Abstract
#IgG4, a subclass of #immunoglobulin G (IgG), can associate various
neurological pathologies in three patterns; (i) pathogenicity of
specific autoantibodies in IgG4 subclass (e.g. anti-neurofascin IgG4
antibodies), (ii) neurological manifestation (e.g. hypertrophic
pachymeningitis) of IgG4-related diseases (IgG4-RD) and (in)
participation of IgG4 with other clinical entity (e.g. antineutrophil
cytoplasmic antibody (ANCA)-associated #vasculitis) [1-5]. However,
accurate prevalence and characteristics of neurological abnormality
associated with IgG4 are not completely disclosed, especially in daily
practice of clinical neurology. Thereby, we authors were motivated to
examine serum IgG4 level as the simple and efficient way because it
often elevates in IgG4 RD (especially>135 mg/dL as a commonly used
cutoff) and other inflammatory/dysimmune disorders, i.e. pattern (ii)
and (iii) above [3,6,7]. On the contrary, it was difficult to examine
specific #autoantibodies in IgG4 subclass in pattern (i) above in our
situation.This is a retrospective chart-review of clinical information with serum
IgG4 level (reference value; 4.5-117 mg/dL) and total IgG (reference
value; 870-1700mg/dL), measured by immunonephelometry (SRL Inc., Tokyo,
Japan) in our institution. All the enrolled patients were Japanese
origin.
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