Biomarkers for C9orf7-ALS in Symptomatic and Pre-symptomatic Patients: State-of-the-art in the New Era of Clinical Trials

G Querin, MG Biferi, PF Pradat - Journal of neuromuscular …, 2022 - content.iospress.com
The development of new possible treatments for C9orf72-related ALS and the possibility of
early identification of subjects genetically at risk of developing the disease is creating a …

Primary lateral sclerosis: consensus diagnostic criteria

MR Turner, RJ Barohn, P Corcia, JK Fink… - Journal of Neurology …, 2020 - jnnp.bmj.com
Primary lateral sclerosis (PLS) is a neurodegenerative disorder of the adult motor system.
Characterised by a slowly progressive upper motor neuron syndrome, the diagnosis is …

[HTML][HTML] Neuroimaging in genetic frontotemporal dementia and amyotrophic lateral sclerosis

S Häkkinen, SA Chu, SE Lee - Neurobiology of disease, 2020 - Elsevier
Frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) have a strong
clinical, genetic and pathological overlap. This review focuses on the current understanding …

Propagation patterns in motor neuron diseases: Individual and phenotype-associated disease-burden trajectories across the UMN-LMN spectrum of MNDs

M Tahedl, SLH Shing, E Finegan, RH Chipika… - Neurobiology of …, 2022 - Elsevier
Motor neuron diseases encompass a divergent group of conditions with considerable
differences in clinical manifestations, survival, and genetic vulnerability. One of the key …

Astrocyte regional diversity in ALS includes distinct aberrant phenotypes with common and causal pathological processes

C Gomes, C Sequeira, M Barbosa, C Cunha… - Experimental Cell …, 2020 - Elsevier
Astrocytes are major contributors of motor neuron (MN) degeneration in amyotrophic lateral
sclerosis (ALS). We investigated whether regional and cell maturation differences influence …

Promises and pitfalls of imaging-based biomarkers in motor neuron diseases

EL Tan, P Bede, PF Pradat - Current Opinion in Neurology, 2023 - journals.lww.com
Although we acknowledge the academic achievements of large descriptive studies, an
unmet priority of neuroimaging in MND is the development of robust diagnostic, prognostic …

Biomarkers in amyotrophic lateral sclerosis: A review of new developments

N Verber, PJ Shaw - Current Opinion in Neurology, 2020 - journals.lww.com
Biomarkers in amyotrophic lateral sclerosis: a review of new... : Current Opinion in
Neurology Biomarkers in amyotrophic lateral sclerosis: a review of new developments …

The imaging signature of C9orf72 hexanucleotide repeat expansions: Implications for clinical trials and therapy development

S Li Hi Shing, MC McKenna, WF Siah… - Brain Imaging and …, 2021 - Springer
While C9orf72-specific imaging signatures have been proposed by both ALS and FTD
research groups and considerable presymptomatic alterations have also been confirmed in …

Cervical spinal cord atrophy in amyotrophic lateral sclerosis across disease stages

A Nigri, E Dalla Bella, S Ferraro… - Annals of Clinical …, 2023 - Wiley Online Library
Objective Spinal cord degeneration is a hallmark of amyotrophic lateral sclerosis. The
assessment of gray matter and white matter cervical spinal cord atrophy across clinical …

Association between brain and upper cervical spinal cord atrophy assessed by MRI and disease aggressiveness in amyotrophic lateral sclerosis

MM El Mendili, A Verschueren, JP Ranjeva, M Guye… - Neuroradiology, 2023 - Springer
Purpose To study the relative contributions of brain and upper cervical spinal cord
compartmental atrophy to disease aggressiveness in amyotrophic lateral sclerosis (ALS) …