paraplegia with thin corpus callosum: Variation of phenotypic expression over time (2004) (13)
Hereditary motor neuropathies and motor neuron diseases: which is which (2002) (13)
Histological correlates of postmortem ultra-high-resolution single-section MRI in cortical cerebral microinfarcts (2020) (13)
Different regional brain volume loss in pure and complicated hereditary spastic paraparesis: A voxel‐based morphometric study (2007) (13)
Psychiatric aspects of writer's cramp (2005) (13)
Pyrimethamine Significantly Lowers CSF/SOD1 in ALS Patients With SOD1 Mutations (2017) (13)
Pyrimethamine significantly lowers cerebrospinal fluid Cu/Zn superoxide dismutase in amyotrophic lateral sclerosis patients with SOD1 mutations (2017) (13)
Recessively inherited amyotrophic lateral sclerosis: a German family with the D90A CuZn-SOD mutation (2000) (13)
Microstructure of the Midbrain and Cervical Spinal Cord in Idiopathic Restless Legs Syndrome: A Diffusion Tensor Imaging Study. (2016) (13)
Preclinical Trials – An Update on Translational Research in ALS (2006) (13)
The multisystem degeneration amyotrophic lateral sclerosis - neuropathological staging and clinical translation. (2017) (13)
Impairment of callosal and corticospinal system function in adolescents with early-treated phenylketonuria: a transcranial magnetic stimulation study (1999) (13)
Informal Caregiving in Amyotrophic Lateral Sclerosis (ALS): A High Caregiver Burden and Drastic Consequences on Caregivers’ Lives (2021) (13)
Non-invasive assessment of the pyramidal tract and motor pathway of primates. (1987) (13)
Segmental involvement of the corpus callosum in C9orf72-associated ALS: a tract of interest-based DTI study (2021) (13)
CSF filtration is an effective treatment of Guillain–Barré syndrome (2001) (13)
Altered migration and adhesion potential of pro-neurally converted human bone marrow stromal cells. (2008) (13)
Symptomatic pharmacotherapy in ALS: data analysis from a platform-based medication management programme (2020) (12)
Longitudinal Serum Neurofilament Levels of Multiple Sclerosis Patients Before and After Treatment with First-Line Immunomodulatory Therapies (2020) (12)
The role of de novo mutations in the development of amyotrophic lateral sclerosis (2017) (12)
Protease-resistant SOD1 aggregates in amyotrophic lateral sclerosis demonstrated by paraffin-embedded tissue (PET) blot (2014) (12)
Statins, diabetes mellitus and prognosis of amyotrophic lateral sclerosis: data from 501 patients of a population‐based registry in southwest Germany (2020) (12)
Benign symmetric lipomatosis with axonal neuropathy and abnormalities in specific mitochondrial tRNA regions. (2006) (12)
Parvalbumin Interneurons Shape Neuronal Vulnerability in Blunt TBI. (2019) (12)
Non-invasive ventilation in amyotrophic lateral sclerosis (2019) (12)
Severe white matter damage in SHANK3 deficiency: a human and translational study (2019) (12)
[Management of dysphagia in acute stroke : A prospective study for validation of current recommendations]. (2017) (12)
Cognitive assessment of executive functions using brain computer interface and eye-tracking (2013) (12)
Sequence variations in C9orf72 downstream of the hexanucleotide repeat region and its effect on repeat-primed PCR interpretation: a large multinational screening study (2017) (12)
Existential decision-making in a fatal progressive disease: how much do legal and medical frameworks matter? (2017) (12)
The Neuroprotective Effect of Ethanol Intoxication in Traumatic Brain Injury Is Associated with the Suppression of ErbB Signaling in Parvalbumin-Positive Interneurons. (2018) (12)
Linking neuron and skin: matrix metalloproteinases in amyotrophic lateral sclerosis (2009) (12)
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Cytoplasmic accumulation of FUS triggers early behavioral alterations linked to cortical neuronal hyperactivity and defects in inhibitory synapses (2020) (1)
[Electromagnetic stimulation of the nervous system. II. Normal values in the peripheral nervous system and comparison with electrical stimulation methods]. (1989) (1)
Predictive parameters of early respiratory decline in amyotrophic lateral sclerosis (2022) (1)
Association of Serum GFAP with Functional and Neurocognitive Outcome in Sporadic Small Vessel Disease (2022) (1)
