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Logopenic Progressive Aphasia: A rare dementia

What is dementia?
Dementia is a general term used to describe a group of symptoms that can affect people’s memory, thinking skills, language, communication and mood. These changes mean that over time people start to have difficulties with day to day tasks. The symptoms of dementia get worse over time so people will start to need more and more support with every day life. There are many different types of dementia.

What is Logopenic Progressive Aphasia?
Logopenic Progressive Aphasia (LPA) is a rare type of dementia. In this condition people’s language and communication skills are affected first. This is different from more common types of dementia where the first sign is usually a change in somebody’s memory. The term Logopenic comes from a combination of two ancient Greek words: logo meaning speech and penic meaning ‘lack of’. Progressive refers to the fact that the disease gets worse over time. Aphasia is a word that also refers to changes in a person’s speech. LPA is one of three language based dementias. Sometimes these dementias are grouped together and called Primary Progressive Aphasia (PPA).


What are the symptoms?
Because this condition causes damage to the parts of the brain that control language most of the symptoms that people notice first are related to their speech and ability to communicate. As LPA progresses people start to develop more general symptoms typical of other types of dementia. Early symptoms
• Speaking more slowly, with frequent pauses.
• Lots of ‘tip-of-the-tongue’ moments.
• Substituting a word with something that sounds similar but isn’t a word. For example saying ‘lelephone’ instead of ‘telephone’.
• Difficulty repeating phrases or sentences.
• Using new terms that aren’t real words.
• Making more spellings errors when writing.
• Difficulties with everyday calculations.
• Becoming easily distracted or finding it harder to concentrate.
These changes get worse over time so it becomes harder for people to express themselves or say what they need. It is difficult to say how quickly this will happen as dementia affects everyone differently.
Later symptoms
• Memory change
• Confusion about the date or day of the week
• Getting lost or disorientated
• Changes in mood or behaviour
• Difficulties performing day to day tasks

Towards the end of their life someone with LPA will need increasing help and support.

What causes Logopenic Progressive Aphasia?
LPA is caused by shrinkage (atrophy) and the build up of abnormal proteins in the parts of the brain responsible for controlling speech and language. Most often these changes are caused by the same process in the brain as the ones seen in Alzheimer’s Disease. However, because a different part of the brain is affected first, the early symptoms are very different. Over time, as these changes begin to affect
other areas of the brain, a person with LPA will start to develop symptoms similar to that of someone with Alzheimer’s Disease. Very rarely LPA is caused by changes related to a different type of dementia, called Frontotemporal Dementia. People with LPA caused by Frontotemporal Dementia are more likely to have early changes in their behaviour and mood.

Is there any medication that can help?
There is no medication available for LPA that will cure the disease, slow it down or stop it from getting worse. However we do have some medications called acetylcholinesterase inhibitors, which can be helpful at boosting a chemical (acetylcholine) in the brain that supports memory and thinking skills. Although these medications do not undo the changes in the brain or slow them down, for some people they can temporarily reduce some of the symptoms of dementia and help a person to function slightly
better day to day. The improvements from this medication are usually fairly mild. They do not work for everyone and it is difficult to say exactly what benefit you would get from them until you tried them.

Taking medication
Acetylcholinesterase inhibitors can be prescribed if your doctor believes the type of LPA you have is due to the same process as Alzheimer’s Disease.
There are three types of acteylcholinestarse inhibitors:
• Donepezil (Aricept) - this is the most commonly prescribed tablet for LPA.
• Rivastgimine (Exelon)
• Galantamine (Reminyl)
All these tablets work in the same way and although most people tolerate them fairly well, they can sometimes give people sideeffects. Your doctor will talk to you more about these before you
take the medication. If your doctor believes that the type of LPA that you have is caused by Frontotemporal Dementia they will not prescribe you any medication. This is because the medications we have available do not work for this type of dementia and may make the symptoms worse.

What else can be helpful?
Research has shown that keeping the brain active in ways that you enjoy can help to enhance brain health. This might involve activities such as socialising, playing board games, completing puzzles or playing music. Gentle regular exercise, getting enough sleep, not smoking and reducing alcohol intake are other important ways to keep the brain healthy.

Your GP will also review your physical health to ensure that illnesses that affect the brain, such as diabetes, high blood pressure and high cholesterol are treated as best as possible.

Genetics
LPA can be sporadic, familial or inherited. In sporadic cases the disease occurs out of the blue. In familial cases genetic abnormalities play a minor role in the risk of developing the disease, whereas in hereditary forms genetic mutations have a significant impact.

It is very rare for LPA to be hereditary but if you are concerned about this you can discuss it more with the team.

Driving with Logopenic Progressive Aphasia
As with all types of dementia it is your legal responsibility to inform the DVLA and your insurance company of your diagnosis. This is because having dementia can affect a person’s ability to drive. However this does not necessarily mean they will automatically stop you driving, as lots of people with dementia are able to drive safely. Once you have informed the DVLA they will write to you and your doctor to collect as much information as possible before making a decision.

If you have any worries about driving this is something you can talk to us more about.
DVLA Medical Enquiries: 0300 790 6806
(Monday - Friday: 8am to 7pm, Saturdays: 8am to 2pm)

Help and support
During your time with the Early Intervention Dementia Service we will talk to you more about support that is available and help you to access services that may be useful to you. We run regular educational groups designed to help you learn more about dementia and offer practical advice on managing symptoms.

You may also be referred to a Speech and Language Team (SALT) for strategies to help you manage your speech change.

Our contact details:
Early Intervention Dementia Team
F Block, Kidderminster Hospital
01562 828815
Monday - Friday, 9am to 5pm

Other resources
Other resources that may be helpful include:
Worcestershire Social Services, Adult Social Care Team Telephone: 01905 768053
Website: www.worcestershire.gov.uk/contactus

Worcestershire Admiral Nurse Service with Herefordshire and
Worcestershire Health and Care NHS Trust
Email: WHCNHS.admiralnurses@nhs.net
Telephone: 0300 123 1734

Rare Dementia Support
Website: www.raredementiasupport.org

Dementia UK
Dementia Helpline 0800 888 6678

Age UK Dementia Wellbeing Service
Telephone: 0800 008 6077
Website: www.ageuk.org.uk

Regional Driving Assessment Centre
Telephone: 0300 300 2240
Website: www.rdac.co.uk