Pspassociation.org.uk

PSP and CBD for
General Practitioners
and Community Nurses
Working for a World Free of PSP
PSP and CBD for General Practitioners
and Community Nurses
This booklet is part of a series of new publications for health and social care professionals, to help them support people who have Progressive Supranuclear Palsy (PSP) and Cortico Basal Degeneration (CBD). The PSP Association actively supports both patients with PSP and CBD. References to PSP in this guide should be read, where appropriate, as PSP and CBD. Issues will be similar for both conditions, unless otherwise stated. The purpose of this booklet is to provide General Practitioners with information about PSP and CBD and to assist them to improve the quality of life for those living with these conditions. Decisions regarding treatment should be made by the patient, with guidance and support from their neurologist or movement disorder specialist, GP, PSP Nurse Specialist, (PSPNS), Parkinson’s Disease Nurse Specialist (PDNS) and other members of their multi-disciplinary team members. This booklet is intended as a guide only and cannot be substituted for the advice and experience of the consultant caring for the person living with PSP or CBD. Thanks and gratitude go to the following for their advice and professional input into producing this booklet: Samantha Pavey, RGN (Guide Author).
Maggie Rose, RGN and Jill Lyons, RGN, Specialist Care Adviser, PSP Association.
Dr Gethin Prior, General Practitioner, South Wales.
Hilary Kerby, Community Matron,Warwickshire PCT.
Dr Mark Bayliss, Consultant in Older Adult Medicine, Brighton and Sussex University Hospitals Trust.
Dr Luke Massey, Clinical Research Fellow, Sara Koe PSP Research Centre.
Dr James Rowe, Consultant Neurologist at Addenbrooke’s Hospital and Wellcome Trust Senior
Research Fellow.
Dr Boyd Ghosh, Clinical Research Associate and Honorary Clinical Fellow,
Department of Neurology Addenbrooke’s Hospital.
Professor Andrew Lees, Director of the Reta Lila Weston Institute of Neurological Studies at
University College London, Professor of Neurology at the National Hospital for Neurology and
Neurosurgery, Queen Square, London.
This guide will be reviewed annually and we would welcome your feedback for improvement, which should be sent to:The Helpline and Information Manager at The PSP Association, 167 Watling Street West, Towcester, Northants. NN12 6BX The PSP Association
Our mission is the conquest of PSP and CBD and the objectives of The PSP
Association are to:
• Promote and sponsor research worldwide into PSP and CBD.
• Support affected patients, their families and carers.
• Provide information and engender awareness of PSP and CBD amongst
the medical profession and the public at large. Support for direct carers and people living with PSP or CBD who join the
Association includes:
• Telephone advice and support by the PSP Specialist Care Advisers. • A comprehensive information pack for direct carers, written in everyday • Invitations to local support groups across the UK and Ireland. • Subscription to PSP Matters,The PSP Association magazine, issued three • Reduced rates for the Annual PSP Symposium, held at different locations • Access to the PSP website and web forum. Support for professionals includes:
• Access to the Nurse Specialist telephone advice service. • Training and PSP/CBD awareness sessions (available on request). In addition, access to all of the above carer benefits is available with a subscription to The PSP Association. For access to the PSP Specialist Care Advisers, to arrange a training or awareness session or to obtain a subscription form, please see our contact details on the inside back cover. Progressive Supranuclear Palsy
Progressive Supranuclear Palsy was formerly known as Steele-Richardson-
Olszewski Syndrome after the three doctors who first identified it in 1964. It is
now generally referred to as PSP.
PSP is a neurodegenerative disease, classified as a movement disorder.
Characteristics include the inability to look up or down (known technically as
a supranuclear, vertical, gaze palsy) accompanied by symptoms resembling
Parkinson’s disease (‘extrapyramidal symptoms’ of slowness and stiffness), falls
and cognitive dysfunction.
Definition
Pathologically, PSP is defined by the accumulation of a special ‘junk’ protein
called tau, which forms neurofibrillary tangles in the brain. The principal areas
of the brain affected are:
• the basal ganglia (particularly the subthalamic nucleus, substantia nigra • the dentate nucleus of the cerebellum. In PSP there is premature death of neurons in the brain, as a result of a build up of the excess tau protein1. Causes
Despite the recent advances in the understanding of the biology of PSP, the
cause of the disease is still unknown.
