Table 4

Comparisons between Asian FFI patients and non-Asians

Asian (N=66) (n/%)Non-Asian (N=65) (n/%)P value
Demographic dataSex (F/M)27/3930/330.443
Age of onset (years)46.79±12.6048.25±12.500.507
Disease duration (months)11.82±6.4014.74±11.150.108
Definite familial history51 (77.3%)56 (86.2%)0.189
Clinical symptomsPrevalence of sleep-related symptoms64 (97.0%)55 (84.6%)0.014
Prevalence of neuropsychiatric symptoms66 (100%)58 (89.2%)0.006*
Prevalence of progressive sympathetic symptoms56 (84.8%)39 (60.0%)0.001*
Genetic analysesD178N with 129 MM63 (95.5%)48 (73.8%)0.001*
D178N with 129 MV3 (4.5%)17 (26.2%)
Brain MRICerebral cortical atrophy21 (36.2%)5 (14.7%)0.027*
Hyperintense signals on DWI02 (5.9%)0.062
EEGDiffusive excess of slow waves21 (36.8%)20 (45.5%)0.382
Periodic spike discharges03 (6.8%)0.045*
PSGReduced durations of REM25 (86.2%)22 (95.7%)0.251
Sleep-related involuntary14 (48.3%)6 (26.1%)0.102
Sleep-related dyspnoea17 (58.6%)6 (26.1%)0.019*
Laryngeal stridor9 (31.0%)1 (4.3%)0.015*
PETHypometabolism in thalamus15 (71.4%)18 (85.7%)0.259
SPECTInduced blood flow in thalamus5 (100%)3 (71.4%)0.114
CSFPositive for 1433 protein15 (46.9%)5 (19.2%)0.028
  • For frequency calculation, the number of patients with abnormalities was the numerator. For calculation of the positive rate of clinical symptoms, the number of all patients was the denominator. When calculating the positive rate of auxiliary examination, the number of patients who completed each type of test was the denominator.

  • *Significant difference.

  • CSF, cerebrospinal fluid; DWI, diffusion-weighted imaging; EEG, electroencephalogram; F, female; FFI, fatal familial insomnia; M, male; PET, positron emission tomography; PSG, polysomnography; REM, rapid eye movement; SPECT, single-photon emission CT.