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Progressive Supranuclear Palsy
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Progressive Supranuclear Palsy (PSP), is a neurodegerative disorder also known as Steele-Richardson-Olszewski syndrome after the three neurologists who first described it back in the early 1960's. The most common symptoms of PSP include unexpected falls (usually backwards) and other problems associated with balance, changes in vision, especially difficulty with eye movements in the up and down direction and difficulties with blinking, and changes in personality. As the disease progresses the ability to swallow becomes severely affected, posing a major risk to patients. Many of the early symptoms of PSP are also present in Parkinsons disease, and so there are difficulties in accurate diagnosis of PSP. Current thinking estimates the occurence of PSP in the UK to be roughly 1 in 7000, which is at least as common as Motor Neurone Disease, a much better known disorder. This figure is still likely to be an underestimate due to the number of cases that are misdiagnosed. Awareness of PSP has been raised since the death of Dudley Moore from the disease back in 2002, and the work of the PSP (Europe) Association (see links below) aims to raise awareness further both in the general public and GP's up and down the country. Increased awareness will in turn lead to a better chance of correct diagnosis and appropriate treatment.

Symptoms of PSP generally develop, on average, between 60-65 years of age. As the name suggests PSP is progressive, i.e. it gets worse over time, and the life expectancy of a patient is usually around seven years, the last two of these may be spent in a wheelchair. Currently there is no drug available that slows down the progression of the disease, and treatments tend to concentrate on alleviating the symptoms. For example, special glasses may be used to counteract visual problems, or weighted walking aids may be used to minimise the risk of falls. In later stages of the disease, tube feeding may be necessary in order to minimise the risk of patients choking.

The brains of people with PSP contain Neurofibrillary tangles composed of a protein called tau, similar to those found in the brains of patients with Alzheimers disease. Neurodegenerative disease where tau is deposited in the brain are collectively known as tauopathies, in addition to Alzheimer's disease and PSP, this family of diseases also includes Pick's disease, Cortico Basal Degeneration (CBD) and Frontotemporal Dementia with Parkinsonism related to chromosome 17 (FTDP-17). Unlike Alzheimers disease there are no amyloid plaques present in the brains of PSP patients, so it is known as a primary tauopathy.

The tau tangles found in PSP are very similar to the ones found in Alzheimers disease, except instead of being flame-shaped (see figures in the Alzheimers disease section) they are globular, and on a molecular level as opposed to being paired helical filaments the tangles are composed of straight filaments. In Alzheimers disease the tangles form in the hippocampus and cortex of the brain, which are important for memory and cognition. This is in contrast to PSP where tangles form in the brain regions responsible for controlling eye movements and movements of the mouth tongue and throat, and is the reason why the two diseases present with very different symptoms. Tau which is deposited in PSP is hyperphosphorylated, much like the tau in Alzheimers disease. Research into PSP here at the centre aims to elucidate the mechanisms by which tau becomes hyperphosphorylated and forms tangles.

Links

The Progressive Supranuclear Palsy (PSP-Europe) Association

The Society for Progressive Supranuclear Palsy

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