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Case 31 - Siblings with a Fatal Cause of Rapidly Progressive Dementia

Published online by Cambridge University Press:  09 January 2021

By
Tharick A. Pascoal ,
Mira Chamoun ,
Sara Mohades ,
Monica Shin ,
Liyong Wu ,
Serge Gauthier  and
Pedro Rosa-Neto
Edited by
Pedro Rosa-Neto  and
Serge Gauthier
Pedro Rosa-Neto
Affiliation:
McGill University, Montréal
Serge Gauthier
Affiliation:
McGill University, Montréal
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Summary

A 62-year-old male (Patient 1) was admitted to the Capital Medical University Hospital, in Beijing, China, because of a 3-month history of progressive cognitive impairment and abnormal behaviors including performing motor gestures and talking to himself incoherently. During the first evaluation, it was reported that the patient had insomnia as an early clinical manifestation accompanied by intense dreams and sleep talking. During the 14 days of hospitalization, the patient showed intractable insomnia, progressive cognitive deterioration, mental confusion, visual and auditory hallucinations, and paranoia. Twelve months after the onset of the symptoms, the patient returned to the hospital awake but unresponsive and died due to breathing difficulties.

Type
Chapter
Information
Case Studies in Dementia
Common and Uncommon Presentations
 , pp. 136 - 139
Publisher: Cambridge University Press
Print publication year: 2021

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References

Further Reading

Lugaresi, E, Medori, R, Montagna, P, et al. Fatal familial insomnia and dysautonomia with selective degeneration of thalamic nuclei. N Engl J Med. 1986;315(16):997–1003.CrossRefGoogle ScholarPubMed
Manetto, V, Medori, R, Cortelli, P, et al. Fatal familial insomnia: clinical and pathologic study of five new cases. Neurology. 1992;42:312–319.CrossRefGoogle ScholarPubMed
Medori, R, Tritschler, HJ, LeBlanc, A, et al. Fatal familial insomnia, a prion disease with a mutation at codon 178 of the prion protein gene. N Engl J Med. 1992;326:444–449.CrossRefGoogle ScholarPubMed
Montagna, P. Fatal familial insomnia: a model disease in sleep physiopathology. Sleep Med Rev. 2005;9:339–353.CrossRefGoogle Scholar
Montagna, P, Gambetti, P, Cortelli, P, Lugaresi, E. Familial and sporadic fatal insomnia. Lancet Neurol. 2003;2:167–176.CrossRefGoogle ScholarPubMed

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