Spinal Cord Gray and White Matter Damage in Different Hereditary Spastic Paraplegia Subtypes

BACKGROUND AND PURPOSE: Spinal cord damage is a hallmark of hereditary spastic paraplegias, but it is still not clear whether specific subtypes of the disease have distinctive patterns of spinal cord gray (GM) and white (WM) matter involvement. We compared cervical cross-sectional GM and WM areas in patients with distinct hereditary spastic paraplegia subtypes. We also assessed whether these metrics correlated with clinical parameters. MATERIALS AND METHODS: We analyzed 37 patients (17 men; mean age, 47.3 [SD, 16.5]?years) and 21 healthy controls (7 men; mean age, 42.3 [SD, 13.2]?years). There were 7 patients with spastic paraplegia type 3A (SPG3A), 12 with SPG4, 10 with SPG7, and 8 with SPG11. Image acquisition was performed on a 3T MR imaging scanner, and T2*-weighted 2D images were assessed by the Spinal Cord Toolbox. Statistical analyses were performed in SPSS using nonparametric tests and false discovery rate?corrected P values?< .05. RESULTS: The mean disease duration for the hereditary spastic paraplegia group was 22.4 [SD, 13.8]?years and the mean Spastic Paraplegia Rating Scale score was 22.8 [SD, 11.0]. We failed to identify spinal cord atrophy in SPG3A and SPG7. In contrast, we found abnormalities in patients with SPG4 and SPG11. Both subtypes had spinal cord GM and WM atrophy. SPG4 showed a strong inverse correlation between GM area and disease duration (? = ?0.903, P?

Automated summary

In this study, differences in spinal cord gray and white matter involvement were compared between different subtypes of hereditary spastic paraplegias. Patients with SPG4 and SPG11 showed spinal cord atrophy, while SPG3A and SPG7 did not display this characteristic.