A spontaneous cerebrospinal fluid leak ( SCSFL ) is a medical condition in which cerebrospinal fluid (CSF) around the human brain and spinal cord leaks out of surrounding protective dural sacs for absence clear reasons. Dura, a hard and inflexible tissue, is the outermost of three layers of meninges, a membrane system that surrounds the brain and spinal cord.
CSF spontaneous leakage , as opposed to a traumatically caused CSF leak, appears idiopathically. This is one of several types of cerebrospinal fluid leakage and occurs because of one or more holes in the dura. Loss of CSF greater than the production level causes a decrease in volume within the skull known as intracranial hypotension. Each CSF leak is most often characterized by an orthostatic headache, which worsens in a vertical position and improves when lying down. Other symptoms may include neck pain or stiffness, nausea, vomiting, dizziness, fatigue, and a sense of metal in the mouth. A CT scan can identify the location of a cerebrospinal fluid leak. Once identified, leakage can often be corrected by an epidural blood patch, injection of the patient's own blood at the site of leakage, injection of fibrin glue, or surgery. When the cause can not be identified, the sequence of symptoms is referred to as spontaneous cerebrospinal fluid leak syndrome (SCSFLS).
SCSFLS impinges 5 out of every 100,000 people. On average, this condition develops at age 42, and women are twice as likely as men to develop the condition. Some people with SCSFLS chronically secrete cerebrospinal fluids despite repeated attempts to patch up, leading to long-term disability due to pain. SCSFLS was first described by the German neurologist Georg Schaltenbrand in 1938 and by American physician Henry Woltman of the Mayo Clinic in the 1950s.
Video Spontaneous cerebrospinal fluid leak
Classification
SCSFLS is classified into two main types: leakage of the skull and spine leak. Most of the leaks are spinal. A wind leak occurs in the head. In some of these cases, CSF can be seen dripping out of the nose, or ears. Spinal leak occurs when one or more holes form in the dura along the spinal cord. Both CSF spontaneous cranial and spinal leakage cause neurological symptoms as well as spontaneous intracranial hypotension (SIH), reduced volume, and skull pressure. While these symptoms may be referred to as intracranial hypotension, intracranial pressure may be normal, with underlying problems not low CSF volumes. For this reason, SCSFLS is referred to as CSF hypovolemia as opposed to CSF hypotension .
Maps Spontaneous cerebrospinal fluid leak
Signs and symptoms
Most people who develop SCSFLS feel a sudden onset of severe and acute headaches. These headaches are usually aggravated by standing and usually become prominent throughout the day, with the pain becoming less severe when lying down. Orthostatic headaches can become chronic and paralyze to the point of disability. Some patients with SCSFLS will develop headaches that begin in the afternoon. This is known as a half-day headache . This may be a preliminary presentation of spontaneous CSF leakage or appears after treatment such as an epidural patch, and may show a slow leak of CSF.
In addition to headaches, about 50% of patients experience neck pain or stiffness, nausea, and vomiting. Other symptoms include dizziness and vertigo, numbness or facial weakness, blurred or double vision, neuralgia, fatigue, or metallic taste in the mouth. Leaking CSF can sometimes be felt or observed as discharge from the nose or ear.
Lack of CSF pressure and volume can allow the brain to sag and descend through the foramen magnum (large opening) of the occipital bone, at the base of the skull. The lower part of the brain is believed to stretch or impact one or more cranial nerve complexes, thus causing various sensory symptoms. The affected nerves and related symptoms are detailed in the table to the right.
Cause
Spontaneous CSF leakage is idiopathic, which means the cause is unknown. Two main theories suggest that these leaks result from connective tissue disorders or spinal drainage problems.
Connective network theory
Various scientists and doctors have suggested that SCSFLS may be the result of underlying connective tissue disorders affecting the spinal dura. It can also run in the family and is associated with aortic aneurysm and joint hypermobility.
