Spontaneous Spinal Cerebrospinal Fluid Leaks

Cephalalgia ◽  
2008 ◽  
Vol 28 (12) ◽  
pp. 1345-1356 ◽  
Author(s):  
WI Schievink
Keyword(s):  
Cerebrospinal Fluid ◽  
Spontaneous Intracranial Hypotension ◽  
Csf Leak ◽  
Epidural Blood Patching ◽  
Delay In Diagnosis ◽  
Facial Numbness ◽  
Cerebrospinal Fluid Leaks ◽  
Brain Sagging ◽  
Glue Injection ◽  
Fluid Collections

Spontaneous intracranial hypotension is an uncommon but not rare cause of new onset daily persistent headaches. A delay in diagnosis is the norm. Women are affected more commonly than men and most are in the fifth or sixth decade of life. The underlying cause is a spontaneous spinal cerebrospinal fluid (CSF) leak. Typically the headache is orthostatic in nature but other headache patterns occur as well. Associated symptoms are common and include neck pain, a change in hearing, diplopia, facial numbness, cognitive abnormalities and even coma. Typical imaging findings consist of subdural fluid collections, pachymeningeal enhancement, pituitary hyperaemia and brain sagging, but magnetic resonance imaging may be normal. Myelography is the study of choice to identify the CSF leak but is not always necessary to make the diagnosis. Treatment consists of bedrest, abdominal binder, epidural blood patching, percutaneous fibrin glue injection or surgical CSF leak repair. Outcomes have been poorly studied.

Behavioral Variant Frontotemporal Dementia as a Serious Complication of Spontaneous Intracranial Hypotension

2018 ◽  
Vol 15 (5) ◽  
pp. 505-515 ◽  
Author(s):  
Wouter I Schievink ◽  
M Marcel Maya ◽  
Zachary R Barnard ◽  
Franklin G Moser ◽  
Stacey Jean-Pierre ◽  
...  
Keyword(s):  
Frontotemporal Dementia ◽  
Intracranial Hypotension ◽  
Spontaneous Intracranial Hypotension ◽  
Csf Leak ◽  
Behavioral Variant Frontotemporal Dementia ◽  
Clinical Criteria ◽  
Epidural Blood Patching ◽  
Early Onset Dementia ◽  
Percutaneous Procedures ◽  
Brain Sagging

Abstract BACKGROUND Behavioral variant frontotemporal dementia (bvFTD) is a devastating early onset dementia. Symptoms of bvFTD may be caused by spontaneous intracranial hypotension (SIH), a treatable disorder, but no comprehensive study of such patients has been reported. OBJECTIVE To describe detailed characteristics of a large cohort of patients with SIH and symptoms of bvFTD. METHODS We identified patients with SIH who met clinical criteria for bvFTD. Patients were compared to a cohort of SIH patients without bvFTD. RESULTS The mean age for the 21 men and 8 women was 52.9 yr (range, 37–65 yr). All 29 patients with bvFTD symptoms had hypersomnolence. Magnetic resonance imaging showed brain sagging in all patients, cerebrospinal fluid (CSF) opening pressure low in about half of patients, but a spinal CSF leak could not be detected in any patient. All patients underwent epidural blood patching, but 26 patients eventually underwent 1 or more surgical procedures. Overall, a good outcome was obtained in 21 patients (72%); 20 (91%) of 22 patients who had not undergone prior Chiari surgery compared to 1 (14%) of 7 patients who did undergo Chiari surgery (P < .003). Compared to SIH patients without symptoms of bvFTD (n = 547), those with bvFTD symptoms were older, more often male, less often demonstrated CSF leak on spinal imaging, and more often underwent surgery (P < .02). CONCLUSION bvFTD in SIH is rare and associated with brain sagging and hypersomnolence. Spinal CSF leaks are rarely detected. bvFTD symptoms are often refractory to the usual percutaneous procedures but most patients can be cured.

scholarly journals Ehlers Danlos, POTS, and Occult Cerebrospinal Fluid Leak: A Case Report

2017 ◽  
Vol 2 (2) ◽  
Keyword(s):  
Cerebrospinal Fluid ◽  
Spontaneous Intracranial Hypotension ◽  
Cerebrospinal Fluid Leak ◽  
Csf Leak ◽  
Postural Orthostatic Tachycardia Syndrome ◽  
Mri Imaging ◽  
Epidural Blood Patching ◽  
Blood Patch ◽  
History Of ◽  
Fluid Leak

Ehlers Danlos syndromes (EDS) are associated with spontaneous intracranial hypotension (SIH) and postural orthostatic tachycardia syndrome (POTS). We hypothesized that some POTS patients might in fact have occult SIH due to unappreciated EDS. We describe a 26-year-old female with a history of POTS and headache who presented to us with negative imaging for cerebrospinal fluid leak (CSF). Upon examination with clinical evidence of EDS and supportive history, we performed an epidural blood patch despite repeat imaging evidence negative for CSF leak resulting in resolution of the patient’s POTS, headache, and cognitive symptoms. Patients with POTS and connective tissue disorders associated with dural weakness may be suffering from occult chronic CSF leaks. MRI imaging may be falsely reassuring and dissuade clinicians from performing epidural blood patching among patients who can be cured.

