Understanding Posterior Cortical Atrophy (PCA) Dementia

What is Posterior Cortical Atrophy (PCA) dementia?

PCA dementia stands for Posterior Cortical Atrophy, which is a rare neurodegenerative condition thought to affect less than 5% of people with Alzheimer’s disease. However, some studies suggest this condition is often under-recognised and therefore in reality there could be more cases, possibly in as many as 15% of people with Alzheimer’s disease. PCA dementia is considered to be a visual variant of Alzheimer’s disease, and is unique in that it impacts the posterior area of the brain which is responsible for visual processing, causing the brain to have issuesinterpreting informationsent from the eyes. Unlike typical dementia, this type usually begins with visual symptoms instead of memory loss, so those with PCA dementia may start to experience difficulties with reading, spatial awareness, and recognising objects or faces. Over time, cognitive impairments extend to other areas and start to further impact daily functioning in ways more commonly known in dementia, such as memory issues. Age is the most prominent risk factor for dementia, andPCA dementia typically affects those between the ages of 50 and 65. However, a person’s risk of developing PCA is similar to other types of dementia, such as environmental and lifestyle factors. There is also thought to be a potential genetic risk, but more research is needed to determine the validity of this, and to what extent it may be a factor. 

How does PCA dementia affect the brain, and what causes it to happen?

Posterior Cortical Atrophyprimarily affects the posterior part of the brain, which is responsible for visual processing and spatial awareness, hence visual symptoms being the most prominent. The condition is characterised by the gradual degeneration of cells in this area of the brain, which is similar to the deterioration seen in Alzheimer’s disease with a slightly different progression. The cause of PCA dementia remains unclear, but it is thought to be linked to abnormal protein deposits, including amyloid and tau which cause plaques and tangles in the brain. Studies find that while this is similar to those found in Alzheimer’s disease, there is more of a focus on the primary visual cortices and visual association areas in PCA dementia. This is because initial damage occurs in the functions controlled by the rear sections of the brain.

How does PCA dementia differ from other dementia types?

It is primarily the brain regions affected and the onset of early visual symptoms that set PCA apart from other variations of dementia. While typical dementia begins with memory loss, PCA will usually begin with visual and spatial impairments due to degeneration in the brain’s posterior regions, despite sharing similar underlying pathological features. Uniquely, PCA dementia patients can sometimes retain their memory and language skills throughout the early stages, which often leads to missed diagnoses.PCA dementia is also a lot less common than other types of dementia, and may occur in younger age groups. For example, Alzheimer’s disease is thought to affect 1 in 14 people aged 65 and over, and 1 in 6 people aged 80 and over, while Lewy body dementia (LBD) is thought to affect around 10-15% of all dementia cases, primarily impacting those aged 65 and over. Although it is unclear the true number of PCA dementia cases, it is believed to affect less than 5% of people with Alzheimer’s disease, and studies find PCA dementia usually presents in younger age groups, with 83% occurring in those aged under 65. However, some patients can be affected when they are as young as 40 or as old as 90. You can learn more about the types of dementia in our guide to the different types of dementia.

How does PCA dementia progress?

PCA dementia tends to progress gradually, beginning with visual and spatial difficulties. In the early stages patients may notice symptoms such as having trouble reading and recognising objects. As the condition advances, visual impairments usually worsen, and patients could experience an increased difficulty with coordination and motor skills, and eventually cognition may decline to the extent that their memory, language and executive functions are affected. Throughout this time, a person with PCA dementia may notice daily activities become increasingly challenging, but a focus on independence can help to maintain quality of life. Studies find patients could present with other symptoms such as trouble with simple mathematics, trouble writing, disorientation, and more. Over time, the cognitive symptoms associated with PCA dementia could lead to functional blindness which can cause patients additional distress on top of their other symptoms, as well as depression. In these cases, home care (such as live-in care) could provide necessary assistance and emotional support to improve a person’s quality of life. You can learn more about the general progression of dementia in our guide to managing the stages of dementia.

How is PCA dementia diagnosed and treated? 

