Posterior Cortical Atrophy (PCA) – Symptoms, Causes & Treatment

Posterior Cortical Atrophy (1)

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Posterior Cortical Atrophy is a gradual, progressive degeneration of the cortex in the brain and nervous system. Symptoms that are commonly seen are difficulty reading, struggle with distance, and visual perception when reaching for objects. It is common with those living with Posterior Cortical Atrophy to have difficulty with their eyesight and processing visual information. They also struggle with calculations and recognizing objects and faces. As Posterior Cortical Atrophy progresses, those living with this disease may start to have trouble with cognitive skills.

When you live with Posterior Cortical Atrophy, you may struggle to process visual and spatial information. This is caused because the damage caused in the posterior part of the brain is where your visual processing and spatial reasoning occurs.

Researchers think that Posterior Cortical Atrophy is caused by Alzheimer’s Disease in about 80 percent of the cases but may also be caused by other types of Dementia such as Lewy Body Dementia or Creutzfeldt-Jakob Disease.

What Is Posterior Cortical Atrophy?

What Is Posterior Cortical Atrophy

Posterior Cortical Atrophy is a rare form of Dementia. It is unclear how many people are currently living with Posterior Cortical Atrophy.

Posterior Cortical Atrophy is caused by damage to the brain cells located in the cortex (in the back of the brain.) This part of the brain is responsible for processing information from your eyes and allows individuals to be able to recognize what you are seeing and where things are.

Researchers believe that Alzheimer’s Disease is most often the cause of brain cell damage in the cortex that causes Posterior Cortical Atrophy. Still, they also have concluded that it can come from other forms of Dementia such as Lewy Body Syndrome or Creutzfeldt-Jakob Disease.

Posterior Cortical Atrophy is referred to as ‘visual variant’ or ‘visual-spatial’ Alzheimers. Early signs of Posterior Cortical Atrophy are very different from early signs of Alzheimer’s. The first symptoms of Posterior Cortical Atrophy often are problems with vision and recognizing and understanding what we are seeing and what things are. In contrast, individuals living with Alzheimer’s usually see declines in their memory first.

Although it is unclear how many people are currently living with Posterior Cortical Atrophy, researchers believe that eight to thirteen percent have Posterior Cortical Atrophy symptoms.

It is common for individuals to be diagnosed with Posterior Cortical Atrophy between the ages of 50 and 65, but it can also affect those older.

Is Posterior Cortical Atrophy Referred To By Any Other Name?

Doctors and healthcare providers will sometimes use other names when referring to Posterior Cortical Atrophy. These include:

  • Posterior Cortical Atrophy
  • PCA
  • Benson’s Syndrome

What Causes Posterior Cortical Atrophy?

What Causes Posterior Cortical Atrophy

The loss of neurons called neurodegeneration causes posterior Cortical Atrophy. In PCA, the neurons are affected in the posterior region of the brain. This part of the brain begins to deteriorate, leading to shrinking the posterior cerebral cortex.

The posterior cerebral cortex of the brain is where the left and right occipital lobes are located, which mediate visual perception that helps individuals understand and recognize what they see with their eyes.

Researchers are still investigating the cause of Posterior Cortical Atrophy. At this time, no inherited pattern or gene is connected with the condition.

Is Posterior Cortical Atrophy A Unique Disease Or A Variant Of Alzheimer’s Disease?

It is still unknown whether Posterior Cortical Atrophy is a variant form of Alzheimer’s Disease or a unique disease. In those living with Posterior Cortical Atrophy, the damaged part of the brain shows amyloid plaques and tangles, which are similar to those living with Alzheimer’s Disease. These just appear in a different portion of the brain.

With that being said, others that are living with Posterior Cortical Atrophy mimic changes in the brain of those with Lewy body Dementia or Creutzfeldt-Jakob Disease.

Alzheimer’s Disease Variant

Posterior Cortical Atrophy has been considered as a type of Alzheimer’s Disease. There are many similarities and differences between Posterior Cortical Atrophy and Alzheimer’s Disease.

