Publication Abstracts 2

pubmed
This is a collection of selected publication abstracts about spinal CSF leaks / intracranial hypotension, listed with most recent first, from 2014 and earlier.
* Abstract links are included. (click on the PMID number)
* Note that links to full-text are provided for open access papers (line below PMID).

2014

Spontaneous intracranial hypotension: improving recognition and treatment strategies in the local setting
Lee GK, Abrigo JM, Cheung TC, Siu DY, Chan DT.
Hong Kong Med J. 2014 Dec;20(6):537-40.
Abstract
We report a case of spontaneous intracranial hypotension with classic symptoms of orthostatic headache and acute presentation of subdural haematoma on computed tomographic scan. Conventional approach with conservative treatment was initially adopted. The patient’s condition, however, deteriorated after 2 weeks, requiring surgical evacuation of the intracranial haemorrhage. We reviewed the clinical features of this disease and the correlated magnetic resonance imaging findings with the pathophysiological mechanisms, and described treatment strategies in the local setting. Subtle findings on initial computed tomographic scan are also reported which might improve pathology recognition. Spontaneous intracranial hypotension is not uncommonly encountered in Hong Kong, and physicians must adopt a high level of clinical suspicion to facilitate early diagnosis and appropriate management. In addition, novel therapeutic approaches may be required in those with recurrent symptoms or who are refractory to current treatment strategies.
PMID: 25488033
full text: hkmj.org/article_pdfs/hkm1412p537.pdf

Spinal cerebrospinal fluid leak as the cause of chronic subdural hematomas in nongeriatric patients.
Beck J, Gralla J, Fung C, Ulrich CT, Schucht P, Fichtner J, Andereggen L, Gosau M, Hattingen E, Gutbrod K, Z’Graggen WJ, Reinert M, Hüsler J, Ozdoba C,Raabe A.
J Neurosurg. 2014 Dec;121(6):1380-7.
Object The etiology of chronic subdural hematoma (CSDH) in nongeriatric patients (≤ 60 years old) often remains unclear. The primary objective of this study was to identify spinal CSF leaks in young patients, after formulating the hypothesis that spinal CSF leaks are causally related to CSDH.
Methods All consecutive patients 60 years of age or younger who underwent operations for CSDH between September 2009 and April 2011 at Bern University Hospital were included in this prospective cohort study. The patient workup included an extended search for a spinal CSF leak using a systematic algorithm: MRI of the spinal axis with or without intrathecal contrast application, myelography/fluoroscopy, and postmyelography CT. Spinal pathologies were classified according to direct proof of CSF outflow from the intrathecal to the extrathecal space, presence of extrathecal fluid accumulation, presence of spinal meningeal cysts, or no pathological findings. The primary outcome was proof of a CSF leak.
Results Twenty-seven patients, with a mean age of 49.6 ± 9.2 years, underwent operations for CSDH. Hematomas were unilateral in 20 patients and bilateral in 7 patients. In 7 (25.9%) of 27 patients, spinal CSF leakage was proven, in 9 patients (33.3%) spinal meningeal cysts in the cervicothoracic region were found, and 3 patients (11.1%) had spinal cysts in the sacral region. The remaining 8 patients (29.6%) showed no pathological findings.
Conclusions The direct proof of spinal CSF leakage in 25.9% of patients suggests that spinal CSF leaks may be a frequent cause of nongeriatric CSDH.
PMID: 25036203
Full text

Movement disorders associated with spontaneous CSF leaks: A case series.
Mokri B.
Cephalalgia. 2014 Dec;34(14):1134-41.
Abstract
IMPORTANCE AND OBJECTIVE:
Headache is the most common symptom in spontaneous CSF leaks, frequently associated with additional manifestations. Herein, attention is drawn to movement disorder as a notable manifestation of spontaneous CSF leaks.
DESIGN:
Four women and one man (ages 51-78 years) with spontaneous CSF leaks and movement disorders were evaluated clinically and by pertinent neuroimaging studies with follow-up of one to seven years (mean 3.2 years).
RESULTS:
The movement disorder consisted of choreiform movements in two patients, torticollis in one, mixed tremor in one, and parkinsonism in one. All except the last patient had headaches (orthostatic in one, Valsalva maneuver-induced in one, both orthostatic and Valsalva-induced in two, lingering low-grade headache in one). Diffuse pachymeningeal enhancement and sinking of the brain was noted in all. CT-myelography showed definite CSF leak in three and equivocal leak in one, while no leak could be located in the fifth patient. Two patients improved over time with complete resolution of the movement disorder. One responded to epidural blood patch with complete resolution of his choreiform movements. Two patients required surgery and epidural blood patches. Results were drastic but nondurable in one, while complete recovery was achieved in the other.
CONCLUSION:
Movement disorders are uncommon in spontaneous CSF leaks but occasionally can be one of the major components of the clinical presentation.
PMID: 24728303

Cerebral venous thrombosis in two patients with spontaneous intracranial hypotension
Garcia-Carreira MC, Vergé DC, Branera J, Zauner M, Herrero JE, Tió E, Perpinyà GR.
Case Rep Neurol Med. 2014;2014:528268.
Abstract
Although few patients with spontaneous intracranial hypotension develop cerebral venous thrombosis, the association between these two entities seems too common to be simply a coincidental finding. We describe two cases of spontaneous intracranial hypotension associated with cerebral venous thrombosis. In one case, extensive cerebral venous thrombosis involved the superior sagittal sinus and multiple cortical cerebral veins. In the other case, only a right frontoparietal cortical vein was involved. Several mechanisms could contribute to the development of cerebral venous thrombosis in spontaneous intracranial hypotension. When spontaneous intracranial hypotension and cerebral venous thrombosis occur together, it raises difficult practical questions about the treatment of these two conditions. In most reported cases, spontaneous intracranial hypotension was treated conservatively and cerebral venous thrombosis was treated with anticoagulation. However, we advocate aggressive treatment of the underlying cerebrospinal fluid leak.
PMID: 25525533
full text: PMC4265689

Bariatric surgery as a possible risk factor for spontaneous intracranial hypotension
Schievink WI, Goseland A, Cunneen S
Neurology 2014 Nov 11;83(20):1819-22.
Abstract
Objective: To evaluate a possible link between bariatric surgery and spontaneous intracranial hypotension.
Methods: The frequency of bariatric surgery was examined in a group of 338 patients with spontaneous intracranial hypotension and compared with a group of 245 patients with unruptured intracranial aneurysms.
Results: Eleven (3.3%) of the 338 patients with spontaneous intracranial hypotension had a history of bariatric surgery compared with 2 (0.8%) of the 245 patients with intracranial aneurysms (p = 0.02). Among the 11 patients with spontaneous intracranial hypotension after bariatric surgery, the mean age at the time of bariatric surgery was 40.8 years (range, 26–53 years) and the mean age at the time of onset of spontaneous intracranial hypotension was 45.6 years (range, 31–59 years). Weight at the time of bariatric surgery ranged from 95 to 166 kg (mean, 130 kg) (body mass index range: 34.9–60.1 kg/m2; mean: 44.6). Weight at the time of onset of symptoms of spontaneous intracranial hypotension ranged from 52 to 106 kg (mean, 77.5 kg) (body mass index range: 19.2–32.1 kg/m2; mean: 26.4). The mean weight loss from bariatric surgery to onset of spontaneous intracranial hypotension was 52.5 kg (range, 25–98 kg). Time interval from bariatric surgery to onset of symptoms of spontaneous intracranial hypotension ranged from 3 to 241 months (mean, 56.5 months).
Conclusions: This case-control study shows that bariatric surgery is a potential risk factor for spontaneous intracranial hypotension.
PMID: 25339216

