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. 2021 Oct 19;93(3):291–297. doi: 10.1136/jnnp-2021-327247

Table 4.

Comparisons between Asian FFI patients and non-Asians

Asian (N=66) (n/%) Non-Asian (N=65) (n/%) P value
Demographic data Sex (F/M) 27/39 30/33 0.443
Age of onset (years) 46.79±12.60 48.25±12.50 0.507
Disease duration (months) 11.82±6.40 14.74±11.15 0.108
Definite familial history 51 (77.3%) 56 (86.2%) 0.189
Clinical symptoms Prevalence of sleep-related symptoms 64 (97.0%) 55 (84.6%) 0.014
Prevalence of neuropsychiatric symptoms 66 (100%) 58 (89.2%) 0.006*
Prevalence of progressive sympathetic symptoms 56 (84.8%) 39 (60.0%) 0.001*
Genetic analyses D178N with 129 MM 63 (95.5%) 48 (73.8%) 0.001*
D178N with 129 MV 3 (4.5%) 17 (26.2%)
Brain MRI Cerebral cortical atrophy 21 (36.2%) 5 (14.7%) 0.027*
Hyperintense signals on DWI 0 2 (5.9%) 0.062
EEG Diffusive excess of slow waves 21 (36.8%) 20 (45.5%) 0.382
Periodic spike discharges 0 3 (6.8%) 0.045*
PSG Reduced durations of REM 25 (86.2%) 22 (95.7%) 0.251
Sleep-related involuntary 14 (48.3%) 6 (26.1%) 0.102
Sleep-related dyspnoea 17 (58.6%) 6 (26.1%) 0.019*
Laryngeal stridor 9 (31.0%) 1 (4.3%) 0.015*
PET Hypometabolism in thalamus 15 (71.4%) 18 (85.7%) 0.259
SPECT Induced blood flow in thalamus 5 (100%) 3 (71.4%) 0.114
CSF Positive for 1433 protein 15 (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.