Acta Neurologica Belgica

, Volume 118, Issue 4, pp 617–627 | Cite as

IgG4-related inflammatory pseudotumor of the brain parenchyma: a case report and literature review

  • Zhuqing Zhang
  • Weiwei Fu
  • Minghui Wang
  • Lei Niu
  • Bin Liu
  • Yingbin Jiao
  • Xuejun LiuEmail author
Original Article


A 29-year-old male with a 4-month history of binocular temporal visual field defect, 1-month history of memory loss and 5-day history of headache was admitted to our hospital. Brain MRI showed progressive signal abnormalities in the brain parenchyma. His laboratory tests showed elevated serum IgG4 and liver dysfunction. Abdominal CT, ultrasound and liver biopsy showed chronic liver disease. According to the diagnostic criteria of IgG4-related disease, IgG4-related inflammatory pseudotumor of brain parenchyma was considered. After methylprednisolone treatment, his symptoms improved and brain lesion reduced. This is the second reported case of a tumefactive lesion of the brain with serum IgG4 elevation, which was responsive to steroid treatment. Coincidentally, his mother had similar medical history and imaging findings, and was subspecialty diagnosed with the same disease, but without pathological and immunohistochemical confirmation. This suggests that the disease may be hereditary. It is important to recognize IgG4-related inflammatory pseudotumors of the brain parenchyma so that patients do not undergo unnecessary surgical or other procedures.


IgG4 Inflammatory pseudotumor Brain parenchymal Calcification 



The authors would like to express their gratitude to Qingdao people’s livelihood science and technology Project [17-3-3-18-nsh] for the financial support provided.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical Standards

The authors declare that they acted in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki.

Informed consent

Informed consent for publication was obtained from the patient.


  1. 1.
    Stone JH, Zen Y, Deshpande V (2012) IgG4-related disease. N Engl J Med 366(6):539–551. CrossRefPubMedGoogle Scholar
  2. 2.
    Radi-Bencteux S, Proust F, Vannier JP, Marret S, Laquerriere A (2003) Intracerebral inflammatory pseudotumour in a 16-month-old boy. Neuropediatrics 34(6):330–333. CrossRefPubMedGoogle Scholar
  3. 3.
    Häusler M, Schaade L, Ramaekers VT, Doenges M, Heimann G, Sellhaus B (2003) Inflammatory pseudotumors of the central nervous system: report of 3 cases and a literature review. Hum Pathol 34(3):253–262CrossRefGoogle Scholar
  4. 4.
    Lin YJ, Yang TM, Lin JW, Song MZ, Lee TC (2009) Posterior fossa intracranial inflammatory pseudotumor: a case report and literature review. Surg Neurol 72(6):712–716. (discussion 716) CrossRefPubMedGoogle Scholar
  5. 5.
    Regev K, Nussbaum T, Cagnano E, Giladi N, Karni A (2014) Central nervous system manifestation of IgG4-related disease. JAMA Neurol 71(6):767CrossRefGoogle Scholar
  6. 6.
    Umehara H, Okazaki K, Masaki Y, Kawano M, Yamamoto M, Saeki T, Matsui S, Sumida T, Mimori T, Tanaka Y (2012) A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details. Jpn J Rheumatol 22(1):1–14Google Scholar
  7. 7.
    Deshpande V (2015) IgG4 related disease of the head and neck. Head Neck Pathol 9(1):24–31CrossRefGoogle Scholar
  8. 8.
    Joshi D, Jager R, Hurel S, Pereira SP, Johnson GJ, Chapman M, Fowler R, Winstanley A, Losseff N, Webster GJ (2015) Cerebral involvement in IgG4-related disease. Clin Med 15(2):130–134CrossRefGoogle Scholar
  9. 9.
    Umehara H, Okazaki K (2014) Comprehensive diagnostic criteria for IgG4-related disease. Springer, TokyoCrossRefGoogle Scholar
  10. 10.
    Tanji H, Okada H, Igari R, Yamaguchi Y, Sato H, Takahashi Y, Koyama S, Arawaka S, Wada M, Kawanami T (2016) Inflammatory pseudotumor of the brain parenchyma with IgG4 hypergammaglobulinemia. Intern Med 55(14):1911CrossRefGoogle Scholar
  11. 11.
    Hirschfield GM, Dyson JK, Alexander GJM, Chapman MH, Collier J, Hübscher S, Patanwala I, Pereira SP, Thain C, Thorburn D (2018) The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines. Gut 67(9):1568–1594CrossRefGoogle Scholar
  12. 12.
    Umemura T, Ota M, Hamano H, Katsuyama Y, Kiyosawa K, Kawa S (2006) Genetic association of Fc receptor-like 3 polymorphisms with autoimmune pancreatitis in Japanese patients. Gut 55(9):1367CrossRefGoogle Scholar
  13. 13.
    Kim DS, Na DG, Kim KH, Kim JH, Kim E, Yun BL, Chang KH (2009) Distinguishing tumefactive demyelinating lesions from glioma or central nervous system lymphoma: added value of unenhanced CT compared with conventional contrast-enhanced MR imaging. Radiology 251(2):467–475CrossRefGoogle Scholar
  14. 14.
    Hawkins JM, Dormosh M, Queenan JV, Uppal G, Katsetos CD (2015) Multinucleated giant cell granulomatous reaction to psammomatous calcifications in inflammatory (calcified fibrous) pseudotumor of CNS. Clin Neuropathol 34(7):239–242CrossRefGoogle Scholar
  15. 15.
    Wu A, Andrew NH, McNab AA, Selva D (2015) IgG4-related ophthalmic disease: pooling of published cases and literature review. Curr Allergy Asthma Rep 15(6):27. CrossRefPubMedGoogle Scholar
  16. 16.
    Akiyama M, Takeuchi T (2018) IgG4-related disease: beyond glucocorticoids. Drugs Aging. CrossRefPubMedGoogle Scholar

Copyright information

© Belgian Neurological Society 2018

Authors and Affiliations

  • Zhuqing Zhang
    • 1
  • Weiwei Fu
    • 2
  • Minghui Wang
    • 1
  • Lei Niu
    • 1
  • Bin Liu
    • 3
  • Yingbin Jiao
    • 4
  • Xuejun Liu
    • 1
    Email author
  1. 1.Department of RadiologyThe Affiliated Hospital of Qingdao UniversityQingdaoChina
  2. 2.Department of PathologyThe Affiliated Hospital of Qingdao UniversityQingdaoChina
  3. 3.Department of RheumatologyThe Affiliated Hospital of Qingdao UniversityQingdaoChina
  4. 4.Department of NeurosurgeryThe Affiliated Hospital of Qingdao UniversityQingdaoChina

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