Headache secondary to intracranial hypotension.
Schievink WI, Deline CR.
Curr Pain Headache Rep. 2014 Nov;18(11):457.
Abstract
Intracranial hypotension is known to occur as a result of spinal cerebrospinal fluid (CSF) leaking, which may be iatrogenic, traumatic, or spontaneous. Headache is usually, but not always, orthostatic. Spontaneous cases are recognized more readily than in previous decades as a result of a greater awareness of clinical presentations and typical cranial magnetic resonance imaging findings. An underlying disorder of connective tissue that predisposes to weakness of the dura is implicated in spontaneous spinal CSF leaks. CT, MR, and digital subtraction myelography are the imaging modalities of choice to identify spinal CSF leakage. Spinal imaging protocols continue to evolve with improved diagnostic sensitivity. Epidural blood patching is the most common initial intervention for those seeking medical attention, and may be repeated several times. Surgery is reserved for cases that fail to respond or relapse after simpler measures. While the prognosis is generally good with intervention, serious complications do occur. More research is needed to better understand the genetics and pathophysiology of dural weakness as well as physiologic compensatory mechanisms, to continue to refine imaging modalities and treatment approaches, and to evaluate short- and long-term clinical outcomes.
PMID: 25255993

Computed tomography-guided epidural patching of postoperative cerebrospinal fluid leaks.
Mihlon F, Kranz PG, Gafton AR, Gray L.
J Neurosurg Spine.2014 Nov;21(5):805-10.
Abstract
Object: Cerebrospinal fluid leaks due to unrecognized durotomy during spinal surgery are often managed with a second surgery for dural closure. CT-guided percutaneous patching targeted to the dural defect offers an alternative to surgery since it can be performed in a minimally invasive fashion without the need for general anesthesia. This case series describes the authors’ experience using targeted CT-guided percutaneous patching to repair incidental durotomies incurred during spinal surgery.
Methods: This investigation is a retrospective case series involving patients who underwent CT-guided percutaneous patching of surgical incidental durotomies and were referred between January 2007 and June 2013. Their presenting clinical history, myelographic findings, and clinical outcomes, including the need for eventual surgical duraplasty, were reviewed.
Results: Nine cases were identified, including 7 durotomies incurred during lumbar discectomy, one due to a medial transpedicular screw breach, and one incurred during vertebrectomy for spinal osteosarcoma. All patients who had favorable outcomes with percutaneous intervention alone had 2 common features: dural defect of 4 mm or smaller and absence of a pseudomeningocele. Patients with CSF leaks complicated by pseudomeningocele and those with a dural defect of 6 mm or more all required eventual surgical management.
Conclusions: The authors’ results suggest that findings on CT myelography may help predict which patients with postsurgical durotomy can be treated with percutaneous intervention. In particular, CT-guided patching may be more likely to be successful in those patients with dural defects of less than 5 mm and without pseudomeningocele. In patients with larger dural defects or pseudomeningoceles, percutaneous blood patching alone is unlikely to be successful.
PMID: 25127431

Echocardiographic findings in patients with spontaneous CSF leak.
Pimienta AL, Rimoin DL, Pariani M, Schievink WI, Reinstein E.
J Neurol. 2014 Oct;261(10):1957-60.
Abstract
The presence of cardiovascular abnormalities in patients with spontaneous cerebrospinal fluid (CSF) leaks are not well-documented in the literature, as cardiovascular evaluation is not generally pursued if a patient does not exhibit additional clinical features suggesting an inherited connective tissue disorder. We aimed to assess this association, enrolling a consecutive group of 50 patients referred for spinal CSF leak consultation. Through echocardiographic evaluation and detailed medical history, we estimate that up to 20 % of patients presenting with a spontaneous CSF leak may have some type of cardiovascular abnormality. Further, the increase in prevalence of aortic dilatation in our cohort was statistically significant in comparison to the estimated population prevalence. This supports a clinical basis for echocardiographic screening of these individuals for cardiovascular manifestations that may have otherwise gone unnoticed or evolved into a more severe manifestation.
PMID: 25059392

MR Myelography for Identification of Spinal CSF Leak in Spontaneous Intracranial Hypotension.
Chazen JL, Talbott JF, Lantos JE, Dillon WP.
AJNR Am J Neuroradiol. 2014 Oct;35(10):2007-12.
Abstract
BACKGROUND AND PURPOSE:
CT myelography has historically been the test of choice for localization of CSF fistula in patients with spontaneous intracranial hypotension. This study evaluates the additional benefits of intrathecal gadolinium MR myelography in the detection of CSF leak.
MATERIALS AND METHODS:
We performed a retrospective review of patients with spontaneous intracranial hypotension who underwent CT myelography followed by intrathecal gadolinium MR myelography. All patients received intrathecal iodine and off-label gadolinium-based contrast followed by immediate CT myelography and subsequent intrathecal gadolinium MR myelography with multiplanar T1 fat-suppressed sequences. CT myelography and intrathecal gadolinium MR myelography images were reviewed by an experienced neuroradiologist to determine the presence of CSF leak. Patient records were reviewed for demographic data and adverse events following the procedure.
RESULTS:
Twenty-four patients met both imaging and clinical criteria for spontaneous intracranial hypotension and underwent CT myelography followed by intrathecal gadolinium MR myelography. In 3/24 patients (13%), a CSF leak was demonstrated on both CT myelography and intrathecal gadolinium MR myelography, and in 9/24 patients (38%), a CSF leak was seen on intrathecal gadolinium MR myelography (P = .011). Four of 6 leaks identified independently by intrathecal gadolinium MR myelography related to meningeal diverticula. CT myelography did not identify any leaks independently. There were no reported adverse events.
CONCLUSIONS:
Present data demonstrate a higher rate of leak detection with intrathecal gadolinium MR myelography when investigating CSF leaks in our cohort of patients with spontaneous intracranial hypotension. Although intrathecal gadolinium is an FDA off-label use, all patients tolerated the medication without evidence of complications. Our data suggest that intrathecal gadolinium MR myelography is a well-tolerated examination with significant benefit in the evaluation of CSF leak, particularly for patients with leak related to meningeal diverticula.
PMID: 24852289

Injecting Under Pressure: The Pain of Low CSF Pressure Headache Responsive to Botulinum Toxin Injections.
Mathew PG, Cutrer FM.
Curr Neurol Neurosci Rep. 2014 Sep;14(9):477.
Abstract
Low intracranial pressure headaches can, at times, be refractory to treatment including multiple blood patches and preventative medications. Imaging studies are often unable to demonstrate a cerebrospinal fluid leak that is causing headache and other associated symptoms. Onabotulinum toxin A (BTX) injection is a treatment that has proven efficacy for the treatment of chronic migraine and potentially other headache disorders. We report a patient with a long standing history of refractory low pressure headaches with brain imaging that demonstrated brain sag, and no CSF leak could be identified. She received no sustained benefit from numerous blood patches, and was unresponsive or intolerant to multiple preventative medications. With BTX treatment, the patient continued to have daily headaches, but her pain intensity improved from an average 7/10 to 3/10. This benefit has been sustained over 7 years. This case suggests that BTX may be an effective treatment for headaches due to low intracranial pressure. It also suggests that the beneficial effects of BTX in the treatment of headaches occur through a direct modulation of the nociceptive system rather than merely induction of pericranial muscle relaxation.
PMID: 25027263

