Pachymeningitis

Aetiology

•   Idiopathic

•   Infective

o   Neurosyphilis

o   CNS tuberculosis: tuberculous pachymeningitis

o   CNS cryptococcosis

o   Bacterial meningitis

•   Inflammatory

o   granulomatosis with polyangiitis

o   Erdheim-Chester Disease (Histiocytosis)

o   IgG4-related hypertrophic pachymeningitis

o   giant cell arteritis

o   neurosarcoidosis

o   Microscopic polyangiitis

o   polyarteritis nodosa

o   rheumatoid arthritis

o   relapsing polychondritis

o   Behηet disease

o   Rosai-Dorfman disease

•   Other

o   Haemodialysis

o   mucopolysaccharidoses

DDX (Conditions that look similar but are not true thickening of the pachymeninges)

•   Neoplasm

o   meningeal metastases

o   meningeal involvement with CNS lymphoma

o   multiple meningiomas including lymphoplasmacyte-rich meningioma

•   Spontaneous intracranial hypotension

 

French study of 60 patients in 2018:

•   Idiopathic                         30%

•   GPA                                17%

•   Erdheim-Chester               17%

•   IgG4                                5%

•   TB                                   5%

•   Rosai-Dofman                  3%

•   Microscopic polyangiitis    3%

•   Sarcoidosis                      3%

•   Cryptococcal

•   Lyme disease

•   ENT infection

•   Post-LP

•   CSF hypotension

•   Lymphoma

 

(Mekinian, Arsene et al. “Characteristics, outcome and treatments with cranial pachymeningitis: A multicenter French retrospective study of 60 patients.” Medicine vol. 97,30 (2018): e11413)