Atypical motor and behavioral presentations of Alzheimer disease: a case-based approach

The correlation of clinical presentation to pathology in dementia syndromes is important to correctly classify and ultimately treat these conditions. However, despite careful clinical characterization, it remains difficult to accurately predict an underlying causative pathology in some cases. Alzheimer disease is a well-defined clinical entity having established diagnostic criteria and characteristic neuropathologic findings. Alzheimer pathology, however, can cause varying clinical syndromes, including both atypical motor and behavioral presentations.

Atypical clinical presentations of Alzheimer disease are reviewed in a case-based format. Corticobasal syndrome, with asymmetric Parkinsonism, dystonia, and apraxia, is increasingly recognized as a presentation of Alzheimer pathology. Frontal variant Alzheimer, clinically indistinguishable from behavioral variant frontotemporal dementia (bv-FTD), can present with difficulties in executive function, poor attention, and behavioral issues. Posterior cortical atrophy (the "visual variant" of Alzheimer) has predominant visuospatial dysfunction and can be an Alzheimer presentation. Finally, Alzheimer can present as logopenic progressive aphasia with word-finding difficulty.

Clinicopathologic correlation may be more complex than previously realized, and the location of the microscopic changes may have as much to do with the clinical presentation as the nature of the changes themselves. Recognizing these clinical syndromes can lead to greater accuracy in diagnosis and treatment.