FDG-PET shows weak correlation between focal motor weakness and brain metabolic alterations in ALS

S Sennfält, M Pagani, F Fang, I Savitcheva… - … Lateral Sclerosis and …, 2023 - Taylor & Francis
Objective: Amyotrophic lateral sclerosis (ALS) is a clinically heterogenous disease, typically
presenting with focal motor weakness that eventually generalizes. Weather there is a …

[HTML][HTML] The two-year progression of structural and functional cerebral MRI in amyotrophic lateral sclerosis

RAL Menke, M Proudfoot, K Talbot, MR Turner - NeuroImage: Clinical, 2018 - Elsevier
MRI has emerged as one of several urgently needed candidate disease progression
biomarkers for the neurodegenerative disorder amyotrophic lateral sclerosis (ALS), not least …

Multiparametric MRI study of ALS stratified for the C9orf72 genotype

P Bede, ALW Bokde, S Byrne, M Elamin… - Neurology, 2013 - AAN Enterprises
Objective: To describe the patterns of cortical and subcortical changes in amyotrophic lateral
sclerosis (ALS) stratified for the C9orf72 genotype. Methods: A prospective, single-center …

Brainstem–cortex disconnection in amyotrophic lateral sclerosis: bulbar impairment, genotype associations, asymptomatic changes and biomarker opportunities

M Tahedl, EL Tan, RH Chipika, JC Hengeveld… - Journal of …, 2023 - Springer
Background Bulbar dysfunction is a cardinal feature of ALS with important quality of life and
management implications. The objective of this study is the longitudinal evaluation of a large …

Decreased thickness of primary motor cortex in primary lateral sclerosis

JA Butman, MK Floeter - American journal of …, 2007 - Am Soc Neuroradiology
BACKGROUND AND PURPOSE: Primary lateral sclerosis (PLS) is a rare form of motor
neuron disease characterized by upper motor neuron dysfunction. Because pathologic …

Grey matter correlates of clinical variables in amyotrophic lateral sclerosis (ALS): a neuroimaging study of ALS motor phenotype heterogeneity and cortical focality

P Bede, A Bokde, M Elamin, S Byrne… - Journal of Neurology …, 2013 - jnnp.bmj.com
Background Body region of onset and functional disability are key components of disease
heterogeneity in amyotrophic lateral sclerosis (ALS). Objectives To evaluate patterns of grey …

A novel ALS2 splice-site mutation in a Cypriot juvenile-onset primary lateral sclerosis family

N Mintchev, E Zamba-Papanicolaou, KA Kleopa… - Neurology, 2009 - AAN Enterprises
Background: Primary lateral sclerosis (PLS) is a rare neurodegenerative disease that affects
the upper motor neurons of the CNS. Juvenile-onset PLS (JPLS) is inherited in an …

Cortical function in amyotrophic lateral sclerosis: a positron emission tomography study

JJM Kew, PN Leigh, ED Playford, RE Passingham… - Brain, 1993 - academic.oup.com
Positron emission tomography was used to measure regional cerebral blood flow (rCBF) in
12 patients with amyotrophic lateral sclerosis (ALS) and six age-matched controls. Scans …

Clinical phenomenology and neuroimaging correlates in ALS-FTD

C Lomen-Hoerth - Journal of Molecular Neuroscience, 2011 - Springer
The overlap of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) has
been well documented in FTD patients with co-morbid motor neuron degeneration and in …

Molecular pathology of ALS: what we currently know and what important information is still missing

N Jankovska, R Matej - Diagnostics, 2021 - mdpi.com
Despite an early understanding of amyotrophic lateral sclerosis (ALS) as a disease affecting
the motor system, including motoneurons in the motor cortex, brainstem, and spinal cord …