Almost all cases appear to be sporadic, that is to say without a family
background of PSP. It has been suggested that both environmental and
genetic influences may be involved. However, the latest thinking is that there
is a complex genetic component to PSP. Around twenty percent of the UK
The tau protein is the major component of the fibrillary tangles seen in several neurode-generative disorders, known as tauopathies. The accumulation of hyperphosphorylated tau isoforms comprise the abnormal neuronal and glial inclusions. population carry a gene which provides a weak susceptibility to PSP (though with a very low level of risk), but the disease itself appears then to be triggered environmentally and selectively2.
Prevalence
Research shows a prevalence of 6.4 per 100,000 of the population with PSP,
suggesting that there are at least 4,000 people living with PSP in the UK.
Recent epidemiological studies suggest that the disorder is more common
than previously thought, is frequently misdiagnosed, and that the majority of
cases are not initially referred to a neurologist.
There has been no formal research into the prevalence of CBD, but it is thought
by experts to be half as common as PSP.
“In the early stages PSP can mimic Parkinson’s disease and be misdiagnosed as
such. The two disorders have historically been linked.” Professor Andrew Lees
The most common misdiagnoses were Parkinson’s disease and cerebrovascular
disease.
Uma Nath’s study Epidemiology of Progressive Supranuclear Palsy in the UK in
2002 also highlighted the large number of cases of PSP where the diagnosis is
missed or delayed and how the early manifestations of PSP lead to potential
referral to a wide variety of specialists.
Epidemiology
• Average age of onset is 62 years, but it can affect people over 40.
• Median interval between onset and diagnosis is 3 years. • Slight male predominance in most studies. Familial
Fewer than 1% of those with PSP have a family member with the same
disorder. A variant in the gene for tau protein called the H1 haplotype, located
on chromosome 17, has been linked to PSP, but this genetic variation is
common and is not enough to cause PSP on its own.
Golbe and coworkers performed a questionnaire survey of 75 patients with PSP and matched controls. Surveyed exposures included hydrocarbons, pesticides and herbicides, urban/ rural living, occupation, trauma, education level, maternal age and family history of 3 neurological diseases. Patients with PSP were less likely than controls to have completed 12 years of education. The role of heredity in PSP is currently under investigation, but the likelihood of transmitting PSP to one’s children through genetic mutations is very small. If there is a family history of dementia, Parkinson’s disease or PSP, the risks increase by a small degree, but are still not high. To date worldwide there have only been seven reported families with autopsy-proven PSP spanning more than one generation. It is now accepted that there are several subvariants of PSP. Subdivisions were initially recognized from clinical symptoms, but it is now shown that different areas of the brain are involved. Symptoms
There is a large variation in symptoms with each patient, each of which can
occur at any point in the progression of the disease. However, not every
person gets every symptom.
• Poor balance, unsteady gait with reduced arm swing. Later a curving of the spine may develop, especially the neck. • Falls, which are often backwards, and always without warning. Falls often occur within the first year of symptoms developing. • Nuchal rigidity (stiffness in the nape of the neck, often accompanied by pain and spasm on attempts to move the head). • Restricted eye movement with slowing of the up and down gaze, making mobility difficult. Later, restriction of horizontal saccades can make reading, among other things, difficult. Blurred vision, tunnel vision, photophobia can also occur, while eye closure (blepharospasm and apraxia of eye opening) can render the patient essentially blind. Corneal problems may also occur due to reduced blinking. • Difficulty with swallowing, causing choking, weight loss, requiring possible Percutaneous Endoscopic Gastroscopy (PEG) feeding. People with PSP have a tendency to overfill their mouths and forget to swallow. • A constant risk of aspirating, which can lead to aspirational pneumonia. Sialorrhea and drooling also occur due to poor control of saliva and poor lip seal. • Difficulty communicating as speech becomes slurred, strained and • Behavioural changes including apathy and disinhibition often occur. Depression, frustration, anger and difficulty coming to terms with the diagnosis are also features that arise frequently. • A change in the level of cognition with problems understanding reasoning, loss of short-term memory and apathy. On the whole, the capacity to make decisions remains intact, even when communication is difficult. Often the person with PSP will lose the ability to appreciate danger, i.e. will still attempt to walk alone despite frequent falls. Reckless and impulsive behaviour is commonplace. • Later, bladder control is affected and will eventually necessitate long term management. People with PSP will experience severe constipation and will require regular laxatives. Cortico Basal Degeneration
CBD is a related progressive neurological disorder characterised by nerve cell
loss or deterioration and atrophy of multiple areas of the brain. The progression
of the disease may be slower than the progression of PSP.
