Fatal familial insomnia (FFI): clinical features

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

Fatal familial insomnia (FFI): clinical features. The onset age is between 20-72 years. Fully referenced neurology checklist.

Onset features

  • The onset age is between 20-72 years
  • The average onset age is 49 years

Cognitive features

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Psychiatric features

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Autonomic features

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Movement disorders

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Ophthalmological features

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Bulbar features

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Other neurological features

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Differential diagnosis

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References

  1. Gambetti P, Kong Q, Zou W, Parchi P, Chen SG. Sporadic and familial CJD: classification and characterisation. Br Med Bull 2003; 66:213-239. 
  2. Krasnianski A, Bartl M, Juan PJS, et al. Fatal familial insomnia: clinical features and early identification. Ann Neurol 2008; 63:658-661.
  3. Brown K, Mastrianni JA. The prion diseases. J Geriatr Psychiatry Neurol 2010; 23:277-298.
  4. Wu LY, Zhan SQ, Huang ZY, et al. Expert consensus on clinical diagnostic criteria for fatal familial insomnia. Chin Med J (Engl) 2018; 131:1613-1617.
  5. Yang TW, Park B, Kim KT, et al. Fatal familial insomnia presenting with agrypnia excitata and very low atonia index level: a case report and literature review. Medicine (Baltimore) 2018; 97:e0646.
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