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Journal of Neurology, Neurosurgery, and Psychiatry logoLink to Journal of Neurology, Neurosurgery, and Psychiatry
. 1998 Dec;65(6):881–889. doi: 10.1136/jnnp.65.6.881

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

W Rakowicz 1, J Hodges 1
PMCID: PMC2170423  PMID: 9854965

Abstract

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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Selected References

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  1. Abrahams S., Goldstein L. H., Al-Chalabi A., Pickering A., Morris R. G., Passingham R. E., Brooks D. J., Leigh P. N. Relation between cognitive dysfunction and pseudobulbar palsy in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry. 1997 May;62(5):464–472. doi: 10.1136/jnnp.62.5.464. [DOI] [PMC free article] [PubMed] [Google Scholar]
  2. Berg L. Clinical Dementia Rating (CDR). Psychopharmacol Bull. 1988;24(4):637–639. [PubMed] [Google Scholar]
  3. Capitani E., Della Sala S., Marchetti C. Is there a cognitive impairment in MND? A survey with longitudinal data. Schweiz Arch Neurol Psychiatr (1985) 1994;145(1):11–13. [PubMed] [Google Scholar]
  4. Caselli R. J., Windebank A. J., Petersen R. C., Komori T., Parisi J. E., Okazaki H., Kokmen E., Iverson R., Dinapoli R. P., Graff-Radford N. R. Rapidly progressive aphasic dementia and motor neuron disease. Ann Neurol. 1993 Feb;33(2):200–207. doi: 10.1002/ana.410330210. [DOI] [PubMed] [Google Scholar]
  5. David A. S., Gillham R. A. Neuropsychological study of motor neuron disease. Psychosomatics. 1986 Jun;27(6):441–445. doi: 10.1016/S0033-3182(86)72673-X. [DOI] [PubMed] [Google Scholar]
  6. Folstein M. F., Folstein S. E., McHugh P. R. "Mini-mental state". A practical method for grading the cognitive state of patients for the clinician. J Psychiatr Res. 1975 Nov;12(3):189–198. doi: 10.1016/0022-3956(75)90026-6. [DOI] [PubMed] [Google Scholar]
  7. Gallassi R., Montagna P., Morreale A., Lorusso S., Tinuper P., Daidone R., Lugaresi E. Neuropsychological, electroencephalogram and brain computed tomography findings in motor neuron disease. Eur Neurol. 1989;29(2):115–120. doi: 10.1159/000116391. [DOI] [PubMed] [Google Scholar]
  8. Gubbay S. S., Kahana E., Zilber N., Cooper G., Pintov S., Leibowitz Y. Amyotrophic lateral sclerosis. A study of its presentation and prognosis. J Neurol. 1985;232(5):295–300. doi: 10.1007/BF00313868. [DOI] [PubMed] [Google Scholar]
  9. Gunnarsson L. G., Dahlbom K., Strandman E. Motor neuron disease and dementia reported among 13 members of a single family. Acta Neurol Scand. 1991 Nov;84(5):429–433. doi: 10.1111/j.1600-0404.1991.tb04983.x. [DOI] [PubMed] [Google Scholar]
  10. Hodges J. R., Salmon D. P., Butters N. The nature of the naming deficit in Alzheimer's and Huntington's disease. Brain. 1991 Aug;114(Pt 4):1547–1558. doi: 10.1093/brain/114.4.1547. [DOI] [PubMed] [Google Scholar]
  11. Hudson A. J. Amyotrophic lateral sclerosis and its association with dementia, parkinsonism and other neurological disorders: a review. Brain. 1981 Jun;104(2):217–247. doi: 10.1093/brain/104.2.217. [DOI] [PubMed] [Google Scholar]
  12. Kew J. J., Goldstein L. H., Leigh P. N., Abrahams S., Cosgrave N., Passingham R. E., Frackowiak R. S., Brooks D. J. The relationship between abnormalities of cognitive function and cerebral activation in amyotrophic lateral sclerosis. A neuropsychological and positron emission tomography study. Brain. 1993 Dec;116(Pt 6):1399–1423. doi: 10.1093/brain/116.6.1399. [DOI] [PubMed] [Google Scholar]
