RT Journal Article
SR Electronic
T1 A nationwide survey of hypertrophic pachymeningitis in Japan
JF Journal of Neurology, Neurosurgery & Psychiatry
JO J Neurol Neurosurg Psychiatry
FD BMJ Publishing Group Ltd
SP 732
OP 739
DO 10.1136/jnnp-2013-306410
VO 85
IS 7
A1 Yonekawa, Tomomi
A1 Murai, Hiroyuki
A1 Utsuki, Satoshi
A1 Matsushita, Takuya
A1 Masaki, Katsuhisa
A1 Isobe, Noriko
A1 Yamasaki, Ryo
A1 Yoshida, Mari
A1 Kusunoki, Susumu
A1 Sakata, Kiyomi
A1 Fujii, Kiyotaka
A1 Kira, Jun-ichi
YR 2014
UL http://jnnp.bmj.com/content/85/7/732.abstract
AB 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.