Interleukin-13 and its receptor are synaptic proteins involved in plasticity and neuroprotection (2021) (1)
Reversible focal brain edema in a patient with MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes) syndrome (1996) (1)
Peer recommendations on how to improve clinical research, and Conference wrap-up (2013) (1)
Thoracic trauma promotes alpha-Synuclein oligomerization in murine Parkinson's disease (2022) (1)
Body fat compartment determination by encoder–decoder convolutional neural network: application to amyotrophic lateral sclerosis (2022) (1)
Genotypes of amyotrophic lateral sclerosis in Mongolia (2019) (1)
Genetic variants in presenilins and correlation to amyloid-beta 42 levels in cerebrospinal fluid and diagnosis of Alzheimer's disease (2009) (1)
Amyotrophic lateral sclerosis and other TDP‐43 proteinopathies (2016) (1)
Integrative genetic analysis illuminates ALS heritability and identifies risk genes (2023) (1)
ALSFRS-R-SE: an adapted, annotated, and self-explanatory version of the revised amyotrophic lateral sclerosis functional rating scale (2022) (1)
Relaxation-weighted 23Na magnetic resonance imaging maps regional patterns of abnormal sodium concentrations in amyotrophic lateral sclerosis (2022) (1)
The intrinsically restructured fovea is correlated with contrast sensitivity loss in Parkinson’s disease (2020) (1)
Therapeutic Problems in Patients Suffering from Aluminum Encephalopathy (1987) (1)
Alterations of circulating insulin-like growth factor system components and androgen insensitivity in patients with Kennedy’s disease (2004) (1)
V42. De novo mutations in the FUS gene are a frequent cause of sporadic ALS in very young patients (2015) (1)
Cerebrospinal fluid levels of proenkephalin and prodynorphin are differentially altered in Huntington’s and Parkinson’s disease (2022) (1)
[Adult-onset neuronal ceroid lipofuscinosis]. (2007) (1)
Diffusion Tensor Imaging in Amyotrophic Lateral Sclerosis: Machine Learning for Biomarker Development (2023) (1)
FP57-FR-05 Prognostic markers in amyotrophic lateral sclerosis (ALS): proteome analysis of cerebrospinal fluid (2009) (1)
Variants and differential diagnosis of peripheral nerve hyperexcitability (2006) (0)
Neurodegenerative Diseases: Prion diseases (2005) (0)
GSM1278160: serum_sALS patient_16 (2014) (0)
Prophylactic antibiotics to reduce pneumonia after acute stroke (2016) (0)
Reduction of ephrin-A5 aggravates disease progression in amyotrophic lateral sclerosis (2019) (0)
GSM1278153: serum_sALS patient_9 (2014) (0)
Explorer A large-scale multicentre cerebral diffusion tensor imaging study in amyotrophic lateral sclerosis (2016) (0)
FACIAL PERCEPTION OF TRUSTWORTHINESS IN FRONTOTEMPORAL DEMENTIA (2014) (0)
A novel homozygous p.Ser69Pro SOD1 mutation causes severe young-onset ALS with decreased enzyme activity (2022) (0)
GSM1278116: serum_healthy control_7 (2014) (0)
Reduced T 2 reveal therapeutic effect of the antioxidant Vitamin E in the G 93 A-SOD 1 Mouse Model of ALS (2008) (0)
GSM1278114: serum_healthy control_5 (2014) (0)
195. Differential diagnostic value of eye movement recording in PSP-Parkinsonism, Richardson’s syndrome, and idiopathic Parkinson’s disease (2009) (0)
P100 Deep brain stimulation and medication in Parkinson’s Disease: Changes in impulse control (2011) (0)
Response to Letter Regarding Article, “Cardiomyopathy in a Duchenne Muscular Dystrophy Carrier and Her Diseased Son: Similar Pattern Revealed by Cardiovascular MRI” (2010) (0)
The association between alterations of eye movement control and cerebral intrinsic functional connectivity in Parkinson’s disease (2015) (0)
GSM1278142: serum_ALS mutation carrier_10 (2014) (0)
Adipokines, C-reactive protein and Amyotrophic Lateral Sclerosis – results from a population- based ALS registry in Germany (2017) (0)
GSM1278132: serum_fALS patient_6 (2014) (0)
Front & Back Matter (2012) (0)
Array CGH of ALS patients – new candidate genes (2006) (0)
P21 Executive eye movement impairment in presymptomatic ALS mutation carriers (2020) (0)
Neurochemical markers in the differential diagnosis of primary progressive aphasias: Data from the German FTLD consortium (2016) (0)
FV 5. MRI-based texture analysis of the corpus callosum in progressive supranuclear palsy (2021) (0)
