Progressive Supranuclear Palsy and Corticobasal Degeneration

Progressive Supranuclear Palsy (previously known as Steele-Richardson-Olszewski Syndrome) and Corticobasal Degeneration are rare degenerative neurological disorders that have similar clinical pictures and often overlap. Both are caused by aggregation of tau proteins, which stick together to form neurofibrillary tangles, damaging the neurons around them.

Both of these disorders are commonly misdiagnosed as Parkinson, Alzheimer’s, stroke or Multisystems Atrophy and it can sometimes take several years for them to be correctly diagnosed. Due to their similarities with these more common disorders, they are often diagnosed by process of elimination, as there are no specific diagnosis techniques for PSP or CBD. Definitive diagnosis can only be given with certainty following a postmortem.

PSP CBD
Area of brain effected Basal ganglia, brain stem, cerebral cortex and dentate nucleus of cerebellum basal ganglia and cortex
Average age of onset 62
more common in men
60-70
more common in women
Epidemiology 6.4 in 100,000 4.9-7.3 in 100,000
Symptoms Unable to look up or down
Parkinsonism
Falls (often backwards)
Behavioural changes
Behaviour changes (apathy, impulsive behaviour)
‘alien limb’ unintentional movements or loss of movement of one arm/leg
only one side effected initially
memory problems
dyspraxia and apraxia

How are the currently treated?

There are currently no treatments or cures for either disorder, and management of symptoms and enabling the person to maintain a normal a life as possible is best practice.

What’s it like to live with PSP and CBD?

The initial diagnosis progress can be quite distressing, as often patients must wait an extra three years after the onset of symptoms to get an official diagnosis. This is normally following a course of medications that don’t work, some of which can have very unpleasant side effects such as hallucinations.

In PSP, while memory function is often not greatly effected, they can have issues word finding and can become more inflexible when making decisions. Some PSP patients can also become emotional labile, crying or laughing inappropriately, something that can make those around them feel uncomfortable, especially if this behaviour is inappropriate. Those with CBD have a greater degree of cognitive and behavioural changes, and dementia is common, although not everyone will suffer with these. Patients can find it very frustrating and they can often feel that they are a burden on those around them, especially as the disease progresses. They can also become isolated as they will no longer be allowed to drive and over time it may become dangerous for them to go out alone, leading to social withdrawal and depression.

So what?

Overtime, patients with either disorder will become more unwell, eventually relying on other people for assistance in every part of their day-to-day life. Each individual case of PSP and CBD (especially CBD) can display very different symptoms and these need to be treated case by case. While there is currently no cure, measures can be put in place to optimise the persons quality of life and maintain their independence as long as possible. There are also lots of support out there, with the PSP Association being the key contact, but there is no reason why patients cannot attend support groups for other similar conditions. Some patients can also find it helpful to make advanced plans, ensuring that their wishes are carried out when the time comes that they are unable to make decisions themselves; this can be through an advanced directive or Power of Attorney.

Contacts

PSP Association https://pspassociation.org.uk/    0300 0110 122
Age UK                 www.ageuk.org
Mind                    www.mind.org.uk

References

PSP Association (no date). Available at: https://pspassociation.org.uk/ (Accessed: 18 October 2018).

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