From progressive nonfluent aphasia to corticobasal syndrome: a case report of corticobasal degeneration.
In a previous report, we presented longitudinal clinical, cognitive and anatomical data of a right-handed woman, whose clinical picture evolved from progressive nonfluent aphasia with apraxia of speech to corticobasal syndrome (CBS) in the last stage of the disease. The patient died at age 57 and pathological examination revealed severe atrophy in the left frontal operculum and left premotor area. On histological examination, there was diffuse tau-positive pathology in gray and white cortical hemispheric gray and white matter, basal ganglia and substantia nigra, compatible with corticobasal degeneration (CBD). This case demonstrates the clinical overlap between frontotemporal lobar degeneration and CBD. In this case, early motor speech impairment predicted earlier and more accurately than CBS the presence of underlying tau-pathology and CBD.
Sánchez-Valle
R
R
UCSF Memory and Aging Center, San Francisco, California 94143-1207, USA.
Forman
M S
MS
Miller
B L
BL
Gorno-Tempini
M L
ML
D018888:Aphasia, Primary Progressive / Q000175:diagnosis* / Q000209:etiology* / Q000503:physiopathology; D001479:Basal Ganglia / Q000473:pathology / Q000503:physiopathology; D001921:Brain / Q000473:pathology* / Q000503:physiopathology*; D002540:Cerebral Cortex / Q000473:pathology / Q000503:physiopathology; D003704:Dementia / Q000175:diagnosis / Q000473:pathology / Q000503:physiopathology; D003937:Diagnosis, Differential; D018450:Disease Progression; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008875:Middle Aged; D009413:Nerve Fibers, Myelinated / Q000378:metabolism / Q000473:pathology; D016874:Neurofibrillary Tangles / Q000378:metabolism / Q000473:pathology; D009474:Neurons / Q000378:metabolism / Q000473:pathology; D011237:Predictive Value of Tests; D013378:Substantia Nigra / Q000473:pathology / Q000503:physiopathology; D024801:Tauopathies / Q000150:complications* / Q000175:diagnosis* / Q000503:physiopathology; D016875:tau Proteins / Q000378:metabolism
D016875:tau Proteins