  13. Kew J. J., Leigh P. N., Playford E. D., Passingham R. E., Goldstein L. H., Frackowiak R. S., Brooks D. J. Cortical function in amyotrophic lateral sclerosis. A positron emission tomography study. Brain. 1993 Jun;116(Pt 3):655–680. doi: 10.1093/brain/116.3.655. [DOI] [PubMed] [Google Scholar]
  14. Kew J., Leigh N. Dementia with motor neurone disease. Baillieres Clin Neurol. 1992 Nov;1(3):611–626. [PubMed] [Google Scholar]
  15. Kurtzke J. F. The current neurologic burden of illness and injury in the United States. Neurology. 1982 Nov;32(11):1207–1214. doi: 10.1212/wnl.32.11.1207. [DOI] [PubMed] [Google Scholar]
  16. Lopez O. L., Becker J. T., DeKosky S. T. Dementia accompanying motor neuron disease. Dementia. 1994 Jan-Feb;5(1):42–47. doi: 10.1159/000106693. [DOI] [PubMed] [Google Scholar]
  17. Ludolph A. C., Langen K. J., Regard M., Herzog H., Kemper B., Kuwert T., Böttger I. G., Feinendegen L. Frontal lobe function in amyotrophic lateral sclerosis: a neuropsychologic and positron emission tomography study. Acta Neurol Scand. 1992 Feb;85(2):81–89. doi: 10.1111/j.1600-0404.1992.tb04003.x. [DOI] [PubMed] [Google Scholar]
  18. Massman P. J., Sims J., Cooke N., Haverkamp L. J., Appel V., Appel S. H. Prevalence and correlates of neuropsychological deficits in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry. 1996 Nov;61(5):450–455. doi: 10.1136/jnnp.61.5.450. [DOI] [PMC free article] [PubMed] [Google Scholar]
  19. Mitsuyama Y. Presenile dementia with motor neuron disease. Dementia. 1993 May-Aug;4(3-4):137–142. doi: 10.1159/000107312. [DOI] [PubMed] [Google Scholar]
  20. Mitsuyama Y., Takamiya S. Presenile dementia with motor neuron disease in Japan. A new entity? Arch Neurol. 1979 Sep;36(9):592–593. doi: 10.1001/archneur.1979.00500450086021. [DOI] [PubMed] [Google Scholar]
  21. Neary D., Snowden J. S., Mann D. M., Northen B., Goulding P. J., Macdermott N. Frontal lobe dementia and motor neuron disease. J Neurol Neurosurg Psychiatry. 1990 Jan;53(1):23–32. doi: 10.1136/jnnp.53.1.23. [DOI] [PMC free article] [PubMed] [Google Scholar]
  22. Neary D., Snowden J. S., Mann D. M. The clinical pathological correlates of lobar atrophy. Dementia. 1993 May-Aug;4(3-4):154–159. doi: 10.1159/000107315. [DOI] [PubMed] [Google Scholar]
  23. Rosser A., Hodges J. R. Initial letter and semantic category fluency in Alzheimer's disease, Huntington's disease, and progressive supranuclear palsy. J Neurol Neurosurg Psychiatry. 1994 Nov;57(11):1389–1394. doi: 10.1136/jnnp.57.11.1389. [DOI] [PMC free article] [PubMed] [Google Scholar]
  24. Wikström J., Paetau A., Palo J., Sulkava R., Haltia M. Classic amyotrophic lateral sclerosis with dementia. Arch Neurol. 1982 Nov;39(11):681–683. doi: 10.1001/archneur.1982.00510230007002. [DOI] [PubMed] [Google Scholar]
  25. Yoshida M., Murakami N., Hashizume Y., Takahashi A. [A clinicopathological study on 13 cases of motor neuron disease with dementia]. Rinsho Shinkeigaku. 1992 Nov;32(11):1193–1202. [PubMed] [Google Scholar]
  26. Zigmond A. S., Snaith R. P. The hospital anxiety and depression scale. Acta Psychiatr Scand. 1983 Jun;67(6):361–370. doi: 10.1111/j.1600-0447.1983.tb09716.x. [DOI] [PubMed] [Google Scholar]

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