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Late-onset frontotemporal dementia associated with progressive supranuclear palsy/argyrophilic grain disease/Alzheimer’s disease pathology

2005; Taylor & Francis; Volume: 11; Issue: 3 Linguagem: Inglês

10.1080/13554790590944753

1465-3656

Gregory A. Rippon, Bradley F. Boeve, Joseph E. Parisi, Dennis W. Dickson, R.I. Ivnik, Clifford R. Jack, M. Hutton, Matthew Baker, Keith A. Josephs, David S. Knopman, Ronald C. Petersen,

Neurological diseases and metabolism

Abstract Progressive supranuclear palsy (PSP) is typically manifested by vertical supranuclear gaze palsy, frequent falls early in the disease course, axial rigidity and poor response to levodopa. Prominent anterograde memory dysfunction with subsequent impairment in other cognitive domains is characteristic of Alzheimer's disease (AD). No clear clinical syndrome has been identified in argyrophilic grain disease (AGD). Frontotemporal dementia (FTD) is characterized by apathy, emotional blunting, disinhibition, and impairment in executive functioning despite relatively preserved memory and visuospatial abilities. Cognitive deficits are known to occur in PSP; however, overt clinical FTD without parkinsonism or supranuclear gaze palsy associated with PSP pathology has rarely been documented. We report an elderly patient with the typical clinical, neuropsychometric, and neuroimaging features of FTD who had autopsy findings most consistent with PSP plus AGD and AD in limbic structures. We suggest that PSP with or without coexisting AD and AGD be included in the differential diagnosis of patients presenting with FTD. Acknowledgments Supported by grants P50 AG16574 and PO1 AG07216 from the National Institute on Aging. We extend our appreciation to the family for participating in research on aging and dementia.