Objective: Our aim was to evaluate the occurrence of motor cortex (MC) and cortico-spinal tract (CST) magnetic resonance imaging abnormalities in patients with hereditary spastic paraplegia (HSP).

Background: Conventional MRI can identify abnormalities associated with upper motor neuron (UMN) involvement. MC and CST imaging abnormalities are reported in patients with amyotrophic lateral sclerosis (ALS), but their prevalence in other disease with UMN involvement such as HSP is poorly studied.

Method: We retrospectively evaluated 3T-MRI from 44 HSP patients (27 men, mean age 49.3 ± 14.6), 55 ALS patients (35 men, 56.3 ± 7.6) and 52 controls (34 men, 53.4 ± 5.1). Features of interest were CCS T2/FLAIR hyperintensity, MC SWI hypointensity and selective MC atrophy. Differences among groups were tested using χ2 or ANOVA based on data (p < 0.05). We adjusted the analysis for age using logistic regression.

Results: HSP patients had more frequently CCS FLAIR hyperintensity than controls but less than ALS patients (HSP 40.5%, ALS 67.3%, controls 30%; p<0.001). The prevalence of MC SWI hypointensity (HSP 69.2%, ALS 73.5%, controls 18.8%; p<0.001) and atrophy (HSP 73.8%, ALS 64.2%, controls 42.3%; p<0.01) was similar in HSP and ALS patients, both higher than in controls. The results did not changed adjusting for age.

Conclusion: The prevalence of MC and CCS MRI abnormalities in HSP patients is higher than controls. However, it was similar to ALS patients, except for CCS FLAIR hyperintensity. less frequent in HSP, possibly reflecting pathophysiological differences. The finding of such MRI features support a pyramidal involvement but is not able to discriminate between HSP and ALS.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/pyramidal-pathway-changes-at-conventional-brain-3t-mri-in-patients-with-hereditary-spastic-paraplegia/