Abstract

BackgroundThe clinical presentation in progressive supranuclear palsy (PSP), an atypical parkinsonian disorder, includes varying degrees of frontal dysexecutive symptoms. Using diffusion tensor imaging (DTI) and tractography (DTT), we investigated whether diffusion changes and atrophy of the inferior fronto-occipital fasciculus (IFO) occurs in PSP and if these changes correlate with disease stage and clinical phenotype. The corticospinal tract (CST), which is often involved in PSP, was investigated for comparison.MethodsDTI of the whole brain was performed with a 3 T MR scanner using a single shot-EPI sequence with diffusion encoding in 48 directions. Scans were obtained in patients with PSP (n = 13) and healthy age-matched controls (n = 12). DTT of the IFO and CST was performed with the PRIDE fibre tracking tool (Philips Medical System). Fractional anisotropy (FA) and apparent diffusion coefficient (ADC) were calculated and correlated with disease stage and clinical phenotype.ResultsIn patients with PSP, significantly decreased FA and increased ADC was found in the frontal part of IFO compared with the medial and occipital parts of IFO, as well as compared to controls. Four of the thirteen patients with PSP showed a marked decrease in the number of tracked voxels in the frontal part of IFO. These findings were most pronounced in patients with severe frontal cognitive symptoms, such as dysexecutive problems, apathy and personality change. There was a strong correlation (r2 = -0.84; p < 0,001) between disease stage and FA and ADC values in the CST.ConclusionsDTT for identification of neuronal tracts with subsequent measurement of FA and ADC is a useful diagnostic tool for demonstrating patterns of neuronal tract involvement in neurodegenerative disease. In selected tracts, FA and ADC values might act as surrogate markers for disease stage.

Highlights

  • The clinical presentation in progressive supranuclear palsy (PSP), an atypical parkinsonian disorder, includes varying degrees of frontal dysexecutive symptoms

  • In the present study, we demonstrate that atrophy and diffusion changes occur in two major nerve tracts (IFO and corticospinal tract (CST)) in patients with PSP using aggregated data from diffusion tensor tractography (DTT) performed in individual patients

  • The anatomy of the inferior fronto-occipital fasciculus (IFO) generated by tractography in the present study closely resembles previously published tractography and post-mortem dissection data [12,13,16]

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Summary

Introduction

The clinical presentation in progressive supranuclear palsy (PSP), an atypical parkinsonian disorder, includes varying degrees of frontal dysexecutive symptoms. Progressive supranuclear palsy (PSP) is a neurodegenerative disorder, with progressive motor, behavioural and cognitive symptoms, leading to severe handicap and death [1]. It is one of the most common parkinsonian disorders, after idiopathic Parkinson disease (IPD) and dementia with lewy bodies (DLB) [2]. Recent clinical and neuropathological studies have revealed other clinical presentations of PSP, dominated by bradykinesia and postural instability and lacking many of the frontal behavioural disturbances, so-called PSPparkinsonism [1]. The sensitivity and specificity of these signs vary, but none of them are completely specific for PSP [4,5,6]

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