A nationwide survey of hypertrophic pachymeningitis in Japan

Tomomi Yonekawa, Hiroyuki Murai, Satoshi Utsuki, Takuya Matsushita, Katsuhisa Masaki, Noriko Isobe, Ryo Yamasaki, Mari Yoshida, Susumu Kusunoki, Kiyomi Sakata, Kiyotaka Fujii, Jun Ichi Kira

Research output: Contribution to journalArticlepeer-review

85 Citations (Scopus)

Abstract

Objectives: To clarify the prevalence, frequent causes and distinct features of hypertrophic pachymeningitis (HP) according to background conditions in a nationwide survey in Japan. Methods: The study began with a preliminary survey to determine the approximate number of HP patients diagnosed from 1 January 2005 to 31 December 2009, and was followed by a questionnaire survey for clinical and laboratory findings. HP was defined as a condition with thickening of the cranial or spinal dura mater with inflammation, evidenced by MRI or histology. Results: Crude HP prevalence was 0.949/100 000 population. The mean age at onset was 58.3±15.8 years. Among 159 cases for whom detailed data were collated, antineutrophil cytoplasmic antibody (ANCA)-related HP was found in 54 cases (34.0%) and IgG4/multifocal fibrosclerosis (MFS)-related HP in 14 cases (8.8%). Seventy cases (44.0%) were classified as 'idiopathic' and 21 (13.2%) as 'others'. ANCA-related HP cases showed a female preponderance, a higher age of onset, and higher frequencies of otological symptoms and elevated systemic inflammatory biomarkers, but lower frequencies of diplopia compared with idiopathic HP. IgG4/MFS-related HP cases showed a marked male predominance; all had cranial HP while none had isolated spinal HP or decreased sensation. Conclusions: HP is not extremely rare. ANCA-related HP is the most frequent form, followed by IgG4/MFS-related HP. Both forms have unique features, which may help to differentiate background causes.

Original languageEnglish
Pages (from-to)732-739
Number of pages8
JournalJournal of Neurology, Neurosurgery and Psychiatry
Volume85
Issue number7
DOIs
Publication statusPublished - Jul 2014

All Science Journal Classification (ASJC) codes

  • Surgery
  • Clinical Neurology
  • Psychiatry and Mental health

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