Title: Clinical Aspects of Progressive Supranuclear Palsy
Abstract: This chapter describes that the diagnosis of progressive supranuclear palsy (PSP) should be considered in any patient with progressive Parkinsonism and disturbance of ocular motility. PSP, also known as Steele-Richardson-Olszewski syndrome, is a progressive neurodegenerative disorder. With a median survival of about six years, PSP is characterized by progressive postural instability leading to early falls, vertical ophthalmoparesis, akinetic-rigid features, pseudobulbar palsy, levodopa-unresponsive parkinsonism and frontal cognitive disturbances. In general, symptoms and signs in PSP progress rapidly and steadily. In advanced stages, patients require a wheelchair and may even become bedridden, their swallowing becomes severely impaired, requiring a feeding tube in some cases, and their speech becomes unintelligible. The disorder is categorized as a four–repeat tauopathy, characterized pathologically by neuronal loss, gliosis and intracellular accumulation of tau in neurons and in glia in many brain areas, including the cerebral cortex, basal ganglia and the brainstem. Although typical cases are characterized by akinesia, supranuclear gaze palsy, rigidity, axial dystonia, gait disturbance, and dementia, there are patients with atypical symptoms and typical pathology. Furthermore, patients without the clinical hallmarks of PSP, including ophthalmopares are, have been found to have typical pathological features of PSP at autopsy.
Publication Year: 2008
Publication Date: 2008-01-01
Language: en
Type: article
Indexed In: ['crossref', 'pubmed']
Access and Citation
Cited By Count: 9
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