Progressive Supranuclear Palsy and Diffusion Tensor Imaging

Progressive Supranuclear Palsy and Diffusion Tensor Imaging

Ito Shoichi

European Neurological Review, 2009;4(2):108-11

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Abstract
Progressive supranuclear palsy (PSP) is a neurodegenerative disease affecting multiple neural systems, particularly the extrapyramidal system. Early differentiation of PSP from other diseases mainly featuring parkinsonism, such as Parkinson’s disease and multiple system atrophy, is necessary because the therapeutic strategy and outcome are substantially different. Diffusion tensor imaging is a recently developed magnetic resonance imaging (MRI) sequence that is able to non-invasively evaluate neural tracts. Two approaches may be used to measure diffusion properties. One approach is to measure diffusion properties by setting the regions of interest on circular/square regions or along the tractography. The other approach is to perform voxel-by-voxel analysis of the diffusion properties. There are several reports evaluating diffusion tensor abnormalities in PSP, and regions with diffusion tensor abnormlities are distributed through frontal projection fibres, the anterior part of the corpus callosum, superior longitudinal fasciculus, arcuate fasciculus, posterior thalamic radiations, internal capsule and superior cerebellar peduncles. Here, diffusion tensor studies in PSP are reviewed and clinical applications, limitations and future perspectives of diffusion tensor analysis in PSP are discussed.

Keywords
Progressive supranuclear palsy, diffusion-weighted imaging, diffusion tensor imaging, tractography, statistical parametric mapping, apparent diffusion coefficient, fractional anisotropy, corpus callosum, superior longitudinal fasciculus, arcuate fasciculus, internal capsule, superior cerebellar peduncles

Disclosure: The author has no conflicts of interest to declare.
Received: 9 March 2009 Accepted: 21 July 2009
Correspondence: Shoichi Ito, 1-8-1 Inohana, Chuo-ku, Chiba, 260-8670, Japan. E: sito@faculty.chiba-u.jp

Clinical Features of Progressive Supranuclear Palsy
Progressive supranuclear palsy (PSP) is a neurodegenerative disorder characterised by parkinsonism, supranuclear ophthalmoplegia, dysphagia and cognitive dysfunction. The National Institute of Neurological Disorders and Stroke (NINDS) PSP criteria are widely used for clinical diagnosis.1 However, the clinical phenotypes of pathologically confirmed PSP patients are heterogeneous. Recently, PSP has been clinically classified into two phenotypes: Richardson’s syndrome and PSP-parkinsonism.2 Those patients who have Richardson’s syndrome are neurologically characterised by the classic features, including the early onset of postural instability and falls, supranuclear vertical gaze palsy and cognitive dysfunction. PSP-parkinsonism is frequently misdiagnosed as Parkinson’s disease in its early clinical stage and is characterised by asymmetrical onset, tremor and moderate initial therapeutic response to levodopa. Differentiating between Richardson’s syndrome and Parkinson’s disease is not difficult, but differentiating between PSP-parkinsonism and Parkinson’s disease is occasionally challenging. Early differentiation of these diseases is necessary because the therapeutic strategy and outcome are substantially different (e.g. PSP patients have much worse prognosis).

Diffusion-weighted Imaging
Magnetic resonance imaging (MRI) is a promising non-invasive tool that supports or complements clinical diagnosis. It is also useful for the investigation of the pathophysiology of diseases. Recently, several new MRI techniques have been developed, and one of the most important of these tools is diffusion-weighted imaging,3 which can be used to measure the diffusion of water molecules in the brain.4 Brain diffusion is influenced by tissue density, neuronal axon and vessel direction and capillary flow. The apparent diffusion coefficient (ADC) or mean diffusivity (MD) is a parameter that represents diffusion intensity, and high ADC or MD values indicate increased diffusion intensity (see Figure 1). Diffusion intensities along three orthogonal directions are represented as λ1, λ2 and λ3, and MD values are calculated as [λ1, λ2, and λ3]/3. Axial diffusivity (λ1) and radial diffusivity ([λ2 + λ3]/2) are occasionally used as additional measures to identify whether differences in anisotropy may be caused by diffusion parallel or perpendicular to the white-matter fibres, respectively. Cytotoxic oedema is a typical pathological condition in which decreased ADC values are indicative of cerebral infarction5,6 or other conditions. ADC values are increased by vasogenic oedema7, or demyelination.8 In neurodegenerative diseases, ADC values are increased in affected areas because of disruption of neuronal axons.9,10

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Keywords:
Progressive supranuclear palsy, diffusion-weighted imaging, diffusion tensor imaging, tractography, statistical parametric mapping, apparent diffusion coefficient, fractional anisotropy, corpus callosum, superior longitudinal fasciculus, arcuate fasciculus, internal capsule, superior cerebellar peduncles

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