CBD usually starts on one side of the body first, for example, the loss of use of
one hand, eventually spreading to the other side. It can cause jerky, awkward
movements (myoclonus and apraxia) and sometimes uncontrolled actions
made by the hand (alien limb3). This can make it difficult to do everyday tasks
such as cleaning teeth, dressing or combing hair.
In common with PSP, there may be a disturbance of eye movements, although
it is less striking than in PSP and problems with up gaze and down gaze are
less common.
CBD patients may have difficulties in problem solving and other behavioural
changes. Memory, language and vision can be affected, but not in many
patients. Like PSP, CBD is a very individual disease and the rate of progression
of symptoms varies considerably from person to person.
CBD mainly affects people in their 60s and 70s and symptom management has
much in common with that recommended for PSP.
CBD – similar to PSP except:
• Numbness, jerking fingers, loss of use of one hand. • Asymmetric; progressively affecting arm and leg. • Alien limb. • Less common disturbance of eye movement. • Increased behavioural changes (frontal lobe deficit). • Slower progression generally. Complex unintentional movements of one limb interfering with normal tasks. PSP and CBD are so closely related that we often refer to them interchangeably (usually as ‘PSP’), and The PSP Association supports people living with both. Diagnosis
To date, definitive diagnosis of PSP can only be made by post mortem
examination of the brain. Specialists can make the diagnosis with over 90%
accuracy. A means to achieve earlier and better diagnosis would be of huge
benefit to patients and would enable any new treatments to be administered
at the earliest opportunity. Patients may wait 2-3 years before the diagnosis is
reached.
Initially many of the symptoms present as idiopathic Parkinson’s Disease (IPD)
and it may be some time until their symptoms develop leading the specialist
to suspect PSP. The neurologist may allow further time to elapse before being
able to give a probable diagnosis.
Imaging techniques, including positron emission tomography, single photo
emission computed tomography and magnetic resonance spectrometry, have
improved the accuracy of diagnosis of parkinsonian syndromes. Magnetic
resonance imaging has shown atrophy of midbrain structures in PSP and also
of the pons. There may be some demyelination and gliosis of the superior
cerebellar peduncle in PSP. At present, however, an MRI scan will usually be
used to rule out other possible diagnoses.
Treatment
There is currently no treatment to stop or slow down the course of PSP, or to
cure PSP. Management will be based around symptom control and quality of
life.
The Management of PSP
The multi-disciplinary team offers the best approach to management and
works towards improving quality of life. For most patients it helps if the GP
refers to the following team members once a possible diagnosis has been
made by the neurologist:
• If the neurologist has not already referred the patient to the local Parkinson’s Disease Nurse Specialist (PDNS), the GP should do this. The PDNS will usually cover PSP and CBD within their role. They will have knowledge of medication usage, be able to advise and monitor any changes and also know which services are available to the person with PSP/CBD locally. Some people will also benefit from support at local PD groups. • Referral to the PSP Association will enable the person to access a telephone helpline, information and specialist care advice, to gain an awareness of their illness and support to cope. Joining the Association will also allow their details to be stored on the database for future research/drug trials. They will also receive invitations to their nearest PSP support group. • Early referral to a Physiotherapist (PT) (neuro physiotherapist if available) will allow gait, balance and falls education, as well as advice regarding bed/ chair transfers, walking aids and wheelchair services. • Referral to an Occupational Therapist (OT) as required for assessment of functional ability, adaptations to the home and garden, aids for eating, bathing, etc. Stairs are a particular hazard when eye movements are affected.
