Progressive Supranuclear Palsy PSP

PSP is the second most common cause of Parkinsonism typified by early gait instability and difficulty with vertical eye movement. PSP is characterized by neurofibrillary tangles composed almost entirely of sdaight filaments of four repeats of Tau protein. Although most cases of PSP appear to be sporadic, genetic diatheses have been implicated. De Yebenes described a pattern of inheritance consistent with a Mende-lian autosomal dominant disorder (De Yebenes et al., 1995). Difficulty recognizing the variable phenotypic expression of PSP may be one reason fewer familial cases have been identified than expected (Rojo et al., 1999). The HI haplotype of the Tau gene has also been found to have association with increased risk for PSP, as it has been for PD (Conrad et al.. 

Recently, the HI and H2 haplotypes have been shown to be directly associated with a large chroniosonie inversion involving the Tau gene. (Gijsehnck et al, 2006). 

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Fig. 6.8 Statistical Images showing the F- FDGimagesof glucose utilization in the brains of patients with frontotemporal dementia (FTD) progressive supranuclear palsy (PSP) mild dementia of Alzheimertype (MID) and normal pressure hydrocephalus (NPH).

Although iron is essential for normal functioning of the human body, it may aiso play a deleterious role as a trigger of various diseases [ 1,2]. In this chapter, we will present results of studies on the role of iron in neurodegenerative diseases such as Parkinson s disease (PD), Alzheimer s disease (AD), and progressive supranuclear palsy (PSP). We report on studies whose main tool was Mossbauer spectroscopy, but also other complementary techniques such as electron microscopy, enzyme-linked immunosorbent assay (ELISA), and atomic absorption. 

OSN olfactory sensory neuron PSP progressive supranuclear palsy... 

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