Spontaneous intracranial hypotension (SIH): risk factors
Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .
Spontaneous intracranial hypotension (SIH): risk factors. FBN2 gene mutations: this encodes fibrillin. Fully referenced neurology checklist.
Connective tissue disorders
- FBN2 gene mutations: this encodes fibrillin
- Ehler’s Danlos syndrome (EDS)
- Adult polycystic kidney disease (APKD)
- Neurofibromatosis type 1 (NF1)
- Lehman syndrome (lateral meningocele)
Spontaneous CSF-venous fistulae
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Other risk factors
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References
- Schievink WI. Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension. JAMA 2006; 295:2286-2296.
- Parks CA, Singh M, Wohler E, et al. Spontaneous spinal CSF leaks: a rare variant exome sequencing study and functional analysis. Lancet Neurol 2026; 25:664-672.
- Kranz PG, Amrhein TJ, Gray L. CSF venous fistulas in spontaneous intracranial hypotension: imaging characteristics on dynamic and CT myelography. Am J Roentgenol 2017; 209:1360-1366.
- Schievink WI, Moser FG, Maya MM, Prasad RS. Digital subtraction myelography for the identification of spontaneous spinal CSF-venous fistulas. J Neurosurg Spine 2016; 24:960-964.
- Kranz PG, Amrhein TJ, Schievink WI, Karikari IO, Gray L. The "hyperdense paraspinal vein" sign: a marker of CSF-venous fistula. Am J Neuroradiol 2016; 37:1379-1381.
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