NORD gratefully acknowledges Connie Deline, MD, Co-founder, Spinal CSF Leak Foundation, and Wouter I. Schievink, MD, Professor of Neurosurgery, Department of Neurosurgery, Cedars-Sinai Medical Center, for the preparation of this report.
Spontaneous intracranial hypotension is secondary to a cerebrospinal fluid (CSF) leak at the level of the spine and the resulting loss of CSF volume to support the brain and spinal cord. Men and women of all ages are affected but the diagnosis is more common in women. Annual incidence of 5 per 100,000 is likely an underestimate and overall prevalence is unknown. It most often results in a new onset headache that is worse with upright posture, along with other neurologic signs and symptoms. Variability in presenting signs and symptoms along with low awareness of the disorder contribute to delayed diagnosis, although this is improving with a growing volume of publications. Diagnostic imaging is quite specialized, both in techniques and in interpretation. Because there is a structural cause, a hole or defect in the spinal dura (tough layer of connective tissue) that normally holds cerebrospinal fluid in, it is both treatable and curable. The most common treatment is epidural patching with blood or fibrin sealant but surgery is sometimes needed. Outcomes are good for most patients.
Onset of symptoms and signs may be relatively abrupt or more gradual. Patients may be minimally affected or profoundly disabled with limited ability to function while upright.
The most common symptom at onset is a positional headache that is worse with upright posture and improved with horizontal positioning. Headache is not always present and varies a great deal in severity and characteristics. The headache may not be positional from the onset or may become less positional over time.
There is a very wide variety of additional signs and symptoms which can make the diagnosis challenging.
More common symptoms include nausea (with or without vomiting), neck pain or stiffness, pain between shoulder blades, arm pain, dizziness, imbalance, sensitivity to light or sound, hearing changes such as muffled hearing or ringing and changes in the ability to think clearly.
Less common symptoms include visual changes, facial pain or numbness and changes in taste.
Rare complications include dementia, Parkinsonism (tremor and gait changes), quadriplegia, coma and death.
The underlying cause of spontaneous intracranial hypotension is a loss of cerebrospinal fluid (CSF) volume through a hole or tear in the spinal dura. The dura is the tough outermost layer of the meninges (connective tissues that surround the brain and spinal cord) that holds in the cerebrospinal fluid. When this fluid volume is reduced, there is less fluid available to support the normally floating brain inside the skull. The resulting sagging of the brain and other structures causes headache and other neurological signs, symptoms and complications. When upright, the loss of CSF volume has a greater effect on the brain.
There is evidence that an underlying weakness of the spinal dura is present in many cases. Several heritable (genetic) disorders of connective tissue (HDCT) have been associated with spontaneous intracranial hypotension. See related disorders below.
In addition, many cases are associated with calcified discs and bone spurs of the spine that can tear the dura on the front side of the spinal cord.
CSF leaks that occur spontaneously in the head (base of skull) are not causally associated with intracranial hypotension.
Men, women and children of any age may develop spontaneous intracranial hypotension, although this is diagnosed more often in women. The peak age of diagnosis is age 40.
The prevalence of spontaneous intracranial hypotension is unknown. The best estimate that we have of annual incidence is from an emergency department study done in Los Angeles, California. In this retrospective study, spontaneous intracranial hypotension was diagnosed half as frequently as subarachnoid hemorrhage (brain bleeding) from brain aneurysm, with an estimated incidence of about 5 cases per 100,000 per year. The study had limitations in that it was single center and retrospective and it is likely that many patients went undiagnosed, so this is likely an underestimate.
The diagnosis of spontaneous intracranial hypotension is initially suspected on the basis of presenting signs and symptoms. Many physician specialties may be involved in the care of patients including primary care physicians, emergency medicine physicians, neurologists, neuroradiologists, pain management physicians, anesthesiologists, neurosurgeons and geneticists.
When suspected, it is important that a magnetic resonance imaging (MRI) study of the brain be done with contrast to look for several specific findings. These findings may be absent in up to 20% of cases, more often when this imaging is done weeks or months after onset. The mnemonic SEEPS is used by physicians to recall the findings:
S – subdural fluid collections
E – enhancement of the meninges (layers around brain)
E – engorgement of venous structures
P – pituitary hyperemia (swelling)
S – sagging of the brain
A lumbar puncture may be done to measure the CSF opening pressure, but because this often falls in the normal range, it has limited value.
For most patients, spinal imaging to localize their spinal CSF leak may not be necessary, because 1-2 epidural blood patch procedures will be curative. See treatment below.
A range of specialized spinal imaging techniques, including computed tomography (CT), magnetic resonance (MR), and digital subtraction are used to locate a spinal CSF leak for further treatment planning. Current imaging techniques are not sensitive enough to identify a spinal CSF leak in a significant percentage of cases, so it is important for physicians to recognize that negative imaging does not rule out the disorder. This also impacts treatment options.
Different types of spinal CSF leaks have been observed based on findings of imaging and at surgery. Treatment approaches are then tailored to the type and location of the leak.
When there are findings on history, clinical exam or imaging that suggest the presence of a heritable disorder of connective tissue, referral to a physician with expertise in the these disorders may be of value. These patients may be at higher risk of cardiovascular abnormalities noted above under “Related disorders”. Screening with echocardiography and other testing may be considered on a case by case basis.
An uncertain percentage of patients with spontaneous intracranial hypotension have milder symptoms and/or symptoms that may resolve without any treatment. Simple measures including bedrest, fluids for hydration and caffeine intake may help to reduce the severity of symptoms.
When symptoms are significant or persistent, the most common initial treatment is an epidural blood patch. In this procedure, some blood is taken from the patient’s arm vein and is injected into the spinal canal in the space outside the dura. Epidural patching procedures may also include the use of fibrin sealant. Neuroradiologists and anesthesiologists are physician subspecialties that perform these procedures most often. These procedures may be repeated several times if the improvement is incomplete or does not last.
When non-surgical procedures are ineffective or symptoms relapse, a neurosurgical repair may be necessary but relies upon imaging localization of the spinal CSF leak.
It is not uncommon for patients to develop rebound intracranial hypertension (elevated CSF pressure) following treatment which may persist for a variable length of time. The natural history and optimal treatment for this complication have not yet been well-studied. Currently, acetazolamide is the most commonly used medication.
Most patients do very well although some patients do have relapsing or persistent symptoms and disability.
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Contacts for more information about spontaneous intracranial hypotension:
Connie Deline, MD
Spinal CSF Leak Foundation
170 S. Lincoln Street, Suite 100
Spokane, WA 99201
Wouter I. Schievink, MD
Professor of Neurosurgery
Department of Neurosurgery
Cedars-Sinai Medical Center
127 South San Vicente Boulevard, Sixth Floor
Los Angeles, CA 90048
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