Up to two thirds of those suffering exhibit some type of general connective tissue disorder. Marfan syndrome, Ehlers-Danlos syndrome, and dominant autosomal polycystic kidney disease are the three most common connective tissue disorders associated with SCSFLS. Approximately 20% of patients with SCSFLS feature showed Marfan syndrome, including height, concave chest (pectus excavatum), joint hypermobility, and curved ceiling. However, these patients showed no other Marfan syndrome presentation.
Spinal drainage theory
Several studies have suggested that problems with the spinal venous drainage system may cause CSF leakage. According to this theory, dural holes and intracranial hypotension are symptoms caused by low venous pressure in the epidural space. When the lower leg muscles pump blood toward the heart and the pressure in the vena cava of the inferior vein becomes negative, the epidural vein tissue becomes overdrained, causing the CSF to be sucked into the epidural space. The actual leak can form at the weak points in the spine of the meninges. Therefore, CSF observed hypotension was the result of CSF hypovolemia and reduced epidural venous pressure.
Other causes
Leakage of cranial CSF occurs due to intracranial hypertension in most cases. Increased pressure causes a rupture of the cranial dura mater, leading to CSF ​​leakage and intracranial hypotension. Patients with nude nerve roots , in which the root arm is absent, are at increased risk for developing recurrent CSF leakage. Lumbar herniation has been reported to cause CSF leakage in at least one case. The degenerative spine disc disease causes the disk to penetrate the dura mater, which causes CSF leakage.
Pathophysiology
Cerebrospinal fluid is produced by the choroid plexus in the cerebral ventricle and is contained by the dura and arachnoid layers of the meninges. The brain floats in CSF, which also transports nutrients to the brain and spinal cord. As the hole forms in the spine dura mater, CSF leaks out into the surrounding space. CSF is then absorbed into the epidural spinal vein plexus or soft tissue around the spine. Due to the sterile condition of the soft tissues around the spine, there is no risk of meningitis.
Diagnosis
The first premonition of the first complaint to the doctor is the hospital emergency room. Up to 94% of those suffering from SCSFLS were initially misdiagnosed. Incorrect diagnoses include migraine, meningitis, Chiari malformation, and psychiatric disorders. The average time from onset of symptoms until the definitive diagnosis is 13 months. A 2007 study found a 0% success rate for proper diagnosis in the emergency department.
The diagnosis of leakage CSF can be done with various imaging techniques, chemical tests of body fluids released from the head or clinical examination. The use of CT, MRI, and tests is the most common type of CSF leak test. Many CSF leaks do not appear on imaging and chemical tests, so the diagnostic tool is not definitive to rule out CSF leakage. A doctor may often rely on a patient's history and examination to diagnose, for example: excessive fluid flow from the nose during bending, increased headache after Valsalva maneuver or headache reduction when the patient takes a prone position is a positive indicator.
Clinical examination is often used as a means to diagnose CSF leakage. Increased patient response to conservative treatment can further determine a positive diagnosis. Lack of clinical awareness of CSF signs and diseases is a major challenge for proper diagnosis and treatment, especially: the loss of orthostatic characteristics of headache and any chronic CSF exterminator will have unique symptoms. that overall contribute to the underlying conditions, and the diagnosis, leakage of CSF.
CT
The diagnosis of cerebrospinal fluid leakage is done through a combination of CSF pressure measurements and computed tomography myelogram (CTM) spinal column scan for leakage of fluid. Opening fluid pressure in the spinal canal is obtained by performing a lumbar puncture, also known as spinal tap. Once the pressure is measured, the radiocontrast agent is injected into the spinal fluid. The contrast then diffuses out through the dura sac before leaking through the dural hole. This allows for CTM with fluoroscopy to search and image each site of dura rupture through contrast seen outside the dura sac in the image.