False localizing sign of C1–2 cerebrospinal fluid leak in spontaneous intracranial hypotension

2004 ◽  
Vol 100 (4) ◽  
pp. 639-644 ◽  
Author(s):  
Wouter I. Schievink ◽  
M. Marcel Maya ◽  
James Tourje
Keyword(s):  
Cerebrospinal Fluid ◽  
Intracranial Hypotension ◽  
Fluid Collection ◽  
Spontaneous Intracranial Hypotension ◽  
Cerebrospinal Fluid Leak ◽  
Csf Leak ◽  
Content Type ◽  
The Mean ◽  
Fluid Collections ◽  
Fine Print

Object. Spontaneous intracranial hypotension due to a spinal cerebrospinal fluid (CSF) leak is an important cause of new daily persistent headaches. Spinal neuroimaging is important in the treatment of these patients, particularly when direct repair of the CSF leak is contemplated. Retrospinal C1–2 fluid collections may be noted on spinal imaging and these are generally believed to correspond to the site of the CSF leak. The authors undertook a study to determine the significance of these C1–2 fluid collections. Methods. The patient population consisted of a consecutive group of 25 patients (18 female and seven male) who were evaluated for surgical repair of a spontaneous spinal CSF leak. The mean age of the 18 patients was 38 years (range 13–72 years). All patients underwent computerized tomography myelography. Three patients (12%) had extensive retrospinal C1–2 fluid collections; the mean age of this woman and these two men was 41 years (range 39–43 years). The actual site of the CSF leak was located at the lower cervical spine in these patients and did not correspond to the site of the retrospinal C1–2 fluid collection. Conclusions. A retrospinal fluid collection at the C1–2 level does not necessarily indicate the site of the CSF leak in patients with spontaneous intracranial hypotension. This is an important consideration in the treatment of these patients because therapy may be inadvertently directed at this site.

scholarly journals Spontaneous C1-2 CSF Leak Treated with High Cervical Epidural Blood Patch

2008 ◽  
Vol 35 (1) ◽  
pp. 102-105 ◽  
Author(s):  
Philip WH Peng ◽  
Richard Farb
Keyword(s):  
Epidural Blood Patch ◽  
Spontaneous Intracranial Hypotension ◽  
Memory Loss ◽  
Csf Leak ◽  
Cerebellar Tonsil ◽  
Iodinated Contrast ◽  
Blood Patch ◽  
Magnetic Resonance Imaging Mri ◽  
Fluid Collections ◽  
High Cervical

Spontaneous intracranial hypotension (SIH) is a condition caused by spontaneous spinal cerebrospinal fluid (CSF) leaks. Clinically, it is characterized by orthostatic headache and may respond well to epidural blood patch. Other neurological presentations of SIH include diplopia, memory loss, hearing deficits, Parkinsonism, ataxia, obtundation and even coma.Magnetic resonance imaging (MRI) is crucial in the diagnosis of this condition. Typical radiological features include diffuse pachymeningeal enhancement, descent of the cerebellar tonsil, a decrease in the size of prepontine and perichiasmatic cisterns, and subdural fluid collections. However, radiological imaging such as radionuclide cisternography or spinal MRI are not reliable in detecting the site of leakage. Myelography with iodinated contrast followed by thin-cut computed tomography (CT) or MRI of the entire spine has been shown as the technique of choice in defining the location of the CSF leak.

