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Risk Factors for Progressive Supranuclear Palsy (PSP)

12:06 EDT 20th April 2014 | BioPortfolio

Summary

Progressive supranuclear palsy (PSP) is the most common atypical parkinsonian movement disorder. This study will determine the role of specific genetic, occupational and environmental components in the development of PSP by evaluating patients with this disorder and age and gender matched controls.

Description

This proposal will determine: (1) if there is an association between PSP and specific genes of interest; (2) if there is an association between PSP and occupational and/or environmental chemical exposures functionally or structurally similar to known parkinsonian toxicants; and (3) if hypertension or traumatic brain injury prior to symptom-onset is associated with PSP. To disentangle the complex etiology of PSP, this case-control multicenter study involves 500 PSP cases, 500 age/gender matched primary controls, and 500 secondary controls for genetic confirmation. Understanding the etiology of PSP may also help explain the causes of other related diseases such as Alzheimer's disease. This multidisciplinary team of movement disorder specialists, epidemiologists, geneticists, biostatisticians, industrial hygienist and toxicologist is well suited to unravel the etiology of PSP.

Study Design

Observational Model: Case Control, Time Perspective: Prospective

Conditions

Progressive Supranuclear Palsy

Location

University of Alabama, Birmingham
Birmingham
Alabama
United States
35233

Status

Completed

Source

University of Louisville

Results (where available)

View Results

Links

Medical and Biotech [MESH] Definitions

Neurodegenerative disorders involving deposition of abnormal tau protein isoforms (TAU PROTEINS) in neurons and glial cells in the brain. Pathological aggregations of tau proteins are associated with mutation of the tau gene on chromosome 17 in patients with ALZHEIMER DISEASE; DEMENTIA; PARKINSONIAN DISORDERS; progressive supranuclear palsy (SUPRANUCLEAR PALSY, PROGRESSIVE); and corticobasal degeneration.

A degenerative disease of the central nervous system characterized by balance difficulties; OCULAR MOTILITY DISORDERS (supranuclear ophthalmoplegia); DYSARTHRIA; swallowing difficulties; and axial DYSTONIA. Onset is usually in the fifth decade and disease progression occurs over several years. Pathologic findings include neurofibrillary degeneration and neuronal loss in the dorsal MESENCEPHALON; SUBTHALAMIC NUCLEUS; RED NUCLEUS; pallidum; dentate nucleus; and vestibular nuclei. (From Adams et al., Principles of Neurology, 6th ed, pp1076-7)

Diseases characterized by a selective degeneration of the motor neurons of the spinal cord, brainstem, or motor cortex. Clinical subtypes are distinguished by the major site of degeneration. In AMYOTROPHIC LATERAL SCLEROSIS there is involvement of upper, lower, and brainstem motor neurons. In progressive muscular atrophy and related syndromes (see MUSCULAR ATROPHY, SPINAL) the motor neurons in the spinal cord are primarily affected. With progressive bulbar palsy (BULBAR PALSY, PROGRESSIVE), the initial degeneration occurs in the brainstem. In primary lateral sclerosis, the cortical neurons are affected in isolation. (Adams et al., Principles of Neurology, 6th ed, p1089)

A motor neuron disease marked by progressive weakness of the muscles innervated by cranial nerves of the lower brain stem. Clinical manifestations include dysarthria, dysphagia, facial weakness, tongue weakness, and fasciculations of the tongue and facial muscles. The adult form of the disease is marked initially by bulbar weakness which progresses to involve motor neurons throughout the neuroaxis. Eventually this condition may become indistinguishable from AMYOTROPHIC LATERAL SCLEROSIS. Fazio-Londe syndrome is an inherited form of this illness which occurs in children and young adults. (Adams et al., Principles of Neurology, 6th ed, p1091; Brain 1992 Dec;115(Pt 6):1889-1900)

A form of multiple sclerosis characterized by a progressive deterioration in neurologic function which is in contrast to the more typical relapsing remitting form. If the clinical course is free of distinct remissions, it is referred to as primary progressive multiple sclerosis. When the progressive decline is punctuated by acute exacerbations, it is referred to as progressive relapsing multiple sclerosis. The term secondary progressive multiple sclerosis is used when relapsing remitting multiple sclerosis evolves into the chronic progressive form. (From Ann Neurol 1994;36 Suppl:S73-S79; Adams et al., Principles of Neurology, 6th ed, pp903-914)

Clinical Trials [240 Associated Clinical Trials listed on BioPortfolio]

Effects of Coenzyme Q10 in Progressive Supranuclear Palsy (PSP)

The clinical syndrome of PSP responds poorly to all available forms of therapy used in Parkinson's Disease (PD). Currently, no effective treatment exists. Coenzyme Q10 in high doses has b...

Trial of Valproic Acid in Patients With Progressive Supranuclear Palsy

Progressive Supranuclear Palsy (PSP) is a relentlessly progressive neurodegenerative disorder, clinically characterized by parkinsonism with prominent axial involvement and postural instab...

Study to Evaluate the Safety and Efficacy of Davunetide for the Treatment of Progressive Supranuclear Palsy

The purpose of the study is to evaluate the safety and efficacy of davunetide for the treatment of Progressive Supranuclear Palsy.

Efficacy Study for Treatment of Dementia in Progressive Supranuclear Palsy

to show that 1. patients improve and stabilize after 12 -24 week treatment with rivastigmine in memory function 2. use of rivastigmine has a positive effect on apathy in P...

A Pilot Clinical Trial of Pyruvate, Creatine, and Niacinamide in Progressive Supranuclear Palsy.

This study intends to study the safety and tolerance of the combination of pyruvate, creatine, and niacinamide over 6 months in patients with PSP.

PubMed Articles [1485 Associated PubMed Articles listed on BioPortfolio]

A case of atypical progressive supranuclear palsy.

Progressive supranuclear palsy (PSP) is a neurodegenerative extrapyramidal syndrome. Studies have demonstrated that PSP can present clinically as an atypical dementing syndrome dominated by a progress...

Changes in the Thalamus in Atypical Parkinsonism Detected Using Shape Analysis and Diffusion Tensor Imaging.

BACKGROUND AND PURPOSE:The thalamus is interconnected with the nigrostriatal system and cerebral cortex and has a major role in cognitive function and sensorimotor integration. The purpose of this stu...

Progressive supranuclear palsy misdiagnosed as Parkinson's disease: a case report and review of literature.

Because of its increasingly recognized clinical diversity, progressive supranuclear palsy (PSP) may be difficult to diagnose, particularly in resource-poor settings where the means of getting supporti...

Progressive supranuclear palsy-like syndrome after aortic aneurysm repair: a case series.

The syndrome of progressive supranuclear palsy-like syndrome is a rare complication of ascending aortic aneurysm repair. We report two patients with videos and present a table of prior reported cases....

Compassionate trial of levodopa carbidopa intestinal gel infusion in two patients with progressive supranuclear palsy.

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