• Early referral to a Speech & Language Therapist (SLT) who will be able to advise on communication needs and also assess swallowing difficulties. As silent aspiration can become an issue the team should be guided by the regular input and advice from the SLT. • Referral to a Dietician who can advise on calorific intake (weight loss is common) and types of foods to avoid/eat safely as dysphagia worsens. The Dietician will liaise with the SLT if PEG feeding becomes necessary and the person with PSP chooses this option. • Referral to the Community Matron (CM) who will often case manage the complex issues of the condition, with support from the MDT. Where there is no CM referral should be made to the district nurse for support and case management. • Referral to an Orthoptist or eye specialist. As the ocular function itself is unaffected, it is not always necessary to refer to an optician. However, other ocular problems (cataracts, glaucoma) must not be ignored. The orthoptist can advise on the effectiveness of prism glasses. Some patients, especially those with CBD, may qualify for a certificate of sight impairment, allowing access to talking books, etc. The GP can treat any infections of the conjunctiva and the use of artificial tears is helpful with the reduced blinking in PSP. • As the illness progresses, referral to the Continence Nurse Advisor will be beneficial to allow the person with PSP support to manage their bladder dysfunction (urgency/frequency/nocturia/retention) and constipation which can be severe. N.B. Early bladder involvement may indicate a diagnosis of Multiple
System Atrophy (MSA4) rather than PSP.
• The Social Worker can assist with a needs assessment and carer assessment and then help to provide an appropriate package of care. As the illness progresses they can also advise on regular respite periods or suitable long term nursing care homes if this becomes necessary. • Referral to a Community Psychiatric Nurse (CPN) may be required if the person with PSP develops behavioural problems or signs of early dementia. Hallucinations and psychotic episodes can also occur, although often in association with medications or infection. Unresponsive depression may require a referral to a psychiatrist or a counsellor. • Palliative / Hospice Outreach Team. As the illness progresses, respite for the carer becomes necessary. As many care homes will be unfamiliar with the person with PSP/CBD’s needs, respite periods can vary in success. Fortunately, hospices now cater for long term neurological conditions, and regular respite periods can thus ensure an excellent standard of care for the person with PSP, and offer essential support for the carer. When end of life care is then required, the person with PSP and their families will be better supported by staff they already have a rapport with and who understand the complexities of a neurodegenerative illness. They are also skilled in broaching end of life issues and assisting people to consider their end of life wishes, so early referral to the palliative team can be very helpful. Medication
There is no specific treatment for PSP, but the medications used for Parkinson’s
disease are often prescribed by the specialist in movement disorders. For
around one third of people there will be limited symptom improvement. In
general, they do not provide dramatic or long lasting improvements and can
cause unwanted side effects.
Levodopa
Sinemet /Madopar is often tried in the early stages and may also be used
to rule out Pakinson’s disease. Small doses are tried and then titrated up
gradually to prevent side effects; commonly nausea for the first few weeks,
which can be improved with domperidone (other antiemetics may make the
extra pyramidal symptoms worse).
MSA is a similar movement disorder characterised by parkinsonism, autonomic
dysfunction and cerebellar problems. www.msaweb.co.uk
Levodopa may help with initiation of movement, muscle rigidity, and
bradykinesia. If it doesn’t benefit the person with PSP, it can be reduced slowly
and discontinued, although a trial of at least 3 months should be allowed.
Dopamine agonists
Drugs such as Mirapexin (Pramipexole) and Ropinirole (Requip) may be tried
to improve the parkinsonian symptoms. This is very individual and it may
take some time to find a working dose. These medications may be used in
conjunction with Levodopa. Rotigotine (neupro) patches may be useful when
swallow is impaired.
Amantadine (Symmetrel)
Amantadine is considered by many neurologists to be one of the best
treatments available for PSP. It can be helpful for improving balance and
preventing falls. There is no research based evidence for Amantadine but
anecdotal studies show it is beneficial in some cases. It can be used in
conjunction with Levodopa or dopamine agonists. In some patients it can
cause hallucinations which are dose dependant.
Amitriptyline
This can often be helpful for improving sleep, lifting mood and can also be
beneficial for pain from muscle rigidity. A small dose at night can be increased
slowly as required. This may also help with drying up secretions. Higher doses
may lead to confusion or cognitive dysfunction.
