Portal de Periódicos da CAPES

Characteristics, outcome and treatments with cranial pachymeningitis

2018; Wolters Kluwer; Volume: 97; Issue: 30 Linguagem: Inglês

10.1097/md.0000000000011413

1536-5964

A. Mékinian, Lucas Maisonobe, L. Boukari, Cléa Melenotte, Benjamin Terrier, Xavier Ayrignac, N. Schleinitz, D. Sène, M. Hamidou, Amadou T. Konaté, Philippe Guilpain, Noémie Abisror, E. Ghrenassia, Florence Lachenal, R. Cévallos, Richard Roos-Weil, Lê Thi Huong Dû, François Lhote, C. Larroche, Jean‐François Bergmann, S. Humbert, Jean Baptiste Fraison, Jean Charles Piette, Loı̈c Guillevin, Robin Dhôte, Zahir Amoura, Julien Haroche, O. Fain,

Sarcoidosis and Beryllium Toxicity Research

The aim of this study was to determine the characteristics, treatment, and outcome according to each etiology of pachymeningitis. We conducted a retrospective multicenter French nationwide study between 2000 and 2016 to describe the characteristics, outcome, and treatment of pachymeningitis. We included 60 patients (median age 55.5 years; interquartile range [IQR] 30–80, female/male ratio 0.43). Neurologic signs were present in 59 patients (98%) and consisted of headache in 43 (72%), cranial nerve palsy in 33 (55%), confusion in 10 (17%), seizures in 7 (12%), and focal neurologic signs in 9 (15%). Fever and weight loss were present in 8 (13%) and 13 cases (22%), respectively. Cerebral venous thrombosis was present in 8 cases (13%). Analysis of cerebrospinal fluid showed moderate hyperproteinorachia (median 0.68 g/L; IQR 0.46–3.2) with or without pleiocytosis. Diagnosis included idiopathic pachymeningitis (n = 18; 30%); granulomatosis with polyangiitis (n = 13; 17%); Erdheim-Chester disease (n = 10; 17%); IgG4-related disease and tuberculosis (n = 3; 5% each); Rosai-Dofman disease, microscopic polyangiitis, and sarcoidosis (n = 2, 3% each); cryptococcal meningitis, Lyme disease, ear-nose-throat infection, postlumbar puncture, low spinal-fluid pressure syndrome, and lymphoma (n = 1 each). We found no difference in demographics and neurologic presentation among idiopathic pachymeningitis, Erdheim-Chester disease, and granulomatosis with polyangiitis. In contrast, frequencies were lower with idiopathic pachymeningitis than Erdheim-Chester disease for general signs (6% and 40%, respectively, P = .041) and complete neurologic response (0% vs 39%, P = .045). The detection of extraneurologic signs and routine screening are needed to classify the pachymeningitis origin. Prospective studies are warranted to determine the best treatment in each case.