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. 1983 Nov;46(11):971–980. doi: 10.1136/jnnp.46.11.971

Cognitive and personality function in myotonic muscular dystrophy.

T D Bird, C Follett, E Griep
PMCID: PMC491732  PMID: 6655483

Abstract

Twenty-nine patients with myotonic dystrophy from 14 families were tested with the Wechsler and Shipley measures of cognitive function. Forty-one per cent of the subjects had little or no physical handicap. Approximately one-third had low Wechsler scores, whereas 7% had relatively high scores. There was a trend for affected females to have poorer cognitive function than males. Limited cognitive ability correlated with maternal inheritance of the gene and severe physical handicap, but there were individual exceptions. Strongest cognitive abilities were verbal and informational, whereas the weakest were immediate recall, abstraction and spatial manipulation and orientation. There was no evidence of intellectual decline with time. Signs of cerebral atrophy on CT scans were uncommon, occurring for certain in only one of 19 subjects. Personality profiles were also constructed for 25 myotonic subjects using interview and MMPI techniques. Forty-four per cent of the subjects had unremarkable personality profiles, 24% had mild personality difficulties and 32% had prominent personality abnormalities. Serious personality difficulty was most common in patients with low cognitive ability and advanced physical handicap. There was no "typical" personality pattern representative of the entire group. It is likely that many personality problems were the result of individuals with limited resources attempting to cope with their physically deforming and debilitating neuromuscular disorder.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Ambrosini P. G., Nurnberg H. G. Psychopathology: a primary feature of myotonic dystrophy. Psychosomatics. 1979 Jun;20(6):393-5, 398-9. doi: 10.1016/s0033-3182(79)70797-3. [DOI] [PubMed] [Google Scholar]
  2. Bird T. D. Myotonic dystrophy associated with Down syndrome (trisomy 21). Neurology. 1981 Apr;31(4):440–442. doi: 10.1212/wnl.31.4.440. [DOI] [PubMed] [Google Scholar]
  3. Bundey S. Clinical evidence for heterogeneity in myotonic dystrophy. J Med Genet. 1982 Oct;19(5):341–348. doi: 10.1136/jmg.19.5.341. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Calderon R. Myotonic dystrophy: a neglected cause of mental retardation. J Pediatr. 1966 Mar;68(3):423–431. doi: 10.1016/s0022-3476(66)80246-9. [DOI] [PubMed] [Google Scholar]
  5. Coccagna G., Mantovani M., Parchi C., Mironi F., Lugaresi E. Alveolar hypoventilation and hyperosmnia in myotonic dystrophy. J Neurol Neurosurg Psychiatry. 1975 Oct;38(10):977–984. doi: 10.1136/jnnp.38.10.977. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Culebras A., Feldman R. G., Merk F. B. Cytoplasmic inclusion bodies within neurons of the thalamus in myotonic dystrophy. A light and electron microscope study. J Neurol Sci. 1973 Jul;19(3):319–329. doi: 10.1016/0022-510x(73)90095-6. [DOI] [PubMed] [Google Scholar]
  7. DODGE P. R., GAMSTORP I., BYERS R. K., RUSSELL P. MYOTONIC DYSTROPHY IN INFANCY AND CHILDHOOD. Pediatrics. 1965 Jan;35:3–19. [PubMed] [Google Scholar]
  8. Dubowitz V. Involvement of the nervous system in muscular dystrophies in man. Ann N Y Acad Sci. 1979;317:431–439. doi: 10.1111/j.1749-6632.1979.tb56558.x. [DOI] [PubMed] [Google Scholar]
  9. Karagan N. J., Sorensen J. P. Intellectual functioning in non-Duchenne muscular dystrophy. Neurology. 1981 Apr;31(4):448–452. doi: 10.1212/wnl.31.4.448. [DOI] [PubMed] [Google Scholar]
  10. Lacks P. B., Keefe K. Relationships among education, the MMPI, and WAIS measures of psychopathology. J Clin Psychol. 1970 Oct;26(4):468–470. doi: 10.1002/1097-4679(197010)26:4<468::aid-jclp2270260418>3.0.co;2-6. [DOI] [PubMed] [Google Scholar]
  11. Lynch H. T., Tips R. L., Krush A. Psychodynamics in a chronic debilitating hereditary disease. Myotonia dystrophica. Arch Gen Psychiatry. 1966 Feb;14(2):153–157. doi: 10.1001/archpsyc.1966.01730080041007. [DOI] [PubMed] [Google Scholar]
  12. Peyser J. M., Edwards K. R., Poser C. M. Psychological profiles in patients with multiple sclerosis. A preliminary investigation. Arch Neurol. 1980 Jul;37(7):437–440. doi: 10.1001/archneur.1980.00500560067009. [DOI] [PubMed] [Google Scholar]
  13. Refsum S., Lonnum A., Sjaastad O., Engeset A. Dystrophia myotonica. Repeated pneumoencephalographic studies in ten patients. Neurology. 1967 Apr;17(4):345–348. doi: 10.1212/wnl.17.4.345. [DOI] [PubMed] [Google Scholar]
  14. Rosman N. P., Kakulas B. A. Mental deficiency associated with muscular dystrophy. A neuropathological study. Brain. 1966 Dec;89(4):769–788. doi: 10.1093/brain/89.4.769. [DOI] [PubMed] [Google Scholar]
  15. Watters G. V., Williams T. W. Early onset myotonic dystrophy. Clinical and laboratory findings in five families and a review of the literature. Arch Neurol. 1967 Aug;17(2):137–152. doi: 10.1001/archneur.1967.00470260027003. [DOI] [PubMed] [Google Scholar]
  16. Wiśniewski H. M., Berry K., Spiro A. J. Ultrastructure of thalamic neuronal inclusions in myotonic dystrophy. J Neurol Sci. 1975 Mar;24(3):321–329. doi: 10.1016/0022-510x(75)90252-x. [DOI] [PubMed] [Google Scholar]

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