· To familiarise the non-expert radiologist with ALS.
· To highlight imaging findings according to modality,
especially in everyday practice.
· To accentuate the role of imaging in ALS.
Amyotrophic lateral sclerosis (ALS; also known as motor neuron disease or Lou Gehrig’s disease) is a widely heterogeneous multisystem neurodegenerative syndrome,
which affects both the upper and lower motor neurons with a welter of pathophysiological mechanisms and multiple clinical phenotypes.
To add to the complexity of the entity,
an overlap between ALS and frontotemporal dementia has been suggested,
attributed to a common genetic cause (hexanucleotide repeat expansions in C9orf72),
forming a probable continuum of a single multispectral disease.
Special attention should be given to ALS...
Findings and procedure details
Six (6) patients with progressive motor deficits presented to our Institution’s Imaging Department with a working diagnosis of ALS,
according to the revisited criteria El Escorial and Awaji.
All patients underwent conventional MRI (3 Tesla) of the brain and cervical spine and,
according to findings,
further DTI tractography was performed.
The first ever abnormality described in imaging was high signal intensity along the CST from the centrum semiovale to the brain stem,
on T2-weighted images (T2W),
including fluid attenuated inversion recovery (FLAIR) images,
ALS is a paralytic disorder characterised by degeneration of motor neurons in the brain and spinal cord with extreme phenotypic heterogeneity.
Imaging findings have been indicative but not conclusive of ALS and clinical correlations have been limited until recent years.
Even though they are not pathognomonic,
familiarity of the radiologist with findings in conventional MRI along with more state-of-the-art methods is mandatory.
The emergence of advanced neuroimaging techniques (DTI,
high field MRI,
Proton magnetic resonance spectroscopy etc.) has shed new light...
 van Es,
& van den Berg,
(2017). Amyotrophic lateral sclerosis.The Lancet.
Amyotrophic lateral sclerosis.New England Journal of Medicine,377(2),
What does imaging reveal about the pathology of amyotrophic lateral sclerosis?.Current neurology and neuroscience reports,15(7),