Characteristic | RCVS, n = 13 | PACNS, n = 8 | p | ||
---|---|---|---|---|---|
Male/female | 1/12 | 3/5 | NS | ||
Age, yrs | 52.9 (21–71) | 40.5 (26–60) | NS | ||
Clinical presentation | |||||
Duration of symptoms before admission, days (range) | 3.7 (0–13) | 4.8 (1–15) | NS | ||
Patients with headaches (%) | 13/13 (100) | 3/8 (37) | 0.002 | ||
Patients with TCH (%) | 10/13 (77) | 1/8 (11) | 0.007 | ||
Patients with focal deficit (%) | 7/13 (54) | 8/8 (100) | 0.045 | ||
Patients with seizure (%) | 0/13 (0) | 2/8 (25) | NS | ||
Patients with emesis (%) | 5/13 (38) | 0/8 (0) | NS | ||
Modified Rankin score at onset | |||||
0–2 | 10/13 (77) | 2/8 (25) | 0.04 | ||
3–5 | 3/13 (23) | 6/8 (75) | |||
MRI result (%) | |||||
Normal | 3/13 (33) | 0/8 (0) | NS | ||
cSAH | 8/13 (62) | 0/8 (0) | 0.006 | ||
Ischemia | 1/13 (8) | 6/8 (75) | 0.003 | ||
ICH | 3/13 (33) | 0/8 (0) | NS | ||
Contrast enhancement | 0/13 (0) | 2/8 (25) | NS | ||
Laboratory results (%) | |||||
CSF pleiocytosis (%) | 1/13 (8) | 3/8 (37) | NS | ||
CSF protein > 0.5 g/l (%) | 4/10 (40) | 5/8 (63) | NS | ||
Mean CSF protein level, g/l (range) | 0.51 (0.31–1.07) | 0.47 (0.2–0.85) | NS | ||
CRP > 10 mg/l (%) | 3/13 (23) | 1/8 (12) | NS | ||
Noninvasive vascular imaging* | Onset | Followup | Onset | Followup | — |
Normal | 4/10 (40) | 6/10 (60) | 6/8 (12) | 4/8 (25) | NS |
Mild abnormalities | 0/10 (0) | 4/10 (40) | 1/8 (12) | 2/8 (25) | — |
Severe abnormalities | 6/10 (60) | 0/10 (0) | 1/8 (12) | 2/8 (25) | — |
Improvement at first reimaging | 6/6 (100) | 1/2 (50) | — | ||
Worsening at reimaging | 0/12 (0) | 2/8 (25) | — | ||
Time to first reimaging, mo | Median 3.7, range 1–8 | Median 7, range 4–9 | — | ||
Time to last reimaging, mo | Median 5, range 3–10 | Median 32, range 20–66 | — | ||
Conventional angiogram | Onset | Followup | Onset | Followup | — |
Normal | 0/13 (0) | 4/4 (100) | 5/8 (63) | 1/4 (25) | — |
Consistent with vasculitis | 13/13 (100) | 0/4 (0) | 3/8 (37) | 3/4 (75) | 0.002 |
Mild abnormalities** | 0/13 (0) | 0/4 (0) | 2/8 (25) | 2/4 (50) | — |
Severe abnormalities** | 13/13 (100) | 0/4 (0) | 1/8 (12) | 1/4 (25) | — |
Improvement at reimaging | 4/4 (100) | 1/4 (25) | |||
Time to first reimaging, mo | Median 3.5, range 1–9 | Median 12, range 0.5–24 | |||
Time to last reimaging, mo | Median 3.5, range 1–9 | Median 46, range 0.5–100 | |||
Cerebral biopsy | |||||
Not performed | 13/13 (100) | 5/8 (62) | — | ||
Not diagnostic | 0/13 (0) | 2/8 (25) | — | ||
Vasculitis | 0/13 (0) | 1/8 (12) | — | ||
Mean followup, mo (range) | 33 (3–98) | 44 (18–108) | — | ||
Corticosteroids | 4/13 (30) | 6/8 (75) | — | ||
Immunosuppressants† | 0/13 (0) | 4/8 (50) | — | ||
Relapse | |||||
None | 12/13 (92) | 3/8 (37) | — | ||
1 | 1/13 (8) | 1/8 (12) | — | ||
2–3 | 0/13 (0) | 3/8 (37) | — | ||
> 3 | 0/13 (0) | 1/8 (12) | — |
↵* MR or CT angiography.
↵** Mild abnormalities: vascular irregularities or single stenosis; severe abnormalities: “string of beads” and/or multiple stenosis.
↵† Cyclophosphamide, azathioprine. CSF: cerebrospinal fluid; NS: nonsignificant (p > 0.05); CRP: C-reactive protein; cSAH: cortical subarachnoid hemorrhage; ICH: intracerebral hemorrhage; CSF pleiocytosis: > 10 leukocytes/mm3; TCH: thunderclap headache, MRI: magnetic resonance imaging.