Similarities of Posterior Cortical Atrophy and Alzheimer’s Disease

When comparing Posterior Cortical Atrophy and Alzheimer’s Disease, researchers see the following similarities.

  • Both Diseases have similar after death research studies.
  • In individuals living with both conditions have amyloid plaques and neurofibrillary tangles. These are the proteins that cause the brain cells to begin to die.

Differences of Posterior Cortical Atrophy and Alzheimer’s Disease

Researchers see the following differences when comparing Posterior Cortical Atrophy and Alzheimer’s Disease.

  • The first symptoms of Posterior Cortical Atrophy and Alzheimer’s Disease are different. With those living with Posterior Cortical Atrophy, the first signs often report problems with vision and recognizing what they are seeing. Those living with Alzheimer’s Disease will see declines in their memory as one of the first signs.

When Do Individuals First see Symptoms of Posterior Cortical Atrophy?

When Do Individuals first see Symptoms of Posterior Cortical Atrophy

The first symptoms of Posterior Cortical Atrophy usually appear in individuals in their 50s or 60s. With that being said, the first symptoms of Posterior Cortical Atrophy are generally very subtle and not noticed for quite some time. 

What Are The Risk Factors Of Posterior Cortical Atrophy?

The cause of Posterior Cortical Atrophy is unknown, and currently, there are no known genetic mutations that are linked to Posterior Cortical Atrophy. Therefore there are no known risk factors for Posterior Cortical Atrophy. With that being said, researchers believed that an individual’s risk of developing Posterior Cortical Atrophy is similar to those of other types of Dementia. These include:

  • Age
  • Lifestyle
  • Genetics
  • Environment

Scientists continue to research to determine the risk factors for developing Posterior Cortical Atrophy.

Symptoms of Posterior Cortical Atrophy

Symptoms of Posterior Cortical Atrophy

The first symptoms of individuals living with Posterior Cortical Atrophy usually begin in adults between the ages of fifty and sixty-five. Once symptoms start, they progressively get worse at a slower pace than some other conditions tied to Dementia, such as Creutzfeldt-Jakob Disease. Problems with vision are the most prominent symptoms that are reported in the early stages of Posterior Cortical Atrophy. Other symptoms may include depression, anxiety, Dementia, loss of cognitive skills. These usually are seen in later stages of Posterior Cortical Atrophy.

Those with Posterior Cortical Atrophy may struggle with the following vision symptoms.

  • Those living with Posterior Cortical Atrophy may see things with an unusual color. They may also look distorted or appear to be moving.
  • Those living with PCA struggle to recognize objects out of the corner of their eye or may see many things as one object. Objects become less recognizable to those living with Posterior Cortical Atrophy.
  • Depth and surfaces appear different. Those living with PCA may think a black object on the floor is a hole or a puddle.
  • Those living with Posterior Cortical Atrophy may still see an image of an object after looking away from the original thing.
  • It is common for those living with Posterior Cortical Atrophy to bump into objects because they cannot see what is right in front of them.

Those living with Posterior Cortical Atrophy may also have the following symptoms.

  • Difficulty reading and writing. Those living with Posterior Cortical Atrophy often lose their place on the page, miss lines while reading, or struggle to read some fonts and handwriting. This can affect day-to-day tasks such as entering your pin to pay for your groceries.
  • Impaired visual recognition of objects and people. Those living with Posterior Cortical Atrophy may struggle with recognizing objects and faces, especially when they are not right in front of them. Those with PCA may also struggle reading a clock or sign.
  • Trouble with distance perception. Depths and distances become more and more difficult. This can lead to having difficulty with things like using the elevator or stairs. Individuals living with Posterior Cortical Atrophy may reach out to touch something but miss it completely.
  • Light Sensitivity. Those with PCA may find bright lights and glares very uncomfortable. They may even see patches of color when it is dark.
  • Struggles to identify moving objects
  • Spatial Awareness. Those living with Posterior Cortical Atrophy may struggle with their sense of direction. When basic terms like left and right are used it may be difficult for those with PCA to follow and understand.
  • Confusion when looking at multiple objects
  • Coordination. Those living with Posterior Cortical Atrophy may struggle with dressing themselves. This includes finding sleeves of jackets, buttoning buttons, and zipping zippers. Often those with Posterior Cortical Atrophy will put their clothes on backward.
  • Mood. Those with Posterior Cortical Atrophy struggle with their mood. These struggles include depression, anxiety, becoming irritable, and anxious. Often those with PCA lose interest in things they once loved.
  • Trouble with calculations. Those who are living with Posterior Cortical Atrophy struggle with simple calculations and math, which make balancing a checkbook and dealing with money very difficult.
  • Literacy. Literacy is often a challenge for those living with PCA. Individuals with Posterior Cortical Atrophy have difficulty remembering the space or names of certain letters.
  • Visual Hallucinations. Those with PCA may have hallucinations and see things that are not really there.