Atropine
In the form of eye drops used sublingually, this can be very helpful with
sialorrhea and is often the first line of treatment. (N.B. it is not licensed for
use in the mouth). Normal dose is Atropine 0.5% two drops t.d.s. If not
effective, Glycoprronium (Glycoprrolate) can be used in tablet form (or in liquid
for a PEG) to aid sialorrhea. The use of hyoscine should usually be avoided as
this can often cause confusion and hallucinations in PSP/CBD.
For severe sialorrhea, the patient may consider Botulinum toxin injections
to the salivary glands, but this is usually performed by an ENT surgeon and
therefore may not be available in all areas.
Botulinum Toxin
Often offered by neurologists in a specific botox clinic, this may be beneficial
for involuntary eyelid closure. It may last up to 4 months and therefore needs
to be repeated regularly. It may not be effective in all cases.
Painful or disabling Dystonia in some neck muscles, hands and feet may also
respond to botox injections.
Muscle relaxants
These may be helpful for muscle rigidity, the commonest used are Baclofen
10mg t.d.s. and Clonazepam 0.5mg nocte.
Clonazepam may also benefit sleep disorders.
Gabapentin may be of benefit for neuropathic pain. However, this is difficult
to treat, and it may be necessary to refer back to the neurologist and to a pain
clinic if required.
Laxatives
Will be required on a daily basis for the majority of people living with PSP.
Despite a healthy high fibre diet, constipation will be a common symptom,
often severe. Movicol would be used initially, with other laxatives added as
required. Parkinson’s disease medications will be ineffective if the person with
PSP is constipated.
Pain Control
Although not common, pain relief may be required at end of life.
Diamorphine will often be used by the palliative care team if anxiety is
present.
Difficult Conversations
In many instances, difficult conversations about end of life issues are put off
by families as being too difficult to address. The PSP Specialist Care Advisers
frequently take calls from distressed family members who have not felt able to
discuss end of life issues and who are now faced with advocacy for the person
with PSP, often not knowing what their wishes are. Often the professional will
need to broach these subjects whilst the person with PSP can still make their
wishes clear as verbal communication can become increasingly difficult as the
disease progresses.
Personal Choice
For many people with PSP the time will come when they lose the ability to
swallow safely and following assessment by the SLT, the neurologist may
suggest PEG feeding. The person with PSP should be supported to make an
informed decision regarding this. For many, once artificial feeding is in place,
they will no longer struggle to get adequate nutrition and hydration and may
indeed gain weight. For others, they may decide their quality of life is so poor
that they no longer wish to prolong their life with artificial feeding. Patients should be supported in either decision. If a PEG is declined by the person with PSP, it is often the family members who have difficulty accepting this decision. It is also important to know the individual’s wishes with regard to admission to hospital, treatment with antibiotics, and resuscitation measures. As it is so difficult for a spouse or family member to act as an advocate without knowing the person’s wishes, the PSP Specialist Care Advisers try to encourage discussions early on. This enables the person living with PSP to consider advance decisions and enduring power of attorney. The PSP Association has information leaflets on advanced directives (living wills) and the PSP Specialist Care Advisers can discuss this with the patient and their family.
Cognition and Behaviour
Cognitive dysfunction and personality changes are common, but they are not
considered to be a ‘true dementia’. There is some debate regarding this.
Steele Richardson et al describe:
• slowing of thought processes, emotional or personality changes (apathy or depression with occasional outbursts of irritability), • impaired ability to manipulate acquired knowledge. In many neurological disease states associated with subcortical pathology a similar pattern of dementia exists. The neurobehavioural changes of progressive supranuclear palsy thus typify a clinical pattern which may be referred to as subcortical dementia. PSP patients can experience cognitive and behavioural changes suggesting abnormal functions in the frontal lobes. Cognitive changes consist of a decline in frontal lobe functioning, such as slow information processing and retrieval, concrete thinking, impaired reasoning and difficulty in planning tasks. Behaviourally, patients often exhibit apathy, leading to decreased motivation. Family members can find the personality changes very distressing and often state this as the most difficult symptom to deal with. Key Issues: • A delayed response• Poor judgement• Lack of self awareness • Difficulty in organisational skills and sequencing • Lack of motivation• Lack of empathy.
