IgG4-related disease: central features

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

IgG4-related disease: central features. Hypertrophic pachymeningitis, Lymphocytic hypophysitis. Fully referenced neurology checklist.

Major central features

  • Hypertrophic pachymeningitis
  • Lymphocytic hypophysitis

Inflammatory pseudotumour: sites

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Limbic encephalitis

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Otologic features

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Other central features

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Pathological features

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References

  1. Huijbers MG, Querol LA, Niks EH, et al. The expanding field of IgG4-mediated neurological autoimmune disorders. Eur J Neurol 2015; 22:1151-1161.
  2. Dalmau J. Name a brain protein, and an autoantibody shall be found! Neurol Neuroimmunol Neuroinflamm 2015; 2:e159.
  3. Massey J. IgG4-related hypertrophic pachymeningitis coexpressing antineutrophil cytoplasmic antibodies. Neurol Neuroimmunol Neuroinflamm. 2017; 4:e341.
  4. Wallace ZS, Carruthers MN, Khosroshahi A, et al. IgG4-related disease and hypertrophic pachymeningitis. Medicine (Baltimore) 2013; 92:206-216.
  5. Schubert RD, Wood M, Levin MH, Perry A, Gelfand JM. The severe side of the IGG4-related hypertrophic pachymeningitis disease spectrum. Neurol Neuroimmunol Neuroinflamm 2016; 3: e197.
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