Safety, Tolerability and Monitoring of Long-Term G-CSF Compassionate Use in ALS Patients (P3.181) (2016) (0)
Partial agenesis of the corpus callosum in spastin related hereditary spastic paraplegia (2004) (0)
Increased Chitotriosidase1 Concentration Following Nusinersen Treatment in Spinal Muscular Atrophy (2021) (0)
GSM1278124: serum_healthy control_15 (2014) (0)
Sequential alterations in diffusion metrics as correlates of disease severity in amyotrophic lateral sclerosis (2023) (0)
FUS mutations dominate TBK1 mutations in FUS/TBK1 double-mutant ALS/FTD pedigrees (2021) (0)
GSM1278122: serum_healthy control_13 (2014) (0)
The SETX missense variation spectrum as evaluated in patients with ALS4-like motor neuron diseases (2012) (0)
Imaging of neurodegenerative diseases using Tau PET/CT with [11C]PBB3 in a clinical setting (2019) (0)
FV 11 Patterns of abnormal functional connectivity in progressive supranuclear palsy are associated with impaired behavioral performance (2017) (0)
Correction to: Short-term safety results from compassionate use of risdiplam in patients with spinal muscular atrophy in Germany (2022) (0)
The pathoanatomy of primary lateral sclerosis: whole-brain based DTI depicts widespread white matter changes (2009) (0)
Three new SOD-1 mutations in familial ALS. (1995) (0)
Longitudinal diffusion tensor magnetic resonance imaging analysis at the cohort level reveals disturbed cortical and callosal microstructure with spared corticospinal tract in the TDP-43G298S ALS mouse model (2019) (0)
GSM1278130: serum_fALS patient_4 (2014) (0)
FV 1 In vivo staging in ALS – A mono-centric cross-sectional and longitudinal DTI-based study (2017) (0)
News about the etiology and pathogenesis of ALS (2012) (0)
5 CLINICAL AND ELECTROPHYSIOLOGICAL FINDINGS IN ADOLESCENT PATIENTS WITH PHENYLKETONURIA (PKU) (1990) (0)
A GENOME-WIDE ASSOCIATION STUDY IN PROGRESSIVE SUPRANUCLEAR PALSY (2015) (0)
Author and Subject Index (2006) (0)
Quantitative mass spectrometry suggests beta‐synuclein as promising blood marker for synaptic degeneration in Alzheimer's disease (2020) (0)
Contents Vol. 12, 2013 (2013) (0)
P24Changes in cortical networks in Parkinson's disease: Covariance analysis of functional connectivity and oculomotor performance (2013) (0)
EPV 2. Spreading patterns of behavioral variant frontotemporal dementia: Analysis of longitudinal in vivo diffusion tensor imaging data (2016) (0)
“Reporting biomarker” development: Update in als patients treated with G-CSF -mobilized hematopoietic stem cells (2013) (0)
MRI AS OBJECTIVE FOLLOW-UP PARAMETER IN PATIENTS OF PRIMARY PROGRESSIVE APHASIAS (2019) (0)
GSE52917: Early pre-symptomatic and progressive signatures of non-coding RNAs in ALS patients and mutation carriers (2014) (0)
3.062 ATAXIN-2 INTERMEDIATE POLYGLUTAMINE EXPANSIONS CONTRIBUTE TO RISK OF ALS AND OF EARLY ONSET HEREDITARY PD (2012) (0)
Multiparametric MRI analysis of patients with amyotrophic lateral sclerosis compared to controls (2009) (0)
P 5 Volumetric analysis of the hypothalamus in a large cohort of patient with amyotrophic lateral sclerosis (2017) (0)
GSM1278156: serum_sALS patient_12 (2014) (0)
GSM1278112: serum_healthy control_3 (2014) (0)
Corticoefferent pathology distribution in amyotrophic lateral sclerosis: in vivo evidence from a meta-analysis of diffusion tensor imaging data (2018) (0)
GSM1278158: serum_sALS patient_14 (2014) (0)
Met/HGFR triggers detrimental reactive microglia in TBI (2021) (0)
Author response: Stage-dependent remodeling of projections to motor cortex in ALS mouse model revealed by a new variant retrograde-AAV9 (2018) (0)
Amygdala Size Reduction Is Associated with Memory Deficits in Complicated Hereditary Spastic Paraparesis: An MRI Study (2010) (0)
GSM1278123: serum_healthy control_14 (2014) (0)
P 9 A multi-modal in vivo staging approach to amyotrophic lateral sclerosis (2022) (0)
Natural History and Neuroprotection in Parkinson Plus Syndromes Parkinson Plus Scale (2014) (0)
Comment: Braak staging in clinical practice? (2014) (0)
Investigating brain structure in different FTLD subtypes using voxel-based morphometry – Data from the multicentric FTLD consortium’s study Germany (2014) (0)
Neurofilament light and heterogeneity of disease progression in amyotrophic lateral sclerosis: development and validation of a prediction model to improve interventional trials (2021) (0)