These are generally more prominent later in the disease course. Most people living with PSP express a sense of isolation when first diagnosed. As many professionals will not be familiar with PSP the person can feel very uncertain and alone. When coming to terms with the diagnosis and changes to the life they may have planned for themselves they can experience a sense of loss and grief. Antidepressants and anxiolytics can help ease these symptoms and may also help with the emotional lability. As one’s intellect remains largely intact, the person with PSP will be aware of their general deterioration and will naturally be very frightened, however they also have a lack of awareness regarding their abilities and will continue to attempt to walk unaided. Loss of higher intellect, reckless behaviour and the added problem of not being able to look down, all contribute to the possibility of falls and injury. People with moderately advanced PSP are at higher risk if left alone due to the risk of falling or choking. End of Life Care
Although PSP has an individual rate of progression, deterioration is inevitable
and the person living with PSP may eventually become immobile, with severe
communication difficulty, poor swallow, restricted vision, incontinent, yet with
their intellect largely intact. It is very difficult to give an accurate individual
prognosis.
For most people end of life will occur within 5 to 9 years from the onset of
symptoms, but there is very wide variation in life expectancy.
Frequent bouts of infection (UTI or pneumonia) with poor recovery in
between, increased apathy, increased periods spent sleeping, weight loss,
worsening bulbar symptoms (speech and swallowing), may all indicate
progression of the disease. Often infections are difficult to diagnose as pyrexia
or other symptoms may not always be present, but early treatment with
antibiotics is crucial to be effective.
Many people living with PSP (and their families) fear that their end of life will
be caused by a traumatic choking episode and may be painful. This is rarely
the case and they require reassurance regarding this. The commonest cause
of death is from aspiration pneumonia, which can be peaceful and pain free if
managed well. Towards end of life, anxiolytics and analgesia may be required.
Involvement of the palliative care team can ensure that those who remain alert can have appropriate medication to ease any anxiety and pain relief if necessary. PSP should be included on the GP palliative care register and included as part of the Gold Standards Framework. Support for Carers
Being a carer for someone with a long term condition can be overwhelming.
There can be many strains upon the relationship especially where
communication and empathy from the patient is restricted or cognition issues
are present. Carer stress is common and they may experience any of the
following:
• guilt
• disbelief
• helplessness
• anger
• depression
• sleep disturbance
• inability to concentrate
• weight loss/gain
• inability to cope
• anxiety about loss of income
• embarrassment
• lack of knowledge
• exhaustion.
Often the carer will require psychological support (and even sometimes
antidepressants) in order to cope with the illness. Regular support and respite
periods will be necessary in order for the carer to continue to deal with
supporting the person living with PSP/CBD. Professional counselling may also
be required.
Referral to support groups, carers groups and local organisations may be
beneficial and may make the carer feel less isolated. Hospices do not treat a
patient in isolation and a lot of the services they offer to the person living with
PSP/CBD are available to their carers also.
Social Services should carry out a carer’s assessment to ensure their needs are
being catered for.
Many of the carers will not ask for help until they reach crisis point; GP’s
and Community Matrons are in a position to prevent this. Carers should
be registered as carers on the practice register and be given priority for
treatment. Most people living with PSP should be booked for double
appointments due to slowness of speech and movement. Home visits will be
necessary as the disease progresses.
N.B.Where the term ‘carer’ is used throughout the text the author is
referring to the main family member caring for the person with PSP,
rather than the paid carer/professional.
Brain Donation
Brain Banks have been established to collect tissue samples and whole brains
donated by people with PSP and others with neuro-degenerative disease.
Examples include the Queen Square Brain Bank in London and the Cambridge
Brain Bank. These are an invaluable resource for research which will enable
researchers to better understand what causes PSP/CBD and how the diseases
progress. In the long term, this will enable us to develop a much needed
diagnostic test and an effective treatment.
The Sara Koe PSP Research Centre (SKRC), the first dedicated PSP Research
Centre in the world, was established in January 2002 at the Institute of
Neurology in London. The SKRC’s role is to co-ordinate PSP research within the
UK, provide support to those involved in PSP research and to act as a link with
PSP research worldwide.
The SKRC administers a PSP DNA and Brain Bank and is closely linked to the
Queen Square Brain Bank which stores the brains of people who have died
from various neurodegenerative diseases as well as the brains from disease
free donors.
People living with PSP will often enquire about brain donation, as will their
family members who wish to do something positive to help with research.
