Posterior cortical atrophy: clinical, neuroimaging, and neuropathological features

Area of Science:

• Neurodegenerative Disorders
• Neuroscience
• Ophthalmology

Background:

• Posterior Cortical Atrophy (PCA) is a neurodegenerative syndrome primarily affecting higher-order visual processing due to parietal and occipital lobe atrophy.
• While the underlying pathology varies, Alzheimer's disease is the most frequent cause, with symptom onset typically before age 65.
• Delayed diagnosis is common, as symptoms are often mistaken for ocular issues rather than cortical dysfunction.

Approach:

• This review provides a comprehensive analysis of Posterior Cortical Atrophy (PCA) syndrome.
• It covers clinical presentations, neuroimaging findings, pathological hallmarks, and genetic factors.
• Management strategies and therapeutic interventions are also discussed.

Key Points:

• Patients often present with visual-perceptual and visuospatial deficits, with other cognitive impairments developing over time.
• Neuroimaging reveals predominant parieto-occipital or temporo-occipital atrophy.
• Cerebrospinal fluid biomarkers and amyloid/tau PET imaging aid in diagnosing underlying Alzheimer's disease.

Conclusions:

• Management of PCA primarily relies on non-pharmacological interventions.
• Prompt etiological diagnosis is essential, particularly with the advent of disease-modifying therapies for Alzheimer's disease.