V90 Longitudinal description of Magnetic Resonance Imaging (MRI) findings in a large prospective cohort of patients with Progressive Supranuclear Palsy (PSP) and Multiple System Atrophy (MSA) (2011) (0)
GSM1278141: serum_ALS mutation carrier pool 4 (2014) (0)
GSM1278144: serum_ALS mutation carrier_12 (2014) (0)
Neurologic Diseases Associated with Use of Plant Components with Toxic Potential1 (1994) (0)
Identification and functional characterization of extracellular signals affecting the expression of astroglial glutamate transporters (2002) (0)
GSM1278126: serum_healthy control_17 (2014) (0)
GSM1278134: serum_ALS mutation carrier_2 (2014) (0)
Internalized capillaries in skeletal muscle biopsy (2015) (0)
P17 MRI volumetry and microtexture analysis of focal alterations of the callosal area III in primary lateral sclerosis (2020) (0)
Can we turn mice into men? (2019) (0)
Ribosomal transcription is regulated by PGC-1alpha and disturbed in Huntington’s disease (2017) (0)
ALS/MND: the spectrum of clinical and pathological phenotypes (2015) (0)
Adenomatous Polyposis, Familial (2009) (0)
GSM1278138: serum_ALS mutation carrier pool 2 (2014) (0)
P79 TRACKING DISEASE PROGRESSION BY MRI: CLINICAL FEATURES OF AMYOTROPHIC LATERAL SCLEROSIS CORRELATE WITH FOCAL CORTICAL THINNING OF THE MOTOR CORTEX (2013) (0)
FXN GAA repeat expansions in amyotrophic lateral sclerosis (2014) (0)
GSM1278117: serum_healthy control_8 (2014) (0)
Amyloid precursor protein-fragments-containing inclusions in cardiomyocytes with basophilic degeneration and its association with cerebral amyloid angiopathy and myocardial fibrosis (2018) (0)
DELETION OF 14-3-3GAMMA EXACERBATES DISEASE IN MOUSE MODELS OF NEURODEGENERATIVE PROTEINOPATHY (2014) (0)
DTI data analysis: application of fiber tracking to group averaged data sets (2010) (0)
ALS Mimics (2018) (0)
[Amyotrophic lateral sclerosis]. (2014) (0)
GSM1278147: serum_sALS patient_3 (2014) (0)
Books Reviews (2004) (0)
P146 Human body fat percentage determination in Parkinson’s disease: A pilot MRI study (2011) (0)
Developmental and Functional Nature of Human iPSC Derived Motoneurons (2011) (0)
Towards a European Registry and Biorepository for Patients with Spinal and Bulbar Muscular Atrophy (2016) (0)
The p150 subunit of dynactin gene in multiple sclerosis (2006) (0)
GSM1278161: serum_sALS patient_17 (2014) (0)
Subject Index Vol. 28, 1988 (1988) (0)
Support vector machine classification enables detection of primary progressive aphasia subtypes with MRI (2015) (0)
Association of Presenilin2 genetic variations and beta-amyloid 42 level in cerebrospinal fluid of early-onset Alzheimer's disease patients (2008) (0)
Contents, Vol. 28, 1988 (1988) (0)
GSM1278159: serum_sALS patient_15 (2014) (0)
Cardiovascular magnetic resonance imaging based comparison of myocardial involvement in a patient with LGMD 2C and a patient with Becker muscular dystrophy (2010) (0)
P18. Pattern of ‘resting-state’ functional hyperconnectivity in Amyotrophic lateral sclerosis reflects neuropathological disease stages (2015) (0)
Evaluation of Visual Rating Scales for MRI Supported Diagnosis of Frontotemporal Lobar Degeneration (2012) (0)
Micronutritional status in dementia (2008) (0)
GSM1278150: serum_sALS patient_6 (2014) (0)
PAW22 Does cortical atrophy take place after basal ganglia damage in multiple system atrophy and progressive supranuclear palsy? (2010) (0)
Depression and Quality of Life in Patients With Amyotrophic Lateral Sclerosis: In Reply (2008) (0)
Sporadic ALS with early-onset respiratory failure is not associated with IGHMBP2 gene mutations (2007) (0)
5-variability of the glutamate transporter EAAT2-A candidate gene of chronic neurodegeneration (1999) (0)
Endothelial damage, vascular bagging and remodeling of the microvascular bed in human microangiopathy with deep white matter lesions (2018) (0)
ID 221 – Disturbed eye movement control in Parkinson’s disease correlates with intrinsic functional connectivity within cortical networks (2016) (0)
P 70 MR imaging correlates of impaired eye movement control in parkinsonian syndromes (2017) (0)
The clinical picture and concept of ALS in 2016 (2016) (0)
