Bookmarks
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
TERMINOLOGY
Abbreviations
- Amyotrophic lateral sclerosis (ALS)
Synonyms
- Lou Gehrig disease, motor neuron disease (MND)
Definitions
- Selective degeneration of somatic motor neurons of brainstem/spinal cord {lower motor neurons (LMN) & large pyramidal neurons of motor cortex [upper motor neurons (UMN)]}
- Eventual loss of corticospinal tract (CST) fibers
- Selective degeneration of somatic motor neurons of brainstem/spinal cord {lower motor neurons (LMN) & large pyramidal neurons of motor cortex [upper motor neurons (UMN)]}
IMAGING
General Features
CT Findings
MR Findings
Nuclear Medicine Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
- Braun N et al: The revised El Escorial criteria "clinically probable laboratory supported ALS"-once a promising now a superfluous category? Amyotroph Lateral Scler Frontotemporal Degener. 1-5, 2019
- Lai JD et al: C9ORF72 protein function and immune dysregulation in amyotrophic lateral sclerosis. Neurosci Lett. 134523, 2019
- Barthel H et al: PET/MR in dementia and other neurodegenerative diseases. Semin Nucl Med. 45(3):224-33, 2015
- Goveas J et al: Diffusion-MRI in neurodegenerative disorders. Magn Reson Imaging. 33(7):853-76, 2015
- Verstraete E et al: Neuroimaging as a new diagnostic modality in amyotrophic lateral sclerosis. Neurotherapeutics. 12(2):403-16, 2015
- Chiò A et al: Neuroimaging in amyotrophic lateral sclerosis: insights into structural and functional changes. Lancet Neurol. 13(12):1228-40, 2014
- Vucic S et al: Advances in treating amyotrophic lateral sclerosis: insights from pathophysiological studies. Trends Neurosci. 37(8):433-42, 2014
- Wang S et al: Neuroimaging in amyotrophic lateral sclerosis. Neurotherapeutics. 8(1):63-71, 2011
- Filippi M et al: EFNS guidelines on the use of neuroimaging in the management of motor neuron diseases. Eur J Neurol. 17(4):526-e20, 2010
- Wijesekera LC et al: Amyotrophic lateral sclerosis. Orphanet J Rare Dis. 4:3, 2009
- Sage CA et al: Quantitative diffusion tensor imaging in amyotrophic lateral sclerosis. Neuroimage. 34(2):486-99, 2007
- Unrath A et al: Brain metabolites in definite amyotrophic lateral sclerosis. A longitudinal proton magnetic resonance spectroscopy study. J Neurol. 254(8):1099-106, 2007
- Kalra S et al: Rapid improvement in cortical neuronal integrity in amyotrophic lateral sclerosis detected by proton magnetic resonance spectroscopic imaging. J Neurol. 253(8):1060-3, 2006
- Cosottini M et al: Diffusion-tensor MR imaging of corticospinal tract in amyotrophic lateral sclerosis and progressive muscular atrophy. Radiology. 237(1):258-64, 2005
- Sach M et al: Diffusion tensor MRI of early upper motor neuron involvement in amyotrophic lateral sclerosis. Brain. 127(Pt 2):340-50, 2004
- Kalra S et al: Gabapentin therapy for amyotrophic lateral sclerosis: lack of improvement in neuronal integrity shown by MR spectroscopy. AJNR Am J Neuroradiol. 24(3):476-80, 2003
- Kalra S et al: Neuroimaging in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord. 4(4):243-8, 2003
- Strong M et al: Amyotrophic lateral sclerosis: a review of current concepts. Amyotroph Lateral Scler Other Motor Neuron Disord. 4(3):136-43, 2003
- Heath PR et al: Update on the glutamatergic neurotransmitter system and the role of excitotoxicity in amyotrophic lateral sclerosis. Muscle Nerve. 26(4):438-58, 2002
- Bowen BC et al: MR imaging and localized proton spectroscopy of the precentral gyrus in amyotrophic lateral sclerosis. AJNR Am J Neuroradiol. 21(4):647-58, 2000
- Chan S et al: Motor neuron diseases: comparison of single-voxel proton MR spectroscopy of the motor cortex with MR imaging of the brain. Radiology. 212(3):763-9, 1999
- Ellis CM et al: Diffusion tensor MRI assesses corticospinal tract damage in ALS. Neurology. 53(5):1051-8, 1999
- Kato Y et al: Detection of pyramidal tract lesions in amyotrophic lateral sclerosis with magnetization-transfer measurements. AJNR Am J Neuroradiol. 18(8):1541-7, 1997
- Mascalchi M et al: Corticospinal tract degeneration in motor neuron disease. AJNR Am J Neuroradiol. 16(4 Suppl):878-80, 1995
Related Anatomy
Loading...
Related Differential Diagnoses
Loading...
