Related Experiment Video
Updated: Jun 5, 2026

Intracranial Pressure Monitoring In Nontraumatic Intraventricular Hemorrhage Rodent Model
Published on: February 8, 2022
Connective tissue disorders in patients with spontaneous intracranial hypotension
Fang-Chun Liu1, Jong-Ling Fuh, Yen-Feng Wang
1Suao Veterans Hospital, Taiwan.
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).
Conclusion:
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.
Related Concept Videos
Increased Intracranial Pressure l: Introduction
Increased Intracranial Pressure ll: Pathophysiology
Cerebral Edema ll: Pathophysiology
Cerebral Edema l: Introduction
Cytotoxic Edema: Pathophysiology
Hemorrhagic Stroke ll: Pathophysiology
