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In Vivo imaging reveals distinct inflammatory activity of CNS microglia versus PNS macrophages in a mouse model for ALS. PLoS One (2011) 1.10

Multi-parametric spinal cord MRI as potential progression marker in amyotrophic lateral sclerosis. PLoS One (2014) 1.01

Parkinson's disease-like midbrain hyperechogenicity is frequent in amyotrophic lateral sclerosis. J Neurol (2012) 0.81

Patterns of cerebral and cerebellar white matter degeneration in ALS. J Neurol Neurosurg Psychiatry (2014) 0.81

Neuroimaging to investigate multisystem involvement and provide biomarkers in amyotrophic lateral sclerosis. Biomed Res Int (2014) 0.77

Disabling Parkinsonism following brief exposure to lithium carbonate in amyotrophic lateral sclerosis. J Neurol (2010) 0.76

TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis. Nat Genet (2008) 9.51

Genome-wide association study identifies 19p13.3 (UNC13A) and 9p21.2 as susceptibility loci for sporadic amyotrophic lateral sclerosis. Nat Genet (2009) 4.38

Chromosome 9p21 in sporadic amyotrophic lateral sclerosis in the UK and seven other countries: a genome-wide association study. Lancet Neurol (2010) 3.61

Three families with amyotrophic lateral sclerosis and frontotemporal dementia with evidence of linkage to chromosome 9p. Arch Neurol (2007) 2.89

Muscle Nogo-A expression is a prognostic marker in lower motor neuron syndromes. Ann Neurol (2007) 2.37

TARDBP mutations in motoneuron disease with frontotemporal lobar degeneration. Ann Neurol (2009) 2.30

Mutations in the profilin 1 gene cause familial amyotrophic lateral sclerosis. Nature (2012) 2.08

Reduced expression of the Kinesin-Associated Protein 3 (KIFAP3) gene increases survival in sporadic amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A (2009) 2.03

SOD1, ANG, VAPB, TARDBP, and FUS mutations in familial amyotrophic lateral sclerosis: genotype-phenotype correlations. J Med Genet (2010) 1.85

Phenotype variability in progranulin mutation carriers: a clinical, neuropsychological, imaging and genetic study. Brain (2008) 1.84

Progranulin null mutations in both sporadic and familial frontotemporal dementia. Hum Mutat (2007) 1.69

A novel locus for familial amyotrophic lateral sclerosis, on chromosome 18q. Am J Hum Genet (2001) 1.65

Diaphragm pacing improves sleep in patients with amyotrophic lateral sclerosis. Amyotroph Lateral Scler (2011) 1.58

Association of chorea and motor neuron disease. Mov Disord (2002) 1.43

Mutations of the ANG gene in French patients with sporadic amyotrophic lateral sclerosis. Arch Neurol (2008) 1.39

Gene profiling of skeletal muscle in an amyotrophic lateral sclerosis mouse model. Physiol Genomics (2007) 1.31

Neural correlates of cognitive impairment in posterior cortical atrophy. Brain (2011) 1.30

Cognitive impairment in patients with multiple system atrophy and progressive supranuclear palsy. Brain (2010) 1.30

Guidelines for the preclinical in vivo evaluation of pharmacological active drugs for ALS/MND: report on the 142nd ENMC international workshop. Amyotroph Lateral Scler (2007) 1.27

Diffusion tensor imaging and voxel based morphometry study in amyotrophic lateral sclerosis: relationships with motor disability. J Neurol Neurosurg Psychiatry (2007) 1.24

Phase 1 trial of a CpG oligodeoxynucleotide for patients with recurrent glioblastoma. Neuro Oncol (2006) 1.23

Impaired glucose tolerance in patients with amyotrophic lateral sclerosis. Amyotroph Lateral Scler (2010) 1.20

Mutations other than null mutations producing a pathogenic loss of progranulin in frontotemporal dementia. Hum Mutat (2007) 1.20

Phenotype difference between ALS patients with expanded repeats in C9ORF72 and patients with mutations in other ALS-related genes. J Med Genet (2012) 1.19

Quantitative simultaneous (99m)Tc-ECD/123I-FP-CIT SPECT in Parkinson's disease and multiple system atrophy. Eur J Nucl Med Mol Imaging (2005) 1.18

Glutamate levels in cerebrospinal fluid in amyotrophic lateral sclerosis: a reappraisal using a new HPLC method with coulometric detection in a large cohort of patients. J Neurol Sci (2002) 1.18

