Dementia and aphasia in motor neuron disease: an underrecognised association?
OBJECTIVE
To determine the prevalence and nature of global cognitive dysfunction and language deficits in an unselected population based cohort of patients with motor neuron disease (MND).


METHODS
A battery of neuropsychological and language tests was administered to patients presenting consecutively over a 3 year period to a regional neurology service with a new diagnosis of sporadic motor neuron disease.


RESULTS
The 18 patients could be divided on the basis of their performance into three groups: Three patients were demented and had impaired language function (group 1); two non-demented patients had an aphasic syndrome characterised by word finding difficulties and anomia (group 2). Major cognitive deficits were therefore found in five of the 18 patients (28%). The remaining 13 performed normally on the test battery apart from decreased verbal fluency (group 3).


CONCLUSIONS
The prevalence of cognitive impairment in MND in this population based study of an unselected cohort was higher than has been previously reported. Language deficits, especially anomia, may be relatively frequent in the MND population. Aphasia in MND may be masked by dysarthria and missed if not specifically examined.
Rakowicz
W P
WP
Department of Neurology, Norfolk and Norwich Health Care NHS Trust, UK.
Hodges
J R
JR
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001037:Aphasia / Q000175:diagnosis* / Q000209:etiology*; D003704:Dementia / Q000175:diagnosis* / Q000209:etiology*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000150:complications*; D009483:Neuropsychological Tests; D012720:Severity of Illness Index