TABLE 1.

Demographic data, SHs, ACs, and NLF according to clinical presentation

	Clinical Presentation Leading to CCM Diagnosis
Characteristic	Incidental & Seizure (n = 118)	SH (n = 74)	Total (n = 192)	p Value*
Age at diagnosis, yrs (range)	37.7 ± 18.0 (0.5–76)	33.7 ± 20.1 (0.3–72)	36.2 ± 18.9 (0.3–76)	0.2
Age at first visit, yrs (range)	41.7 ± 17.7 (4–80)	38.5 ± 19.2 (1–72)	40.5 ± 18.3 (1–80)	0.2
Sex
Male	27 (22.9%)	29 (39.2%)	56 (29.2%)	0.1
Female	91 (77.1%)	45 (60.8%)	136 (70.8%)	0.3
Genotype
Sporadic/solitary	75 (63.6%)	33 (44.6%)	108 (56.3%)	0.2
Familial/multifocal
CCM1	12 (10.2%)	15 (20.3%)	27 (14.1%)	0.1
CCM2	7 (5.9%)	3 (4.1%)	10 (5.2%)	0.7
CCM3	10 (8.5%)	12 (16.2%)	22 (11.5%)	0.2
Multifocal unknown genotype	14 (11.9%)	11 (14.9%)	25 (13.0%)	0.6
Primary CCM location†
Lobar	72 (61.0%)	19 (25.7%)	91 (47.4%)	0.003
Deep	20 (17.0%)	10 (13.5%)	30 (15.6%)	0.6
Cerebellar	13 (11.0%)	3 (4.1%)	16 (8.3%)	0.1
Brainstem	13 (11.0%)	42 (56.8%)	55 (28.7%)	<0.0001
No. of AC & SH
AC	20	33	53
SH	12	19	31
Total follow-up
Patient-years	244.0	167.7	411.7
Lesion-years	2338.6	164.7	2503.3
Rate in patient-years (95% CI)
AC	8.2% (5.4–12.5)	19.7% (15.0–26.7)	12.9% (10.0–16.6)	0.003
SH	4.9% (2.8–8.5)	11.3% (7.4–17.3)	7.5% (5.4–10.6)	0.03
Rate in lesion-years (95% CI)
AC	2.1% (1.6–2.7)	3.0% (1.3–7.2)	2.1% (1.6–2.8)	0.4
SH	0.7% (0.4–1.1)	9.1% (5.6–14.8)	1.2% (0.9–1.8)	<0.0001
NLF
SWI	22	40	62
T2-weighted imaging	5	19	24
Rate of NLF in familial/multifocal cases (95% CI)
SWI	23.2% (16.1–33.5)	34.0% (26.5–43.8)	29.2% (23.7–36.0)	0.2
T2-weighted imaging	5.3% (2.2–12.4)	16.2% (10.7–24.4)	11.3% (7.8–16.5)	0.03

^*Comparison between patients with SH and those presenting incidentally or with seizures.

^†In cases with multiple lesions, the symptomatic lesion was considered primary, and the largest lesion was considered primary if all were asymptomatic.