title: Granulomatosis With Polyangiitis-Associated Hypertrophic Pachymeningitis Mimicking Spontaneous Intracranial Hypotension: A Case Report.
abstract: BACKGROUND
  Dural enhancement is a characteristic finding in both spontaneous intracranial hypotension and hypertrophic pachymeningitis. Positional headache is the most important feature that distinguishes the two diseases. We report a patient with granulomatosis with polyangiitis (formerly Wegener's granulomatosis) who initially manifested like spontaneous intracranial hypotension.
  
  
  METHODS
  We report here the case of a 63-year old man who presented with severe positional headache. The patient had typical symptoms, symmetric dural enhancement, and a recent history of nontraumatic subdural hygroma which led to the diagnosis of spontaneous intracranial hypotension, but was finally diagnosed as granulomatosis with polyangiitis-associated secondary hypertrophic pachymeningitis. Cyclophosphamide therapy was effective for the maintenance of remission.
  
  
  CONCLUSIONS
  Hypertrophic pachymeningitis associated with granulomatosis with polyangiitis can present with positional headache and subdural hygroma, mimicking spontaneous intracranial hypotension. Granulomatosis with polyangiitis should be suspected when patients with spontaneous intracranial hypotension or hypertrophic pachymeningitis show atypical features.
authors.affiliation: Department of Neurology, Neuroscience Center, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
authors.forename: Young Hee
authors.initials: YH
authors.lastname: Jung
authors.affiliation: Department of Neurology, Neuroscience Center, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
authors.forename: Mi Ji
authors.initials: MJ
authors.lastname: Lee
authors.affiliation: Department of Neurology, Neuroscience Center, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
authors.forename: Chungbin
authors.initials: C
authors.lastname: Lee
authors.affiliation: Department of Radiology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
authors.forename: Jihoon
authors.initials: J
authors.lastname: Cha
authors.affiliation: Department of Neurology, Neuroscience Center, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
authors.forename: Chin-Sang
authors.initials: CS
authors.lastname: Chung
mesh_terms: D003520:Cyclophosphamide / Q000627:therapeutic use; D014890:Granulomatosis with Polyangiitis / Q000150:complications* / Q000000981:diagnostic imaging; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D019585:Intracranial Hypotension / Q000503:physiopathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D008581:Meningitis / Q000150:complications* / Q000000981:diagnostic imaging; D008875:Middle Aged; D015898:Tomography Scanners, X-Ray Computed
chemical_list: D007166:Immunosuppressive Agents; D003520:Cyclophosphamide
keywords: Wegener's granulomatosis; hypertrophic pachymeningitis; spontaneous intracranial hypotension