Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Case Study
  • Published:

The case of a 48 year-old woman with bizarre and complex delusions

Abstract

Background. A 48 year-old woman presented with an 18 month history of bizarre and complex delusions on a background of social, behavioral and cognitive decline over several years. Her psychosis progressed despite receiving high doses of antipsychotics. The patient's father also had a psychotic episode in his 40s. He subsequently developed motor neuron disease, which caused his death at 68 years of age.

Investigations. Physical examination, neuropsychological testing, nerve conduction studies, brain MRI and transcranial magnetic stimulation.

Diagnosis. On the basis of the patient's age at onset of the delusions, imaging findings and family history, a diagnosis of frontotemporal dementia (FTD) was favored over a primary psychotic disorder. The ubiquitin-positive and TAR DNA binding protein 43-positive inclusions that were found at autopsy confirmed the diagnosis of FTD.

Management. The patient was treated with various antipsychotics at high doses; however, her delusions continued to progress. No disease-specific treatments for FTD currently exist.

This is a preview of subscription content, access via your institution

Access options

Buy this article

Prices may be subject to local taxes which are calculated during checkout

Figure 1: MRI of the patient's brain.
Figure 2: Neuropathology of the patient's brain.

References

  1. Mioshi, E., Dawson, K., Mitchell, J., Arnold, R. & Hodges, J. R. The Addenbrooke's Cognitive Examination Revised (ACE-R): a brief cognitive test battery for dementia screening. Int. J. Geriatr. Psychiatry 21, 1078–1085 (2006).

    Article  Google Scholar 

  2. Vucic, S., Nicholson, G. A. & Kiernan, M. C. Cortical hyperexcitability may precede the onset of familial amyotrophic lateral sclerosis. Brain 131, 1540–1550 (2008).

    Article  Google Scholar 

  3. American Psychiatric Association. Diagnostic and Statistical Manual of Mental Disorders DSM-IV-TR (American Psychiatric Association, Washington DC, 2000).

  4. Kessler, R. C. et al. Age of onset of mental disorders: a review of recent literature. Curr. Opin. Psychiatry 20, 359–364 (2007).

    Article  Google Scholar 

  5. Rajji, T. K. & Mulsant, B. H. Nature and course of cognitive function in late-life schizophrenia: a systematic review. Schizophr. Res. 102, 122–140 (2008).

    Article  Google Scholar 

  6. Mitelman, S. A. et al. A comprehensive assessment of gray and white matter volumes and their relationship to outcome and severity in schizophrenia. Neuroimage 37, 449–462 (2007).

    Article  Google Scholar 

  7. Neary, D. et al. Frontotemporal lobar degeneration: a consensus on clinical diagnostic criteria. Neurology 51, 1546–1554 (1998).

    Article  CAS  Google Scholar 

  8. Hodges, J. R. et al. Clinicopathological correlates in frontotemporal dementia. Ann. Neurol. 56, 399–406 (2004).

    Article  Google Scholar 

  9. Omar, R. et al. Delusions in frontotemporal lobar degeneration. J. Neurol. 256, 600–607 (2009).

    Article  Google Scholar 

  10. Bak, T. H., O'Donovan, D. G., Xuereb, J. H., Boniface, S. & Hodges, J. R. Selective impairment of verb processing associated with pathological changes in Brodmann areas 44 and 45 in the motor neurone disease-dementia-aphasia syndrome. Brain 124, 103–120 (2001).

    Article  CAS  Google Scholar 

  11. Velakoulis, D., Walterfang, M., Mocellin, R., Pantelis, C. & McLean, C. Frontotemporal dementia presenting as schizophrenia-like psychosis in young people: clinicopathological series and review of cases. Br. J. Psychiatry 194, 298–305 (2009).

    Article  CAS  Google Scholar 

  12. Luty, A. A. et al. Pedigree with frontotemporal lobar degeneration–motor neuron disease and Tar DNA binding protein-43 positive neuropathology: genetic linkage to chromosome 9. BMC Neurol. 8, 32 (2008).

    Article  Google Scholar 

  13. Vance, C. et al. Familial amyotrophic lateral sclerosis with frontotemporal dementia is linked to a locus on chromosome 9p13.2–213. Brain 129, 868–876 (2006).

    Article  Google Scholar 

  14. Kril, J. J., Macdonald, V., Patel, S., Png, F. & Halliday, G. M. Distribution of brain atrophy in behavioral variant frontotemporal dementia. J. Neurol. Sci. 232, 83–90 (2005).

    Article  Google Scholar 

  15. Hoffman, R. E., Anderson, A. W., Varanko, M., Gore, J. C. & Hampson, M. Time course of regional brain activation associated with onset of auditory/verbal hallucinations. Br. J. Psychiatry 193, 424–425 (2008).

    Article  Google Scholar 

  16. Neumann, M. et al. Frontotemporal lobar degeneration with FUS pathology. Brain doi:10.1093/brain/awp214.

    Article  Google Scholar 

  17. Huey, E. D., Putnam, K. T. & Grafman, J. A systematic review of neurotransmitter deficits and treatments in frontotemporal dementia. Neurology 66, 17–22 (2006).

    Article  CAS  Google Scholar 

  18. Knopman, D. S. et al. Development of methodology for conducting clinical trials in frontotemporal lobar degeneration. Brain 131, 2957–2968 (2008).

    Article  Google Scholar 

  19. Schymick, J. C. et al. Progranulin mutations and amyotrophic lateral sclerosis or amyotrophic lateral sclerosis-frontotemporal dementia phenotypes. J. Neurol. Neurosurg. Psychiatry 78, 754–756 (2007).

    Article  CAS  Google Scholar 

Download references

Acknowledgements

Written consent for publication was obtained from the patient's family. This study was supported by a NHMRC Postgraduate Scholarship to C. T. Loy.

Désirée Lie, University of Calfornia, Irvine, CA, is the author of and is solely responsible for the content of the learning objectives, questions and answers of the MedscapeCME-accredited continuing medical education activity associated with this article.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to John R. Hodges.

Ethics declarations

Competing interests

The authors and the Journal Editor H. Wood declare no competing interests. The CME questions author D. Lie has served as a nonproduct speaker for "Topics in Health" for Merck Speaker Services.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Loy, C., Kril, J., Trollor, J. et al. The case of a 48 year-old woman with bizarre and complex delusions. Nat Rev Neurol 6, 175–179 (2010). https://doi.org/10.1038/nrneurol.2010.3

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1038/nrneurol.2010.3

This article is cited by

Search

Quick links

Nature Briefing

Sign up for the Nature Briefing newsletter — what matters in science, free to your inbox daily.

Get the most important science stories of the day, free in your inbox. Sign up for Nature Briefing