* important recent paper
CSF-venous fistula in spontaneous intracranial hypotension: demonstration by digital subtraction myelography.
Schievink W, Moser F, Maya M.
Neurology. 2014 Jul;83:472-473
PMID: 24951475

Radioisotope Cisternography in Spontaneous CSF Leaks: Interpretations and Misinterpretations.
Mokri B.
Headache. 2014 Jul 4. [Epub ahead of print]
Abstract
A broadening of the clinical and imaging features of the spontaneous cerebrospinal fluid (CSF) leaks is now well recognized, far beyond what was thought only two decades ago. This has resulted in increasing number of patients with atypical and unusual features who, not unexpectedly, are directed to headache specialists and tertiary referral centers. In many cases, obviously the fundamental question of presence or absence of CSF leak will need to be addressed prior to proceeding with further and often more involved, more invasive, and more costly diagnostic and therapeutic considerations. Radioisotope cisternography often proves to be very helpful in these situations by demonstrating reliable, although indirect, evidences of CSF leak while it is less helpful in directly identifying the exact site of the CSF leakage. In this overview article, the expectations from and the limitations of this diagnostic method are described along with some personal observations in the past 25 years.
PMID: 25041119

Rapid resolution of subdural hematoma after targeted epidural blood patch treatment in patients with spontaneous intracranial hypotension.
Wang J, Zhang D, Gong X, Ding M.
Chin Med J (Engl). 2014 Jun;127(11):2063-6.
Abstract
BACKGROUND:
Subdural hematoma (SDH) is a common complication of spontaneous intracranial hypotension (SIH). To date, the management of SDH caused by SIH remains controversial. In this paper, we reviewed the clinical course of SDH in patients with SIH, and discuss the underlying mechanism and attributing factors for rapid resolution of subdural hematomas after epidural blood patch (EBP) surgery.
METHODS:
We retrospectively reviewed a cohort of seventy-eight SIH patients diagnosed and treated with targeted EBP in our neurology center. Patients who received early CT/MRI follow-up after EBP operation were included.
RESULTS:
A series of four cases of SIH complicated with SDHs were evaluated. Early follow-up neuroimages of these patients revealed that SDHs could be partially or totally absorbed just two to four days after targeted epidural blood patch treatment.
CONCLUSION:
Targeted epidural blood patch can result in rapid hematoma regression and good recovery in some patients with a combination of SDH and SIH.
PMID: 24890153

Rebound Intracranial Hypertension: A Complication of Epidural Blood Patching for Intracranial Hypotension.
Kranz PG, Amrhein TJ, Gray L.
AJNR Am J Neuroradiol. 2014 Jun;35(6):1237-40.
doi: 10.3174/ajnr.A3841.
Abstract
SUMMARY: Rebound intracranial hypertension is a complication of epidural blood patching for treatment of intracranial hypotension characterized by increased intracranial pressure, resulting in potentially severe headache, nausea, and vomiting. Because the symptoms of rebound intracranial hypertension may bear some similarity to those of intracranial hypotension and literature reports of rebound intracranial hypertension are limited, it may be mistaken for refractory intracranial hypotension, leading to inappropriate management. This clinical report of 9 patients with confirmed rebound intracranial hypertension reviews the clinical characteristics of patients with this condition, emphasizing factors that can be helpful in discriminating rebound intracranial hypertension from refractory spontaneous intracranial hypotension, and discusses treatment.
PMID: 24407273

** review article
Spontaneous CSF leaks: low CSF volume syndromes.
Mokri B.
Neurol Clin. 2014 May;32(2):397-422.
doi: 10.1016/j.ncl.2013.11.002.
Abstract
Practically all cases of spontaneous intracranial hypotension results from spontaneous cerebral spinal fluid (CSF) leaks, often at the level of the spine and only rarely from the skull base. The triad of orthostatic headaches, diffuse pachymeningeal enhancement on head imaging and low CSF opening pressure is considered the hallmark of these leaks but substantial variability is noted in most aspects of this disorder including in features of the headaches, imaging and CSF findings, response to treatment and outcome.
PMID: 24703536

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2013

Sensitivity of MRI of the spine compared with CT myelography in orthostatic headache with CSF leak.
Starling A, Hernandez F, Hoxworth JM, Trentman T, Halker R, Vargas BB, Hastriter E, Dodick D
Neurology. 2013 Nov 12;81(20):1789-92.
doi: 10.1212/01.wnl.0000435555.13695.22.
Abstract
OBJECTIVE:
To investigate the sensitivity of MRI of the spine compared with CT myelography (CTM) in detecting CSF leaks.
METHODS:
Between July 1998 and October 2010, 12 patients with orthostatic headache and a CTM-confirmed spinal CSF leak underwent an MRI of the spine with and without contrast. Using CTM as the gold standard, we retrospectively investigated the sensitivity of spinal MRI in detecting a CSF leak.
RESULTS:
Eleven of 12 patients with a CSF leak documented by CTM also had extradural fluid collections on spinal MRI (sensitivity 91.7%). Six patients with extradural fluid collections on spinal MRI also had spinal dural enhancement.
CONCLUSION:
When compared with the gold standard of CTM, MRI of the spine appears to be a sensitive and less invasive imaging modality for detecting a spinal CSF leak, suggesting that MRI of the spine should be the imaging modality of first choice for the detection of spinal CSF leaks.
PMID:
24107860

Spontaneous intracranial hypotension in childhood and adolescence.
Schievink WI, Maya MM, Louy C, Moser FG, Sloninsky L.
J Pediatr. 2013 Aug;163(2):504-10.
doi: 10.1016/j.jpeds.2013.01.055.
Abstract
OBJECTIVES:
To describe the clinical and radiographic manifestations of spontaneous intracranial hypotension, a rarely diagnosed cause of headache in children.
STUDY DESIGN:
This study included patients 19 years of age or younger evaluated between January 1, 2001, and June 30, 2012, for spontaneous intracranial hypotension.
RESULTS:
We evaluated 24 children (18 girls and 6 boys) with spontaneous intracranial hypotension (age at onset of symptoms: 2-19 years, mean 14.3 years). Twenty-three patients presented with orthostatic headaches and 1 presented with a nonpositional headache. A generalized connective tissue disorder was diagnosed in 54% of patients. Magnetic resonance imaging showed the typical changes of spontaneous intracranial hypotension in most patients (79%). Spinal imaging demonstrated a cerebrospinal fluid (CSF) leak with or without an associated meningeal diverticulum in 12 patients (50%) and with dural ectasia or meningeal diverticula in 10 patients (42%), and it was normal in 2 patients (8%). Twenty-three patients initially underwent epidural blood patching, but 8 patients also were treated with percutaneous injections of fibrin glue and 11 patients eventually required surgical correction of the underlying CSF leak. There was no morbidity or mortality associated with any of the treatments, but 5 patients required acetazolamide for rebound high intracranial pressure headache. Overall, outcome was good in 22 patients (92%) and poor in 2 patients (8%).
CONCLUSIONS:
Spontaneous intracranial hypotension in childhood is rare. Most patients can be treated effectively using a combination of epidural blood patching and percutaneous injections of fibrin glue or surgical CSF leak repair in refractory cases.
PMID: 23453548

