Cerebrospinal fluid leak

A cerebrospinal fluid leak (CSF leak) is a medical condition where the cerebrospinal fluid (CSF) in the brain or spinal cord leaks out of one or more holes or tears in the arachnoid and dura mater.[1][2]

Cerebrospinal fluid leak
Other namesCSF leak
SpecialtyNeurology 

CSF leaks are often further classified according to the cause. An iatrogenic or traumatic CSF leak has an identified cause such as a lumbar puncture (noted by a post-dural-puncture headache), surgery or physical trauma; while a spontaneous cerebrospinal fluid leak has an unknown cause. CSF leaks are associated with heritable connective tissue disorders including Marfan syndrome and Ehlers-Danlos syndrome.[3]

Signs and symptoms

The most common symptom of a CSF leak is a fast-onset, extremely painful orthostatic headache or thunderclap headache.[4]

Patients with a spinal leak may suffer intracranial hypotension (low CSF pressure) because the body cannot replenish the CSF fast enough to keep pace with the leak. As a result, the brain may sag inside the skull and into the foramen magnum, which is visible (and measurable) with an MRI of the brain. Patients with a cranial leak are more likely to suffer intracranial hypertension (high CSF pressure), and are at risk of meningitis. Some patients with either a cranial or spinal leak can fluctuate between high and low CSF pressure.

While high CSF pressure can make lying down unbearable, low CSF pressure due to a leak can be relieved by lying flat on the back.[5]

Other symptoms of a CSF leak can include neck pain, photophobia, dizziness, gait disturbances, tinnitus, visual disturbances, brain fog, nausea, fluid dripping from the nose or ears, and a metallic taste in the mouth. An untreated CSF leak can result in coma or death.[6]

Diagnosis

CSF leaks are frequently misdiagnosed by physicians as migraine, Chiari malformation, dysautonomia or conversion disorder. Imaging can be helpful in diagnosing a CSF leak, and in identifying its location, typically using a CT scan or an MRI scan. A myelogram can be used to more precisely identify the location of a CSF leak by injecting a dye to further enhance the imaging. However, CSF leaks are frequently not visible on imaging.

Treatment

Symptomatic treatment usually involves analgesics for both cranial and spinal CSF leaks. Caffeine and short-term bed rest can alleviate symptoms of low CSF pressure, while elevated rest and acetazolamide can alleviate symptoms of high CSF pressure.

Sometimes a CSF leak will heal spontaneously. Otherwise, symptoms may last months or even years. An epidural blood patch is the typical treatment for a CSF leak, where up to 20 cubic centimeters of the patient's blood is drawn, then injected into either the lumbar or cervical spine, close to the known or suspected site of the leak. Fibrin glue patching is an alternative where blood patching is unsuccessful. If the site of the leak is known, neurosurgical repair of the dura mater is an option.

The use of antibiotics to prevent meningitis in those with a CSF leak due to a skull fracture is of unclear benefit.[7]

References
  1. MedlinePlus Encyclopedia CSF leak
  2. Cerebrospinal Fluid Leak Imaging at eMedicine
  3. 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.
  4. "Thunderclap headaches - Symptoms and causes". Mayo Clinic. Retrieved 2018-08-01.
  5. "CSF Leak: A Curable Cause of Headache". www.cedars-sinai.edu. Retrieved 2018-08-01.
  6. "Headache Secondary to Intracranial Hypotension, Schievink, W and Deline, C". www.researchgate.net. Retrieved 2018-09-22.
  7. Ratilal, BO; Costa, J; Pappamikail, L; Sampaio, C (28 April 2015). "Antibiotic prophylaxis for preventing meningitis in patients with basilar skull fractures". The Cochrane Database of Systematic Reviews (4): CD004884. doi:10.1002/14651858.CD004884.pub4. PMID 25918919.
Classification
External resources
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