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Case 15 - Talking about Family Matters

from Part 3 - Missing Important Clues in the History

Published online by Cambridge University Press:  03 November 2020

Keith Josephs
Affiliation:
Mayo Clinic Alzheimer’s Disease Research Center
Federico Rodriguez-Porcel
Affiliation:
Medical University of South Carolina
Rhonna Shatz
Affiliation:
University of Cincinnati
Daniel Weintraub
Affiliation:
University of Pennsylvania
Alberto Espay
Affiliation:
University of Cincinnati
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Summary

This 58-year-old right-handed man presented with a 3-year history of personality changes and imbalance. His family first noticed he was more withdrawn from social activities and seemed to care less about his appearance. In addition, he was not as affectionate as before and would often make inappropriate comments (e.g., telling people they were overweight). After a recent death in the family, he did not seem to be emotionally affected. More recently, he became more disorganized, which affected his work performance. His gait slowed, and he started to fall frequently. Despite all these changes, he did not seem to be bothered by his symptoms. On neurological examination, he exhibited a resting tremor of the left leg as well as symmetric bradykinesia and upward vertical gaze limitation. His gait was mildly wide-based and unstable, with quick turning (Video 15.1). When asked about family history of neurological disease, he reported his father recently died with Parkinson disease. In addition, out of his five siblings, two older brothers had been diagnosed with dementia and a younger sister with late-onset schizophrenia. His neuropsychological evaluation revealed prominent deficits in executive function, including response inhibition (i.e., ability to suppress inappropriate responses), fluency (i.e., producing words in a limited amount of time), and set-shifting (i.e., switching between different tasks).

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Chapter
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Publisher: Cambridge University Press
Print publication year: 2020

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References

Deleon, J. and Miller, B. L. 2018. Frontotemporal dementia. Handb Clin Neurol 148 409430.Google Scholar
Goldman, J. S. et al. 2005. Comparison of family histories in FTLD subtypes and related tauopathies. Neurology 65(11) 18171819.CrossRefGoogle ScholarPubMed
Josephs, K. A. et al. 2011. Neuropathological background of phenotypical variability in frontotemporal dementia. Acta Neuropathol 122(2) 137153.CrossRefGoogle ScholarPubMed
Neumann, M. and Mackenzie, I. R. A. 2019. Review: neuropathology of non-tau frontotemporal lobar degeneration. Neuropathol Appl Neurobiol 45(1) 1940.CrossRefGoogle ScholarPubMed
Rademakers, R., Neumann, M. and Mackenzie, I. R. 2012. Advances in understanding the molecular basis of frontotemporal dementia. Nat Rev Neurol 8(8) 423434.Google Scholar
Rohrer, J. D. et al. 2009. The heritability and genetics of frontotemporal lobar degeneration. Neurology 73(18) 14511456.CrossRefGoogle ScholarPubMed

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