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Research paper
A nationwide survey of hypertrophic pachymeningitis in Japan
  1. Tomomi Yonekawa1,
  2. Hiroyuki Murai1,
  3. Satoshi Utsuki2,
  4. Takuya Matsushita1,
  5. Katsuhisa Masaki1,
  6. Noriko Isobe1,
  7. Ryo Yamasaki3,
  8. Mari Yoshida4,
  9. Susumu Kusunoki5,
  10. Kiyomi Sakata6,
  11. Kiyotaka Fujii2,
  12. Jun-ichi Kira1
  1. 1Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
  2. 2Department of Neurosurgery, Kitasato University School of Medicine, Sagamihara, Japan
  3. 3Department of Neurological Therapeutics, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
  4. 4Department of Neuropathology, Institute for Medical Science of Aging, Aichi Medical University, Nagakute, Japan
  5. 5Department of Neurology, School of Medicine, Kinki University, Osaka, Japan
  6. 6Department of Hygiene and Preventive Medicine, Iwate Medical University School of Medicine, Morioka, Japan
  1. Correspondence to Professor Jun-ichi Kira, Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka 812-8582, Japan; kira{at}neuro.med.kyushu-u.ac.jp

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.

  • Immunology
  • MRI
  • Bacteriology
  • Infectious Diseases

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