Midbrain atrophy in patients with presymptomatic progressive supranuclear palsy-Richardson's syndrome

Abstract

Introduction: In the present study, midbrain atrophy and the pons-to-midbrain area ratio (P/M ratio) were investigated as diagnostic markers for presymptomatic progressive supranuclear palsy-Richardson's syndrome (Pre-PSP-RS).

Methods: The present study included 27 patients with probable PSP-RS who underwent brain MRI at least twice before and after the development of clinical symptoms, age- and sex-matched participants with Parkinson's disease (PD, n = 27), and healthy controls (n = 27). The midbrain area, pons area, and P/M ratio of the Pre-PSP-RS, PD, and control subjects were measured using midsagittal images from brain MRI, and the parameters were compared among the groups.

Results: The midbrain area decreased and the P/M ratio increased significantly in the Pre-PSP-RS patients compared with both the PD and control subjects (midbrain, Pre-PSP-RS vs. PD = 1.01 cm²vs. 1.29 cm², p < 0.001, Pre-PSP-RS vs. controls = 1.01 cm²vs. 1.29 cm², p < 0.001; P/M ratio, Pre-PSP-RS vs. PD = 5.27 vs. 4.03, p < 0.001, Pre-PSP-RS vs. controls = 5.27 cm²vs. 4.06 cm², p < 0.001). The P/M ratio had high sensitivity (vs. PD, 96.3%, vs. control, 88.9%) and specificity (vs. PD, 81.5%, vs. control, 96.3%) in differentiating Pre-PSP-RS patients from PD and control subjects.

Conclusion: Midbrain atrophy precedes the clinical symptoms of PSP-RS and could be a useful diagnostic imaging biomarker for Pre-PSP-RS. Furthermore, this information could play an important role in the development of future treatment strategies.

Keywords: Biomarker; Midbrain atrophy; Pons-to-midbrain ratio; Presymptomatic PSP; Progressive supranuclear palsy; Richardson's syndrome.