Increased Signal in the Superior Cerebellar Peduncle of Patients with Progressive Supranuclear Palsy

J Mov Disord. 2019 Sep;12(3):166-171. doi: 10.14802/jmd.19002. Epub 2019 Aug 9.

Abstract

Objective: The provisional diagnosis of progressive supranuclear palsy (PSP) depends on a combination of typical clinical features and specific MRI findings, such as atrophy of the tegmentum in the midbrain. Atrophy of the superior cerebellar peduncle (SCP) distinguishes PSP from other types of parkinsonism. Histological factors affect the conventional fluid-attenuated inversion recovery (FLAIR) signals, such as the extent of neuronal loss and gliosis.

Methods: We investigated patients with PSP to verify the percentage of patients with various PSP phenotypes presenting a high signal intensity in the SCP. Three interviewers, who were not informed about the clinical data, visually inspected the presence or absence of a high signal intensity in the SCP on the FLAIR images. We measured the pixel value in the SCP of each patient. Clinical characteristics were evaluated using the Mann-Whitney test, followed by the χ2 test.

Results: Ten of the 51 patients with PSP showed a high signal intensity in the SCP on FLAIR MRI. Higher pixel values were observed within the SCP of patients with a high signal intensity in the SCP than in patients without a high signal intensity (p < 0.001). The sensitivity and specificity of the high signal intensity in the SCP of patients with PSP was 19.6% and 100%, respectively. This finding was more frequently observed in patients with PSP with Richardson's syndrome (PSP-RS) (25.7%) than other phenotypes (6.2%).

Conclusion: The high signal intensity in the SCP on FLAIR MRI might be an effective diagnostic tool for PSP-RS.

Keywords: Atrophy; FLAIR; Magnetic resonance imaging; Neurodegenerative disease; Progressive supranuclear palsy; Superior cerebellar peduncle.