Benson’s Syndrome, or posterior cortical atrophy, is a rare, progressive degenerative condition and is a form of dementia not considered a type of Alzheimer’s disease.
Though PCA and Alzheimer’s disease both involve the loss and dysfunction of neurons, or brain cells, they affect different parts of the brain.
For the most part, Alzheimer’s usually first impacts the sides of the brain, those areas that play an important role in memory, which causes the first symptoms of memory loss.
PCA, first described in 1988 by Dr. D. Franck Benson, develops slowly and first affects the back of the brain known as the occipital lobe, which is responsible for vision. Often, those first experiencing this condition describe visual problems or complex visual behaviors.
Those with PCA symptoms would have difficulty perceiving objects like glass doors, difficulty in recognizing people and can struggle with reading, writing and numerals. Additionally, they might have difficulty with some tasks, such as buttoning up a shirt.
The syndrome is sometimes caused by other diseases, such as dementia with Lewy Bodies.
Signs of depression and anxiety tend to be prominent in the early stages of PCA. Early detection and treatment of psychiatric symptoms can affect function and independence in daily life.
The first symptoms of PCA typically occur in those in their late 40s, 50s or early 60s, and are often subtle and difficult to diagnose. As the brain deteriorates and the disease progresses, more Alzheimer’s-like symptoms occur, such as memory loss and confusion.
Risk factors for developing the disease are unknown. PCA is said to be the most poorly understood type of dementia, and there is no specific treatment. Most often the same medications prescribed for Alzheimer’s disease are used.
While researchers have observed that PCA and Alzheimer’s disease have the same changes in the brain and the same protein buildup and the same pathology, it is a mystery why this specific dementia affects different parts of the brain.
Because of the nature of the disease, PCA presents unique challenges for clinicians who counsel those affected and their families on clinical status and prognosis, and experts designing clinical trial of interventions.