Table 1.

Characteristics of the patients with CAPS

		n = 24	%
Female sex		14	58
Familial history of CAPS		22	92
Genotype	R260W	12	50
	A439V	4	17
	T348M	4	17
	Other mutations	4	17
Age at onset (years)		0 [0–14]
Age at diagnosis (years)		20 [0–53]
Mean follow-up duration (years)		10,4 ± 1,94
Fever		22	92
Skin involvement		24	100
	Urticarial rash	23	96
	Maculopapular rash	17	71
Myalgia		15	62
Arthralgia		21	87
Conjunctivitis		20	83
Neurological signs		17	71
	Headaches	15	62
	Migrainous headaches	4	27
	Chronic daily headaches	8	54
	Hearing loss	10	59
	Optic nerve atrophy	2	8
	Mental retardation	1	0,04
Learning difficulties		14	58
Abnormal brain MRI (n = 23)		2	0,09
CSF assays (n = 5)	Aseptic meningitis	2	40
	Elevated opening pressure	1	20