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Progressive supranuclear palsy (PSP) (or the Steele-Richardson-Olszewski syndrome, after the Canadian physicians who described it in 1963 ) is a rare degenerative disorder involving the gradual deterioration and death of selected neurons in the brain. Typical effects are problems with control of gait and balance (axial dystonia) and/or losing conciousness (collapsing, fainting etc), and, in some cases later on, an inability to aim the eyes properly (ophthalmoplegia), especially in the vertical directions (downward gaze palsy). Other symptoms may be alterations of mood and behavior, depression and apathy as well as mild dementia. In some cases it can prove to be fatal. There is currently no effective treatment for the disease.
Unlike most palsies, which result in the wholesale death of patches of cerebral or spinal tissue, PSP generally starts with the deterioration of synaptic connections between neurons. Because of the insidious, often lifelong and gradual nature of this deterioration, it is difficult to diagnose until it is fairly advanced.
Grossly, a widespread neuronal loss and gliosis is observed. Subcortical sites are primarily targets, with relative sparing of the Cerebral or Cerebellar Cortex. Neurofibrillary tangles--distinct from those seen for Alzheimer's--may be seen under microscope
Geneticists are studying the human genome to determine if there is a hereditary trait that predisposes one for this disease; its rarity as well as its progressiveness makes it difficult to study objectively. Two genes have been linked to PSP, but these are only abnormal in a minority of the patients.
Actor Dudley Moore, suffering from the disease and dying from its complications, increased public awareness of this disease. In the United Kingdom alone, there could be as many as 10,000 cases of PSP.
Reference
- Richardson JC, Steele J, Olszewski J. Supranuclear ophthalmoplegia, pseudobulbar palsy, nuchal dystonia and dementia. A clinical report on eight cases of "heterogenous system degeneration". Trans Am Neurol Assoc 1963;88:25-9. PMID 14272249.
- Steele JC, Richardson JC, Olszewski J. Progressive supranuclear palsy: a heterogeneous degeneration involving brain stem, basal ganglia and cerebellum with vertical gaze and pseudobulbar palsy, nuchal dystonia and dementia. Arch Neurol 1964;10:333-359. PMID 14107684.
External links
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