Characteristics, outcome and treatments with cranial pachymeningitis

Arsene Mekinian 1, 2 Lucas Maisonobe 1, 2 Latifatou Boukari 3 Clea Melenotte 4 Benjamin Terrier 5 Xavier Ayrignac 6, 7 Nicolas Scheinlitz 4 Damien Sène 8 Mohamed Hamidou 9 Amadou Konate 10 Philippe Guilpain 10, 11 Noémie Abisror 1, 2 Etienne Ghrenassia 1, 2 Florence Lachenal 12 Ramiro Cevallos 13 Richard Roos-Weil 14 Le Thi Huong Du 15, 16 Francois Lhote 17 Claire Larroche 18 Jean-Francois Bergmann 8 Sébastien Humbert 19 Jean Fraison 5 Jean Piette 15 Loïc Guillevin 5 Robin Dhote 18 Zahir Amoura 15, 16 Julien Haroche 15, 16 Olivier Fain 1, 2
Abstract : 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.
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Arsene Mekinian, Lucas Maisonobe, Latifatou Boukari, Clea Melenotte, Benjamin Terrier, et al.. Characteristics, outcome and treatments with cranial pachymeningitis. Medicine, Lippincott, Williams & Wilkins, 2018, 97 (30), pp.e11413. ⟨10.1097/MD.0000000000011413⟩. ⟨hal-02318130⟩

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