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Progressive aphasia with Lewy bodies.
Richard J Caselli1, Thomas G Beach, Lucia I Sue
1Department of Neurology, Mayo Clinic Scottsdale, 13400 East Shea Boulevard, AZ 85259, USA. Caselli.Richard@Mayo.edu
Dementia and Geriatric Cognitive Disorders
|July 30, 2002
Summary
This case study details a rare instance of progressive aphasia preceding dementia with Lewy bodies (DLB), Alzheimer's disease (AD), and Lewy body pathology. It highlights the complex interplay between these neurodegenerative conditions.
Area of Science:
- Neurology
- Neuroscience
- Pathology
Background:
- Dementia with Lewy bodies (DLB) typically presents with cognitive decline, parkinsonism, and hallucinations.
- Primary progressive aphasia (PPA) is a neurodegenerative syndrome primarily affecting language.
- Co-occurrence of Alzheimer's disease (AD) and Lewy body pathology is known, but PPA as a presenting symptom in DLB is undocumented.
Observation:
- A 69-year-old woman presented with progressive aphasia, later developing visual hallucinations and parkinsonism.
- Autopsy revealed coexisting Alzheimer's disease pathology and Lewy bodies in cortical and subcortical regions.
- Specific findings included cortical Lewy bodies, and depigmentation and Lewy bodies in the substantia nigra and locus ceruleus.
Findings:
- The patient's aphasia was attributed to the initial Alzheimer's disease pathology.
- Subsequent onset of psychosis and parkinsonism were linked to the Lewy body pathology.
- This case represents the first reported instance of PPA within the context of combined AD and Lewy body pathology.
Implications:
- This case uniquely illustrates the clinical and pathological relationships between Alzheimer's disease and Lewy body pathology.
- It demonstrates that the syndrome of PPA can occur in the context of what might otherwise be classified as Dementia with Lewy Bodies.
- The findings suggest limitations in the current diagnostic terminology for DLB when overlapping pathologies are present.