Table 1.

Clinical characteristics of patients with IAs and mutations in the PKD1 or PKD2 gene

Case No.	Sex	Age at diagnosis of IA years	Symptoms, HH scale	Location of IAs	IAs, n	Size of IA, mm	Therapy	Diagnosis of IA before ESRF	Family history of IAs
1	M	49	sy	right and left middle cerebral artery	2	8 × 8 × 8, 10 × 10 × 10	clip	no	no
2	F	32	asy	left posterior inferior cerebellar artery	1	4.8 × 3.4	clip	yes	no; relative of case 22
3	M	46	asy	left internal carotid artery	multiple	n.a.	no	yes	no
4	M	36	asy	2 × anterior communicating artery	2	n.a.	coil 2 ×	yes	no
5	M	50	asy	right middle cerebral artery	1	4 × 4	clip	yes	relative of cases 6 and 29
6	M	46	asy	right middle cerebral artery	1	n.a.	no	yes	relative of cases 5 and 29
7	M	43	asy	right middle cerebral artery	1	5.1 × 4.7 × 4.6	clip	yes	yes; relative of cases 12 and 20
8	F	71	asy	right middle cerebral artery	1	n.a.	no	no	no
9	F	43	asy	right middle cerebral artery, callosomarginal artery, ophthalmic artery, left middle cerebral artery	4	ophthalmic left 7.5 × 5; right middle cerebral artery 4 × 6	clip media right	no	no
10	F	47	asy	middle cerebral artery, anterior communicating artery	2	n.a.	clip	yes	no
11	F	50	asy	internal carotid artery	1	2 × 3 × 3	no treatment	yes	no
12	M	41	asy	basilar artery	1	9.5 × 5.5 × 4	coil	yes	yes; relative of cases 7 and 20
13	F	34	asy	left middle cerebral artery, left pericallosal artery, left ophthalmic artery	3	n.a.	clip 1 ×, coil 1 ×, 1 not treated	no	no
14	M	47	headache	right middle cerebral artery and ophthalmic artery	2	n.a.	clip 1 ×	yes	no
15	F	49	HH1	right middle cerebral artery	1	4.2 × 4.5	clip	yes	mother with cerebral aneurysm
16	F	55	HH4	right pericallosal artery	1	3.7 × 3 × 4.5	coil	yes	no
17	F	45	HH4	right middle cerebral artery	1	6 × 6	coil	yes	no
18	F	38	SAB	n.a.	1	n.a.	clip	yes	no
19	F	31	SAB	anterior cerebral artery, anterior communicating artery	2	n.a.	coil 1 ×, 1 not treated	yes	no
20	M	43	SAB, HH1	left posterior communicating artery	1	2.9 × 2.2 × 2.8	clip	yes	yes; relative of cases 7 and 12
21	M	37	SAB, HH1	right middle cerebral artery	1	2.5 i.D.	clip	yes	no
22	M	29	SAB, HH1	anterior communicating artery	1	3 i.D	clip	yes	later*; relative of case 2
23	F	49	SAB, HH1, Fisher 2	ophthalmic artery 3 ×	3	1.5 i.D, 2.2 i.D., 2.5 i.D.	coil, clip 2 ×	yes	no
24	F	29/50	SAB/SAB,HH2–3	anterior communicating artery, right internal carotid artery	2	2 i.D.	clip 2 × in 21 years	yes	no
25	F	33	SDB, HH2	left vertebral artery	1	n.a.	coil	yes	no
26	M	42	sy	anterior communicating artery	1	n.a.	clip	yes	no
27	F	50	sy	left middle cerebral artery	1	n.a.	clip	yes	no
28	M	42	sy	anterior communicating artery	1	n.a.	clip	yes	relative of cases 5 and 6
29	F	27	sy	n.a.	1	9.5 × 7.9 × 10.6	n.a.	yes	father with cerebral hemorrhage
30	F	57	sy	2× right middle cerebral artery and anterior communicating artery	3	n.a.	middle right cerebral artery clip, others not treated	no	no
31	F	2	sy	n.a.	1	n.a.	clip	yes	no
32	F	48	sy	2× left middle cerebral artery, left pericallosal artery and left callosomarginal artery	4	n.a.	3 × coil 1 × clip	no	no

Cases 5, 6, and 29 are members of the same family, cases 2 and 22 are from another family, and cases 7, 12, and 20 from a 3rd family. HH = Cerebral dysfunction according to the Hunt and Hess grading scale;

ESRD = end-stage renal disease;

sy = symptomatic;

asy = asymptomatic;

SAB = subarachnoid hemorrhage;

n.a. = data not available;

i.D. = in diameter.

* The family history of IAs for this case was clarified by our study and was not known to the patient.