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The syndrome of progressive posterior cortical dysfunction: A multiple case study and review
Renata Areza-Fegyveres1, Paulo Caramelli2, Claudia Sellitto Porto3
1Neurologist, member of the Behavioral and Cognitive Neurology Unit, Department of Neurology, University of São Paulo School of Medicine, São Paulo, SP, Brazil.
Dementia & Neuropsychologia
|December 8, 2017
Summary
Dementia with significant visual symptoms is challenging to diagnose. This study highlights visuospatial and visuoperceptive impairments in progressive dementia, often associated with Alzheimer disease.
Area of Science:
- Neurodegenerative diseases
- Cognitive neurology
- Neuro-ophthalmology
Background:
- Dementia with prominent higher-order visual symptoms is difficult to diagnose.
- These symptoms can occur in various neurodegenerative conditions.
Purpose of the Study:
- To describe progressive dementia cases with predominant visual cortical symptoms.
- To identify clinical features and potential diagnoses.
Main Methods:
- Retrospective search of dementia cases with predominant visual symptoms.
- Reviewed patient data from 1996 to 2006.
Main Results:
- Twelve patients (49-67 years) with progressive dementia and visual symptoms were identified.
- Predominant symptoms included visuospatial (Balint syndrome) and visuoperceptive impairments.
- Associated symptoms: constructional apraxia, Gerstmann syndrome, ideomotor apraxia, hemineglect, alien hand phenomenon, prosopagnosia. Memory loss was not the primary complaint.
- Diagnoses included Alzheimer disease (AD), corticobasal degeneration, and dementia with Lewy body.
Conclusions:
- Consider dementia with prominent higher-order visual symptoms in presenile patients.
- This presentation can occur in Alzheimer disease and other neurodegenerative conditions.
