Interdisciplinary Neurosurgery (Sep 2016)

Normal pressure hydrocephalus, a possible complication in IgG4-related disease

  • Hiroshi Kobayashi,
  • Masaaki Uehara,
  • Aya Oda,
  • Erika Matsubara,
  • Hiroyuki Baba,
  • Ryo Matsumiya,
  • Noritada Yoshikawa,
  • Osamu Hosono,
  • Hirotoshi Tanaka

DOI
https://doi.org/10.1016/j.inat.2016.03.009
Journal volume & issue
Vol. 5, no. C
pp. 1 – 2

Abstract

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We report a 75-year-old man with glucocorticoid-responsive normal pressure hydrocephalus (NPH) complicated with tubulointerstitial renal disease, protein-losing gastroenteropathy (PLG) and elevated serum IgG4, which might be a possible subset of IgG4-related disease (IgG4-RD). Although either PLG or NPH, especially in combination, has rarely been reported in IgG4-RD, the glucocorticoid-responsive nature of every abnormality observed in the patient supports the diagnosis of IgG4-RD. Of course, pathological confirmation is essential to fulfill the recently raised diagnostic criteria, however, such invasive procedure might not always be indicated in high-risk patients. Our report illustrates that IgG4-RD might be considered as one of the underlying causes and/or the aggravating factors of NPH before shunt operation.

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