Posterior cortical atrophy (PCA) or also known as Benson’s Syndrome is a rare degenerative illness. It is considered as a atypical type of Altziemer’s due to the similarities in symptoms. Benson’s syndrome, which affects on average adults between 50 and 60 years old, is currently incurable. Read on to discover more about this rare illness.
What is Benson’s Syndrome?
Benson’s syndrome affects at least 5% of Alzheimer’s patients and is considered as an atypical form of this illness. In fact, PCA is not considered as an illness in the proper sense but as a syndrome that consists of many developmental stages.
Benson’s syndrome can be associated with several illnesses:
- Alzheimer’s disease ;
- Lewy body dementia (a neuro-degenerative illness that produces similar symptoms to Alzheimer’s and Parkinson’s disease);
- Cortico-basal syndrome (atypical syndrome related to Parkinson’s)
- Creutzfeld-Jakob disease (prion disease, causing degeneration of the nervous system).
You can also read: Alzheimer’s disease: Learn to recognise 10 early signs
What are the symptoms of Posterior cortical atrophy?
As it’s name might suggest, posterior cortical atrophy affects the high function of the posterior cortex. This means that Benson’s syndrome affects capacities generated at the back of the brain. This area is where our visual functions are located. The atrophied which is coloured in purple below is affected during PCA. This is why most symptoms are related to vision.
Although PCA is associated with Alzheimer’s disease, a patient’s memory abilities remain intact for a long time.
Orphanet reports numerous clinical signs such as:
- Dysfunctional Perception and Spatial Vision (have difficulty orientating in open spaces)
- Apraxia (difficulty in carrying out voluntary movement) and Alexia (an inability to recognize written elements)
- Sumultanagnosis (inability to see multiple objects simultaneously)
- Ataxia (movement coordination disorder) and oculomotor apraxia
- Have difficulty or is impossible to write, read, read the time, drive
- Left to right depreciation,
- Strange visual images (colored consecutive images, perception of movements from images or immobile objects) .
These patients also suffer from anxiety and premature depression.
You can also read: Parkinson’s disease: causes, symptoms and treatments
How is it diagnosed and treated?
After a meeting has been set up with a neurologist, the neuro-psychologist will carry out a new exam using a number of tools:
- Ophthalmological examination: to determine the presence of an eye disease
- MRI: to detect if there is a loss of volume (atrophy) in the region’s of the brain responsible for vision (the posterior part of the brain);
- PET scan: to detect the presence or absence of abnormal cells in the areas of the brain responsible for vision
- A lumbar puncture: to clarify the origin of the disorder by comparing the biomarkers of the patient to those of Alzheimer’s disease.
Benson’s syndrome is not a curable disease. Proposed treatments are substantially the same as for Alzheimer’s disease. That said, patients can also undergo rehabilitation to improve their living conditions. Follow-ups with a mental health professional are also a necessity given the importance of psychic symptoms.