TABLE 2.

Immunodeficiencies associated with recurrent bacterial meningitisa

ConditionTotal no. of casesMedian age at onsetb (range)Median age at diagnosis (range)Organism(s)c isolated (no. of occasionsd)Reference(s)
HIV infectione43n.a.n.a.S. pneumoniae (23), Salmonella spp. (10), Hib (2), E. coli (1)1, 68, 135,f 136
Complement deficiencies
    C factor I122 yrn.a.N. meningitidis (2)67
    C251 yr (1 mo-1.3 yr)2 yr (1 yr-37 yr)S. pneumoniae (7), N. meningitidis (2), group B streptococcus (1)86, 193
    C373 yr (1 mo-6 yr)4 yr (3.5 yr-41 yr)S. pneumoniae (29), N. meningitidis (3)18, 36, 50, 52, 54, 180, 210
    C3b inhibitor14 mon.a.N. meningitidis (4), S. pneumoniae (1)124
    C4151 yrn.a.S. pneumoniae (2)124
    C4b13 mo17 yrn.a.83
    C558.5 yr (3 yr-15 yr)16 yr (7 yr-44 yr)N. meningitidis (9)29, 38, 124, 175, 180
    C62623 yr (2 yr-37 yr)43 yr (25 yr-45 yr)N. meningitidis (54)33, 56, 148, 170,g 194
    C7510 yr (1 yr-18 yr)20 yr (11 yr-20 yr)N. meningitidis (13)7, 124, 150, 168, 223
    C81915 yr (1 yr-28 yr)21 yrN. meningitidis (35)168, 174
    C9123 yr56 yrN. meningitidis (1)229
Agammaglobulinemia21.8 yr (6 mo-3 yr)2.8 yr (8 mo-5 yr)S. pneumoniae (2)49, 104
IgG subclass deficiency32.5 yr (11 mo-16 yr)4 yr (14 mo-18 yr)S. pneumoniae (7)56, 73, 153
Common variable immunodeficiency17 yr8 yrS. pneumoniae (2)157
IRAK-4 deficiency1n.a.n.a.S. pneumoniae (2)129
Asplenism (iatrogenic and congenital)e1013 yr (3 mo-38 yr)14 yr (6 mo-39 yr)S. pneumoniae (21), Streptococcus bovis (1)1, 42, 62, 63, 78, 102, 136, 167, 189, 201
  • a Patients with more than one risk factor were recorded in all the relevant categories. All patients included had at least two episodes of meningitis (clinical features and results suggestive of bacterial meningitis), but the causative organism was not identified in all episodes. n.a., no data available.

  • b Age at onset indicates the age at the first episode of meningitis.

  • c Organisms were determined by CSF culture, CSF antigen testing, or positive blood culture with CSF analysis suggestive of bacterial meningitis.

  • d Number of separate occasions at which an organism was isolated; i.e., one patient may have had several episodes caused by different organisms.

  • e One patient was HIV positive and had a splenectomy before the onset of recurrent meningitis.

  • f Incorporates additional data provided by author that were not included in the original publication; E. M. Molyneux provided more detailed data on the cohort described in the text.

  • g Incorporates additional data provided by author that were not included in the original publication; the number of episodes of meningitis in some individuals was considerably greater than 2, but exact figures were unclear (P. C. Potter and A. Orren, personal communication).