Posterior = Back
Cortical = Outer layer of brain
Atrophy = Loss of neural cells and connections
Posterior cortical atrophy (PCA) is a progressive neurodegenerative syndrome that initially affects the visual perception system located in the back of the brain. Most cases are found to stem from Alzheimer’s pathology and therefore PCA has been traditionally classified as a rare visual variant of that disease. Over time, the atrophy can spread into other regions of the brain resulting in typical dementia symptoms (e.g., memory issues).
Alzheimer’s with dementia normally develops after the age of 65 but PCA can have a much younger onset age. Although most people living with PCA are diagnosed with the syndrome between the ages of 50 and 60, others may begin suffering symptoms in their forties.
I see the teacup with my eyes, but my brain refuses to send me the teacup message. It's very Zen. First, there is no teacup and then, because I know there is a teacup, the teacup will appear the next time I look.
Sir Terry Pratchet
Author
who was “thoroughly annoyed that he had [PCA] dementia”
Common Symptoms
The symptoms of PCA can vary across patients and change as the condition progresses.
PCA Patients May Present With
Blurry Vision
Light Sensitivity
Or may have Difficulty With
Reading
Writing
Drawing
Dressing
Navigating Familiar Places
Reaching for Objects
Stepping Off Curbs
Tripping
Descending Stairs
Finding Objects "Right in Front of Their Eyes"
Using Keyboards
Dialing Phones
Using Remotes
Missing Table When Setting Objects Down
Running into Doorways
Environmental Disorientation
Loss of Peripheral Vision
Basic Calulations
Visual Hallucinations
The ability to interact with and make sense of the visual world is often severely affected in PCA.
I Can See But I Can't See
Objects may appear to move, popping in and out of the visual field. It is not unusual for someone living with PCA to be able to see something as small as a pill on the ground but be unable to see things right in front of their faces.
Seeing the Trees But Not the Forest
One symptom of PCA is being able to recognize individual elements of a complex visual scene but they are unable to grasp the overall meaning.
Language
Although language abilities are relatively spared, some individuals diagnosed with PCA have difficulty with word finding and language comprehension. Therefore, they may exhibit tangential conversational styles that lack focus.
Living with PCA
A Typical Diagnostic Story May Look Like
“It took years to get a diagnosis. Every ophthalmologist I consulted said my eyes were healthy. Four neurologist were unable to give me any answers. Finally, neurologist #5 looked at my MRI and said that he suspected that I had PCA but he had never treated anyone else with the syndrome. Ophthalmologist #6 sent me to a year’s worth of therapies before reluctantly agreeing that some form of cortical blindness was a possible explanation for my visual issues. Even the doctors who diagnosed me, could not provide me with much information about my condition.”
Research
Crutch, S. J., Schott, J. M., Rabinovici, G. D., Murray, M., Snowden, J. S., van der Flier, W. M., et al. (2017). Consensus classification of posterior cortical atrophy. Alzheimer’s & Dementia, 13, 870-884. Article
Firth, N. C., Primativo, S. Marinescu, R. V., Shakespeare, T. J. Suarez-Gonzalez, A. Lehmann, M., et al. (2019). Longitudinal neuroanatomical and cognitive progression of posterior cortical atrophy, Brain, 142(7), 2082–2095. Article
Holden, S. K., Bettcher, B. M., & Pelak, V. S. (2020). Update on posterior cortical atrophy. Current Opinion in Neurology, 33(1), 68-73. Article
Wong, B., Lucente, D. E., MacLean, J., Padmanabhan, J., Quimby, M., Brandt, K. D., et al. (2019). Diagnostic evaluation and monitoring of patients with posterior cortical atrophy. Neurodegenerative Disease Management, 9(4), 217–239. Article
It Is Time To Talk About
Posterior Cortical Atrophy
Feeling lost after receiving a PCA diagnosis, Dr. Mize began an educational outreach effort. Her goal is to bring together accessible information about PCA from those living with the syndrome and from medical professionals.
Even if PCA has taken away your ability to read and write, your story can have a powerful impact. If you, or a loved one, are living with PCA, contact Dr. Mize to discuss how sharing your PCA journey can help others.
Dr. Mize is working on a book that will include the stories of those living with PCA. A defining feature of the book will be a collection of PCA-specific tips and tools that those affected by the condition have found work to make their lives better.
About Me
Educator • Researcher • Author • Guide Dog Handler
I am currently an Assistant Professor at Florida Gulf Coast University. Using evolutionary psychological theory as an overarching theme, I focus on behavioral and psychophysiological measures to study development across the lifespan. My primary interests are social relationships and emotions although I often explore other areas of research-including PCA.
As a PCA Patient...
I am learning to live again with the assistance of a good medical team, family support, and my guide dog.
Achievements
PhD
Experimental Psychology
MA
Psychology
BA
Experimental Psychology