title: Idiopathic hypertrophic spinal pachymeningitis: a case report and review of literature.
abstract: OBJECTIVE
  To report an unusual case of idiopathic hypertrophic spinal pachymeningitis (IHSP) with a review of relevant literature and to discuss the etiology, clinical features, imaging, treatment and prognosis of IHSP.
  
  
  METHODS
  The case of a 44-year-old woman is reported. MEDLINE was used to search relevant literatures written in English since 2004.
  
  
  RESULTS
  The patient suffered from progressive mild thoracic backache followed by truncal and lower extremity weakness, numbness and urinary retention. The diagnosis was confirmed by magnetic resonance (MR) imaging and histopathologic examination. Although she received corticosteroid therapy and decompressive surgery, the patient suffered a rapid relapse probably because of the withdrawal of postoperative steroid therapy.
  
  
  CONCLUSIONS
  IHSP is a rare disease characterized by inflammatory hypertrophy of the dura mater without identifiable cause and featured clinical progress of radiculalgia to myelopathy. It is a diagnosis of exclusion. In our view, surgical decompression with postoperative steroid therapy may be optimal. Furthermore,we speculated that increased levels of protein and cell count in cerebrospinal fluid (CSF) might be positively related to the disease progression. High inflammatory signs or CSF protein and cell levels before surgery or postoperative residual lesions are possible reasons of poor prognosis in patients with IHSP.
authors.affiliation: Department of Neurology, The Second Xiangya Hospital, Central South University, 139 # the People Road, Changsha, 410011, P.R. China.
authors.forename: Li-xia
authors.initials: LX
authors.lastname: Qin
authors.forename: Chun-yu
authors.initials: CY
authors.lastname: Wang
authors.forename: Zhi-ping
authors.initials: ZP
authors.lastname: Hu
authors.forename: Liu-wang
authors.initials: LW
authors.lastname: Zeng
authors.forename: Li-ming
authors.initials: LM
authors.lastname: Tan
authors.forename: Hai-nan
authors.initials: HN
authors.lastname: Zhang
mesh_terms: D000328:Adult; D001416:Back Pain / Q000209:etiology; D003131:Combined Modality Therapy; D019299:Decompression, Surgical / Q000379:methods; D004388:Dura Mater / Q000473:pathology; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D006984:Hypertrophy / Q000150:complications / Q000175:diagnosis / Q000628:therapy; D006987:Hypesthesia / Q000209:etiology; D008279:Magnetic Resonance Imaging; D008581:Meningitis / Q000150:complications / Q000175:diagnosis* / Q000628:therapy; D011379:Prognosis; D035583:Rare Diseases / Q000473:pathology; D012008:Recurrence
chemical_list: D005938:Glucocorticoids