MRI
There is disagreement about whether MRI should be the diagnostic tool of choice. Magnetic resonance imaging is less effective than CT at CSF leak direct site imaging. MRI studies may show an increase in pachymeningeal (when the dura mater appears thick and inflamed), sagging of the brain, pituitary enlargement, subdural hygiene, cerebral venous sinus swelling, and other abnormalities. For 20% of patients, MRI is present as completely normal. MRI performed with patients sitting upright (vs lying supine) is not better for diagnosing CSF leakage, but more than twice as effective in diagnosing ectopia of cerebellar tonsils, also known as Chiari malformation. Serebellar tonsillar ectopia shares many of the same symptoms with CSF leakage, but originates either congenitally or from trauma, including whiplash strains to the dura.
An alternative method to find the location of CSF leakage is to use T2-weighted MR myelography. It has been effective in identifying CSF leak sites without the need for CT scans, lumbar punctures, and contrasts and at liquids collection sites such as CSF collection. Another highly successful method of finding CSF leakage is the intrathecal contrast and MR myelography.
Assay
When cranial CSF leakage is suspected due to discharge from the nose or ear of potentially CSF, fluids can be collected and tested with a beta-2 transferin test. This test can positively identify if the fluid is a cerebrospinal fluid.
CSF Analysis
Patients with CSF leak have been known to have very low or even negative opening pressure during lumbar puncture. However, patients with confirmed CSF leak may also exhibit normal opening pressures. In 18-46% of cases, CSF pressure is measured in the normal range. Spinal fluid analysis may show lymphocytic pleocytosis and high protein content or xanthochromia. This is hypothesized because of increased permeability of the widened meningeal blood vessels and decreased CSF flow in the lumbar subarachnoid space.
Clinical presentation
The diagnostic criteria for SCSFLS are based on International Headache Criteria 2004, 2nd ed. (ICHD-II) (Table 1) (50) criteria. However, the presentation of patients with a confirmed diagnosis may be very different from the clinical diagnostic criteria and can not be considered authoritative.
Treatment
The initial steps may include rest, caffeine intake (through coffee or intravenous infusion), and hydration. Corticosteroids may provide temporary relief for some patients. A stomach binder, which increases intracranial pressure by pressing the abdomen, may temporarily relieve symptoms for some people.
Epidural blood patch
Treatment options for this condition are epidural blood patch surgery applications, which have a higher success rate than conservative care from bed rest and hydration. By injection of one's own blood into the hole area of ​​the dura, epidural blood patches use blood clotting factors to clot the hole site. The volume of autologous blood and the number of patch attempts for patients vary greatly. A quarter to one-third of SCSFLS patients do not have symptomatic relief from epidural blood patching.
Lemant adhesive Fibrin
If the blood patch alone fails to close the dural tear, percutaneous fibrin glue placement can be used in the blood-clotting site, increasing the effectiveness of clot formation and capturing CSF leakage.
Surgical drainage techniques
In the extreme cases of CSF leaks that are difficult to solve, surgical lumbar channels have been used. This procedure is believed to decrease CSF volume of the spine while increasing the intracranial pressure and volume of CSF. This procedure restores normal intracranial CSF volume and pressure while promoting dural tear healing by lowering pressure and volume in the dura. This procedure has produced positive results leading to symptom relief for up to a year.
Neurosurgical repair
For patients who do not respond to either epidural blood patching or fibrin glue, neurosurgery is available to directly correct the leakage of meningeal diverticula. The dura leak area can be bonded together in a process called ligation and then a metal clip can be placed to hold closed ligation. Alternatively, a small compress called pledget can be placed over a dura leak and then covered with foam gel and fibrin glue. Primary suturing is rarely able to repair CSF leakage, and in some patients dura exploration may be necessary to locate all CSF leak locations appropriately.
Prognosis
Long-term outcomes for people with SCSFLS remain poorly studied. Symptoms may disappear within two weeks, or persist for months. Less commonly, patients may suffer persistent symptoms for years. People with chronic SCSFLS can be disabled and can not work. Recurring CSF leaks on alternative sites after recent improvements is common.
Complications
Some complications can occur as a result of SCSFLS including decreased cranial pressure, brain herniation, infections, blood pressure problems, temporary paralysis, and coma. The main and most serious complication of SCSFLS is spontaneous intracranial hypotension, where the pressure in the brain is greatly reduced. This complication leads to typical symptoms of severe orthostatic headaches.
