August 22, 2019 e-Pearl of the Week: Benson syndrome

Benson syndrome

Benson syndrome or posterior cortical atrophy (PCA), also known as a visual variant of Alzheimer disease presents with visuospatial and perceptual difficulties.  Memory, insight, and judgment are preserved in early stages¹. The exact etiology is unknown with no genetic causation found. The MRI shows atrophy of parietal and occipitotemporal cortices³. Management is using voice recognition software, audio books and lighting tools at home. Currently, no data supports the use of acetylcholinesterase inhibitors^2,3. Patients usually succumb within 8–12 years from the time of onset.

References

1. Grover S, Amitava AK, Kumari N. One glasses too many: A case report of Benson's syndrome. Indian J Ophthalmol 2015;63: 277–279.

2. Crutch SJ, Lehmann N, Schott JM, Rabinovici GD, Rossor MN, Fox NC. Posterior Cortical Atrophy. Lancet Neurol 2012;11: 170–178.

3. Schott, J., & Crutch, S. Posterior Cortical Atrophy. CONTINUUM: Lifelong Learning in Neurology 2019;25; 52-75.

Submitted by Naveed Ahmad, MBBS, Professional Trainee, Psychiatry department, University of Texas, Houston, and Faisal Khan, MD, DABSM, Consultant Neurologist, Sugar Land Sleep and Neurology.

Naveed Ahmad and Dr. Khan report no disclosures.