The meninges of the central nervous system impacted by CSF leak
Specialty
Neurology
A cerebrospinal fluid leak (CSF leak or CSFL) is a medical condition where the cerebrospinal fluid (CSF) that surrounds the brain and spinal cord leaks out of one or more holes or tears in the dura mater.[1] A CSF leak is classed as either nonspontaneous (primary), having a known cause, or spontaneous (sCSF leak) (secondary) where the cause is not readily evident.[2][3] Causes of a primary CSF leak are those of trauma including from an accident or intentional injury, or arising from a medical intervention known as iatrogenic. A basilar skull fracture as a cause can give the sign of CSF leakage from the ear nose or mouth.[4] A lumbar puncture can give the symptom of a post-dural-puncture headache.
A cerebrospinal fluid leak can be either cranial or spinal, and these are two different disorders.[5] A spinal CSF leak can be caused by one or more meningeal diverticula or CSF-venous fistulas not associated with an epidural leak.[6][7][8] A spontaneous spinal cerebrospinal fluid leak may occur sometimes in those with predisposing heritable connective tissue disorders including Marfan syndrome and Ehlers–Danlos syndromes.[5][9] A loss of CSF greater than its rate of production leads to a decreased volume inside the skull known as intracranial hypotension.
Any CSF leak is most often characterized by orthostatic headaches, which worsen when standing, and improve when lying down. Other symptoms can include neck pain or stiffness, nausea, vomiting, dizziness, fatigue, and a metallic taste in the mouth. A CT myelography scan can identify the site of a cerebrospinal fluid leakage. Once identified, the leak can often be repaired by an epidural blood patch, an injection of the patient's own blood at the site of the leak, a fibrin glue injection, or surgery.
A spontaneous CSF leak is a rare condition, affecting at least one in 20,000 people and many more who go undiagnosed every year. On average, the condition develops at age 42, and women are twice as likely to be affected. Some people with a sCSF leak have a chronic leak despite repeated patching attempts, leading to long-term disability due to pain and being unable to be upright, and surgery is often needed. The symptoms of a spontaneous CSF leak were first described by German neurologist Georg Schaltenbrand in 1938 and by American neurologist Henry Woltman of the Mayo Clinic in the 1950s.
^MedlinePlus Encyclopedia: CSF leak
^Tai, YC; Tai, YS; Ou, CH; Lui, CC; Wang, HK; Kuo, HC; Hsu, SP (2 March 2022). "Treatment, Outcome, and Relapse of Spontaneous and Nonspontaneous Cerebrospinal Fluid Leak". Brain Sciences. 12 (3): 340. doi:10.3390/brainsci12030340. PMC 8945904. PMID 35326296.
^ ab"Are all CSF leaks similar?". Spinal CSF Leak Foundation. 29 April 2018.
^Kranz, Peter G.; Luetmer, Patrick H.; Diehn, Felix E.; Amrhein, Timothy J.; Tanpitukpongse, Teerath Peter; Gray, Linda (2015-12-23). "Myelographic Techniques for the Detection of Spinal CSF Leaks in Spontaneous Intracranial Hypotension". American Journal of Roentgenology. 206 (1): 8–19. doi:10.2214/AJR.15.14884. ISSN 0361-803X. PMID 26700332. S2CID 5430158.
^Kranz, Peter G.; Amrhein, Timothy J.; Gray, Linda (December 2017). "CSF Venous Fistulas in Spontaneous Intracranial Hypotension: Imaging Characteristics on Dynamic and CT Myelography". AJR. American Journal of Roentgenology. 209 (6): 1360–1366. doi:10.2214/AJR.17.18351. ISSN 1546-3141. PMID 29023155.
^Cite error: The named reference :2 was invoked but never defined (see the help page).
^Reinstein, E; Pariani, M; Bannykh, S; Rimoin, D; Schievink, WI (April 2013). "Connective tissue spectrum abnormalities associated with spontaneous cerebrospinal fluid leaks: a prospective study". European Journal of Human Genetics. 21 (4): 386–390. doi:10.1038/ejhg.2012.191. PMC 3598315. PMID 22929030.
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