J Neurol Neurosurg Psychiatry 65:881-889 doi:10.1136/jnnp.65.6.881
  • Paper

Dementia and aphasia in motor neuron disease: an underrecognised association?

  1. Wojtek P Rakowicza,b,
  2. John R Hodgesb
  1. aDepartment of Neurology, Norfolk and Norwich Health Care NHS Trust, Norwich, UK, bDepartment of Neurology, Addenbrooke’s Hospital and MRC Cognition and Brain Sciences Unit, Cambridge, UK
  1. Professor J R Hodges, MRC Cambridge Cognition and Brain Sciences Unit, 15 Chaucer Road, Cambridge CB2 2EF, UK. Telephone 0044 1223 355294; fax 0044 1223 359062.
  • Received 6 June 1997
  • Revised 21 May 1998
  • Accepted 15 June 1998


OBJECTIVES 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.


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