Spontaneous Cerebrospinal Fluid Leak

Spontaneous Cerebrospinal Fluid Leak

Spontaneous cerebrospinal fluid leak syndrome (SCSFLS) 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. (The other two meningeal layers are the pia mater and the arachnoid mater).

A spontaneous cerebrospinal fluid leak is one of several types of cerebrospinal fluid leaks and occurs due to the presence of one or more holes in the dura. A spontaneous CSF leak, as opposed to traumatically caused CSF leaks, arises idiopathically. 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 which 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 amongst others. A CT 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, fibrin glue injection or surgery.

SCSFLS afflicts 5 out of every 100,000 people. On average, the condition is developed at the age of 42, and women are twice as likely as men to develop the condition. Some people with SCSFLS chronically leak cerebrospinal fluid despite repeated attempts at patching, leading to long-term disability due to pain and nerve damage. SCSFLS was first described by German neurologist Georg Schaltenbrand in 1938 and by American physician Henry Woltman of the Mayo Clinic in the 1950s.