In the first instance they can discuss this with their specialist, or call the
telephone number below for information and support.
Queen Square Brain Bank Contact Details
Susan Stoneham Tel: 020 7679 4266 or 0207 837 8370.
Cambridge Brain Bank Contact Details
Beverley Haynes / Jenny Wilson Tel: 01223 217336
Other local brain banks can be located at:
www.hta.gov.uk/donations/howtodonateyourbody/donatingyourbrain.cfm
Future Treatments - Stem cell treatment
For many people with neurodegenerative diseases, including PSP, the potential
of stem cell treatment lies in the possibility that it could lead to ways of
producing new neurons to replace those lost in the brain through the PSP/CBD
process. Whilst exciting, stem cell research is still in its infancy and there is a long
way to go before treatments are available.
It is important that this is recognized as all too often stem cell research is
overhyped and misrepresented in the media. This gives false hope to those who
find themselves in very desperate situations.
Research
The PSP Association is the only UK organisation dedicated to funding research
which aims to:
• develop a better understanding of the causes of PSP
• establish the best ways of caring for people with PSP
• develop an effective treatment for PSP
• find a cure for PSP.
Ongoing Research at The PSP Association
• Control of tau gene expression.
• High field quantitative Magnetic Resonance Imaging in PSP.
• Randomized placebo-controlled trial of valproic acid in patients with PSP.
• Cerebrospinal fluid biomarker discovery in PSP and related tauopathies.
• Haplotype regulation of alternative splicing at the MAPT locus.
Useful Resources
Carers UK Equal Partners
Tel: 0808 808 7777 Web: www.carersuk.org
Counsel + Care
Tel: 0845 300 7585 Web: www.counselandcare.org.uk
Carers Trust
Web: www.carers.org/ Tel: 0844 800 4361
Scotland (The Princess Royal Trust for Carers) Tel: 0141 221 5066
Cure PSP
Web: www.psp.org
Department for Work and Pensions
Web: www.dwp.gov.uk/directgov
Disabled Holiday Directory
Tel: 01348 875592 Web: www.disabledholidaydirectory.co.uk
National Care Forum
Tel: 024 7624 3619 Web: www.nationalcareforum.org.uk
National Care Association
Tel: 020 7831 7090 Web: www.nationalcareassociation.org.uk
NHS Carers Blog
Web: http://talk.nhs.uk/blogs/carers/default.aspx
NHS Carers Direct
Tel: 0808 802 0202 Web: www.nhs.uk/Carersdirect/Pages/CarersDirectHome.
aspx
Remap Charity
Tel: 0845 130 0456 Web: www.remap.org.uk
Respite Care
Tel: 0845 644 4932 Web: www.bettercaring.com
Telecare Services Association
Tel: 01625 520320 Web: www.telecare.org.uk
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Williams D R, de Silva R, Paviour D C, Pittman A,Watt H C, Kilford L, Holton J L, Revesz T, Lees A J.The Queen
Square Brain Bank for Neurological Disorders, University College London, UK, (Jun 2005) Characteristics of
two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson’s
syndrome and PSP-parkinsonism. Brain 128(Pt 6):1247-58.
Williams D R, Holton J, Strand C, Pittman A, de Silva R, Lees A J, Revesz T.The Queen Square Brain Bank for
Neurological Disorders, University College London, UK, (2007) Pathological tau burden and distribution
distinguishes progressive supranuclear palsy-parkinsonism from Richardson’s syndrome. Brain
130(6):1566-1576.
Further Reading
www.brt.org.uk/dr-rohan-de-silva
www.brt.org.uk/prof-john-hardy
The PSP Association
PSP House,167 Watling Street West
Towcester, Northants NN12 6BX
Website: www.pspassociation.org.uk
Helpline: 0300 0110 122
The PSP Association is a Company limited by guarantee Registered number: 2920581 Registered Charity numbers: England and Wales 1037087 / Scotland SC041199 Registered Office: PSP House, 167 Watling Street West, Towcester, Northamptonshire NN12 6BX

Source: http://www.pspassociation.org.uk/wp-content/uploads/2012/07/For-Professionals-Resources-Publications-and-Training-A-Guide-for-GPs-download.pdf?e21992