Selective impairment of verb processing associated with pathological changes in Brodmann areas 44 and 45 in the motor neurone disease-dementia-aphasia syndrome.
We report six patients with clinically diagnosed and electrophysiologically confirmed motor neurone disease (MND), in whom communication problems were an early and dominant feature. All patients developed a progressive non-fluent aphasia culminating in some cases in complete mutism. In five cases, formal testing revealed deficits in syntactic comprehension. Comprehension and production of verbs were consistently more affected those that of nouns and this effect remained stable upon subsequent testing, despite overall deterioration. The classical signs of MND, including wasting, fasciculations and severe bulbar symptoms, occurred over the following 6-12 months. The behavioural symptoms ranged from mild anosognosia to personality change implicating frontal-lobe dementia. In three cases, post-mortem examination has confirmed the clinical diagnosis of MND-dementia. In addition to the typical involvement of motor and premotor cortex, particularly pronounced pathological changes were observed in the Brodmann areas 44 (Broca's area) and 45. The finding of a selective impairment of verb/action processing in association with the dementia/aphasia syndrome of MND suggests that the neural substrate underlying verb representation is strongly connected to anterior cortical motor systems.
Bak
T H
TH
Medical Research Council Cognition and Brain Sciences Unit, The University of Cambridge, UK.
O'Donovan
D G
DG
Xuereb
J H
JH
Boniface
S
S
Hodges
J R
JR
D000368:Aged; D001037:Aphasia / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology; D001933:Brain Stem / Q000473:pathology; D003704:Dementia / Q000150:complications / Q000175:diagnosis* / Q000503:physiopathology; D018450:Disease Progression; D004576:Electromyography; D017809:Fatal Outcome; D005260:Female; D005625:Frontal Lobe / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009044:Motor Cortex / Q000473:pathology; D016472:Motor Neuron Disease / Q000150:complications / Q000473:pathology* / Q000503:physiopathology; D009483:Neuropsychological Tests; D010296:Parietal Lobe / Q000473:pathology; D013116:Spinal Cord / Q000473:pathology; D013577:Syndrome; D013702:Temporal Lobe / Q000473:pathology; D015899:Tomography, Emission-Computed, Single-Photon; D014057:Tomography, X-Ray Computed