People with cranial CSF leak, a more rare form, have a 10% risk of developing meningitis per year. If a skull leak lasts more than seven days, the likelihood of developing meningitis is significantly higher. Spinal leak CSF can not cause meningitis because of the sterile condition of the leak site. When CSF leak occurs in temporal bone surgery becomes necessary to prevent infection and repair leakage. Orthostatic hypotension is another complication that occurs due to autonomic dysfunction when blood pressure drops significantly. Autonomic dysfunction is caused by brainstem compression, which controls breathing and circulation.
A low CSF volume can cause the cerebellar tonsillar position to decrease, which can be misinterpreted as Chiari malformation; but when CSF leak is repaired, the tonsil position often returns to normal (as seen on the upright MRI) under these "pseudo-Chiari" conditions. Further complications, though uncommon, CSF leakage complications are transient quadriplegia due to sudden and significant CSF loss. This loss causes back cerebral herniation and causes major compression in the upper cervical spinal cord. The quadriplegia disappears after the patient lies on his back. A very rare SCSFLS complication is a third nerve paralysis, in which the ability to divert the eye becomes difficult and disturbed by the compression of the third cranial nerve.
There are documented cases of reversible and comatant frontotemporal dementia. Coma because CSF leak has been successfully treated by using blood patch and/or fibrin glue and placing the patient in Trendelenburg position. Sella empty syndrome, a boney structure that surrounds the pituitary gland, occurs in patients with CSF leakage.
Epidemiology
A 1994 community-based study showed that two out of every 100,000 people suffer from SCSFLS, while an emergency room-based study in 2004 showed five per 100,000. SCSFLS generally affects young and middle age; the mean age for onset is 42.3 years, but the onset may range from ages 22 to 61 years. In an 11-year study, women were found twice as likely to be affected as men.
Studies have shown that SCSFLS runs in families. Suspected genetic similarities in the family include weakness in the dura mater leading to SCSFLS. Large scale population-based studies have not yet been conducted. While the majority of cases of SCSFLS continue to be undiagnosed or misdiagnosed, increased incidence is not likely to occur.
History
Spontaneous CSF leakage has been described by renowned physicians and reported in medical journals dating back to the early 1900s. The German neurologist Georg Schaltenbrand reported in 1938 and 1953 what he called "aliquorrhea", a condition characterized by very low CSF pressure, unreachable, or even negative. Symptoms include orthostatic headaches and other features that are now recognized as spontaneous intracranial hypotension. Several decades earlier, the same syndrome has been described in the French literature as "spinal fluid hypotension" and "ventricular collapse". In 1940, Henry Woltman of the Mayo Clinic wrote about "headaches associated with decreased intracranial pressure". The full clinical manifestations of intracranial hypotension and CSF leakage are described in several publications reported between the 1960s and early 1990s. Modern reports of spontaneous CSF leakage have been reported to medical journals since the late 1980s.
Research
Tetracosactide is a corticosteroid that causes the brain to produce additional spinal fluid to replace the lost volume of CSF and relieve symptoms, and has been given intravenously to treat CSF leakage.
In three small studies of 1-2 patients suffering from recurrent CSF leak where recurrent blood patch failed to form clots and relieve symptoms, the patient received temporary symptoms but complete with epidural saline infusion. The saline infusion temporarily restores the volume required for the patient to avoid SIH until the leak can be repaired properly. Intrathecal saline infusion is used in urgent cases such as unbearable pain or decreased consciousness.
The TGFBR2 gene has been implicated in several connective tissue disorders including Marfan's syndrome, arterial torture, and thoracic aortic aneurysm. A patient study with SCSFLS showed no mutations in this gene. Minor features of Marfan syndrome have been found in 20% of patients with CSF leakage. The abnormal findings of fibrillin-1 have been documented in patients with CSF leakage, but only one patient showed a fibrillin-1 defect consistent with Marfan syndrome.
See also
- Subdural Effects
References
Source of the article : Wikipedia
0 Comments