Table 1.
Patient characteristics and diagnosis.
| Characteristic | Categories | Number (%) |
|---|---|---|
| Age (yrs) (N = 13) | Median (range) | 43 (17–69) |
| Gender (N = 13) | Male | 4 (30.8 %) |
| Female | 9 (69.2 %) | |
| Country (N = 13) | China | 7 (53.8 %) |
| Japan | 3 (23.1 %) | |
| India | 2 (15.4 %) | |
| Turkey | 1 (7.7 %) | |
| Location of ischemia (N = 13) | Anterior circulation | 10 (76.9 %) |
| Posterior circulation | 2 (15.4 %) | |
| Anterior and posterior circulation | 1 (7.7%s) | |
| Involved cervical and intracranial large vessels (N = 13) | ICA | 6 (46.2 %) |
| MCA | 6 (46.2 %) | |
| CCA | 1 (7.7 %) | |
| PCA | 1 (7.7 %) | |
| SubA | 1 (7.7 %) | |
| BA | 1 (7.7 %) | |
| Presenting symptoms (N = 13) | Cerebral infarction | 11 (84.6 %) |
| TIA | 2 (15.4 %) | |
| Infarction type (N = 10) | Watershed infarction | 7 (70.0 %) |
| Perforating branch | 3 (30.0 %) | |
| Duration of PSS symptoms | Duration before stroke, N = 4, yrs (Median (range)) | 2.0 (0.5–10.0) |
| Diagnose after stroke (N) | 9 (69.2 %) | |
| Risk factors of vascular diseases (N = 13) | HTN | 3 (23.1 %) |
| CHD | 2 (15.4 %) | |
| Smoking | 2 (15.4 %) | |
| DM | 1 (7.7 %) | |
| Drinking | 1 (7.7 %) | |
| Hyperlipidemia | 0 (0 %) | |
| AF | 0 (0 %) | |
| Comorbidities of other disease (N = 13) | APS | 1 (7.7 %) |
| SLE | 0 (0 %) | |
| TKA | 0 (0 %) | |
| GCA | 0 (0 %) | |
| Serum profile | SSA positive (N = 12) | 12 (100.0 %) |
| SSB positive (N = 9) | 5 (55.6 %) | |
| ANA≥1:320 (N = 4) | 3 (75.0 %) | |
| CSR (N = 6, Median (range), mm/h) | 20.0 (2.0–84.0) | |
| CRP (N = 6, Median (range), mg/L) | 3.61 (0.70–12.57) | |
| LDL-C (N = 6, Median (range), mmol/L) | 1.64 (0.67–2.84) | |
| HbA1c (N = 5, Median (range), %) | 5.6 (5.0–6.4) | |
| Labial gland biopsy confirmed lymphocyte foci | N = 13 | 10 (76.9 %) |
| Holter (N = 6) | Normal | 6 (100 %) |
| Cardiac ultrasound (N = 6) | Normal | 6 (100 %) |
| MR-VWI (N = 7) | Vessel wall thickening and enhancement | 5 (71.4 %) |
| Intravascular thrombi | 2 (28.6 %) |
CCA: common carotid artery, ICA: intracranial carotid artery, MCA: middle cerebral artery, PCA: posterior cerebral artery, BA: basilar artery, SubA: subclavian artery, HTN: hypertension, DM: diabetes mellitus, CHD: chronic heart disease, AF: atrial fibrillation, SLE: systemic lupus erythematosus, APS: antiphospholipid antibody syndrome, TKA: Takayasu arteritis, GCA: giant cell arteritis, ANA: antinuclear antibody, SSA: Sjögren's-syndrome-related antigen A, SSB: Sjögren's-syndrome-related antigen B, CSR: erythrocyte sedimentation rate, CRP: C-reactive protein, LDL-C: low density lipoprotein cholesterol, MR-VWI: MR vessel wall imaging.