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-fl uent aphasia culminating in some cases in complete mutism. In five cases, f ormal testing revealed deficits in syntactic comprehension. Comprehension a nd production of verbs were consistently more affected those that of nouns and this effect remained stable upon subsequent testing, despite overall de terioration. The classical signs of MND, including wasting, fasciculations and severe bulbar symptoms, occurred over the following 6-12 months. The be havioural symptoms ranged from mild anosognosia to personality change impli cating frontal-lobe dementia, In three cases, post-mortem examination has c onfirmed the clinical diagnosis of MND-dementia. In addition to the typical involvement of motor and premotor cortex, particularly pronounced patholog ical changes were observed in the Brodmann areas 44 (Broca's area) and 45. The finding of a selective impairment of verb/action processing in associat ion with the demential aphasia syndrome of MND suggests that the neural sub strate underlying verb representation is strongly connected to anterior cor tical motor systems.