PCA dementia may be more difficult to diagnose due to the early symptoms suggesting possible vision problems could be to blame, rather than dementia-related issues. As a result, often people with PCA dementia will have their condition missed by clinical evaluations or misdiagnosedas a visual or psychological issue. Studies suggest there is often an average delay in diagnosis of around 4 years, with symptoms present for months or years before the patient is connected with the relevant clinical staff. If you suspect you or a loved one may be experiencing symptoms of any type of dementia, it is important to speak to your GP and discuss signs you have noticed, so they can explore a possible dementia diagnosis. If PCA dementia is suspected, it may bediagnosedthrough a combination of clinical assessments, neuroimaging, and cognitive tests. A neurologist may assess visual and spatial abilities, and review medical history, or a neuro-ophthalmologist may be brought in as a specialist in neurology in relation to vision. Other tests may be carried out, such as psychological tests to determine the person’s mental status, or blood tests to find out if there are any vitamin deficiencies, thyroid disorders, or something else causing the symptoms. The person may also be referred for MRI, CT or PET scans to help identify any brain atrophy, particularly in the posterior regions often affected by PCA dementia, and occasionally spinal fluid tests may be done to measure amyloid and tau proteins. There is no cure for PCA dementiaor treatments to slow its onset, but it has been suggested certain medications used to manage the symptoms of Alzheimer’s disease may also be beneficial for PCA patients due to their similar makeup. However, more research is needed to determine how effective this may be. Some other treatments can focus on managing symptoms to offer relief, such as reducing symptoms of depression or anxiety common with a dementia diagnosis, or visual aids to help with processing difficulties. 

What else can someone with PCA dementia do to make their life easier?

Symptoms of PCA dementia will continue to progress, so someone with the condition could benefit from making early changes to their environment and utilising various assistive aids. This can help to adapt their lifestyle to fit with their symptom onset and ensure they maintain independence in their home environment for as long as possible. Here are some things that could help: 

• Begin engaging in cognitive and visual exercises to help stimulate brain function, such as games and activities for dementia patients
• Usetailored assistive aidsand technologies, like screen readers and magnifiers, to help with reading or performing daily tasks
• Create a structured, clutter-free environment that makes moving around easier for someone with visual difficulties, reduces confusion, and enhances safety – you can learn more about the best ways to do this in our guide to home adaptations for disabled older people
• Engage in regular physical activity and eat a healthy diet with plenty of nutritional foods and adequate hydration to support overall wellbeing
• Join support groups providing emotional support and practical advice for those going through dementia 
• Create a structured routine to make working with healthcare professionals easier, such as keeping a calendar of upcoming appointments with occupational therapists and neurologists
• Consider what can be done to create tailored interventions that have a positive impact on the person’s symptoms, rather than employing one-size-fits-all strategies
• Enlist the help of home care services to provide adaptable levels of care as the person’s condition progresses, such as companionship, personal care assistance, housekeeping, specialist dementia care, and more

Why is home care helpful for people with PCA dementia?

For those with any type of dementia, home care can offer both physical and emotional support as their condition progresses, while allowing them the safety, companionship and assistance they need to remain in the home they know and love. This is particularly helpful for those with PCA dementia where visual issues may be a key symptom. The person may prefer to remain in a familiar environment where they can learn to easily move around safely and live their life in a place they know well to reduce confusion and anxiety. There are many home care options available (you can read more in our guides to what home care is and the benefits of home care vs a care home). At Home Instead, we can offer personalised care plans tailored to the individual needs of you or your loved one, to help manage daily activities, enhance safety, and promote independence as much as is possible throughout the progression of dementia. If you arecaring for someone with dementia at homeand wondering if home care could be helpful, our Care Professionals can assist with everything from creating visual aids, to navigating the environment, to improving communication, which are all important for PCA patients experiencing visual and spatial challenges. In addition, we can help with medication adherence, monitor health changes to facilitate timely medical interventions, and provide emotional support and companionship to help with feelings of isolation, so no one with PCA dementia needs to feel alone with their diagnosis. Ultimately, home care can help to promote a better quality of life for dementia patients, and support them along with their families throughout the progression of their condition.If you are looking into specialist care for dementia, you may want to ask a home care provider questions such as:

• How can my/my loved one’s environment be adapted to help with symptoms?
• Will I/my loved one receive a carer with specialist training and experience in dementia? 
• How often can I/my loved one receive specialist dementia care?
• Can I/my loved one receive respite care? 
• Do I/does my loved one need a live-in carer, and is this an option?
• Can care adapt as my/my loved one’s symptoms progress through the different stages of dementia? 

At Home Instead, we offer specialist, person-centred care, meaning as long as the person is able to make decisions about their own care and how they spend their time, our Care Professionals will respect their wishes provided they are safe and well. They will also provide a high standard of dementia-focused care through our unique training programme created by ageing experts, dementia specialists and key medical practitioners from across the world. This training is assured by City & Guilds, so you can be confident you are in safe hands. You may find more helpful information in our guide on how to choose and arrange home care services.We’re an award-winning home care provider and part of a worldwide organisation devoted to providing the highest-quality relationship-led care for older people in their own homes. Arranging care for yourself or your loved one shouldn’t be stressful, so whatever questions you would like answered, feel free to reach out to the Home Instead team to discuss your needs.