Clinical Spectrum of Amyotrophic Lateral Sclerosis (ALS)

Prion Properties of SOD1 in Amyotrophic Lateral Sclerosis and Potential Therapy

(2017) Caroline Sibilla et al.   Cold Spring Harbor Perspectives in Biology

Amyotrophic lateral sclerosis: Is the spinal fluid pathway involved in seeding and spread?

(2015) Richard Smith et al.   MEDICAL HYPOTHESES

From molecule to molecule and cell to cell: Prion-like mechanisms in amyotrophic lateral sclerosis

(2015) Leslie I. Grad et al.   NEUROBIOLOGY OF DISEASE

From nucleation to widespread propagation: A prion-like concept for ALS

(2015) Zuzanna Maniecka et al.   VIRUS RESEARCH

TDP-43 pathology and neuronal loss in amyotrophic lateral sclerosis spinal cord

(2014) Johannes Brettschneider et al.   ACTA NEUROPATHOLOGICA

Focality, stochasticity and neuroanatomic propagation in ALS pathogenesis

(2014) John Ravits   EXPERIMENTAL NEUROLOGY

Intercellular propagated misfolding of wild-type Cu/Zn superoxide dismutase occurs via exosome-dependent and -independent mechanisms

(2014) Leslie I. Grad et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Stages of pTDP-43 pathology in amyotrophic lateral sclerosis

(2013) Johannes Brettschneider et al.   ANNALS OF NEUROLOGY

Spreading of amyotrophic lateral sclerosis lesions--multifocal hits and local propagation?

(2013) T. Sekiguchi et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

State of play in amyotrophic lateral sclerosis genetics

(2013) Alan E Renton et al.   NATURE NEUROSCIENCE

Amyotrophic lateral sclerosis—a model of corticofugal axonal spread

(2013) Heiko Braak et al.   Nature Reviews Neurology

Global Epidemiology of Amyotrophic Lateral Sclerosis: A Systematic Review of the Published Literature

(2013) A. Chiò et al.   NEUROEPIDEMIOLOGY

Progressive hemiparesis (Mills syndrome) with aphasia in amyotrophic lateral sclerosis

(2013) D. Baumer et al.   NEUROLOGY

C9ORF72 repeat expansions in cases with previously identified pathogenic mutations

(2013) M. van Blitterswijk et al.   NEUROLOGY

Unconventional Translation of C9ORF72 GGGGCC Expansion Generates Insoluble Polypeptides Specific to c9FTD/ALS

(2013) Peter E.A. Ash et al.   NEURON

RAN proteins and RNA foci from antisense transcripts in C9ORF72 ALS and frontotemporal dementia

(2013) T. Zu et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The C9orf72 GGGGCC Repeat Is Translated into Aggregating Dipeptide-Repeat Proteins in FTLD/ALS

(2013) K. Mori et al.   SCIENCE

Deciphering amyotrophic lateral sclerosis: What phenotype, neuropathology and genetics are telling us about pathogenesis

(2013) John Ravits et al.   Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration

Clinic, neuropathology and molecular genetics of frontotemporal dementia: a mini-review

(2013) Xiao-dong Pan et al.   Translational Neurodegeneration

The genetics and neuropathology of frontotemporal lobar degeneration

(2012) Anne Sieben et al.   ACTA NEUROPATHOLOGICA

Clinical and pathological features of amyotrophic lateral sclerosis caused by mutation in the C9ORF72 gene on chromosome 9p

(2012) Heather Stewart et al.   ACTA NEUROPATHOLOGICA

Young-onset amyotrophic lateral sclerosis: historical and other observations

(2012) M. R. Turner et al.   BRAIN

Regional spread pattern predicts survival in patients with sporadic amyotrophic lateral sclerosis

(2012) G. M. Gargiulo-Monachelli et al.   EUROPEAN JOURNAL OF NEUROLOGY

Can regional spreading of amyotrophic lateral sclerosis motor symptoms be explained by prion-like propagation?

(2012) Tadashi Kanouchi et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Predicting Regional Neurodegeneration from the Healthy Brain Functional Connectome

(2012) Juan Zhou et al.   NEURON

Aberrant Localization of FUS and TDP43 Is Associated with Misfolding of SOD1 in Amyotrophic Lateral Sclerosis

(2012) Edward Pokrishevsky et al.   PLoS One

Motor neuron disease clinically limited to the lower motor neuron is a diffuse TDP-43 proteinopathy

(2011) Felix Geser et al.   ACTA NEUROPATHOLOGICA

p62 positive, TDP-43 negative, neuronal cytoplasmic and intranuclear inclusions in the cerebellum and hippocampus define the pathology of C9orf72-linked FTLD and MND/ALS

(2011) Safa Al-Sarraj et al.   ACTA NEUROPATHOLOGICA

The Seeds of Neurodegeneration: Prion-like Spreading in ALS

(2011) Magdalini Polymenidou et al.   CELL

What Are the Different Initial Presentations of Frontotemporal Dementia?

