Cognitive and motor assessment in autopsy-proven corticobasal degeneration.
OBJECTIVE
To investigate the clinical features of autopsy-proven corticobasal degeneration (CBD).


METHODS
We evaluated symptoms, signs, and neuropsychological deficits longitudinally in 15 patients with autopsy-proven CBD and related these observations directly to the neuroanatomic distribution of disease.


RESULTS
At presentation, a specific pattern of cognitive impairment was evident, whereas an extrapyramidal motor abnormality was present in less than half of the patients. Follow-up examination revealed persistent impairment of apraxia and executive functioning, worsening language performance, and preserved memory. The motor disorder emerged and worsened as the condition progressed. Statistical analysis associated cognitive deficits with tau-immunoreactive pathology that is significantly more prominent in frontal and parietal cortices and the basal ganglia than temporal neocortex and the hippocampus.


CONCLUSIONS
The clinical diagnosis of corticobasal degeneration should depend on a specific pattern of impaired cognition as well as an extrapyramidal motor disorder, reflecting the neuroanatomic distribution of disease in frontal and parietal cortices and the basal ganglia.
Murray
R
R
Department of Neurology, University of Pennsylvania School of Medicine, Philadelphia, PA 19104-4283, USA.
Neumann
M
M
Forman
M S
MS
Farmer
J
J
Massimo
L
L
Rice
A
A
Miller
B L
BL
Johnson
J K
JK
Clark
C M
CM
Hurtig
H I
HI
Gorno-Tempini
M L
ML
Lee
V M-Y
VM
Trojanowski
J Q
JQ
Grossman
M
M
D017668:Age of Onset; D000368:Aged; D001072:Apraxias / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology; D001480:Basal Ganglia Diseases / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology; D001921:Brain / Q000473:pathology / Q000503:physiopathology*; D003072:Cognition Disorders / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology; D018450:Disease Progression; D005260:Female; D006801:Humans; D007806:Language Disorders / Q000175:diagnosis / Q000209:etiology / Q000503:physiopathology; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D009069:Movement Disorders / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology; D009434:Neural Pathways / Q000473:pathology / Q000503:physiopathology; D019636:Neurodegenerative Diseases / Q000175:diagnosis* / Q000503:physiopathology / Q000523:psychology; D016874:Neurofibrillary Tangles / Q000378:metabolism / Q000473:pathology; D009460:Neurologic Examination; D009483:Neuropsychological Tests; D011237:Predictive Value of Tests; D012042:Registries; D024801:Tauopathies / Q000175:diagnosis* / Q000503:physiopathology / Q000523:psychology; D016875:tau Proteins / Q000378:metabolism
D016875:tau Proteins