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J Neurol Neurosurg Psychiatry. 2014 Jul;85(7):732-9. doi: 10.1136/jnnp-2013-306410. Epub 2013 Nov 22.

A nationwide survey of hypertrophic pachymeningitis in Japan.

Author information

1
Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
2
Department of Neurosurgery, Kitasato University School of Medicine, Sagamihara, Japan.
3
Department of Neurological Therapeutics, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan.
4
Department of Neuropathology, Institute for Medical Science of Aging, Aichi Medical University, Nagakute, Japan.
5
Department of Neurology, School of Medicine, Kinki University, Osaka, Japan.
6
Department of Hygiene and Preventive Medicine, Iwate Medical University School of Medicine, Morioka, Japan.

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.

KEYWORDS:

Bacteriology; Immunology; Infectious Diseases; MRI

PMID:
24273222
DOI:
10.1136/jnnp-2013-306410
[Indexed for MEDLINE]

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