The midbrain-to-pons ratio distinguishes progressive supranuclear palsy from non-fluent primary progressive aphasias

作者：Sil**y M; Tweedie Cullen R Y; Murray C R; Halliday G M; Hodges J R; Burrell J R

来源：European Journal of Neurology, 2017, 24(7): 956-965.

DOI：10.1111/ene.13314

• clinicopathological correlation
• hummingbird bird sign
• logopenic progressive aphasia
• primary progressive aphasia
• progressive non-fluent aphasia
• progressive supranuclear palsy

Background and purpose: To determine the clinical utility of the midbrain-to-pons (M/P) ratio as a clinical biomarker of progressive supranuclear palsy (PSP) in patients with non-fluent primary progressive aphasia syndromes.
Methods: Patients with PSP, progressive non-fluent aphasia (PNFA) and logopenic progressive aphasia (LPA) were recruited. Patients were diagnosed clinically, but pathological confirmation was available in a proportion of patients. Midbrain and pons areas were measured using Osirix Lite, a free DICOM viewer. The M/P ratio and Magnetic Resonance Parkinsonism Index were calculated and their diagnostic utility compared.
Results: A total of 72 participants were included (16 PSP, 18 PNFA, 16 LPA and 22 controls). Patients with PSP had motor features typical of the syndrome. Both the M/P ratio and Magnetic Resonance Parkinsonism Index differed significantly in PSP compared with controls. The M/P ratio was disproportionately reduced in PSP compared with PNFA and LPA (PSP, 0.182 +/- 0.043; PNFA, 0.255 +/- 0.034; LPA, 0.258 +/- 0.033; controls, 0.292 +/- 0.031; P < 0.001). An M/P ratio of <= 0.215 produced a positive predictive value of 77.8% for the diagnosis of PSP syndrome. Pathological examination revealed Alzheimer's disease in three cases (all LPA), pathological PSP in two cases (one clinical PSP and one PNFA) and corticobasal degeneration in one case (PNFA). The M/P ratio was <= 0.215 in both pathological cases of PSP.
Conclusions: The M/P ratio was disproportionately reduced in PSP, suggesting its potential as a clinical marker of the PSP syndrome. Larger studies of pathologically confirmed cases are needed to establish the M/P ratio as a biomarker of PSP pathology.

• 出版日期2017-7