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Table 1 Characteristics of the patients with CAPS

From: Neurological outcome of patients with cryopyrin-associated periodic syndrome (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