References
Tables
Tables
KEY FACTS
Terminology
Imaging
Top Differential Diagnoses
Pathology
Clinical Issues
TERMINOLOGY
Abbreviations
- Amyotrophic lateral sclerosis (ALS)
Synonyms
- Lou Gehrig disease, motor neuron disease (MND)
Definitions
- Selective degeneration of somatic motor neurons of brainstem/spinal cord {lower motor neurons (LMN) & large pyramidal neurons of motor cortex [upper motor neurons (UMN)]}
- Eventual loss of corticospinal tract (CST) fibers
- Selective degeneration of somatic motor neurons of brainstem/spinal cord {lower motor neurons (LMN) & large pyramidal neurons of motor cortex [upper motor neurons (UMN)]}
IMAGING
General Features
CT Findings
MR Findings
Nuclear Medicine Findings
Imaging Recommendations
DIFFERENTIAL DIAGNOSIS
PATHOLOGY
General Features
Gross Pathologic & Surgical Features
Microscopic Features
CLINICAL ISSUES
Presentation
Demographics
Natural History & Prognosis
Treatment
DIAGNOSTIC CHECKLIST
Consider
Image Interpretation Pearls
Selected References
- Braun N et al: The revised El Escorial criteria "clinically probable laboratory supported ALS"-once a promising now a superfluous category? Amyotroph Lateral Scler Frontotemporal Degener. 1-5, 2019
- Lai JD et al: C9ORF72 protein function and immune dysregulation in amyotrophic lateral sclerosis. Neurosci Lett. 134523, 2019
- Barthel H et al: PET/MR in dementia and other neurodegenerative diseases. Semin Nucl Med. 45(3):224-33, 2015
- Goveas J et al: Diffusion-MRI in neurodegenerative disorders. Magn Reson Imaging. 33(7):853-76, 2015
- Verstraete E et al: Neuroimaging as a new diagnostic modality in amyotrophic lateral sclerosis. Neurotherapeutics. 12(2):403-16, 2015
- Chiò A et al: Neuroimaging in amyotrophic lateral sclerosis: insights into structural and functional changes. Lancet Neurol. 13(12):1228-40, 2014
- Vucic S et al: Advances in treating amyotrophic lateral sclerosis: insights from pathophysiological studies. Trends Neurosci. 37(8):433-42, 2014
- Wang S et al: Neuroimaging in amyotrophic lateral sclerosis. Neurotherapeutics. 8(1):63-71, 2011
- Filippi M et al: EFNS guidelines on the use of neuroimaging in the management of motor neuron diseases. Eur J Neurol. 17(4):526-e20, 2010
- Wijesekera LC et al: Amyotrophic lateral sclerosis. Orphanet J Rare Dis. 4:3, 2009
- Sage CA et al: Quantitative diffusion tensor imaging in amyotrophic lateral sclerosis. Neuroimage. 34(2):486-99, 2007
- Unrath A et al: Brain metabolites in definite amyotrophic lateral sclerosis. A longitudinal proton magnetic resonance spectroscopy study. J Neurol. 254(8):1099-106, 2007
- Kalra S et al: Rapid improvement in cortical neuronal integrity in amyotrophic lateral sclerosis detected by proton magnetic resonance spectroscopic imaging. J Neurol. 253(8):1060-3, 2006
- Cosottini M et al: Diffusion-tensor MR imaging of corticospinal tract in amyotrophic lateral sclerosis and progressive muscular atrophy. Radiology. 237(1):258-64, 2005
- Sach M et al: Diffusion tensor MRI of early upper motor neuron involvement in amyotrophic lateral sclerosis. Brain. 127(Pt 2):340-50, 2004
- Kalra S et al: Gabapentin therapy for amyotrophic lateral sclerosis: lack of improvement in neuronal integrity shown by MR spectroscopy. AJNR Am J Neuroradiol. 24(3):476-80, 2003
- Kalra S et al: Neuroimaging in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord. 4(4):243-8, 2003
- Strong M et al: Amyotrophic lateral sclerosis: a review of current concepts. Amyotroph Lateral Scler Other Motor Neuron Disord. 4(3):136-43, 2003
- Heath PR et al: Update on the glutamatergic neurotransmitter system and the role of excitotoxicity in amyotrophic lateral sclerosis. Muscle Nerve. 26(4):438-58, 2002
- Bowen BC et al: MR imaging and localized proton spectroscopy of the precentral gyrus in amyotrophic lateral sclerosis. AJNR Am J Neuroradiol. 21(4):647-58, 2000
- Chan S et al: Motor neuron diseases: comparison of single-voxel proton MR spectroscopy of the motor cortex with MR imaging of the brain. Radiology. 212(3):763-9, 1999
- Ellis CM et al: Diffusion tensor MRI assesses corticospinal tract damage in ALS. Neurology. 53(5):1051-8, 1999
- Kato Y et al: Detection of pyramidal tract lesions in amyotrophic lateral sclerosis with magnetization-transfer measurements. AJNR Am J Neuroradiol. 18(8):1541-7, 1997
- Mascalchi M et al: Corticospinal tract degeneration in motor neuron disease. AJNR Am J Neuroradiol. 16(4 Suppl):878-80, 1995