EPV 3. Regional microstructural impairment is associated with characteristic altered oculomotor performance in Parkinsonian syndromes (2016) (0)
P18 A human and translational study of severe white matter damage in SHANK3 deficiency (2020) (0)
PREDICT-juvenile-stroke: PRospective evaluation of a prediction score determining individual clinical outcome three months after ischemic stroke in young adults – a study protocol (2023) (0)
Biomarker - Discovery in G-CSF mediated clinical ALS Stabilization (P6.005) (2015) (0)
Amyotrophe Lateralsklerose (ALS) (2020) (0)
The m.9143T>C Variant: Recurrent Infections and Immunodeficiency as an Extension of the Phenotypic Spectrum in MT-ATP6 Mutations? (2020) (0)
Evaluation of C-terminal cross-linling telopeptide of type I collagen ( CTx ) in the urine of breast cancer bone metastsis female patients treated with zoledronic acid (2005) (0)
Author Correction: Common and rare variant association analyses in amyotrophic lateral sclerosis identify 15 risk loci with distinct genetic architectures and neuron-specific biology (2022) (0)
GSM1278113: serum_healthy control_4 (2014) (0)
Alterations of eye movements in Parkinson's disease with high frequency subthalamic nucleus deep brain stimulation (2009) (0)
Polyneuropathy in type 1 and type 2 diabetes: do we need different examinations? (2010) (0)
Histological correlates of postmortem ultra-high-resolution single-section MRI in cortical cerebral microinfarcts (2020) (0)
GSM1278110: serum_healthy control_1 (2014) (0)
MUSCLE IMAGING – MRI P.158 The impact of muscle MRI in diagnostics of myopathies (2020) (0)
Patient-Centered Approach Might Effectively Tackle The Definition Of Progression In Chronic Neurological Diseases: Results From the EmBioProMS Trial in Progressive Multiple Sclerosis. (2021) (0)
B21 Ribosomal transcription is regulated by PGC-1alpha and disturbed in huntington’s disease (2016) (0)
Well‐being on supportive techniques in amyotrophic lateral sclerosis: from neurologists' perspective (2022) (0)
FV 3. Diagnostic value of oculomotor parameters in progressive supranuclear palsy: a monocentric study in 100 patients (2021) (0)
Novel CSF Biomarkers of Disease Progression of Amyotrophic Lateral Sclerosis (2011) (0)
Preclinical Development NFk B Is Required for SmacMimetic-Mediated Sensitization of Glioblastoma Cells for g-Irradiation – Induced Apoptosis (2011) (0)
Safety and Tolerability of Dexpramipexole for the Treatment of ALS: Results from the Randomized, Double-Blind, Placebo-Controlled Trial, EMPOWER (PL02.003) (2013) (0)
FV7. Tract of interest-based DTI analysis in upper and lower motor neuron disease variants of ALS (2018) (0)
Exome array analysis of rare and low frequency variants in amyotrophic lateral sclerosis (2019) (0)
Auditory evoked potentials in chronic schizophrenics (1990) (0)
The effect of experimentelly induced painful thermal stimuli on chronic pain patients (1987) (0)
[Normal and pathological aging of the brain]. (2001) (0)
Digital Scientific Platform for Independent Content in Neurology: Rigorous Quality Guideline Development and Implementation (2022) (0)
PB 1 In vivo assessment of neurodegeneration in the brain by serial longitudinal diffusion tensor imaging – an application to amyotrophic lateral sclerosis (2017) (0)
Biomarkers Reflect ALS Clinical Courses (P5.039) (2016) (0)
Multimodality approach to neuroimaging in amyotrophic lateral sclerosis/frontotemporal dementia (2012) (0)
FV 21 Heading for imaging read-outs in Huntington’s disease: Sample size calculations for longitudinal automatic MRI volumetry (2019) (0)
Familial Amyotrophic Lateral Sclerosis – risk carriers in focus (2016) (0)
FV 29 Executive eye movement impairment in presymptomatic amyotrophic lateral sclerosis mutation carriers (2019) (0)
Explorative study of emerging blood biomarkers in progressive multiple sclerosis (EmBioProMS) (2350) (2020) (0)
Studies on the therapeutic potential of adult stem cells in the G 93 A animal model of amyotrophic lateral sclerosis ( ALS ) (2011) (0)
Abstract 14014: Early Detection of Myocardial Involvement in Idiopathic Inflammatory Myopathies Assessed by Cardiac Magnetic Resonance Imaging (2015) (0)
Histological verified focal myositis in familial Mediterranean fever (2006) (0)
[Amyotrophic lateral sclerosis and frontotemporal dementia-On the way to common gene-specific treatment approaches]. (2021) (0)
