Progressive Supranuclear Palsy

A/Prof David R Williams
Text Box: 1. Introduction 2. Tauopathies 3. Ambiguities in defining PSP 4. Hypothesis 5. Clinical heterogeneity in pathologically diagnosed PSP 6. Patterns of clinical presentation in pathologically diagnosed PSP 6.1. Analysis of clinical features in PSP 6.2. Post-hoc analysis of QSBB database – pure akinesia with gait freezing 7. Pathological heterogeneity in pathologically diagnosed PSP 8. Patterns of regional pathological involvement in PSP Biochemical heterogeneity in PSP 10. Genetics of progressive supranuclear palsy 10.1. MAPT gene mutations in PSP 10.2. Tau haplotype analysis in PSP 10.3. Apolipoprotein E 11. The diagnosis of PSP-P 11.1. Diagnostic experiments in clinically diagnosed patients 11.1.1. A visual hallucination inventory that differentiates PD from PSP 11.1.2. Olfaction in PSP 11.1.3. The auditory startle response in the diagnosis PSP 11.2. Retrospective studies in pathologically diagnosed patients 11.2.1. Clinical differences between RS, PSP-P & other Parkinsonism 11.2.2. Falls in PSP 11.2.3. VH in pathologically diagnosed bradykinetic rigid syndromes 12. Sample size calculations using clinical phenotypes 13. Summary Text Box: PSP thesis Text Box: What is PSP? Information sheet—click here for PDF version

PSP is a rare neurological condition affecting the part of the brain that controls walking, eye movements, balance, speech and swallowing. PSP has a wide range of symptoms, but few people experience them all and symptoms vary from person to person. It is a progressive condition, meaning that the symptoms continue to get worse over time, however the severity and rate of progression varies widely between individuals. Symptoms can fluctuate from day to day or even from hour to hour.

 

There is no known cure for PSP and we do not know of any way of preventing it from occurring. Treatments, therapies and strategies are available to help people with PSP and their families and carers to make the most of living with the condition. The information here is a guide; it is important to discuss all symptoms and management strategies with your neurologist and other health professionals.

 

‘P’         Progressive                  means the symptoms get worse over time

 

‘S’        Supranuclear               refers to the part of the brain that is affected

 

‘P’         Palsy                            means a weakness or paralysis in part of the body.

 

PSP occurs in only about five or six people per 100 000 (this is about the same as motor neurone disease). It is a difficult condition for doctors to diagnose, so actual numbers of people with PSP may be higher. Approximately 180 Australians will develop the first signs of PSP each year and currently around 1300 Australians are living with PSP. Only 22 per cent of people with PSP are given the correct diagnosis in the first 2 years, with many receiving an initial diagnosis of Parkinson’s disease due to the similarity of symptoms.

 

PSP was first described and named by doctors in 1964. Before then people with the condition were thought to have Parkinson’s disease. In the past PSP was sometimes called ‘Steele-Richardson-Olszewski Syndrome’. In its different manifestations it may be called ‘Richardson’s disease’ after the doctors who first described the condition, PSP-parkinsonism (PSP-P) or pure akinesia with gait freezing.

 

What causes PSP?

PSP is associated with an over-production of a protein called ‘tau’ in certain areas of the brain. Clumps of tau (called ‘tangles’) build up and these are believed to cause damage to nerve cells. We do not know what causes the over-production or the build up of tau, however research is currently underway. Researchers have found no evidence of any links between PSP and environmental factors such as exposure to toxins or chemicals. There is ongoing research going on in Australia, and study participants are always needed. Parkinson’s Australia or your treating neurologist may know about these studies.

 

Who gets PSP?

People with PSP usually start to have symptoms between the ages of 60 and 70. PSP can affect people from all nationalities and lifestyles and many of those affected have always lived a healthy lifestyle.

 

 

Is PSP inherited or passed on through families?

PSP does not run in families. However a common genetic type has been found to be more common in people with PSP than the rest of the population. In other words, there may be some genetic susceptibility that makes some people more at risk than others, but PSP rarely affects more than one person in a family.

 

 

Can it be treated?

Treatments and therapies are available to help manage symptoms of PSP, however there is no cure and we do not know of any way of slowing its progression.

 

It is important that people with PSP see a neurologist, preferably one with expertise in movement disorders such as PSP and Parkinson’s. Depending on symptoms, it is also important to seek the advice of other health professionals with expertise in the condition, such as a physiotherapist, occupational therapist, speech pathologist (speech therapist) or counsellor. Parkinson’s Australia can provide information about Neurologists and other health professionals in your area.

 

For further information about treatments click on this link: