Table 2

Clinical characteristics of all FFI patients

ParameterCommonFrequentLess frequentRare
Cluster A (sleep-related symptoms)
 Insomnia87.0%
 Sleep-related involuntary movements46.6%
 Sleep-related dyspnoea25.2%
 Laryngeal stridor32.1%
Cluster B (neuropsychiatric symptoms)
 Rapidly progressive dementia83.2%
 Psychiatric symptoms66.4%
 Ataxia56.5%
 Pyramidal sign25.2%
 Parkinsonism36.6%
 Myoclonus29.8%
 Bulbar syndrome25.2%
 Diplopia22.1%
Cluster C (progressive sympathetic symptoms)
 Sweating55.0%
 Hypertension33.6%
 Weight loss30.5%
 Tachycardia24.4%
 Irregular breathing16.8%
 Hyperthermia19.8%
 Others*13.0%
  • *Others in cluster C represent constipation, urinary retention and sexual dysfunction.

  • FFI, fatal familial insomnia.;