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Posterior Cortical Atrophy

Causes, Symptoms, Diagnosis & Treatment
  • Key Points
  • Overview
  • Causes
  • Symptoms
  • Diagnosis
  • Treatment
  • When to Seek Care
  • Next Steps

Key Points

  • Posterior Cortical Atrophy is a rare form of dementia affecting visual processing areas of the brain.
  • Early symptoms usually involve visual and spatial difficulties rather than memory loss.
  • The disease shares similarities with Alzheimer’s Disease at a cellular level.
  • There is no cure, but treatments can help manage symptoms. Early diagnosis can help patients and families adapt, plan care and connect with support services.

Overview

What is Posterior Cortical Atrophy?

Posterior Cortical Atrophy (PCA) is a rare and progressive form of dementia that affects the outer layer of the brain, known as the cortex, in the posterior (back) region. These affected areas of the brain are primarily responsible for processing visual information. As the disease progresses, nerve cells in this region deteriorate, causing increasing difficulty with visual awareness and spatial perception.

Researchers continue to study whether PCA is a distinct condition or a variant of Alzheimer’s Disease. The underlying cellular changes are similar to Alzheimer’s Disease, but the symptoms resemble Lewy Body Dementia, especially with visual and perceptual problems.

Who is Affected?

Posterior Cortical Atrophy most often affects adults in midlife (50s – early 60s), but it can occur at other ages.

It affects men and women at similar rates.

Many individuals with PCA are otherwise healthy at the time symptoms begin.

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Causes & Risk Factors

What Are the Causes and Risk Factors?

Posterior Cortical Atrophy is caused by the degeneration of brain cells due to abnormal protein build up in the posterior (back) regions of the cortex. The exact reason why PCA develops in some people is not fully understood.

Uncontrollable Risk Factors

  • Age
  • Underlying neurodegenerative processes similar to Alzheimer’s Disease
  • Possible genetic influences, though most cases are not inherited


Controllable Risk Factors

  • None known at this time

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Signs & Symptoms

What Are the Signs and Symptoms of PCA?

Early symptoms of posterior cortical atrophy usually involve visual processing problems before memory symptoms appear.

Common early symptoms include:

  • Difficulty judging distances
  • Trouble distinguishes between objects
  • Problems with reading
  • Difficulty navigating familiar environments
  • Challenges with visual coordination


Other symptoms include:

  • Visual hallucinations in some individuals
  • Anxiety or depression related to visual difficulties
  • Trouble with complex visual tasks


As the disease progresses, additional symptoms may include:

  • Memory impairment
  • Difficulty with planning or decision-making
  • Problems with language or communication

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Diagnosis

How is Posterior Cortical Atrophy Diagnosed?

Diagnosing PCA can be challenging as early symptoms often present as vision problems rather than neurological disorders. Diagnosis is based on detailed medical history, neurological examination and assessment of cognitive and visual-spatial abilities.

Tests used may include:

  • Neurological examination
  • Cognitive and visual spatial testing
  • Brain imaging (such as MRI or CT)
  • Additional testing to rule out causes of visual symptoms

 

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Treatments

How is Posterior Cortical Atrophy Treated?

There is no cure for the disease and no treatment proven to slow disease progression. Treatment focuses on managing symptoms and supporting quality of life.

Lifestyle

  • Create a safe living environment
  • Adjusting daily activities to accommodate visual challenges
  • Using structured routines


Devices

  • Visual aids and adaptive tools
  • Assistive technology for reading or navigation


Medical

  • Medications to help manage mood symptoms such as anxiety or depression
  • Medication sometimes used in Alzheimer’s Disease to help manage cognitive symptoms


Surgical

  • There are no surgical treatments for PCA


Other

  • Occupational therapy to support daily function
  • Vision rehabilitation strategies
  • Counseling and support for patients and caregivers

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When to Seek Care

When to See Your Doctor

Talk to your doctor if you or your loved one experiences ongoing visual difficulties that cannot be explained by eye disease, especially if accompanied by problems with reading, navigation or coordination.

When to Seek Emergency Care

Seek immediate medical care if symptoms appear suddenly, worsen rapidly or are accompanied by sudden confusion, vision loss or other neurological changes as it could indicate another neurological emergency such as a stroke.

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Next Steps

If you're noticing persistent changes in your vision that can't be explained by an eye condition, don't ignore them. Talk with your doctor, especially if these changes are affecting reading, driving, navigation or other everyday activities.

Visit Understanding Memory to learn more about the many things than can impact memory.

To learn more and connect with support, visit Riverside Health’s Martha W. Goodson Center.


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