With a connective tissue disorder such as EDS the collagen-containing „sleeve“ of the spinal cord (meninges) can spontaneously tear without any external influences which then leads to the discharge of cerebrospinal fluid.
Instabilities, however, can irritate the meninges mechanically and therefore cause small holes.
A CSF leak leads to discharge of the liquid that surrounds and protects the brain and spinal cord, called liquor cerebrospinalis. As a result a vacuum develops around the brain which causes intracranial hypotension and leads to the typical orthostatic headaches. These occur shortly after changing the bodies position from lying to standing or sitting upright.
Other symptoms that can occur with CSF leaks:
Visual and hearing disturbances
A comorbid condition of Chiari malformation can be exacerbated by the intracranial hypotension which is why CSF leaks should be treated immediately.
EDS patients have a higher risk of developing CSF leaks after lumbar puncture or spinal anesthesia due to healing problems of the puncture wound.
CSF leaks can basically occur in any part of the spine.
CSF leaks are often missed and diagnosis can be delayed due to the absence of orthostatic headaches or other unspecific symptoms
MRI is usually the first measure
Lumbar puncture with opening pressure
But even if intracranial hypotension has been found the main difficulty still is to find the leak.
Non-invasive: bed rest and caffeine tablets
Invasive: blood patch