While Posterior Cortical Atrophy is a variant of Alzheimer’s Disease, those living with Posterior Cortical Atrophy often will not have symptoms that affect their memory until years later. 

Diagnosis of Posterior Cortical Atrophy

Diagnosis of Posterior Cortical Atrophy

Posterior Cortical Atrophy is challenging to diagnose. In fact, it is often misdiagnosed because of how rare of a disease it is. Those who have Posterior Cortical Atrophy often first go to an ophthalmologist because they have vision problems.

At this time, there are no standard diagnostic criteria for those with Posterior Cortical Atrophy. Healthcare providers rely on a variety of tests and examinations to diagnose as well as rule out other causes. The following tests and assessments may be done to find a diagnosis.

Physical Examination

If you or a loved one think you may have Posterior Cortical Atrophy, your healthcare provider will perform a physical examination as well as a neurological examination. The following things will be assessed.

  • Muscle strength
  • Reflexes
  • Sensation
  • Coordination
  • Walking
  • Vision
  • Speech
  • Memory

Vision Testing

Those individuals who may have Posterior Cortical Atrophy will undergo a visual acuity test and eye exam. The visual acuity test measures your ability to see objects clearly at a distance. This is usually done on a reading chart.

Neuropsychological Testing

Those who may have PCA may undergo Neuropsychological testing. This will evaluate your memory, concentration, problem-solving, and judgment. These tests take several hours to complete and are pretty interactive.

Imaging Tests

Often, if your healthcare may think you have Posterior Cortical Atrophy, you will likely do brain imaging testing. This can induce a CT scan or an MRI. 

By taking these tests, your doctor will be able to identify specific abnormalities in the brain, such as damage from strokes and traumatic brain injuries.

With those living with Posterior Cortical Atrophy, the occipital lobes usually appear smaller than they should be, and this is usually seen on a CT scan or MRI examinations.

Blood Tests

Your healthcare provider may order blood tests, including a complete blood count and electrolyte tests. These tests help rule out other problems and infections or metabolic issues.

Spinal Tap

A spinal tap may be done. This may help identify infection or inflammation to rule out other diagnoses. Results typically will be normal if you are living with Posterior Cortical Atrophy.

If you are unfamiliar with what a spinal tap is, it is a pretty invasive diagnostic test that is also known as a lumbar puncture. Your doctor would place a needle in your lower back and extract spinal fluid. This takes about five to ten minutes.

Tips for Visiting Your Doctor

You want to get the most out of your visits with your doctor. By reviewing these tips, you can get the most out of your visit with your doctor.

  • Know the reason for your visit.
  • Write down all your questions beforehand. This way you remember to ask all your questions.
  • Bring someone with you to help you remember your questions as well as the critical points of your visit.
  • Write down any new diagnosis, treatment options, and new medications.
  • Understand why new medications are being prescribed and their side effects.
  • Ask about new available treatments that may help or clinical trials.
  • Write down all follow-up appointments, including the date, time, location, and purpose of the visit.
  • Be sure you know how to contact your doctor if questions arise before your next visit.
  • Get a care summary from your doctor to remember the key points of the visit.

