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European Radiology
Published in: European Radiology 12/2022

20-05-2022 | Spastic Paraplegia | Neuro

Iron-sensitive MR imaging of the primary motor cortex to differentiate hereditary spastic paraplegia from other motor neuron diseases

Authors: Mirco Cosottini, Graziella Donatelli, Ivana Ricca, Francesca Bianchi, Daniela Frosini, Vincenzo Montano, Gianmichele Migaleddu, Eleonora Del Prete, Alessandra Tessa, Paolo Cecchi, Claudio D’Amelio, Gabriele Siciliano, Michelangelo Mancuso, Filippo Maria Santorelli

Published in: European Radiology | Issue 12/2022

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Abstract

Objectives

Hereditary spastic paraplegia (HSP) is a group of genetic neurodegenerative diseases characterised by upper motor neuron (UMN) impairment of the lower limbs. The differential diagnosis with primary lateral sclerosis (PLS) and amyotrophic lateral sclerosis (ALS) can be challenging. As microglial iron accumulation was reported in the primary motor cortex (PMC) of ALS cases, here we assessed the radiological appearance of the PMC in a cohort of HSP patients using iron-sensitive MR imaging and compared the PMC findings among HSP, PLS, and ALS patients.

Methods

We included 3-T MRI scans of 23 HSP patients, 7 PLS patients with lower limb onset, 8 ALS patients with lower limb and prevalent UMN onset (UMN-ALS), and 84 ALS patients with any other clinical picture. The PMC was visually rated on 3D T2*-weighted images as having normal signal intensity, mild hypointensity, or marked hypointensity, and differences in the frequency distribution of signal intensity among the diseases were investigated.

Results

The marked hypointensity in the PMC was visible in 3/22 HSP patients (14%), 7/7 PLS patients (100%), 6/8 UMN-ALS patients (75%), and 35/84 ALS patients (42%). The frequency distribution of normal signal intensity, mild hypointensity, and marked hypointensity in HSP patients was different than that in PLS, UMN-ALS, and ALS patients (p < 0.01 in all cases).

Conclusions

Iron-sensitive imaging of the PMC could provide useful information in the diagnostic work - up of adult patients with a lower limb onset UMN syndrome, as the cortical hypointensity often seen in PLS and ALS cases is apparently rare in HSP patients.

Key Points

• The T2* signal intensity of the primary motor cortex was investigated in patients with HSP, PLS with lower limb onset, and ALS with lower limb and prevalent UMN onset (UMN-ALS) using a clinical 3-T MRI sequence.
• Most HSP patients had normal signal intensity in the primary motor cortex (86%); on the contrary, all the PLS and the majority of UMN-ALS patients (75%) had marked cortical hypointensity.
• The T2*-weighted imaging of the primary motor cortex could provide useful information in the differential diagnosis of sporadic adult-onset UMN syndromes.
Appendix
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Metadata
Title
Iron-sensitive MR imaging of the primary motor cortex to differentiate hereditary spastic paraplegia from other motor neuron diseases
Authors
Mirco Cosottini
Graziella Donatelli
Ivana Ricca
Francesca Bianchi
Daniela Frosini
Vincenzo Montano
Gianmichele Migaleddu
Eleonora Del Prete
Alessandra Tessa
Paolo Cecchi
Claudio D’Amelio
Gabriele Siciliano
Michelangelo Mancuso
Filippo Maria Santorelli
Publication date
20-05-2022
Publisher
Springer Berlin Heidelberg
Published in
European Radiology / Issue 12/2022
Print ISSN: 0938-7994
Electronic ISSN: 1432-1084
DOI
https://doi.org/10.1007/s00330-022-08865-6

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