Open AccessCorticobasal Ganglionic Degeneration and Progressive Supranuclear Palsy Presenting with Cognitive Decline
Author(s) -
Bergeron Catherine,
Davis Andrea,
Lang Anthony E.
Publication year - 1998
Publication title -
brain pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.986
H-Index - 132
eISSN - 1750-3639
pISSN - 1015-6305
DOI - 10.1111/j.1750-3639.1998.tb00159.x
Subject(s) - corticobasal degeneration , progressive supranuclear palsy , dementia , lewy body , primary progressive aphasia , dementia with lewy bodies , tangle , pathology , neuroscience , medicine , pathological , psychology , amyotrophic lateral sclerosis , disease , frontotemporal dementia , mathematics , pure mathematics
Corticobasal ganglionic degeneration (CBGD) and progressive supranuclear palsy (PSP) were originally described in the sixties as predominantly motor syndromes. Over the years, the detailed study of additional cases of CBGD has shown that it is a distinctive histological entity which can often present as dementia or aphasia. Although some pathological features of CBGD overlap with those of other forms of non‐Alzheimer non‐Lewy body dementia, the distribution and relative number of these abnormalities and the distinctive pattern of tau immunodeposits allows the distinction of CBGD from Pick's disease and fronto‐temporal dementia. In contrast, PSP only rarely presents with prominent dementia or behavioral changes. In these unusual PSP cases, care must be taken to exclude the diagnoses of CBGD and familial tangle‐only dementia.