Posterior cortical atrophy (PCA), also called Benson's syndrome, is a form of dementia which is usually considered 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. The disease causes atrophy of the back (posterior) part of the cerebral cortex, resulting in the progressive disruption of complex visual processing.
Although no cure for posterior cortical atrophy exists, several medications as well as many non-pharmaceutical approaches can potentially improve daily functioning and quality of life. Patients with posterior cortical atrophy can often benefit from physical and occupational therapy. Cholinesterase inhibitors approved for Alzheimer's disease, like donepezil (Aricept®), rivastigmine (Exelon®) and galantamine (Razadyne®/Reminyl®), can help the symptoms of PCA by boosting the function of brain cells to compensate for damage caused by Alzheimer's disease. Patients experiencing depression, irritability, frustration and a loss of self-confidence may benefit from antidepressant medication. In sporadic CJD, the disease usually progresses within a few months. Early symptoms include minor lapses of memory, mood changes and loss of interest. Within weeks the person may complain of clumsiness and feeling muddled, become unsteady walking, and have slow or slurred speech. Symptoms progress to jerky movements, shakiness, stiffness of limbs, incontinence and loss of the ability to move or speak. By this stage the person is unlikely to be aware of their surroundings or disabilities.