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Amyotrophic Lateral Sclerosis (ALS)
Miral D. Jhaveri, MD; Lubdha M. Shah, MD
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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]) and large pyramidal neurons of motor cortex (upper motor neurons [UMN])
                • Eventual loss of corticospinal tract (CST) fibers

            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

                                          1. Barthel H et al: PET/MR in Dementia and Other Neurodegenerative Diseases. Semin Nucl Med. 45(3):224-233, 2015
                                          2. Goveas J et al: Diffusion-MRI in neurodegenerative disorders. Magn Reson Imaging. ePub, 2015
                                          3. Verstraete E et al: Neuroimaging as a new diagnostic modality in amyotrophic lateral sclerosis. Neurotherapeutics. 12(2):403-16, 2015
                                          4. Chiò A et al: Neuroimaging in amyotrophic lateral sclerosis: insights into structural and functional changes. Lancet Neurol. 13(12):1228-40, 2014
                                          5. Vucic S et al: Advances in treating amyotrophic lateral sclerosis: insights from pathophysiological studies. Trends Neurosci. 37(8):433-42, 2014
                                          6. Wang S et al: Neuroimaging in amyotrophic lateral sclerosis. Neurotherapeutics. 8(1):63-71, 2011
                                          7. Wijesekera LC et al: Amyotrophic lateral sclerosis. Orphanet J Rare Dis. 4:3, 2009
                                          8. Sage CA et al: Quantitative diffusion tensor imaging in amyotrophic lateral sclerosis. Neuroimage. 34(2):486-99, 2007
                                          9. 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
                                          10. 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
                                          11. 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
                                          12. Sach M et al: Diffusion tensor MRI of early upper motor neuron involvement in amyotrophic lateral sclerosis. Brain. 127(Pt 2):340-50, 2004
                                          13. 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
                                          14. Kalra S et al: Neuroimaging in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord. 4(4):243-8, 2003
                                          15. Strong M et al: Amyotrophic lateral sclerosis: a review of current concepts. Amyotroph Lateral Scler Other Motor Neuron Disord. 4(3):136-43, 2003
                                          16. 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
                                          17. 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
                                          18. 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
                                          19. Ellis CM et al: Diffusion tensor MRI assesses corticospinal tract damage in ALS. Neurology. 53(5):1051-8, 1999
                                          20. 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
                                          21. Mascalchi M et al: Corticospinal tract degeneration in motor neuron disease. AJNR Am J Neuroradiol. 16(4 Suppl):878-80, 1995
                                          Related Anatomy
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                                          Related Differential Diagnoses
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                                          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]) and large pyramidal neurons of motor cortex (upper motor neurons [UMN])
                                                        • Eventual loss of corticospinal tract (CST) fibers

                                                    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

                                                                                  1. Barthel H et al: PET/MR in Dementia and Other Neurodegenerative Diseases. Semin Nucl Med. 45(3):224-233, 2015
                                                                                  2. Goveas J et al: Diffusion-MRI in neurodegenerative disorders. Magn Reson Imaging. ePub, 2015
                                                                                  3. Verstraete E et al: Neuroimaging as a new diagnostic modality in amyotrophic lateral sclerosis. Neurotherapeutics. 12(2):403-16, 2015
                                                                                  4. Chiò A et al: Neuroimaging in amyotrophic lateral sclerosis: insights into structural and functional changes. Lancet Neurol. 13(12):1228-40, 2014
                                                                                  5. Vucic S et al: Advances in treating amyotrophic lateral sclerosis: insights from pathophysiological studies. Trends Neurosci. 37(8):433-42, 2014
                                                                                  6. Wang S et al: Neuroimaging in amyotrophic lateral sclerosis. Neurotherapeutics. 8(1):63-71, 2011
                                                                                  7. Wijesekera LC et al: Amyotrophic lateral sclerosis. Orphanet J Rare Dis. 4:3, 2009
                                                                                  8. Sage CA et al: Quantitative diffusion tensor imaging in amyotrophic lateral sclerosis. Neuroimage. 34(2):486-99, 2007
                                                                                  9. 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
                                                                                  10. 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
                                                                                  11. 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
                                                                                  12. Sach M et al: Diffusion tensor MRI of early upper motor neuron involvement in amyotrophic lateral sclerosis. Brain. 127(Pt 2):340-50, 2004
                                                                                  13. 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
                                                                                  14. Kalra S et al: Neuroimaging in amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord. 4(4):243-8, 2003
                                                                                  15. Strong M et al: Amyotrophic lateral sclerosis: a review of current concepts. Amyotroph Lateral Scler Other Motor Neuron Disord. 4(3):136-43, 2003
                                                                                  16. 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
                                                                                  17. 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
                                                                                  18. 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
                                                                                  19. Ellis CM et al: Diffusion tensor MRI assesses corticospinal tract damage in ALS. Neurology. 53(5):1051-8, 1999
                                                                                  20. 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
                                                                                  21. Mascalchi M et al: Corticospinal tract degeneration in motor neuron disease. AJNR Am J Neuroradiol. 16(4 Suppl):878-80, 1995