title: Connective tissue disorders in patients with spontaneous intracranial hypotension.
abstract: OBJECTIVE
  Spontaneous intracranial hypotension (SIH) is caused by spinal cerebrospinal fluid (CSF) leakage. An underlying connective tissue disorder has been hypothesized to cause dural weakness and predisposition to CSF leak. We conducted a case-controlled study to investigate the role of connective tissue disorders in SIH patients.
  
  
  METHODS
  We recruited 55 consecutive SIH patients (38 F, 17 M; mean age, 40.8 ± 9.8 years) and 55 age- and sex-matched control individuals (mean age, 38.0 ± 8.9 years) for this study. The connective tissue disorders were evaluated by: (i) Beighton hypermobility scores and revised diagnostic criteria for benign joint hypermobility syndrome; (ii) skin and skeletal manifestations of Ehlers-Danlos syndrome (EDS); and (iii) skeletal features of Marfan syndrome.
  
  
  RESULTS
  The frequencies of joint hypermobility according to Beighton scores >4/9 (SIH 23.6% vs controls 16.4%, P = 0.48) and revised benign joint hypermobility syndrome criteria (SIH 23.6% vs controls 34.5%, P = 0.29) did not differ between SIH patients and controls. Sixteen patients and 16 controls had one or more skin features of EDS (P = 1.0). Nine SIH patients (16.4%) demonstrated the skeletal features of Marfan syndrome; this frequency did not differ from that of the control group (9.1%; P = 0.262). Only dolichostenomelia (disproportionately long limbs) was more prominent in SIH patients than in controls (34.5% vs 9.1%; P = 0.002).
  
  
  CONCLUSIONS
  Compared with Western studies, the frequencies of connective tissue disorders were higher in our SIH patients. However, these frequencies did not differ between SIH patients and control individuals, except for dolichostenomelia.
authors.affiliation: Suao Veterans Hospital, Taiwan.
authors.forename: Fang-Chun
authors.initials: FC
authors.lastname: Liu
authors.forename: Jong-Ling
authors.initials: JL
authors.lastname: Fuh
authors.forename: Yen-Feng
authors.initials: YF
authors.lastname: Wang
authors.forename: Shuu-Jiun
authors.initials: SJ
authors.lastname: Wang
mesh_terms: D000328:Adult; D016022:Case-Control Studies; D065634:Cerebrospinal Fluid Leak; D002559:Cerebrospinal Fluid Rhinorrhea / Q000134:cerebrospinal fluid / Q000453:epidemiology; D003240:Connective Tissue Diseases / Q000453:epidemiology*; D004535:Ehlers-Danlos Syndrome / Q000453:epidemiology; D005260:Female; D006801:Humans; D019585:Intracranial Hypotension / Q000134:cerebrospinal fluid / Q000453:epidemiology*; D007593:Joint Instability / Q000453:epidemiology; D008297:Male; D008382:Marfan Syndrome / Q000453:epidemiology; D008875:Middle Aged; D011446:Prospective Studies; D013624:Taiwan / Q000453:epidemiology