Posterior cortical atrophy
Encyclopedia
Posterior cortical atrophy (PCA), also called Benson's syndrome, is most usually considered to be an atypical variant of Alzheimer's disease
. The disease causes atrophy
of the back (posterior) part of the cerebral cortex
, resulting in the progressive disruption of complex visual processing. PCA was first described by D. Frank Benson in 1988.
Despite being caused by the same disease process, the effects of posterior cortical atrophy and typical Alzheimer’s disease upon the behaviour, thought processes and skills of individuals with each condition are very different. Typical Alzheimer’s disease is most commonly associated with deterioration in memory, language, perception and a host of other skills and abilities. By contrast, individuals with posterior cortical atrophy tend to have well preserved memory and language but instead show a progressive, dramatic and relatively selective decline in vision and/or literacy skills such as spelling, writing and arithmetic.
In rare cases, PCA "can also be due to other diseases including dementia with Lewy bodies
and Creutzfeldt–Jakob disease."
PCA usually affect people at an earlier age than typical cases of Alzheimer's disease, with initial symptoms often experienced in people in their mid-fifties or early sixties. This was the case with writer Terry Pratchett
, who went public in 2007 about being diagnosed with PCA.
Alzheimer's disease
Alzheimer's disease also known in medical literature as Alzheimer disease is the most common form of dementia. There is no cure for the disease, which worsens as it progresses, and eventually leads to death...
. The disease causes atrophy
Atrophy
Atrophy is the partial or complete wasting away of a part of the body. Causes of atrophy include mutations , poor nourishment, poor circulation, loss of hormonal support, loss of nerve supply to the target organ, disuse or lack of exercise or disease intrinsic to the tissue itself...
of the back (posterior) part of the cerebral cortex
Cerebral cortex
The cerebral cortex is a sheet of neural tissue that is outermost to the cerebrum of the mammalian brain. It plays a key role in memory, attention, perceptual awareness, thought, language, and consciousness. It is constituted of up to six horizontal layers, each of which has a different...
, resulting in the progressive disruption of complex visual processing. PCA was first described by D. Frank Benson in 1988.
Despite being caused by the same disease process, the effects of posterior cortical atrophy and typical Alzheimer’s disease upon the behaviour, thought processes and skills of individuals with each condition are very different. Typical Alzheimer’s disease is most commonly associated with deterioration in memory, language, perception and a host of other skills and abilities. By contrast, individuals with posterior cortical atrophy tend to have well preserved memory and language but instead show a progressive, dramatic and relatively selective decline in vision and/or literacy skills such as spelling, writing and arithmetic.
In rare cases, PCA "can also be due to other diseases including dementia with Lewy bodies
Dementia with Lewy bodies
Dementia with Lewy bodies , also known under a variety of other names including Lewy body dementia, diffuse Lewy body disease, cortical Lewy body disease, and senile dementia of Lewy type, is a type of dementia closely allied to both Alzheimers and Parkinson's Diseases...
and Creutzfeldt–Jakob disease."
PCA usually affect people at an earlier age than typical cases of Alzheimer's disease, with initial symptoms often experienced in people in their mid-fifties or early sixties. This was the case with writer Terry Pratchett
Terry Pratchett
Sir Terence David John "Terry" Pratchett, OBE is an English novelist, known for his frequently comical work in the fantasy genre. He is best known for his popular and long-running Discworld series of comic fantasy novels...
, who went public in 2007 about being diagnosed with PCA.
External links
- What is posterior cortical atrophy? from the Dementia Research CentreDementia Research CentreThe Dementia Research Centre is part of University College London's Department of Neurodegenerative Disease. The DRC is part of the National Hospital for Neurology and Neurosurgery in Queen Square, London and is affiliated with the Hospital's Specialist Cognitive Disorders Clinic...
PCA support group