Table 1 Summary of demographic and clinical information

Demographic information

Sex

Male: female = 12:20

Age onset (years, \(\overline{\mathrm{x}}\pm \mathrm{s }\))

54.3 \(\pm\) 10.2

Duration of disease (months, \(\overline{\mathrm{x}}\pm \mathrm{s }\))

57.8 \(\pm\) 51.4

Family history (%)

9/32 (28.1%)

Clinical presentations

Dementia (%)

27/32 (84.4%)

Autonomic nervous system dysfunction (%)

22/32 (68.8%)

Urinary and/or fecal dysfunction (%)

19/32 (59.4%)

Orthostatic hypotension (%)

3/32 (9.4%)

Nausea/ vomit (%)

3/32 (9.4%)

Miosis (%)

2/32 (6.3%)

Hidrosis (%)

2/32 (6.3%)

Erectile dysfunction (%)

1/32 (3.1%)

Episodes of fever (%)

12/32 (37.5%)

Movement disorder (%)

11/32 (34.4%)

Parkinsonism (%)

5/32 (15.6%)

Tremor (%)

6/32 (18.8%)

Encephalopathy like attacks (%)

11/32 (34.4%)

Headache (%)

11/32 (34.4%)

Stroke like attacks (%)

10/32 (31.3%)

Seizure (%)

4/32 (12.5%)

Examinations

MMSE (0–30, \(\overline{\mathrm{x}}\pm \mathrm{s }\))

19.6 \(\pm\) 9.2

MoCA (0–30, \(\overline{\mathrm{x}}\pm \mathrm{s }\))

16.7 \(\pm\) 8.9

Abnormal electromyogram (%)

5/12 (41.7%)

Abnormal electroencephalogram (%)

12/16 (75.0%)

Abnormal CSF analysis (%)

0/20 (0%)

CSF Aβ₄₂ (pg/ml, \(\overline{\mathrm{x}}\pm \mathrm{s }\))

882.6 \(\pm\) 316.3

CSF t-tau (pg/ml, \(\overline{\mathrm{x}}\pm \mathrm{s }\))

172.4 \(\pm\) 97.4

CSF p-tau (pg/ml, \(\overline{\mathrm{x}}\pm \mathrm{s }\))

52.2 \(\pm\) 22.8

Abnormal ¹⁸F-FDG-PET/CT (%)

2/2 (100%)