Advocacy in amyotrophic lateral sclerosis (2019) (0)
[Methodology and normal values in recording evoked motor potentials following transcranial stimulation in the human]. (1987) (0)
The differential diagnostic value of video-oculography in the early differentiation of PSP-P and IPD (2008) (0)
A natural history comparison of SOD1-mutant patients with amyotrophic lateral sclerosis between Chinese and German populations (2021) (0)
Natural history and clinical features of amyotrophic lateral sclerosis in Mongolia (2017) (0)
Retinoids and Amyotrophic Lateral Sclerosis-Reply. (2018) (0)
CARDIAC INVOLVEMENT IN IDIOPATHIC INFLAMMATORY MYOPATHY DETECTED BY CARDIAC MAGNETIC RESONANCE IMAGING (2014) (0)
Frontiers of amyotrophic lateral sclerosis cognitive assessment: The use of Eye-tracking and Brain Computer Interface in the eBrain project (2013) (0)
European Incidence of Syndromes Associated with Frontotemporal Lobar Degeneration (2022) (0)
Functional reorganization during cognitive function tasks in patients with amyotrophic lateral sclerosis (2017) (0)
ROLE OF FREE AND EXOSOMAL TDP-43 AS A DIAGNOSTIC TOOL IN NEURODEGENERATIVE DISEASES (2014) (0)
PB1. Functional network alterations in multisystem atrophy (MSA) (2018) (0)
TDP-43 and beta-amyloid precursor protein processing products in cerebrospinal fluid of patients with amyotrophic lateral sclerosis and frontotemporal lobar degeneration (2009) (0)
Which Nerve Cell Types Develop TDP-43-immunoreactive Inclusions in sALS? (2017) (0)
Early onset sporadic ALS – phenotype of eight patients (2005) (0)
P 6 Eye movement disturbances are associated with regional brain volume loss in neurodegenerative Parkinsonism (2017) (0)
FUS mutations dominate TBK1 mutations in FUS/TBK1 double-mutant ALS/FTD pedigrees (2021) (0)
P19 A tract of interest-based DTI study for in-vivo-staging in ALS patients with C9orf72 mutation (2020) (0)
Clinical and experimental neuroprotection in neurodegenerative diseases is a therapeutic option for the future of amyotrophic lateral sclerosis (ALS). Preface. (2000) (0)
Glial Fibrillary Acidic Protein in Cerebrospinal Fluid of Nusinersen-Treated Patients with Spinal Muscular Atrophy (2021) (0)
Treatment of amyotrophic lateral sclerosis— What is the next step? (2000) (0)
V94 Voxel-based MRI volumetry of the caudate in Huntington’s disease, compared with manual volumetry (2011) (0)
Amyotrophic lateral sclerosis: disease stage related changes of biochemical markers (tau protein, creatine kinase, sCD14) in CSF and serum (2005) (0)
MRI IN ALS . REPLY (1992) (0)
GSM1278149: serum_sALS patient_5 (2014) (0)
Erratum to: The epidemiology of CuZn-SOD mutations in Germany: a study of 217 families (2010) (0)
Association of Insulin-like Growth Factor 1 Concentrations with Risk for and Prognosis of Amyotrophic Lateral Sclerosis – Results from the ALS Registry Swabia (2020) (0)
GSM1278135: serum_ALS mutation carrier_3 (2014) (0)
V13. Alterations of ‘resting-state’ functional connectivity in cognitively unimpaired and impaired patients with Parkinson’s disease (2015) (0)
Profilin 1 with the amyotrophic lateral sclerosis associated mutation T109M displays unaltered actin binding and does not affect the actin cytoskeleton (2015) (0)
Patient-centred decision support tools for ALS/MND (2015) (0)
Cross-sectional clinical description of a large prospective cohort of patients with progressive supranuclear palsy or multiple system atrophy: The NNIPPS project (2003) (0)
Neurodegenerative Diseases: Preface (2005) (0)
Neurodegenerative Diseases: Cerebellar degenerations (2005) (0)
A language-based sum score for the course and therapeutic intervention in primary progressive aphasia (2018) (0)
The involvement of the GGA protein family in BACE transport and APP processing in Alzheimer's disease (2008) (0)
T-cell dysregulation is associated with disease severity in Parkinson’s Disease (2021) (0)
FV 14. Longitudinal recordings of eye movements confirm the model of sequential oculomotor alterations in amyotrophic lateral sclerosis consistent with the neuropathological staging scheme (2016) (0)
FV 9 Diffusion tensor imaging in pure lower motor neuron disease (2017) (0)
Differential effect of ethanol intoxication on peripheral markers of cerebral injury in murine blunt TBI (2020) (0)