Nogo provides a molecular marker for diagnosis of amyotrophic lateral sclerosis. Neurobiol Dis (2002) 1.17

Mutations in SQSTM1 encoding p62 in amyotrophic lateral sclerosis: genetics and neuropathology. Acta Neuropathol (2013) 1.16

C9ORF72 repeat expansions in the frontotemporal dementias spectrum of diseases: a flow-chart for genetic testing. J Alzheimers Dis (2013) 1.16

Disease severity and progression in progressive supranuclear palsy and multiple system atrophy: validation of the NNIPPS--Parkinson Plus Scale. PLoS One (2011) 1.15

Efficacy and safety of xaliproden in amyotrophic lateral sclerosis: results of two phase III trials. Amyotroph Lateral Scler Other Motor Neuron Disord (2004) 1.15

Progression in ALS is not linear but is curvilinear. J Neurol (2010) 1.14

Association of long ATXN2 CAG repeat sizes with increased risk of amyotrophic lateral sclerosis. Arch Neurol (2011) 1.13

Clinical outcome in 19 French and Spanish patients with valosin-containing protein myopathy associated with Paget's disease of bone and frontotemporal dementia. Neuromuscul Disord (2009) 1.12

Accumulation of TDP-43 and alpha-actin in an amyotrophic lateral sclerosis patient with the K17I ANG mutation. Acta Neuropathol (2009) 1.12

Up-regulation of mitochondrial uncoupling protein 3 reveals an early muscular metabolic defect in amyotrophic lateral sclerosis. FASEB J (2003) 1.11

Nogo expression in muscle correlates with amyotrophic lateral sclerosis severity. Ann Neurol (2005) 1.11

Increased peripheral lipid clearance in an animal model of amyotrophic lateral sclerosis. J Lipid Res (2007) 1.09

A frameshift deletion in peripherin gene associated with amyotrophic lateral sclerosis. J Biol Chem (2004) 1.08

Demographic, neurological and behavioural characteristics and brain perfusion SPECT in frontal variant of frontotemporal dementia. Brain (2006) 1.08

Memantine in behavioral variant frontotemporal dementia: negative results. J Alzheimers Dis (2011) 1.07

Implications for the kynurenine pathway and quinolinic acid in amyotrophic lateral sclerosis. Neurodegener Dis (2005) 1.04

Age of onset of amyotrophic lateral sclerosis is modulated by a locus on 1p34.1. Neurobiol Aging (2012) 1.02

FUS mutations in frontotemporal lobar degeneration with amyotrophic lateral sclerosis. J Alzheimers Dis (2010) 1.02

Development of a French isometric strength normative database for adults using quantitative muscle testing. Arch Phys Med Rehabil (2007) 1.02

UBQLN2 mutations are rare in French and French-Canadian amyotrophic lateral sclerosis. Neurobiol Aging (2012) 1.01

Chromogranin B P413L variant as risk factor and modifier of disease onset for amyotrophic lateral sclerosis. Proc Natl Acad Sci U S A (2009) 1.01

Amyotrophic lateral sclerosis: all roads lead to Rome. J Neurochem (2007) 0.99

Involvement of spinal sensory pathway in ALS and specificity of cord atrophy to lower motor neuron degeneration. Amyotroph Lateral Scler Frontotemporal Degener (2012) 0.99

A genome-wide association meta-analysis identifies a novel locus at 17q11.2 associated with sporadic amyotrophic lateral sclerosis. Hum Mol Genet (2013) 0.98

Amyotrophic lateral sclerosis with neuronal intranuclear protein inclusions. Acta Neuropathol (2004) 0.96

Muscle histone deacetylase 4 upregulation in amyotrophic lateral sclerosis: potential role in reinnervation ability and disease progression. Brain (2013) 0.96

Mapping of gene expression reveals CYP27A1 as a susceptibility gene for sporadic ALS. PLoS One (2012) 0.95

Abnormalities of satellite cells function in amyotrophic lateral sclerosis. Amyotroph Lateral Scler (2011) 0.95

Amyotrophic lateral sclerosis: a consensus viewpoint on designing and implementing a clinical trial. Amyotroph Lateral Scler Other Motor Neuron Disord (2004) 0.95

Causes of death in a post-mortem series of ALS patients. Amyotroph Lateral Scler (2008) 0.94