title: Focal status epilepticus as a manifestation of idiopathic hypertrophic cranial pachymeningitis.
abstract: BACKGROUND
  Idiopathic hypertrophic cranial pachymeningitis (IHCP) is an uncommon disease of unknown etiology characterized by thickening of the cerebral dura mater with possible associated inflammation. The most frequently described clinical symptoms include headache, cranial nerve palsy, and cerebellar dysfunction. Epilepsy and/or status epilepticus as main presentation is very uncommon.
  
  
  METHODS
  Two consecutive cases are presented of patients manifesting focal status epilepticus secondary to IHCP, with clinical, laboratory [blood test and cerebrospinal fluid (CSF) analysis], neuroradiologic [magnetic resonance imaging (MRI) at 3 Tesla and digital subtraction angiography (DSA)], and therapeutic data. One patient underwent meningeal biopsy; pathology findings are also included. Corticosteroid therapy resulted in clinical improvement in both cases, and neuroimaging showed decreased abnormal morphology, compared to initial findings.
  
  
  CONCLUSIONS
  In the diagnostic approach to focal status epilepticus or epilepsy, IHCP must be considered a potential, although extremely infrequent, cause. Anti-inflammatory treatment is an effective addition to antiepileptic drug therapy in patients with IHCP.
authors.affiliation: Department of Neurology, Hospital Universitari del Mar, Barcelona, Spain. Electronic address: irene.navalpotro@biodonostia.org.
authors.forename: Irene
authors.initials: I
authors.lastname: Navalpotro-Gómez
authors.affiliation: Department of Neurology, Hospital Universitari del Mar, Barcelona, Spain; Neurovascular Research Group, IMIM-Hospital del Mar (Institut Hospital del Mar d'Investigacions Mèdiques), Universitat Autònoma de Barcelona, Barcelona, Spain.
authors.forename: Rosa María
authors.initials: RM
authors.lastname: Vivanco-Hidalgo
authors.affiliation: Department of Neurology, Hospital Universitari del Mar, Barcelona, Spain; Neurovascular Research Group, IMIM-Hospital del Mar (Institut Hospital del Mar d'Investigacions Mèdiques), Universitat Autònoma de Barcelona, Barcelona, Spain.
authors.forename: Elisa
authors.initials: E
authors.lastname: Cuadrado-Godia
authors.affiliation: Department of Radiology, Hospital Universitari del Mar, Barcelona, Spain.
authors.forename: Santiago
authors.initials: S
authors.lastname: Medrano-Martorell
authors.affiliation: Department of Pathology, Hospital Universitari del Mar, Barcelona, Spain.
authors.forename: Francisco
authors.initials: F
authors.lastname: Alameda-Quitllet
authors.affiliation: Department of Neurosurgery, Hospital Universitari del Mar, Barcelona, Spain.
authors.forename: Gloria
authors.initials: G
authors.lastname: Villalba-Martínez
authors.affiliation: Department of Neurology, Hospital Universitari del Mar, Barcelona, Spain; Neurovascular Research Group, IMIM-Hospital del Mar (Institut Hospital del Mar d'Investigacions Mèdiques), Universitat Autònoma de Barcelona, Barcelona, Spain.
authors.forename: Jaume
authors.initials: J
authors.lastname: Roquer
mesh_terms: D000328:Adult; D000369:Aged, 80 and over; D001921:Brain / Q000000981:diagnostic imaging; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008297:Male; D008578:Meninges / Q000473:pathology; D008581:Meningitis / Q000150:complications* / Q000000981:diagnostic imaging / Q000503:physiopathology*; D013226:Status Epilepticus / Q000000981:diagnostic imaging / Q000209:etiology* / Q000503:physiopathology*
keywords: Cerebral edema; Dural enhancement; Focal seizures; Idiopathic hypertrophic pachymeningitis; Status epilepticus