Evaluation on a clinical course of subdural hematoma in patients undergoing epidural blood patch for spontaneous cerebrospinal fluid leak.
Hashizume K, Watanabe K, Kawaguchi M, Fujiwara A, Furuya H.
Clin Neurol Neurosurg. 2013 Aug;115(8):1403-6.
doi: 10.1016/j.clineuro.2013.01.022.
Abstract
OBJECTIVE:
Subdural hematoma (SDH) is a frequent complication of spontaneous intracranial hypotension (SIH), in which epidural blood patch (EBP) may be applied as a treatment to stop cerebrospinal fluid (CSF) leak. However, a clinical course of SDH in SIH patients has not been sufficiently evaluated. We retrospectively evaluated the temporal relationships between EBP and SDH in the patients with SIH.
METHODS:
Twenty-nine consecutive patients, diagnosed as SIH, were studied. Clinical records and images were retrospectively evaluated. When orthostatic headache continued for 2 weeks regardless of conservative treatment, EBP was performed under fluoroscopy.
RESULTS:
We detected 13(45%) cases of SDH (mean age 44 years, 8 males and 5 females). In 6 patients, SDHs disappeared after effective EBP, i.e., after the disappearance of orthostatic headache. In 3 patients, SDHs were enlarged or recurred after effective EBP, and in 4 patients, SDHs were first detected after effective EBP.
CONCLUSION:
The knowledge of the presence of these types of SDH (enlarged or recurred or detected after EBP) may deserve clinical attention.
PMID: 23462186

** important paper reviewing diagnostic imaging
Novel neuroimaging modalities in the evaluation of spontaneous cerebrospinal fluid leaks.
Schievink WI.
Curr Neurol Neurosci Rep. 2013 Jul;13(7):358.
doi: 10.1007/s11910-013-0358-z.
Abstract
Although for the vast majority of patients with spontaneous intracranial hypotension knowledge of the exact site of the underlying spinal CSF leak is not necessary, it is for patients with recalcitrant symptoms. Such patients may require directed treatments such as percutaneous fibrin glue injections or surgery. A variety of MRI techniques have been shown to be able to detect CSF leaks as well and sometimes better than the “gold standard” – CT-myelography. For unusually rapid CSF leaks – particularly those ventral to the spinal cord – digital subtraction myelography or dynamic CT-myelography are indicated. Some patients with spontaneous intracranial hypotension verified by intracranial MRI are never found to have a spinal CSF leak using current techniques.
PMID: 23703239

Spontaneous low pressure, low CSF volume headaches: spontaneous CSF leaks.
Mokri B.
Headache. 2013 Jul-Aug;53(7):1034-53.
doi: 10.1111/head.12149.
Abstract
Spontaneous intracranial hypotension typically results from spontaneous cerebrospinal fluid (CSF) leak, often at spine level and only rarely from skull base. Once considered rare, it is now diagnosed far more commonly than before and is recognized as an important cause of headaches. CSF leak leads to loss of CSF volume. Considering that the skull is a rigid noncollapsible container, loss of CSF volume is typically compensated by subdural fluid collections and by increase in intracranial venous blood which, in turn, causes pachymeningeal thickening, enlarged pituitary, and engorgement of cerebral venous sinuses on magnetic resonance imaging (MRI). Another consequence of CSF hypovolemia is sinking of the brain, with descent of the cerebellar tonsils and brainstem as well as crowding of the posterior fossa noted on head MRI. The clinical consequences of these changes include headaches that are often but not always orthostatic, nausea, occasional emesis, neck and interscapular pain, cochleovestibular manifestations, cranial nerve palsies, and several other manifestations attributed to pressure upon or stretching of the cranial nerves or brain or brainstem structures. CSF lymphocytic pleocytosis or increase in CSF protein concentration is not uncommon. CSF opening pressure is often low but can be within normal limits. Stigmata of disorders of connective tissue matrix are seen in some of the patients. An epidural blood patch, once or more, targeted or distant, at one site or bilevel, has emerged as the treatment of choice for those who have failed the conservative measures. Epidural injection of fibrin glue of both blood and fibrin glue can be considered in selected cases. Surgery to stop the leak is considered when the exact site of the leak has been determined by neurodiagnostic studies and when less invasive measures have failed. Subdural hematomas sometimes complicate the CSF leaks; a rebound intracranial hypertension after successful treatment of a leak is not rare. Cerebral venous sinus thrombosis as a complication is fortunately less common, and superficial siderosis and bibrachial amyotrophy are rare. Short-term recurrences are not uncommon, and long-term recurrences are not rare.
PMID: 23808630
Full text

Spinal meningeal diverticula in spontaneous intracranial hypotension: analysis of prevalence and myelographic appearance.
Kranz PG, Stinnett SS, Huang KT, Gray L.
Am J Neuroradiol. 2013 Jun-Jul;34(6):1284-9.
doi: 10.3174/ajnr.A3359.
Abstract
BACKGROUND AND PURPOSE:
Spinal meningeal diverticula have been implicated in the pathogenesis of spontaneous intracranial hypotension and have been proposed as both diagnostic features of and therapeutic targets for the condition. We compared the prevalence and myelographic appearance of spinal diverticula in patients with SIH and healthy controls.
MATERIALS AND METHODS:
Patients satisfying the ICHD-2 criteria for SIH were retrospectively identified. CT myelograms of 19 patients with SIH were compared with CT myelograms of 18 control patients. Images were reviewed by 2 blinded neuroradiologists. The prevalence, morphology (round versus multilobulated), size, and location (cervical, upper thoracic, lower thoracic, or lumbar) of spinal meningeal diverticula were analyzed.
RESULTS:
There was no difference in the proportion of patients with diverticula in the SIH group compared with the control group (68% versus 44%, P = .14) or in the mean number of diverticula per patient (6.3 versus 2.2, P = .099). No difference was seen in the morphology (P = .95) or size (P = .71) of diverticula between groups. There was a difference between groups that just reached statistical significance (P = .050) in the location of the diverticula along the spinal axis, but substantial overlap was seen between groups for all spinal locations.
CONCLUSIONS:
Despite the well-established association between spinal meningeal diverticula and SIH, we found no difference in the prevalence or myelographic appearance of diverticula in patients with SIH compared with controls. Further investigation into the role of diverticula in the diagnosis and treatment of SIH is necessary.
PMID: 23221945
full text: ajnr.org/content/34/6/1284.long

Connective tissue spectrum abnormalities associated with spontaneous cerebrospinal fluid leaks: a prospective study.
Reinstein E, Pariani M, Bannykh S, Rimoin DL, Schievink WI.
Eur J Hum Genet. 2013 Apr;21(4):386-90.
doi: 10.1038/ejhg.2012.191.
Abstract
We aimed to assess the frequency of connective tissue abnormalities among patients with cerebrospinal fluid (CSF) leaks in a prospective study using a large cohort of patients. We enrolled a consecutive group of 50 patients, referred for consultation because of CSF leak. All patients have been carefully examined for the presence of connective tissue abnormalities, and based on findings, patients underwent genetic testing. Ancillary diagnostic studies included echocardiography, eye exam, and histopathological examinations of skin and dura biopsies in selected patients. We identified nine patients with heritable connective tissue disorders, including Marfan syndrome, Ehlers-Danlos syndrome and other unclassified forms. In seven patients, spontaneous CSF leak was the first noted manifestation of the genetic disorder. We conclude that spontaneous CSF leaks are associated with a spectrum of connective tissue abnormalities and may be the first noted clinical presentation of the genetic disorder. We propose that there is a clinical basis for considering spontaneous CSF leak as a clinical manifestation of heritable connective tissue disorders, and we suggest that patients with CSF leaks should be screened for connective tissue and vascular abnormalities.
PMID: 22929030
Full text PMC: 3598315