A placebo-controlled study to evaluate the safety, tolerability, and preliminary efficacy of BVS857 in patients with spinal and bulbar muscular atrophy (S13.003) (2017) (0)
Subject Index Vol. 26, 1987 (1987) (0)
Andersen disease (2019) (0)
Phthisis (1889) (0)
GSM1278128: serum_fALS patient pool 2 (2014) (0)
Commentary: Effects of ALS-associated TANK binding kinase 1 mutations on protein-protein interactions and kinase activity (2020) (0)
Can Pyrimethamine Lower CSF SOD1 Levels in Familial ALS? Results from a Multicenter Phase Ib Trial (S43.007) (2016) (0)
Biomarkers for axonal damage in amyotrophic lateral sclerosis indicate upper motor neuron involvement and correlate with disease progression (2005) (0)
GSM1278143: serum_ALS mutation carrier_11 (2014) (0)
GSM1278125: serum_healthy control_16 (2014) (0)
Clinical Trials in Spinal and Bulbar Muscular Atrophy—Past, Present, and Future (2015) (0)
GSM1278111: serum_healthy control_2 (2014) (0)
Correction to: MR‑imaging pattern is not a predictor of occult atrial fibrillation in patients with cryptogenic stroke (2020) (0)
GSM1278136: serum_ALS mutation carrier_4 (2014) (0)
Correspondence (reply): In Reply. (2008) (0)
Etiology, Pathology, and Pathogenesis (2010) (0)
Joint genome-wide association study of progressive supranuclear palsy identifies novel susceptibility loci and genetic correlation to neurodegenerative diseases (2018) (0)
Therapeutic Response to Phenytoin in a Patient Suffering from Kleine-Levin Syndrome (1987) (0)
Serotonergic neurotoxicity — the example MDMA (2005) (0)
GSM1278127: serum_fALS patient pool 1 (2014) (0)
Comparison of whole retinal thickness and single layer analysis of optical coherence tomography in Multiple System Atrophy and Parkinson's Disease (2013) (0)
THE COTTONSEED PIGMENT GOSSYPOL: A CHRONIC NEUROTOXIN IN THE CAT (1986) (0)
Longitudinal 1H MRS in advanced amyotrophic lateral sclerosis (2006) (0)
Handbook of Experimental Neurology: Experimental models of motor neuron disease/amyotrophic lateral sclerosis (2006) (0)
[Methodology and normal values for the recording of evoked muscle potentials following stimulation over motor roots]. (1988) (0)
Editorial: Amyotrophic lateral sclerosis. (2022) (0)
“Reporting Biomarkers” in ALS: Experiences from a Pilot Compassionate Use Program Applying G-CSF - Mobilized Hematopoietic Stem Cells in ALS-Patients (P1.082) (2014) (0)
GSM1278140: serum_ALS mutation carrier_8 (2014) (0)
GSM1278137: serum_ALS mutation carrier_5 (2014) (0)
EP 50. Correlation between oculomotor changes and atlas-based volumetry in patients with different neurodegenerative Parkinson syndromes (2016) (0)
Identification of candidate genes in patients with amyotrophic lateral sclerosis by breakpoint characterisation (2005) (0)
Cognition in the course of ALS—a meta-analysis (2022) (0)
Incidence of Syndromes Associated With Frontotemporal Lobar Degeneration in 9 European Countries (2023) (0)
University of Groningen Investigation of common , low-frequency and rare genome-wide variation in anorexia nervosa Kas (2017) (0)
1.3 Proteinaggregation und Neurodegeneration (2004) (0)
GSM1278119: serum_healthy control_10 (2014) (0)
Digital Neurology Platform: Developing and implementing a rigorous content quality guideline. (2021) (0)
Aortic Root to Right Heart Shunts (2009) (0)
Health-Related Quality of Life in Spinal Muscular Atrophy Patients and Their Caregivers—A Prospective, Cross-Sectional, Multi-Center Analysis (2023) (0)
The quality of life and depression in primary caregivers of patients with amyotrophic lateral sclerosis is affected by patient-related and culture-specific conditions (2022) (0)
Protease-resistant SOD1 aggregates in amyotrophic lateral sclerosis demonstrated by paraffin-embedded tissue (PET) blot (2014) (0)
Voxel-based morphometry in primary progressive aphasia and its subtypes–data from the multicentric FTLD consortium’s study Germany (2014) (0)
Factors influencing atrophy progression in primary progressive aphasia (2021) (0)
P116 TAUROS: A clinical trial on Progressive Supranuclear Palsy with the GSK-3 inhibitor Tideglusib (Zentylor®) (2011) (0)
Contents Vol. 14, 2014 (2015) (0)
1 NF-κ B is required for Smac mimetic-mediated sensitization of glioblastoma cells for γ-irradiation-induced apoptosis 1 (2011) (0)
