Uncommon Spinal Cord MRI Findings in a Patient With Early-Onset Amyotrophic Lateral Sclerosis

A 26-year-old man presented with an 8-month history of progressive lower limb weakness extending to upper limbs over 3 months, cramps, and a 27 kg weight loss.Examination showed weakness in upper and lower right limbs with signs of upper motor neuron (MN) involvement in bulbar, cervical, and lumbar regions, and signs of lower MN involvement in the cervical region.Brain and spinal MRI were abnormal (Figures 1 and2).Lumbar puncture and blood tests (including immunologic, infectious, and metabolic tests) were normal.Electroneuromyography confirmed lower MN involvement with signs of acute and chronic motor denervation in cervical and thoracic regions, leading to the diagnosis of amyotrophic lateral sclerosis (ALS).Although brain MRI anomalies are well-documented in ALS, 1 spine MRI is usually normal and used to exclude differential diagnoses.T2 hyperintensities in the cervical spinal cord on a 7-Tesla MRI were previously reported. 2Our case confirms that 1.5-Tesla spine MRI can reveal abnormalities which, although rare, are consistent with the diagnosis of ALS.Coronal fluid-attenuated inversion recovery-weighted MR sequence (A) shows bilateral T2 hyperintensities of the corticospinal tracts in the internal capsules (arrows).Axial susceptibility-weighted imaging (B) shows slight loss of signal in the precentral gyrus, predominating on the left side, consistent with iron deposition in the cortex, known as motor band sign (arrow).

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Publication Details

Journal
Neurology
Published
2025-03-04
DOI
https://doi.org/10.1212/wnl.0000000000213503
Primary Topic
Amyotrophic Lateral Sclerosis Research
Type
article
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article

Uncommon Spinal Cord MRI Findings in a Patient With Early-Onset Amyotrophic Lateral Sclerosis

Bruno Law-Ye, Manh‐Louis Nguyen, Andrei Haddad, Adèle Hesters
Neurology
Amyotrophic Lateral Sclerosis Research
article

Uncommon Spinal Cord MRI Findings in a Patient With Early-Onset Amyotrophic Lateral Sclerosis

Bruno Law-Ye, Manh‐Louis Nguyen, Andrei Haddad, Adèle Hesters
article en

Abstract

A 26-year-old man presented with an 8-month history of progressive lower limb weakness extending to upper limbs over 3 months, cramps, and a 27 kg weight loss.Examination showed weakness in upper and lower right limbs with signs of upper motor neuron (MN) involvement in bulbar, cervical, and lumbar regions, and signs of lower MN involvement in the cervical region.Brain and spinal MRI were abnormal (Figures 1 and2).Lumbar puncture and blood tests (including immunologic, infectious, and metabolic tests) were normal.Electroneuromyography confirmed lower MN involvement with signs of acute and chronic motor denervation in cervical and thoracic regions, leading to the diagnosis of amyotrophic lateral sclerosis (ALS).Although brain MRI anomalies are well-documented in ALS, 1 spine MRI is usually normal and used to exclude differential diagnoses.T2 hyperintensities in the cervical spinal cord on a 7-Tesla MRI were previously reported. 2Our case confirms that 1.5-Tesla spine MRI can reveal abnormalities which, although rare, are consistent with the diagnosis of ALS.Coronal fluid-attenuated inversion recovery-weighted MR sequence (A) shows bilateral T2 hyperintensities of the corticospinal tracts in the internal capsules (arrows).Axial susceptibility-weighted imaging (B) shows slight loss of signal in the precentral gyrus, predominating on the left side, consistent with iron deposition in the cortex, known as motor band sign (arrow).

NeurologyVol. 104(7)
Sorbonne Université (FR), Assistance Publique – Hôpitaux de Paris (FR), Pitié-Salpêtrière Hospital (FR)
Openalex Percentile: Top 93%
Amyotrophic Lateral Sclerosis Research
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Uncommon Spinal Cord MRI Findings in a Patient With Early-Onset Amyotrophic Lateral Sclerosis — Bruno Law-Ye, Manh‐Louis Nguyen, et al. · Neurology (2025) | TGRS Research Map | TGRS