M. Mimura et al., Corticobasal degeneration presenting with nonfluent primary progressive aphasia: a clinicopathological study, J NEUR SCI, 183(1), 2001, pp. 19-26
A 62-year-old woman initially presented with slowly progressive nonfluent a
phasia with minimal intellectual involvement. Echolalia and personality cha
nge were prominent whereas parkinsonian features and signs suggesting parie
tal lobe dysfunctions were not present. The patient's language deficit was
consistent with transcortical motor aphasia. She did not manifest extrapyra
midal signs. The patient was diagnosed as having Pick's disease or frontal
lobe dementia. She died at age 65, 2 years and 9 months following disease o
nset. Neuropathological findings including cytoskeletal abnormalities, howe
ver, were clearly distinct from those of classical Pick's disease and were
consistent with those reported in corticobasal degeneration (CBD). The dist
ribution of her cortical lesions was accentuated in the frontal language-re
lated area. The clinical manifestations in CBD are diverse, and primary pro
gressive nonfluent aphasia should be considered as an initial symptom of CB
D. Neuropathological examination of such patients should include cytoskelet
al abnormality studies. (C) 2001 Elsevier Science B.V. All rights reserved.