Brain herniation induced by drainage of subdural hematoma in spontaneous intracranial hypotension.
Chotai S, Kim JH, Kim JH, Kwon TH.
Asian J Neurosurg. 2013 Apr;8(2):112-5.
doi: 10.4103/1793-5482.116390.
Abstract
Spontaneous intracranial hypotension (SIH), typically presents with orthostatic headache, low pressure on lumbar tapping, and diffuse pachymeningeal enhancement on magnetic resonance imaging. SIH is often accompanied by subdural fluid collections, which in most cases responds to conservative treatment or spinal epidural blood patch. Several authors advocate that large subdural hematoma with acute deterioration merits surgical drainage; however, few have reported complications following craniotomy. We describe a complicated case of SIH, which was initially diagnosed as acute subarachnoid hemorrhage with bilateral chronic subdural hematoma (SDH), due to unusual presentation. Burr hole drainage of subdural hematoma was performed due to progressive decrease of consciousness, which then resulted in a huge postoperative epidural hematoma collection. Prompt hematoma evacuation did not restore the patient’s consciousness but aggravated downward brain herniation. Trendelenburg position and spinal epidural blood patch achieved a rapid improvement in patient’s consciousness. This case indicates that the surgical drainage for chronic SDH in SIH can lead to serious complications and it should be cautiously considered.
PMID: 24049555
full text : PMC3775182

Stroke and death due to spontaneous intracranial hypotension.
Schievink WI.
Neurocrit Care. 2013 Apr;18(2):248-51.
doi: 10.1007/s12028-012-9800-3.
Abstract
BACKGROUND:
Spontaneous intracranial hypotension has become a well-recognized cause of headaches and a wide variety of other manifestations have been reported. Recently, several patients with asymptomatic spontaneous intracranial hypotension were reported. I now report two patients with spontaneous intracranial hypotension who developed multiple arterial strokes associated with death in one patient, illustrating the spectrum of disease severity in spontaneous intracranial hypotension.
METHODS:
Medical records and radiologic imaging of the two patients were reviewed.
RESULTS:
Case 1. A 45-year-old man presented with an orthostatic headache. Neurologic examination was normal. MRI showed bilateral subdural fluid collections, brain sagging, and pachymeningeal enhancement. At lumbar puncture, the opening pressure was too low to record. He underwent two epidural blood patches with transient improvement of symptoms. His headaches progressed and a CT-myelogram showed a lower cervical CSF leak. Subsequently, periodic lethargy and confusion was noted and he then rapidly deteriorated. Examination showed coma (GCS: 4 [E1, M2, V1]), a fixed and dilated right pupil, and decerebrate posturing. Bilateral craniotomies were performed for the evacuation of chronic subdural hematomas. Immediate postoperative CT showed bilateral posterior cerebral artery infarcts and a recurrent right subdural hematoma, requiring re-evacuation. Postoperative examination was consistent with brain death and support was withdrawn.
Case 2. A 42-year-old man presented with a non-positional headache. Neurologic examination was normal. CT showed bilateral acute on chronic subdural hematomas and effacement of the basilar cisterns. MRI showed brain sagging, bilateral subdural hematomas, and pachymeningeal enhancement. Bilateral craniotomies were performed and subdural hematomas were evacuated. Postoperatively, the patient became progressively lethargic (GCS: 8 [E2, M4, V2]) and variable degrees of pupillary asymmetry and quadriparesis were noted. MRI now also showed multiple areas of restricted diffusion in the pons and midbrain, consistent with multiple infarcts. CT showed worsening subdural fluid collections with midline shift and increased effacement of the basilar cisterns. Repeat bilateral craniotomies were performed for evacuation of the subdural fluid collections. Neurologic examination was then noted to be fluctuating but clearly improved when lying flat (GCS: 10T [E4, M6, VT]). CT-myelography demonstrated an extensive cervico-thoracic CSF leak. An epidural blood patch was performed. The patient made a good, but incomplete, recovery with residual quadriparesis and dysphagia.
CONCLUSIONS:
Arterial cerebral infarcts are rare, but potentially life-threatening complications of spontaneous intracranial hypotension. The strokes are due to downward displacement of the brain and can be precipitated by craniotomy for evacuation of associated subdural hematomas.
PMID: 23196352

Spontaneous intracranial hypotension: recommendations for management.
Amoozegar F, Guglielmin D, Hu W, Chan D, Becker WJ.
Can J Neurol Sci. 2013 Mar;40(2):144-57.
Abstract
A literature search found no clinical trials or guidelines addressing the management of spontaneous intracranial hypotension (SIH). Based on the available literature and expert opinion, we have developed recommendations for the diagnosis and management of SIH. For typical cases, we recommend brain magnetic resonance (MR) imaging with gadolinium to confirm the diagnosis, and conservative measures for up to two weeks. If the patient remains symptomatic, up to three non-directed lumbar epidural blood patches (EBPs) should be considered. If these are unsuccessful, non-invasive MR myelography, radionuclide cisternography, MR myelography with intrathecal gadolinium, or computed tomography with myelography should be used to localize the leak. If the leak is localized, directed EPBs should be considered, followed by fibrin sealant or neurosurgery if necessary. Clinically atypical cases with normal brain MR imaging should be investigated to localize the leak. Directed EBPs can be used if the leak is localized; non-directed EBPs should be used only if there are indirect signs of SIH.
PMID: 23419561
full text: headachenetwork.ca/wp-content/uploads/Amoozegar-et-al.pdf

Cerebrospinal fluid volume-depletion headaches in patients with traumatic brachial plexus injury.
Hébert-Blouin MN, Mokri B, Shin AY, Bishop AT, Spinner RJ.
J Neurosurg. 2013 Jan;118(1):149-54.
doi: 10.3171/2012.9.JNS112368.
Abstract
OBJECT:
Patients with brachial plexus injury (BPI) present with a combination of motor weakness/paralysis, sensory deficits, and pain. Brachial plexus injury is generally not believed to be associated with headaches. However, CSF leaks may be associated with CSF volume-depletion (low-pressure) headaches and can occur in BPI secondary to nerve root avulsion. Only a few cases of headaches associated with BPI have been reported. It is unknown if headaches in patients with BPI occur so rarely, or if they are just unrecognized by physicians and/or patients in which the focus of attention is the affected limb. The aim of this study was to determine the prevalence of CSF volume-depletion headaches in patients with BPI.
METHODS:
All adult patients presenting at the Mayo brachial plexus clinic with traumatic BPI were asked to complete a questionnaire addressing the presence and quality of headaches following their injury. The patients’ clinical, injury, and imaging characteristics were subsequently reviewed.
RESULTS:
Between December 2008 and July 2010, 145 patients completed the questionnaire. Twenty-two patients reported new onset headaches occurring after their BPI. Eight of these patients experienced positional headaches, suggestive of CSF volume depletion. One of the patients with orthostatic headaches was excluded because the headaches immediately followed a lumbar puncture for a myelogram. Six of the other 7 patients with positional headaches had a clear preganglionic BPI. The available imaging studies in these 6 patients revealed evidence of CSF leaks: pseudomeningoceles (n = 5), CSF tracking into soft tissues (n = 3), CSF tracking into the intraspinal compartment (n = 3), CSF tracking into the pleural space (n = 2), and low-positioned cerebellar tonsils (n = 2).
CONCLUSIONS:
In this retrospective study, 15.2% of patients (22 of 145 patients) with traumatic BPI suffered from a new-onset headache. Seven of these patients (4.8%) experienced postural headaches clearly suggestive of CSF volume depletion likely secondary to a CSF leak associated with the BPI, whereas the other 15 patients (10.3%) suffered headaches that may have represented a variant of CSF depletion headaches without a postural characteristic or a headache from another cause. These data suggest that CSF volume-depletion headaches occur in a significant proportion of patients with BPI and have been underrecognized and underreported.
PMID: 23082886
Full text

