Spontaneous Spinal Fluid Leak Headaches: Detection, Definition of the Diagnosis
Spontaneous Spinal Fluid Leak Headaches: Detection, Definition of the Diagnosis
Abstract & Commentary
By Dara G. Jamieson, MD, Associate Professor of Clinical Neurology, Department of Neurology and Neuroscience, Weill Medical College, Cornell University. Dr. Jamieson reports that she is a retained consultant for Boehringer Ingelheim, Merck, and Ortho-McNeil; and is on the speaker's bureau for Boehringer Ingelheim and Merck.
Synopsis: Specific radiological criteria and response to epidural blood patching can confirm the diagnosis of spontaneous spinal CSF leaks and intracranial hypotension. Non-invasive magnetic resonance myelography can detect the site of CSF leakage.
Sources: Schievink WI, et al. Diagnostic criteria for spontaneous spinal CSF leaks and intracranial hypotension. AJNR Am J Neuroradiol 2008;29:853-856; Yoo HM, et al. Detection of CSF leak in spinal CSF leak syndrome using MR myelography: correlation with radioisotope cisternography. AJNR Am J Neuroradiol 2008;29:649-654.
Spontaneous intracranial hypotension (SIH) can cause positional headache; however, lack of recognition of this not uncommon cause of new daily-persistent headache in young and middle-age adults may result in the prolonged use of ineffective treatment. The usual patient with SIH has an orthostatic headache that is most severe while upright but relieved while recumbent, pachymeningeal enhancement on MRI, and relief with epidural blood patching. However, there is a wide variation in the clinical and radiological presentation of patients with SIH, leading to occasional uncertainty about the diagnosis.
Schievink and coworkers (from the Departments of Neurosurgery and Anesthesiology, and the Imaging Medical Group at Cedars-Sinai Medical Center) used their experience and a review of the medical literature to devise a diagnostic scheme to diagnose spontaneous spinal CSF leaks and intracranial hypotension. The study used criterion A: the demonstration of a spinal CSF leak with extra-thecal spinal fluid on CT myelography, MRI, or radionuclide cisternography. If this criterion cannot be met, then criterion B dictates that there is cranial MRI of intracranial hypotension (i.e., subdural fluid collection, pachymeningeal enhancement, or sagging of the brain) in addition at least one of the following: 1) low opening pressure on lumbar puncture; 2) spinal meningeal diverticulum; or 3) improvement of the headache after epidural blood patching. If criteria A and B are not met, then criterion C requires the presence of all of the following or at least 2 of the following if typical orthostatic headaches are present: 1) low opening pressure; 2) spinal meningeal diverticulum; or 3) improvement of the headache after epidural blood patching. These criteria were applied to a total of 107 patients who were evaluated for a spontaneous spinal CSF leaks and intracranial hypotension over a 6-year period, beginning in 2001. The patients did not have any history of dural puncture or spinal fluid leakage from the nose or ear.
The diagnosis of spontaneous spinal CSF leakage with intracranial hypotension was confirmed in a total of 94 patients. There were abnormal cranial MR imaging in 72% of patients: subdural enhancement in 36%, pachymeningeal enhancement in 56%, and sagging brain in 51%. A spinal fluid leak was demonstrated (criterion A) in 78 patients using CT myelography, MRI, or MR myleography (MRM). Eleven patients had abnormal cranial MRI but were diagnosed using criterion B. Five patients had normal imaging but met criterion C. In the remaining 13 patients, cranial and spinal imaging, along with recording of opening pressure, could not confirm the diagnosis for their orthostatic headaches. Meningeal diverticula (abnormal dilations of the nerve root sleeve) found in the cervical and/or thoracic spine were found on spinal imaging in 43% of patients. Spinal imaging showed a CSF leak in 83% of patients. Of 88 patients treated with epidural blood patching, 84 (95%) had a favorable response.
Yoo and colleagues compared MRM and radioisotope cisternography (RIC) to detect the leakage site in the spine in 15 patients with spinal CSF leak syndrome by clinical criteria. MRM was performed with the 2D turbo spin-echo technique in the entire spine using a 1.5T scanner. The sensitivity, specificity, and accuracy of MR myelography for detecting a CSF leak were in the range of 80.0% to 93.3% for the two readers. The sensitivity of RIC was 93.3%. The authors concluded that MRM is an effective tool for detecting the site of CSF leak in patients with spinal CSF leak syndrome and can be used, in most patients, in the place of the more invasive RIC technique.
Commentary
The diagnosis of SIH can be made easily when there is a typical presentation of orthostatic headaches in a patient with pachymeningeal enhancement on MRI. However, when these headaches persist untreated, the distinctive orthostatic fluctuation in headache may be obscured, leading to the diagnosis of new daily-persistent headache. The evaluation of new daily-persistent headache (a specific headache type defined in the second edition of the International Classification of Headache Disorders) may not include enhanced MRI, spinal imaging, or evaluation of opening pressure unless the evolution of SIH is suspected. Since persistent SIH responds to epidural blood patching in the majority of patients, the accurate diagnosis of this specific headache type is especially important. The use of MRM, a non-invasive technique, in patients with new daily-persistent headache, may detect a source of CSF leak in unsuspected cases of SIH.
Specific radiological criteria and response to epidural blood patching can confirm the diagnosis of spontaneous spinal CSF leaks and intracranial hypotension. Non-invasive magnetic resonance myelography can detect the site of CSF leakage.Subscribe Now for Access
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