(2011) Tiffany W. Chow   JOURNAL OF MOLECULAR NEUROSCIENCE

Onset and spreading patterns of lower motor neuron involvements predict survival in sporadic amyotrophic lateral sclerosis

(2011) C. Fujimura-Kiyono et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Phenotypic heterogeneity of amyotrophic lateral sclerosis: a population based study

(2011) A. Chio et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Generalization of the Prion Hypothesis to Other Neurodegenerative Diseases: An Imperfect Fit

(2011) Will C. Guest et al.   JOURNAL OF TOXICOLOGY AND ENVIRONMENTAL HEALTH-PART A-CURRENT ISSUES

Neuroinflammation in amyotrophic lateral sclerosis: role of glial activation in motor neuron disease

(2011) Thomas Philips et al.   LANCET NEUROLOGY

Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia

(2011) Han-Xiang Deng et al.   NATURE

Astrocytes from familial and sporadic ALS patients are toxic to motor neurons

(2011) Amanda M Haidet-Phillips et al.   NATURE BIOTECHNOLOGY

Expanded GGGGCC Hexanucleotide Repeat in Noncoding Region of C9ORF72 Causes Chromosome 9p-Linked FTD and ALS

(2011) Mariely DeJesus-Hernandez et al.   NEURON

A Hexanucleotide Repeat Expansion in C9ORF72 Is the Cause of Chromosome 9p21-Linked ALS-FTD

(2011) Alan E. Renton et al.   NEURON

An MND/ALS phenotype associated withC9orf72repeat expansion: Abundant p62-positive, TDP-43-negative inclusions in cerebral cortex, hippocampus and cerebellum but without associated cognitive decline

(2011) Claire Troakes et al.   NEUROPATHOLOGY

Primary lateral sclerosis: Upper-motor-predominant amyotrophic lateral sclerosis with frontotemporal lobar degeneration - immunohistochemical and biochemical analyses of TDP-43

(2011) Takayuki Kosaka et al.   NEUROPATHOLOGY

Molecular Determinants and Genetic Modifiers of Aggregation and Toxicity for the ALS Disease Protein FUS/TLS

(2011) Zhihui Sun et al.   PLOS BIOLOGY

Updates on amyotrophic lateral sclerosis: Improving patient care

(2010) Walter G. Bradley   ANNALS OF NEUROLOGY

Extensive FUS-Immunoreactive Pathology in Juvenile Amyotrophic Lateral Sclerosis with Basophilic Inclusions

(2010) Eric J. Huang et al.   BRAIN PATHOLOGY

Patterns of cortical activity differ in ALS patients with limb and/or bulbar involvement depending on motor tasks

(2010) Katja Kollewe et al.   JOURNAL OF NEUROLOGY

Eye movements in amyotrophic lateral sclerosis and its mimics: a review with illustrative cases

(2010) C. Donaghy et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

Cognitive dysfunction in lower motor neuron disease: executive and memory deficits in progressive muscular atrophy

(2010) J. Raaphorst et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

The diagnostic pathway and prognosis in bulbar-onset amyotrophic lateral sclerosis

(2010) Martin R. Turner et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

Clinicopathological characteristics of FTLD-TDP showing corticospinal tract degeneration but lacking lower motor neuron loss

(2010) Zen Kobayashi et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

Energy metabolism in amyotrophic lateral sclerosis

(2010) Luc Dupuis et al.   LANCET NEUROLOGY

Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS

(2010) Andrew C. Elden et al.   NATURE

Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS

(2010) Daryl A Bosco et al.   NATURE NEUROSCIENCE

Novel Antibodies Reveal Inclusions Containing Non-Native SOD1 in Sporadic ALS Patients

(2010) Karin Forsberg et al.   PLoS One

Consensus criteria for the diagnosis of frontotemporal cognitive and behavioural syndromes in amyotrophic lateral sclerosis

(2009) Michael J. Strong et al.   Amyotrophic Lateral Sclerosis

Amyotrophic Lateral Sclerosis–Plus Syndrome With TAR DNA-Binding Protein-43 Pathology

(2009) Leo F. McCluskey et al.   ARCHIVES OF NEUROLOGY

Incidence of amyotrophic lateral sclerosis in Europe

(2009) G. Logroscino et al.   JOURNAL OF NEUROLOGY NEUROSURGERY AND PSYCHIATRY

No evidence for cognitive dysfunction or depression in patients with mild restless legs syndrome

(2009) Erika Driver-Dunckley et al.   MOVEMENT DISORDERS

Study of 962 patients indicates progressive muscular atrophy is a form of ALS

(2009) W. -K. Kim et al.   NEUROLOGY

Transcranial magnetic stimulation in ALS: Utility of central motor conduction tests

(2009) A. G. Floyd et al.   NEUROLOGY

Natural history and clinical features of the flail arm and flail leg ALS variants

(2009) L. C. Wijesekera et al.   NEUROLOGY

Clinical features that distinguish PLS, upper motor neuron-dominant ALS, and typical ALS

(2009) P. H. Gordon et al.   NEUROLOGY

ALS motor phenotype heterogeneity, focality, and spread: Deconstructing motor neuron degeneration

(2009) J. M. Ravits et al.   NEUROLOGY

Neurodegenerative Diseases Target Large-Scale Human Brain Networks

(2009) William W. Seeley et al.   NEURON

Latent Cluster Analysis of ALS Phenotypes Identifies Prognostically Differing Groups

(2009) Jeban Ganesalingam et al.   PLoS One

Evidence of Multisystem Disorder in Whole-Brain Map of Pathological TDP-43 in Amyotrophic Lateral Sclerosis

(2008) Felix Geser et al.   ARCHIVES OF NEUROLOGY

Selective functional, regional, and neuronal vulnerability in frontotemporal dementia

(2008) William W Seeley   CURRENT OPINION IN NEUROLOGY

Upper motor neuron and extra-motor neuron involvement in amyotrophic lateral sclerosis: A clinical and brain imaging review

(2008) M.M. van der Graaff et al.   NEUROMUSCULAR DISORDERS

Chorea-ballism associated with familial amyotrophic lateral sclerosis. A clinical, genetic, and neuropathological study

(2007) Josep Gamez et al.   MOVEMENT DISORDERS