Spontaneous Skull Base Cerebrospinal Fluid Leaks and Their Relationship to Idiopathic Intracranial Hypertension

2020 ◽  
Vol 35 (1) ◽  
pp. 36-43 ◽  
Author(s):  
Samuel Bidot ◽  
Joshua M. Levy ◽  
Amit M. Saindane ◽  
Kannan M. Narayana ◽  
Michael Dattilo ◽  
...  
Keyword(s):  
Cerebrospinal Fluid ◽  
Optic Nerve ◽  
Intracranial Hypertension ◽  
Skull Base ◽  
Optic Nerve Head ◽  
Idiopathic Intracranial Hypertension ◽  
Signs And Symptoms ◽  
Csf Leak ◽  
Imaging Evaluation ◽  
Cerebrospinal Fluid Leaks

Background The association between spontaneous skull base cerebrospinal fluid (CSF) leaks and idiopathic intracranial hypertension (IIH) has been suggested, but its significance remains unclear. Objective To estimate the prevalence of IIH in spontaneous skull base CSF leak patients. Methods Systematic collection of demographics, neuro-ophthalmic and magnetic resonance imaging evaluation of spontaneous skull base CSF leak patients seen pre- and post-leak repair in one neuro-ophthalmology service. Patients with preexisting IIH were diagnosed with definite IIH if adequate documentation was provided; otherwise, they were categorized with presumed IIH. Classic radiographic signs of intracranial hypertension and bilateral transverse venous sinus stenosis were recorded. Results Thirty six patients were included (age [interquartile range]: 50 [45;54] years; 94% women; body mass index: 36.8 [30.5;39.9] kg/m2), among whom six (16.7%, [95% confidence interval, CI]: [6.4;32.8]) had a preexisting diagnosis of definite or presumed IIH. Of the remaining 30 patients, four (13.3%, 95%CI: [3.8;30.7]) had optic nerve head changes suggesting previously undiagnosed IIH, while one was newly diagnosed with definite IIH at initial consultation. One out of 29 patients with normal findings of the optic nerve head at presentation developed new onset papilledema following surgery (3.4%, 95%CI: [0.1;17.8]) and was ultimately diagnosed with definite IIH. Overall, the prevalence of definite IIH was 19.4% (95%CI: [8.2;36.0]). Conclusion Striking demographic overlap exists between IIH patients and those with spontaneous CSF leak. Definite IIH was present in approximately 20% of our patients. However, its true prevalence is likely higher than identified by using classic criteria. We therefore hypothesize that an active CSF leak serves as an auto-diversion for CSF, thereby “treating” the intracranial hypertension and eliminating characteristic signs and symptoms at initial presentation.

Significance of Empty Sella in Cerebrospinal Fluid Leaks

2003 ◽  
Vol 128 (1) ◽  
pp. 32-38 ◽  
Author(s):  
Rodney J. Schlosser ◽  
William E. Bolger
Keyword(s):  
Cerebrospinal Fluid ◽  
Intracranial Hypertension ◽  
Sella Turcica ◽  
Radiographic Finding ◽  
Empty Sella ◽  
Senior Author ◽  
Csf Leak ◽  
Benign Intracranial Hypertension ◽  
Cerebrospinal Fluid Leaks ◽  
Csf Leaks

OBJECTIVE: The role of elevated cerebrospinal fluid (CSF) pressures in the pathophysiology of various CSF leaks is not clear. Empty sella syndrome (ESS) is a radiographic finding that can be associated with elevated CSF pressures and may represent a radiographic indicator of intracranial hypertension. We present our experience with CSF leaks of various causes, the prevalence of ESS in the spontaneous and nonspontaneous categories, and the potential pathophysiology and unique management issues of the spontaneous CSF leak group. METHODS: We conducted a retrospective review of medical records, imaging studies, and surgical treatment of CSF leaks in patients treated by the senior author. RESULTS: Sixteen patients with spontaneous CSF leaks and 12 patients with nonspontaneous CSF leaks were surgically treated from 1996 through 2002. In the spontaneous group, 15 patients had complete imaging of the sella turcica. Ten had completely empty sellae and 5 had partially empty sellae, for a total of 100% (15 of 15). In the nonspontaneous group, 9 patients had complete imaging of the sella. Only 11% (1 of 9) had a partially empty sella and that was a congenital leak. Comparison of proportions between these 2 groups was significant ( P = 0.01). The spontaneous group consisted primarily of obese, middle-aged females (13 of 16 patients). CONCLUSION: Empty sella probably represents a sign of elevated intracranial pressure that leads to idiopathic, spontaneous CSF leaks. Spontaneous CSF leaks are strongly associated with the radiographic finding of an empty sella and are more common in obese females, similar to benign intracranial hypertension. This unique population may require more aggressive surgical and medical treatment to prevent recurrent or multiple leaks.