Ventral spinal cerebrospinal fluid leak as the cause of persistent post-dural puncture headache in children.
Schievink WI, Maya MM.
J Neurosurg Pediatr. 2013 Jan;11(1):48-51.
doi: 10.3171/2012.10.PEDS12353.
Abstract
Headache occurs after dural puncture in about 1%-25% of children who undergo the procedure-a rate similar to that seen in adults. Persistence of post-dural puncture headache in spite of bed rest, increased fluid intake, and epidural blood patch treatment, however, is rare. The authors reviewed the medical records and imaging studies of all patients 19 years of age or younger who they evaluated between 2001 and 2010 for intracranial hypotension, and they identified 8 children who had persistent post-dural puncture headache despite maximal medical treatment and placement of epidural blood patches. A CSF leak could be demonstrated radiologically and treated surgically in 3 of these patients, and the authors report these 3 cases. The patients were 2 girls (ages 14 and 16 years) who had undergone lumbar puncture for evaluation of headache and fever and 1 boy (age 13 years) who had undergone placement of a lumboperitoneal shunt using a Tuohy needle for treatment of pseudotumor cerebri. The boy also had undergone a laminectomy and exploration of the posterior dural sac, but no CSF leak could be identified. All 3 patients presented with new-onset orthostatic headaches, and in all 3 cases MRI demonstrated a large ventral lumbar or thoracolumbar CSF collection. Conventional myelography or digital subtraction myelography revealed a ventral dural defect at L2-3 requiring surgical repair. Through a posterior transdural approach, the dural defect was repaired using 6-0 Prolene sutures and a dural substitute. Postoperative recovery was uneventful, with complete resolution of orthostatic headache and of the ventral cerebrospinal fluid leak on MRI. The authors conclude that persistent postdural puncture headache requiring surgical repair is rare in children. They note that the CSF leak may be located ventrally and may require conventional or digital subtraction myelography for exact localization and that transdural repair is safe and effective in eliminating the headaches.
PMID: 23140214
Full text

Spinal manifestations of spontaneous intracranial hypotension.
Schievink WI, Chu RM, Maya MM, Johnson JP, Cohen HC.
J Neurosurg Spine. 2013 Jan;18(1):96-101.
doi: 10.3171/2012.10.SPINE12469.
Abstract
OBJECT:
The goal of the study was to elucidate the spinal manifestations of spontaneous intracranial hypotension.
METHODS:
The authors reviewed the medical records and imaging studies of 338 consecutive patients with spontaneous intracranial hypotension who were evaluated at their institution between 2001 and 2010.
RESULTS:
Twenty patients (6%; mean age 35.8 [range 16 to 60 years]; 5 males and 15 females) had convincing signs or symptoms referable to the spinal cord or spinal nerve roots. The spinal manifestations consisted of radiculopathy in 11 patients (unilateral in 8 and bilateral in 3), myelopathy in 8 patients, and bibrachial amyotrophy in 1 patient. The cervical spine was involved in 12 patients, the thoracic spine in 5, and the lumbosacral spine in 3. The spinal symptoms were positional in only 3 patients. The spinal manifestations occurred around the time of the headache onset in 16 patients, and months to years after the positional headache had resolved in 4 patients. A large extrathecal CSF collection causing compression of the spinal cord or nerve root was responsible for the spinal manifestations in the majority of patients. Treatment of the spinal CSF leak resulted in resolution of the spinal manifestations along with the headache, except for those in the patient with bibrachial amyotrophy.
CONCLUSIONS:
Spinal manifestations are uncommon in cases of spontaneous intracranial hypotension, occurring in about 6% of patients, but myelopathy and radiculopathy involving all spinal segments do occur. Unlike the headache, the spinal manifestations usually are not positional and are caused by mass effect from an extradural CSF collection.
PMID: 23121651

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2012

The role of digital subtraction myelography in the diagnosis and localization of spontaneous spinal CSF leaks.
Hoxworth JM, Trentman TL, Kotsenas AL, Thielen KR, Nelson KD, Dodick DW.
Am J Roentgenol. 2012 Sep;199(3):649-53.
doi: 10.2214/AJR.11.8238.
Abstract
OBJECTIVE:
The objective of our study was to review the clinical utility of digital subtraction myelography for the diagnosis of spinal CSF leaks in patients with spontaneous intracranial hypotension (SIH) and those with superficial siderosis.
MATERIALS AND METHODS:
Procedure logs from 2007 to 2011 were reviewed to identify cases in which digital subtraction myelography was performed to diagnose spinal CSF leaks. Electronic medical records were reviewed to obtain information regarding diagnosis and outcome. For patients to be included in the study, preprocedural spinal MRI had to show an extradural fluid collection spanning more than one vertebral level and postmyelographic CT had to confirm the presence of an active CSF leak. If digital subtraction myelography successfully showed the site of the CSF leak, the location was documented.
RESULTS:
Eleven patients (seven men and four women; mean age, 49.0 years) underwent digital subtraction myelography during the study period. Six patients had SIH and five patients had superficial siderosis. The extradural fluid collection on spinal MRI averaged a length of 15.5 vertebral levels. Digital subtraction myelography successfully showed the site of the CSF leak in nine of the 11 patients, and all of the dural tears were located in the thoracic spine between T3 and T11.
CONCLUSION:
Digital subtraction myelography is a valuable diagnostic tool for the localization of rapid spinal CSF leaks and should be considered in patients who are clinically suspected to have a dural tear that is accompanied by a
longitudinally extensive extradural fluid collection on spinal MRI.
PMID:
22915407
Full text

