A clinical approach to hypertrophic pachymeningitis.

Abstract:

IMPORTANCE:Hypertrophic pachymeningitis (HP) is a non-usual manifestation of rheumatologic, infectious, and neoplastic diseases. Etiological diagnosis is a challenge, but when made promptly it creates a window of opportunity for treatment, with the possibility of a total reversal of symptoms. OBSERVATIONS:HP is an inflammatory process of the dura mater that can occur as a manifestation of sarcoidosis, granulomatosis with polyangiitis, and IgG4-related disease. The HP case evaluation is extensive and includes central nervous system imaging, cerebrospinal fluid analysis, serology, rheumatologic tests, and systemic survey for other manifestations sites. After systemic investigation, meningeal biopsy might be necessary. Etiology guides HP treatment, and autoimmune disorders are treated with corticosteroids alone or associated with an immunosuppressor. CONCLUSION:HP is a manifestation of several diseases, and a precise etiological diagnosis is crucial because of the difference among treatments. An extensive investigation of patients with HP helps early diagnosis and correct treatment.

journal_name

Arq Neuropsiquiatr

authors

Abrantes FF,Moraes MPM,Rezende Filho FM,Pedroso JL,Barsottini OGP

doi

10.1590/0004-282X20200073

subject

Has Abstract

pub_date

2020-12-01 00:00:00

pages

797-804

issue

12

eissn

0004-282X

issn

1678-4227

pii

S0004-282X2020005033202

journal_volume

78

pub_type

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