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Posterior Cortical Atrophy - Causes, Symptoms, Risk Factors, Diagnosis, and Treatment

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Posterior cortical atrophy is the gradual degeneration of the brain's outer layer in the head's posterior (back) part. Read the article to know more.

Written byDr. Osheen Kour

Medically reviewed byDr. Abhishek Juneja

Published At March 17, 2023
Reviewed AtNovember 24, 2023

Introduction

Posterior cortical atrophy is considered a neurodegenerative syndrome that causes a progressive decline in visuoperceptual, visuospatial, praxis, and literacy skills. The condition causes progressive damage to the temporal, occipital, and parietal lobes of the brain and is often associated with Alzheimer’s disease in most patients. Posterior cortical atrophy, called Benson’s syndrome, is considered a visual variant of Alzheimer’s disease-causing dementia. Posterior cortical atrophy affects people at an early age of about 50 to 60 and can occur due to multiple underlying pathologies. The most common cause of posterior cortical atrophy is considered to be Alzheimer’s disease. Still, other neurological conditions such as corticobasal degeneration and Lewy body dementia may also cause this condition. The etiology of posterior cortical atrophy is still unknown, but researchers are conducting trials to improve identification and increase awareness of the unusual symptoms of posterior cortical atrophy.

What Are the Causes of Posterior Cortical Atrophy?

The actual cause of posterior cortical atrophy is unknown; however, Lewy body dementia and Alzheimer's disease are considered the possible causes of this condition. There is no evidence of genetic mutations linked to posterior cortical atrophy. However, the condition is believed to have similar risk factors as Alzheimer’s disease.

What Are the Risk Factors for Posterior Cortical Atrophy?

There is no substantial evidence to show what effects or causes damage to the posterior part of the brain. Still, the risk of developing posterior cortical atrophy may be similar to dementia of any other form. However, certain common factors may increase the possibility of developing this condition, such as

  • Age.

  • Environmental factors.

  • The overall health of a person.

  • Lifestyle habits.

  • Genetic history of any neurological diseases or posterior cortical atrophy.

What Are the Symptoms of Posterior Cortical Atrophy?

The symptoms of posterior cortical atrophy are as follows:

  • Difficulty driving.

  • Problems in identifying things on the left from right.

  • Difficulty in reading and spelling.

  • Problems in identifying how far objects are placed.

  • Difficulty recognizing the difference between moving objects and stationary or still objects.

  • Difficulty in using everyday tools and objects.

  • Problems with getting dressed up.

Other symptoms include

  • Confusion.

  • Hallucinations.

  • Anxiety.

  • Changes in personality and behavior.

  • Memory problems may also occur later in life.

What Is the Connection Between Posterior Cortical Atrophy and Alzheimer’s Disease?

Some studies show that posterior cortical atrophy can be a variant of Alzheimer’s disease, as both conditions present visual defects as initial symptoms. Both diseases lead to progressive neural degeneration, overlapping brain regions, and loss of synapses and neurons, eventually causing dementia in a person. In Alzheimer’s disease, the hippocampus and prefrontal cortex are more damaged. In posterior cortical atrophy, damage to the cortical region and loss of cell body or gray matter is evident in the posterior part of the brain, including frontal, temporal, and occipital lobes. Alzheimer’s disease impairs episodic memory, whereas posterior cortical atrophy impairs anterograde and working memory but keeps the episodic memory unaffected.

The atrophy caused by posterior cortical atrophy can later spread to the regions damaged by Alzheimer's disease and thus produce combined symptoms such as defects in cognition, memory, learning, and language. However, a person's posterior cortical atrophy condition often remains unknown due to a lack of proper diagnosis. Therefore, researchers are still working to establish standard diagnostic criteria for posterior cortical atrophy.

How Is Posterior Cortical Atrophy Diagnosed?

The doctor diagnoses posterior cortical atrophy by analyzing a person's symptoms and medical history, including vision difficulties, which is often the first symptom of this condition. The doctor also conducts physical and neurological examinations of a person suffering from posterior cortical atrophy. Several tests are recommended to eliminate the conditions that may appear similar to posterior cortical atrophy.

  • Blood Tests: These include tests for thyroid disorders, vitamin deficiencies, and other conditions that may cause symptoms.

  • Magnetic Resonance Imaging (MRI): This test shows a 3D (3 dimensional) image of the brain and reveals any abnormalities causing the symptoms.

  • Neuropsychological Tests and Mental Status: A person's cognitive skills are assessed in this test. The doctor may also suggest psychiatric assessments for a person to test for various mental illnesses such as depression.

  • Ophthalmological Examination: This test is conducted to rule out any other condition or vision defect causing visual symptoms similar to posterior cortical atrophy.

  • Single-photon Emission Computerized Tomography (SPECT) or Positive Emission Tomography (PET): In this test, a minimal amount of radioactive material is injected, and an emission detector is placed on the person’s brain. SPECT helps measure blood flow to various brain parts, and PET gives a visual image of the brain’s activity.

How Is Posterior Cortical Atrophy Treated?

The treatment for posterior cortical atrophy that could cure or halt its progression has yet to be discovered. Since the condition presents symptoms similar to Alzheimer's, medications used for Alzheimer’s are also recommended for posterior cortical atrophy. But the treatment has also not been approved yet by the experts. However, certain medications and therapies can help treat the symptoms of posterior cortical atrophy. These include. The doctor may prescribe medication to treat symptoms such as anxiety and depression.

  • The skills of a person affected by posterior cortical atrophy can be managed with various cognitive, physical, and occupational therapies.

What Is the Differential Diagnosis for Posterior Cortical Atrophy?

Conclusion

Posterior cortical atrophy is an under-recognized and deliberating degenerative syndrome characterized by visual deficits and occurs due to various underlying pathologies. The condition is not confirmed to be a variant of Alzheimer’s or any other unique form of the disease, and research is still going on to confirm its etiology. Posterior cortical atrophy sometimes resembles Alzheimer’s disease as it presents neurofibrillary tangles and amyloid plaques. In some people, it may resemble a disease such as Creutzfeldt-Jakob disease and dementia in Lewy body disease. The condition is often unrecognized due to its challenging diagnosis, and therefore, its diagnostic criteria and standard definition are still under research. However, If a person experiences any initial visual symptoms of posterior cortical atrophy, they must consult the doctor immediately to rule out the possible cause of the disease.

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