Therapeutische Konzepte bei der Amyotrophen Lateral Sklerose (ALS) (2013) (0)
Age-related changes in tau protein isoform expression in mice brains. (2006) (0)
Huntington's Disease: Unraveling the Pathophysiological Cascade Behind a Singular Gene Defect (2007) (0)
P19. The brain’s default mode is associated with executive oculomotor deficits in Parkinson’s disease (2015) (0)
Die Rolle der Elektronystagmographie in der frühen Differentialdiagnose der Parkinson-Syndrome (2005) (0)
GSM1278162: serum_sALS patient_18 (2014) (0)
European-Huntington-Disease-Initiative (EHDI)-Study: Effect of riluzole on disease progression (2005) (0)
GSM1278155: serum_sALS patient_11 (2014) (0)
Increased NF-L levels in the TDP-43G298S ALS mouse model resemble NF-L levels in ALS patients (2022) (0)
ID 231 – Multicentric structural connectome analysis in 240 patients with amyotrophic lateral sclerosis (2016) (0)
P01.056 Tumor Treating Fields therapy in a newly diagnosed glioblastoma patient with multiple sclerosis (2018) (0)
Oral session 21—Motor neuron disease (1) (2005) (0)
The Functional Anatomy of Bimanual Coordination - fMRI Analysis with a New Fast and User-Independent Software Tool (2001) (0)
GSM1278118: serum_healthy control_9 (2014) (0)
Clinical Manifestations and Mechanisms of Action of Environmental Mitochondrial Toxins (2000) (0)
Ludolph Metabolism in Presymptomatic Mutation Carriers of familial Amyotrophic Lateral Sclerosis (2019) (0)
O4-06-02 TDP-43 AND BETA-AMYLOID PRECURSOR PROTEIN PROCESSING PRODUCTS IN CEREBROSPINAL FLUID OF PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS AND FRONTOTEMPORAL LOBAR DEGENERATION (0)
Stages of oculomotor dysfunctions confirms the model of axonal spread of pTDP-43 pathology in amyotrophic lateral sclerosis (2016) (0)
Neurodegenerative Diseases: List of contributors (2005) (0)
Distinct patterns of disease progression in SOD1-associated familial ALS: A study of 25 mutations. Pooled analysis of two trials of pyrimethamine in familial ALS. (S3.005) (2017) (0)
Dysregulation of a novel miR-1825/TBCB/TUBA4A pathway in sporadic and familial ALS (2018) (0)
Motor speech disorders in the nonfluent, semantic and logopenic variants of primary progressive aphasia. Supplementary data: Motor speech characterization per participant (2021) (0)
GSM1278151: serum_sALS patient_7 (2014) (0)
Morphological alterations of the hypothalamus in idiopathic intracranial hypertension (2022) (0)
GSM1278129: serum_fALS patient_3 (2014) (0)
Impaired DNA damage response signaling by FUS-NLS mutations leads to neurodegeneration and FUS aggregate formation (2018) (0)
FV8. Sequential pathology spread in amyotrophic lateral sclerosis: In-vivo evidence from a systematic meta-analysis of structural brain data (2018) (0)
A multimodal imaging approach for phenotyping of dynein heavy chain mutant mice Cra 1 using MRI and PET / CT (2009) (0)
SEPs and Evoked Muscle Responses After Noninvasive Magnetic Stimulation in Patients with Syringomyelia (1990) (0)
GSM1278145: serum_sALS patient_1 (2014) (0)
In vivo amyloid imaging with the new radiotracer [N-Methyl-11C]2-(4'-(methylamino)phenyl)-benzothiazol (C-11-BTA-1) in Alzheimer's disease (2006) (0)
Shank2 expression identifies a subpopulation of glycinergic interneurons involved in nociception and altered in an autism mouse model (2020) (0)
BRAIN LETTER TO THE EDITOR Reply: Two novel mutations in conserved codons indicate that CHCHD10 is a gene associated with motor neuron disease (2014) (0)
ALS - A multisystem degeneration (2017) (0)
P 33. Investigation of microstructural alterations of ALS-specific tracts in asymptomatic ALS-mutation carriers (2021) (0)
GSM1278139: serum_ALS mutation carrier pool 3 (2014) (0)
GSM1278157: serum_sALS patient_13 (2014) (0)

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What Schools Are Affiliated With Albert Christian Ludolph?

Albert Christian Ludolph is affiliated with the following schools:

University of Ulm
University of Cologne
Albert Einstein College of Medicine

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Albert Christian Ludolph's Published Works

Published Works

What Schools Are Affiliated With Albert Christian Ludolph?

Albert Christian Ludolph's AcademicInfluence.com Rankings