| Prevalence | Rare, estimated 1-2% of all AD cases |
| Progressive visual loss | Insidious onset of bilateral visual impairment |
| Cortical blindness | Loss of visual perception without damage to the eyes or optic nerves |
| Visual field defects | Often beginning as hemianopic (half-field) defects |
| Color perception deficits | Difficulty distinguishing colors (achromatopsia) |
| Spatial disorientation | Difficulty judging distances and navigating familiar environments |
| Alexia without agraphia | Inability to read while maintaining writing ability |
| Prosopagnosia | Difficulty recognizing faces |
| Simultanagnosia | Inability to perceive more than one object at a time |
| Posterior cingulate cortex | Often affected early |
| Temporoparietal regions | Variable involvement |
| Neurofibrillary tangles (NFTs) | Abundant in the occipital cortex, often exceeding densities seen in typical AD[@levine1993] |
| Databases | OMIMOrphanetClinicalTrialsPubMed |
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