Intracranial hypotension producing reversible coma: a systematic review, including three new cases.
Loya JJ, Mindea SA, Yu H, Venkatasubramanian C, Chang SD, Burns TC.
J Neurosurg. 2012 Sep;117(3):615-28.
doi: 10.3171/2012.4.JNS112030.
Abstract
Intracranial hypotension is a disorder of CSF hypovolemia due to iatrogenic or spontaneous spinal CSF leakage. Rarely, positional headaches may progress to coma, with frequent misdiagnosis. The authors review reported cases of verified intracranial hypotension-associated coma, including 3 previously unpublished cases, totaling 29. Most patients presented with headache prior to neurological deterioration, with positional symptoms elicited in almost half. Eight patients had recently undergone a spinal procedure such as lumbar drainage. Diagnostic workup almost always began with a head CT scan. Subdural collections were present in 86%; however, intracranial hypotension was frequently unrecognized as the underlying cause. Twelve patients underwent one or more procedures to evacuate the collections, sometimes with transiently improved mental status. However, no patient experienced lasting neurological improvement after subdural fluid evacuation alone, and some deteriorated further. Intracranial hypotension was diagnosed in most patients via MRI studies, which were often obtained due to failure to improve after subdural hematoma (SDH) evacuation. Once the diagnosis of intracranial hypotension was made, placement of epidural blood patches was curative in 85% of patients. Twenty-seven patients (93%) experienced favorable outcomes after diagnosis and treatment; 1 patient died, and 1 patient had a morbid outcome secondary to duret hemorrhages. The literature review revealed that numerous additional patients with clinical histories consistent with intracranial hypotension but no radiological confirmation developed SDH following a spinal procedure. Several such patients experienced poor outcomes, and there were multiple deaths. To facilitate recognition of this treatable but potentially life-threatening condition, the authors propose criteria that should prompt intracranial hypotension workup in the comatose patient and present a stepwise management algorithm to guide the appropriate diagnosis and treatment of these patients.
PMID: 22725982
Full text

Supine digital subtraction myelography for the demonstration of a dorsal cerebrospinal fluid leak in a patient with spontaneous intracranial hypotension: a technical note.
Carstensen M, Chaudhary N, Leung A, Ng W.
J Radiol Case Rep. 2012 Sep;6(9):1-9.
doi: 10.3941/jrcr.v6i9.1002.
Abstract
A patient with spontaneous intracranial hypotension due to a spinal cerebrospinal fluid (CSF) leak required localization of the leakage site prior to surgical management. Conventional, computed tomography and prone digital subtraction myelography failed to localize the dural tear, which was postulated to be dorsally located. We present here a digital subtraction myelographic approach to accurately localize a dorsal site of CSF leakage by injecting iodinated contrast via a lumbar drain with the patient in the supine position.
PMID: 23378882
full text: PMC3558254

Lack of causal association between spontaneous intracranial hypotension and cranial cerebrospinal fluid leaks.
Schievink WI, Schwartz MS, Maya MM, Moser FG, Rozen TD.
J Neurosurg. 2012 Apr;116(4):749-54.
doi: 10.3171/2011.12.JNS111474.
Abstract
OBJECT:
Spontaneous intracranial hypotension is an important cause of headaches and an underlying spinal CSF leak can be demonstrated in most patients. Whether CSF leaks at the level of the skull base can cause spontaneous intracranial hypotension remains a matter of controversy. The authors’ aim was to examine the frequency of skull base CSF leaks as the cause of spontaneous intracranial hypotension.
METHODS:
Demographic, clinical, and radiological data were collected from a consecutive group of patients evaluated for spontaneous intracranial hypotension during a 9-year period.
RESULTS:
Among 273 patients who met the diagnostic criteria for spontaneous intracranial hypotension and 42 who did not, not a single instance of CSF leak at the skull base was encountered. Clear nasal drainage was reported by 41 patients, but a diagnosis of CSF rhinorrhea could not be established. Four patients underwent exploratory surgery for presumed CSF rhinorrhea. In addition, the authors treated 3 patients who had a postoperative CSF leak at the skull base following the resection of a cerebellopontine angle tumor and developed orthostatic headaches; spinal imaging, however, demonstrated the presence of a spinal source of CSF leakage in all 3 patients.
CONCLUSIONS:
There is no evidence for an association between spontaneous intracranial hypotension and CSF leaks at the level of the skull base. Moreover, the authors’ study suggests that a spinal source for CSF leakage should even be suspected in patients with orthostatic headaches who have a documented skull base CSF leak.
PMID: 22264184
full text: thejns.org/doi/pdf/10.3171/2011.12.JNS111474

When should I do dynamic CT myelography? Predicting fast spinal CSF leaks in patients with spontaneous intracranial hypotension.
Luetmer PH, Schwartz KM, Eckel LJ, Hunt CH, Carter RE, Diehn FE.
AJNR Am J Neuroradiol. 2012 Apr;33(4):690-4.
doi: 10.3174/ajnr.A2849.
Abstract
BACKGROUND AND PURPOSE:
Some patients with SIH have fast CSF leaks requiring dynamic CTM for localization; however, patients generally undergo conventional CTM before a dynamic study. Our aim was to determine whether findings on head MR imaging, spine MR imaging, or opening pressure measurements can predict fast spinal CSF leaks.
MATERIALS AND METHODS:
A retrospective review was performed on 151 consecutive patients referred for CTM to evaluate for spinal CSF leak. Head MR imaging was evaluated for diffuse dural enhancement and “brain sag,” and spine MR imaging for presence of an extradural fluid collection. The opening pressure was recorded. The CTM was scored as no leak, slow leak localized on conventional CTM, or fast leak that required dynamic CTM.
RESULTS:
Fast CSF leaks were identified in 32 (21%), slow leaks in 36 (24%), and no leak in 83 (55%) of 151 patients on initial CTM. There was significant association between spinal extra-arachnoid fluid on MR imaging and the presence of a fast leak (sensitivity 85%, specificity 79%, P < .0001). There was not significant association between fast leak and findings on head MR imaging (P = .27) or opening pressure (P = .30).
CONCLUSIONS:
If all patients with spinal extra-arachnoid CSF on MR imaging had been sent directly to dynamic CTM, repeat myelography would have been avoided in most patients with fast leaks (23 of 27; 85%). However, a minority of patients with slow or no leaks would have been converted from conventional to dynamic CTM (16 of 77; 21%). Spinal MR imaging is helpful in premyelographic evaluation of SIH.
PMID: 22194380
full text: www.ajnr.org/content/33/4/690.long

The role of MR myelography with intrathecal gadolinium in localization of spinal CSF leaks in patients with spontaneous intracranial hypotension.
Akbar JJ, Luetmer PH, Schwartz KM, Hunt CH, Diehn FE, Eckel LJ.
AJNR Am J Neuroradiol. 2012 Mar;33(3):535-40.
doi: 10.3174/ajnr.A2815.
Abstract
BACKGROUND AND PURPOSE:
Localization of spinal CSF leaks in CSF hypovolemia is critical in directing focal therapy. In this retrospective review, our aim was to determine whether GdM was helpful in confirming and localizing spinal CSF leaks in patients in whom no leak was identified on a prior CTM.
MATERIALS AND METHODS:
Forty-one symptomatic patients with clinical suspicion of SIH were referred for GdM after undergoing at least 1 CTM between February 2002 and August 2010. A retrospective review of the imaging and electronic medical records was performed on each patient.
RESULTS:
In 17 of the 41 patients (41%), GdM was performed for follow-up of a previously documented leak at CTM. In the remaining 24 patients (59%), in whom GdM was performed for a suspected CSF leak, which was not identified on CTM, GdM localized the CSF leak in 5 of 24 patients (21%). In 1 of these 5 patients, GdM detected the site of leak despite negative findings on brain MR imaging, spine MR imaging, and CTM of the entire spine. Sixteen of 17 patients with previously identified leaks underwent interval treatment, and leaks were again identified in 12 of 17 (71%).
CONCLUSIONS:
GdM is a useful technique in the highly select group of patients who have debilitating symptoms of SIH, a high clinical index of suspicion of spinal CSF leak, and no demonstrated leak on conventional CTM. Intrathecal injection of gadolinium contrast remains an off-label use and should be reserved for those patients who fail conventional CTM.
PMID: 22173753
full text: www.ajnr.org/content/33/3/535.long