Recurrent spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension: a prospective study

2003 ◽  
Vol 99 (5) ◽  
pp. 840-842 ◽  
Author(s):  
Wouter I. Schievink ◽  
M. Marcel Maya ◽  
Mary Riedinger
Keyword(s):  
Cerebrospinal Fluid ◽  
Intracranial Hypotension ◽  
Surgical Repair ◽  
Csf Leak ◽  
Content Type ◽  
Cerebrospinal Fluid Leaks ◽  
The Mean ◽  
A Prospective Study ◽  
Fine Print

Object. Intracranial hypotension due to a spontaneous spinal cerebrospinal fluid (CSF) leak is an increasingly recognized cause of postural headaches, but reliable follow-up data are lacking. The authors undertook a study to determine the risk of a recurrent spontaneous spinal CSF leak. Methods. The patient population consisted of a consecutive group of 18 patients who had been evaluated for consideration of surgical repair of a spontaneous spinal CSF leak. The mean age of the 15 women and three men was 38 years (range 22–55 years). The mean duration of follow up was 36 months (range 6–132 months). The total follow-up time was 654 months. A recurrent spinal CSF leak was defined on the basis of computerized tomography myelography evidence of a CSF leak in a previously visualized but unaffected spinal location. Five patients (28%) developed a recurrent spinal CSF leak; the mean age of these four women and one man was 36 years. A recurrent CSF leak developed in five (38%) of 13 patients who had undergone surgical CSF leak repair, compared with none (0%) of five patients who had been treated non-surgically (p = 0.249). The recurrent leak occurred between 10 and 77 months after the initial CSF leak, but within 2 or 3 months of successful surgical repair of the leak in all patients. Conclusions. Recurrent spontaneous spinal CSF leaks are not rare, and the recent successful repair of such a leak at another site may be an important risk factor.

False localizing signs of spinal CSF–venous fistulas in spontaneous intracranial hypotension: report of 2 cases

2019 ◽  
Vol 31 (5) ◽  
pp. 764-767 ◽  
Author(s):  
Wouter I. Schievink ◽  
M. Marcel Maya ◽  
Franklin G. Moser
Keyword(s):  
Intracranial Hypotension ◽  
Spontaneous Intracranial Hypotension ◽  
The Other ◽  
Ct Scans ◽  
Csf Leak ◽  
Digital Subtraction ◽  
Glue Injection ◽  
Venous Fistula ◽  
Digital Subtraction Myelography ◽  
Treatment Surgery

A spinal CSF–venous fistula is one of three specific types of spinal CSF leak that can be seen in patients with spontaneous intracranial hypotension (SIH). They are best demonstrated on specialized imaging, such as digital subtraction myelography (DSM) or dynamic myelography, but often they are diagnosed on the basis of increased contrast density in the draining veins (the so-called hyperdense paraspinal vein sign) on early postmyelography CT scans. The authors report on 2 patients who underwent directed treatment (surgery in one patient and glue injection in the other) based on the hyperdense paraspinal vein sign, in whom the actual site of the fistula did not correspond to the level or laterality of the hyperdense paraspinal vein sign. The authors suggest consideration of DSM or dynamic myelography prior to undertaking treatment directed at these fistulas.

Spontaneous Intracranial Hypotension and MRI

1998 ◽  
Vol 11 (2) ◽  
pp. 203-206
Author(s):  
I. Muras ◽  
A. Scuotto ◽  
M. Maisto ◽  
F.P. Bernini
Keyword(s):  
Intracranial Hypotension ◽  
Medical Condition ◽  
Spontaneous Intracranial Hypotension ◽  
Bed Rest ◽  
Csf Leak ◽  
Meningeal Enhancement ◽  
Csf Leakage ◽  
Downward Displacement ◽  
Fluid Collections ◽  
The Brain

Postural headache due to low intracranial pressure is a well-known entity and is most commonly encountered following lumbar puncture. It may occur as a consequence of a medical condition (dehydratation, uremia, etc.) but in some cases no precipitating event is apparent and the intracranial hypotension is believed to have developed spontaneously. In such cases the underlying cause of the syndrome is rarely established and treatment is non specific. We describe three patients with spontaneous intracranial hypotension examined with MRI of the brain. Women are more commonly affected than men in the third or fourth decades of life. Schaltenbrand (1938) proposed three mechanisms by which spontaneous intracranial hypotension may be explained: diminished CSF production; CSF hyperabsorption, CSF leakage. The defect causing a CSF leak usually remains obscure. Several cases of diffuse meningeal enhancement on MRI have recently been described, probably due to meningeal hyperaemia resulting from the low CSF pressure. Subdural fluid collections have also been detected as a result of rupture of bridging veins due to the decrease in CSF volume and downward displacement of the brain. In our cases, MRI showed a diffuse dural thickening, hyperintense in T2. Spontaneous intracranial hypotension is often a self-limiting disease, responding well to bed rest and a generous intake of oral or parenteral fluid and salt.

Sign in / Sign up

Export Citation Format

Share Document