Spontaneous cerebrospinal fluid leak syndrome (SCSFLS) [AKA Idiopathic Intracranial Hypotension] is a medical condition in which the cerebrospinal fluid (CSF) held in and around a human brain and spinal cord leaks out of the surrounding protective sac, the dura, for no apparent reason.
The dura, a tough, inflexible tissue, is the outermost of the three layers of the meninges, the system of meninges surrounding the brain and spinal cord.
A loss of CSF greater than its rate of production leads to a decreased volume inside the skull known as intracranial hypotension.
A CSF leak is most often characterized by a severe and disabling headache and a spectrum of various symptoms that occur as a result of intracerebral hemorrhage (ICH). These symptoms can include: dizziness, nausea, fatigue, a metallic taste in the mouth (indicative of a cranial leak), myoclonus, tinnitus, tingling in the limbs, and facial weakness among others.
Some people with SCSFLS chronically leak cerebrospinal fluid despite repeated attempts at patching, leading to long-term disability due to pain and nerve damage.
Actor George Clooney suffered from a CSF leak in 2005. Due to the intense pain Clooney considered suicide.
SCSFLS is classified into two main types, cranial leaks and spinal leaks Cranial leaks occur in the head.
Both cranial and spinal spontaneous CSF leaks cause neurological symptoms as well as spontaneous intracranial hypotension, diminished volume and pressure of the cranium
Most people who develop SCSFLS feel a sudden onset of a severe and acute headache
usually the pain is worse when the person is vertical and less severe when horizontal.
Orthostatic headaches can be incapacitating;
Lack of CSF pressure and volume allows the brain to descend through the foramen magnum, or occipital bone, the large opening at the base of the skull. The lower portion of the brain is believed to stretch or impact one or more cranial nerve complexes
The two main theories as to the underlying cause of SCSFLS are as a result of a connective tissue disorder or spinal drainage problems.
Various scientists and physicians have suggested that this condition may be the result of an underlying connective tissue disorder affecting the spinal dura. It may also run in families and be associated with aortic aneurysms and joint hypermobility.
Up to two thirds of those afflicted demonstrate some type of generalized connective tissue disorder.
Up to 94% of those suffering from SCSFLS are initially misdiagnosed. Incorrect diagnoses include migraines, meningitis, Chiari malformation and psychiatric disorders. The average time from onset of symptoms until definitive diagnosis is 13 months. A study found a 0% success rate for proper diagnosis in the emergency department
Many CSF leaks do not show up on imaging and chemical assays, thus such diagnostic tools are not definitive to rule out CSF leaks.
A clinician may often depend upon patient history and exam to diagnose, for example:
- discharge of excessive amount of clear fluid from the nose upon bending over,
- the increase in headache following a Valsalva maneuver or
- the reduction of headache when the patient takes a prone position are positive indicators.
The lack of clinician awareness of the signs -symptoms and ailments- of a CSF leak is the greatest challenge to proper diagnosis and treatment, in particular: the loss of the orthostatic characteristic of headache and that every chronic CSF leaker will have a unique symptom set that as a whole contributes to the underlying condition, and diagnosis of, a CSF leak.
An abdominal binder — a type of garment that increases intracranial pressure by compressing the abdomen — can temporarily relieve symptoms for some people
Epidural Blood Patch
The treatment of choice for this condition is the surgical application of epidural blood patches, which has a higher success rate than conservative treatments of bed rest and hydration. Through the injection of a person’s own blood into the area of the hole in the dura, an epidural blood patch uses blood’s clotting factors to clot the sites of holes