Treatment of Posterior Cortical Atrophy

Treatment of Posterior Cortical Atrophy

Currently, there are no cures for Posterior Cortical Atrophy. There are no treatments or medications specifically designed to help control or slow its progression. There are some things individuals living with Posterior Cortical Atrophy can do to help relieve symptoms caused by PCA.

Therapy

Vision therapy can be helpful for those living with Posterior Cortical Atrophy. Be sure to find a therapist that is familiar with treatment for those with visuospatial impairment.

Caregivers

Those living with Posterior Cortical Atrophy may require extra help and assistance with day-to-day tasks.

Medications

While there are no specific medications to help treat Posterior Cortical Atrophy, doctors may prescribe medications used by Alzheimer’s patients to help temporarily alleviate brain dysfunction. They may also take medications for depression and anxiety.

Medical Conditions that Cause similar Symptoms to Posterior Cortical Atrophy

Medical Conditions that cause similar symptoms to Posterior Cortical Atrophy

Several other medical conditions have similar symptoms in the early stages of Posterior Cortical Atrophy. This makes it difficult to determine if an individual has Posterior Cortical Atrophy or some other medical condition. These medical conditions include:

Blindness/ Vision Loss

Loss in vision can show similar symptoms as Posterior Cortical Atrophy. A vision test can determine if it is true vision loss.

Schizophrenia

Those living with Schizophrenia often have hallucinations and have mood changes. Both these symptoms are similar to individuals living with Posterior Cortical Atrophy. After a medical and physical examination, your healthcare provider can determine the difference between these conditions.

Dementia

Several types of Dementia have similar effects that Posterior Cortical Atrophy has. These include Alzheimer’s Disease, Lewy Body Dementia, and Vascular Dementia.

As symptoms increase, it is easier to determine the differences in these types of Dementia to get a clear diagnosis.

Stroke

An occipital stroke can cause similar symptoms as Posterior Cortical Atrophy. The difference between the two is that with individuals who have had a stroke, their symptoms will appear suddenly, whereas those living with Posterior Cortical Atrophy will gradually appear. Image testing can help determine if you have had a stroke or have Posterior Cortical Atrophy.

Inflammation

Inflammation can have similar neurological effects of those living with Posterior Cortical Atrophy. To tell the difference between the two, the inflammation usually has other systemic symptoms as well.

What Is The Prognosis Of Posterior Cortical Atrophy?

Posterior Cortical Atrophy is a progressive disease. The life expectancy of those living with Posterior Cortical Atrophy is about eight to twelve years. This is similar to individuals living with Alzheimer’s Disease.

When Should You Contact Your Healthcare Provider?

When Should You Contact Your Healthcare Provider (1)

Posterior Cortical Atrophy is not a medical emergency. If you think you or a loved one is living with Posterior Cortical Atrophy, you will want to make an appointment with your healthcare provider to get a medical examination. While there is no treatment or cure, healthcare providers can help you become more comfortable through therapy and medication if needed.

Key Points of Posterior Cortical Atrophy

  • Posterior Cortical Atrophy is a rare form of Dementia that is a gradual progressive degeneration of the cortex in the brain and nervous system.
  • The first signs of Posterior Cortical Atrophy commonly are difficulty reading, struggle with distance, and visual perception.
  • It is unclear how many individuals are living with Posterior Cortical Atrophy, although researchers believe it is 8-13%.
  • Posterior Cortical Atrophy shares similar symptoms as Alzheimer’s Disease; however first symptoms with Alzheimer’s are usually memory-related.
  • Individuals with Posterior Cortical Atrophy often see symptoms in their late 50s to around 65 years of age.
  • There are no known risk factors of Posterior Cortical Atrophy and none that are linked to genetic mutations.
  • It is challenging to diagnose Posterior Cortical Atrophy because of how rare it is.
  • There is no cure for Posterior Cortical Atrophy, just things that can be done to make those living with PCA more comfortable.
  • The life expectancy of those living with Posterior Cortical Atrophy is similar to those living with Alzheimer’s Disease. This is eight to twelve years.

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