Table 1.
Clinical features of MM2 c and MM1/classic sCJD groups.
| MM2 c (N = 6) | MM1/classic CJD (N = 8) | P | ||
|---|---|---|---|---|
| Male | 4/6 (66.7%) | 2/8 (25%) | ||
| Age at onset (median, range) | 75 (60–83) | 72.5 (51–74) | 0.17 | |
| Time of first consultation from disease onset (months; mean, range) | 8.5 (4–17) | 1.75 (1–3) | 0.002 | |
| Time of first EEG from disease onset (months; mean, range) | 9.17 (4–15) | 1.88 (1–4) | 0.003 | |
| Diagnosis | ||||
| Definite | 2/6 (33.30%) | 3/8 (37.5%) | ||
| Probable | 3/6 (50%) | 5/8 (62.5%) | ||
| Possible | 1/6 (16.7%) | 0/8 (0%) | ||
| Clinical symptoms and signs** | ||||
| Cognitive function (mean, range)*** | (MMSE) | 9.25 (0–23) | 9 (0–20) | |
| (HDS-R)**** | 4.5 (0–12) | 10 (0–26) | ||
| Progressive dementia | (Rapid) | 1/6 (16.7%) | 8/8 (100%) | |
| (Slow) | 5/6 (83.3%) | 0/8 (0%) | ||
| Myoclonus | 1/6 (16.7%) | 8/8 (100%) | ||
| Visual or Cerebellar signs | 5/6 (83.3%) | 7/8 (87.5%) | ||
| Pyramidal/extrapyramidal signs | 2/5 (40%) | 5/8 (62.5%) | ||
| Akinetic mutism | 0/6 (0%) | 0/8 (0%) | ||
**Clinical signs and symptoms, except cognitive function, were observed one year and 1 to 3 months after disease onset in the MM2c-sCJD and the MM1/classic CJD groups, respectively.
***Cognitive function was examined between 4 to 17 months and between 1 to 3 months after disease onset, in the MM2c-sCJD and the MM1/classic CJD groups, respectively.
****Imai Y, Hasegawa K. The revised Hasegawa’s dementia scale (HDS-R)-evaluation of its usefulness as a screening test for dementia. J Hong Kong Coll Psychiatr.1994;4 (2):20–24.
EEG: electroencephalogram, sCJD: sporadic Creutzfeldt-Jakob disease, MM2c: methionine homozygosity type 2 cortical form, MMSE: Mini-Mental State Examination.