Posterior Cortical Atrophy

What is posterior cortical atrophy?

Posterior cortical atrophy is a rare, progressive neurodegenerative disorder. In posterior cortical atrophy, you lose neurons (brain cells) in the part of your brain that manages what you see. Early symptoms include vision issues, like having trouble reading, bumping into things or judging distances. As the disease gets worse, you may develop other symptoms, including memory loss.

Neurodegenerative diseases like Alzheimer’s disease and Lewy body dementia may cause posterior cortical atrophy. Like those diseases, there’s no cure for posterior cortical atrophy. Healthcare providers treat it by determining the underlying cause so they can recommend medication and other treatments that’ll reduce symptoms and help you manage your symptoms.

What are the symptoms of posterior cortical atrophy?

Posterior cortical atrophy symptoms typically start when you’re in your 50s and 60s. The condition affects the posterior cerebral cortex, which includes your right and left parietal and occipital lobes.

These brain regions build the world you see from the information received from your eyes. They’re the reason you understand and recognize what you see.

If you have this condition, you’re losing brain cells in your posterior cerebral cortex so that it shrinks (atrophies). When that happens, your brain doesn’t make sense of what you see, making it hard for you to:

• Read text. When you try to read, you keep losing your place on the page because lines of text seem to move around on the page. Words and letters may look jumbled together. Some people describe a sensation or perception that words are sliding off the page as they try to read.
• Judge distances, like the space between steps on a flight of stairs or between your car tires and the curb. You may feel anxious about using an escalator because you can’t figure out when it’s OK to place your foot on the moving escalator step.
• Follow directions because you can’t tell your right from your left.
• Recognize faces, objects or places.
• Avoid bumping into doorways, furniture or other objects because you don’t appropriately judge where they are even though they might be right in front of you.
• Deal with numbers. For example, you know your best friend’s phone number, but you can’t call them because you can’t find the numbers on your phone.
• Use tools and appliances because the on-and-off switches aren’t located where you “see” them.

Anxiety is another common early symptom of posterior cortical atrophy. Some researchers suspect the condition may affect the brain networks that integrate sensory, emotional and cognitive information. Tests show these networks change in early-stage cortical atrophy and could cause symptoms like anxiety.

Another theory is people feel anxious because they sense there’s something wrong, but they don’t know how to describe their symptoms. Either way, unusual anxiety is a symptom healthcare providers look for when diagnosing posterior cortical atrophy.

What are the complications of posterior cortical atrophy?

Posterior cortical atrophy is a progressive disease, which means early symptoms get worse and you develop new symptoms. Complications include:

• Vision loss (functional blindness).
• Depression and anxiety.
• Increased risk of falling because the condition can affect your balance.
• Increased risk of accidents around the home. You might burn your hand on a stovetop heating element because you thought you were reaching for the control panel but touched the hot spot instead.

What causes posterior cortical atrophy?

It usually happens because people have an underlying neurodegenerative disease. Most people with posterior cortical atrophy also have Alzheimer’s disease or Lewy body dementia. Very rarely, people have other underlying conditions, including:

• Creutzfeldt-Jakob disease.
• Corticobasal degeneration.

Is posterior cortical atrophy a kind of Alzheimer’s disease?

Yes, it is. Even though we typically think of Alzheimer’s disease as having early memory problems, early-stage Alzheimer’s disease can affect parts of your brain that don’t manage memory. Healthcare providers may call this an atypical variant of Alzheimer’s disease. Posterior cortical atrophy is one of the atypical variants of Alzheimer’s disease.

How is posterior cortical atrophy diagnosed?

Healthcare providers may use the following tests to diagnose posterior cortical atrophy:

• Brain MRI (magnetic resonance imaging).
• Eye exam to rule out conditions that affect your eye.
• Neurological exam.
• Neuropsychological assessment.
• PET scan (positron emission tomography scan).
• Spinal tap (lumbar puncture).

What are the treatments for posterior cortical atrophy?

Healthcare providers treat posterior cortical atrophy by diagnosing and treating the underlying causes — Alzheimer’s disease or Lewy body dementia. They may use medications that the U.S. Food and Drug Administration has approved for use in treating those conditions. These medications help to slow down the disease.

Providers may emphasize programs and services that focus on cognitive rehabilitation, occupational and physical rehabilitation for coping strategies and mental health support.

Medications

You may receive one of the following:

• Donepezil (Aricept®).
• Rivagstigmine (Exelon®).
• Galantamine (Reminyl®).
• Lecanemab (Leqembi ®)

If you experience anxiety or depression, you may receive anti-anxiety medication or antidepressants.

What can I expect if my loved one has posterior cortical atrophy?

You can expect their symptoms to get worse over time and eventually develop symptoms that are very similar to symptoms of advanced Alzheimer’s disease.

What is the life expectancy for someone with posterior cortical atrophy?

The life expectancy for someone with posterior cortical atrophy is about the same as it is for someone with Alzheimer’s disease. Most people with Alzheimer’s disease live 10 to 12 years after they first have symptoms. But It’s important to remember that life expectancy estimates are based on the average person with Alzheimer’s disease. Your situation may be different. Ask your healthcare provider what you can expect.

How do I take care of my loved one who has posterior cortical atrophy?

The first step is understanding how the world looks when seen through their eyes. For example, they may run into a table because they can’t “see” it. Their eyes alerted their brain about the table, but their brain didn’t react as it should.

In this case, the solution may be setting up clear pathways to a specific room. That’s just one suggestion — your loved one’s care team will have other suggestions to help your loved one be safe at home and elsewhere without losing their independence.

When should I see my healthcare provider?

Posterior cortical atrophy symptoms develop slowly. If you have this condition, ask your provider what you can expect in the next few months and years. Ask about changes that may be symptoms that your condition is getting worse and would be a reason for you to contact them.

What questions should I ask my healthcare provider?

Posterior cortical atrophy is a rare neurogenerative disorder. You may have questions like:

• How do you know I have posterior cortical atrophy?
• What’s causing the change in my brain function?
• What can I do about my current symptoms?
• Are there medications to ease my symptoms?
• How will this condition affect my daily life?
• What changes should I expect to experience in the coming months and years?
• How can I best plan for life ahead?

A note from Cleveland Clinic

In posterior cortical atrophy, there’s a disconnect in how your brain makes sense of what you see. That disconnect can disrupt your daily life, making it difficult for you to do things like read letters and words, understand numbers or judge distances. Having posterior cortical atrophy may make you feel anxious and afraid about other ways that the disease could change your life, including losing your independence.

Healthcare providers can’t cure this condition or restore the abilities that you’ve lost. This may be hard to hear, but there are many things you can do to manage your symptoms, navigate your new way of life and maintain your independence. If you have posterior cortical atrophy, talk to your care team about supportive programs and services. They’ll be glad to help you find the right resources.