Second-half-of-the-day headache as a manifestation of spontaneous CSF leak.
Leep Hunderfund AN, Mokri B.
J Neurol. 2012 Feb;259(2):306-10.
doi: 10.1007/s00415-011-6181-z.
Abstract
Orthostatic headache related to spontaneous cerebrospinal fluid leak (CSF) appears within 2 h of sitting or standing in most patients. However, longer delays to headache onset have been observed, including some patients who have headaches only in the afternoon. The objective of this study is to describe second-half-of-the-day headache as a manifestation of spontaneous CSF leak and propose potential mechanisms. From 142 patients evaluated by one of us (B.M.) during a 10-year period for spontaneous intracranial hypotension, those describing headache occurring exclusively in the afternoon accompanied by typical changes of intracranial hypotension on head MRI were retrospectively identified and their medical records reviewed. Five patients met our pre-defined inclusion criteria (5/142, 3.5%; three women; mean age 50 years). Second-half-of-the-day headache was an initial symptom of intracranial hypotension in one patient, spontaneously evolved from prior all-day orthostatic headache in one patient, and was a residual or recurrent symptom after epidural blood patch in three patients. Head MRI changes due to intracranial hypotension were decreased during second-half-of-the-day-headache compared to typical all-day orthostatic headache in three out of four patients. The timing of second-half-of-the-day headache and orthostatic headache in the clinical course of patients with spontaneous CSF leaks and related MRI findings suggest that second-half-of-the-day headache is likely a manifestation of a slowed or slow-flow CSF leak.
PMID: 21811806

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2011

** Diagnostic criteria
Diagnostic criteria for headache due to spontaneous intracranial hypotension: a perspective.
Schievink WI, Dodick DW, Mokri B, Silberstein S, Bousser MG, Goadsby PJ.
Headache. 2011 Oct;51(9):1442-4.
doi: 10.1111/j.1526-4610.2011.01911.x.
Abstract
The clinical and radiographic manifestations of spontaneous intracranial hypotension are highly variable and many patients do not satisfy the 2004 International Classification of Headache Disorders criteria. We developed new diagnostic criteria for spontaneous intracranial hypotension based on cases we have seen reflecting the variable manifestations of the disorder. These criteria provide a basis for change when the classification criteria are next revised. The diagnostic criteria consist of A, orthostatic headache; B, the presence of at least one of the following: low opening pressure (≤ 60 mm H(2) O), sustained improvement of symptoms after epidural blood patching, demonstration of an active spinal cerebrospinal fluid leak, cranial magnetic resonance imaging changes of intracranial hypotension (eg, brain sagging or pachymeningeal enhancement); C, no recent history of dural puncture; and D, not attributable to another disorder.
PMID:
21658029

Chronic cerebellar hemorrhage in spontaneous intracranial hypotension: association with ventral spinal cerebrospinal fluid leaks: clinical article.
Schievink WI, Maya MM, Nuño M.
J Neurosurg Spine. 2011 Oct;15(4):433-40.
doi: 10.3171/2011.5.SPINE10890.
Abstract
OBJECT:
Spontaneous intracranial hypotension is an important cause of new-onset daily persistent headache. Cerebellar hemorrhage has been identified as a possible feature of spontaneous intracranial hypotension. The authors reviewed the MR imaging studies from a group of patients with spontaneous intracranial hypotension to assess the presence of cerebellar hemorrhage.
METHODS:
Medical records and radiological images were reviewed in 262 cases involving patients with spontaneous intracranial hypotension who had undergone MR imaging of the brain as well as spinal imaging.
RESULTS:
Chronic cerebellar hemorrhages were found in 7 (2.7%) of the 262 patients with spontaneous intracranial hypotension. These hemorrhages were found in 7 (19.4%) of the 36 patients with a ventral spinal CSF leak and in none of the 226 patients who did not have such a CSF leak (p < 0.0001). The degree of hemosiderin deposits was variable, ranging from mild involvement of the cerebellar folia to widespread superficial siderosis. Only the 1 patient with superficial siderosis had symptoms due to the hemorrhages. The time period between the onset of symptoms due to spontaneous intracranial hypotension and MR imaging examination was significantly longer in those patients with cerebellar hemorrhage than in those with a ventral spinal CSF leak and no evidence for cerebellar hemorrhage (mean 19.6 years vs 2.3 months, p < 0.0001).
CONCLUSIONS:
Chronic cerebellar hemorrhage should be included among the manifestations of spontaneous intracranial hypotension. The severity is variable, but the hemorrhage generally is asymptomatic. The underlying spinal CSF leak is ventral and mostly of long duration.
PMID: 21740128
Full text

Frequency of intracranial aneurysms in patients with spontaneous intracranial hypotension.
Schievink WI, Maya MM.
J Neurosurg. 2011 Jul;115(1):113-5.
doi: 10.3171/2011.2.JNS101805.
Abstract
OBJECT:
Spontaneous intracranial hypotension (SIH) is a significant cause of new-onset daily persistent headache. A generalized connective tissue disorder also involving the intracranial arteries has been suspected in the population with SIH. Therefore, the authors reviewed angiographic studies for the presence of intracranial aneurysms in a group of patients with SIH.
METHODS:
Magnetic resonance angiography studies of the brain were performed in 93 patients with SIH (mean age 43 years, range 14-86 years) and in 291 controls (mean age 56 years, range 28-78 years).
RESULTS:
Intracranial aneurysms were detected in 8 (8.6%) of the 93 patients with SIH (95% CI 2.9%-14.3%). This incidence was higher than in the control population (3 (1.0%) of 291 (95% CI 0%-2.2%; p = 0.0007). In 7 patients the aneurysms were incidental, and in 1 patient SIH developed 5 weeks after an aneurysmal subarachnoid hemorrhage.
CONCLUSIONS:
In this retrospective case-control study, the frequency of intracranial aneurysms among patients with SIH was significantly higher than in the control population.
PMID: 21395391
full text: thejns.org/doi/full/10.3171/2011.2.JNS101805

Connective tissue disorders in patients with spontaneous intracranial hypotension.
Liu FC, Fuh JL, Wang YF, Wang SJ.
Cephalalgia. 2011 Apr;31(6):691-5.
doi: 10.1177/0333102410394676.
Abstract
OBJECTIVE:
Spontaneous intracranial hypotension (SIH) is caused by spinal cerebrospinal fluid (CSF) leakage. An underlying connective tissue disorder has been hypothesized to cause dural weakness and predisposition to CSF leak. We conducted a case-controlled study to investigate the role of connective tissue disorders in SIH patients.
METHODS:
We recruited 55 consecutive SIH patients (38 F, 17 M; mean age, 40.8 ± 9.8 years) and 55 age- and sex-matched control individuals (mean age, 38.0 ± 8.9 years) for this study. The connective tissue disorders were evaluated by: (i) Beighton hypermobility scores and revised diagnostic criteria for benign joint hypermobility syndrome; (ii) skin and skeletal manifestations of Ehlers-Danlos syndrome (EDS); and (iii) skeletal features of Marfan syndrome.
RESULTS:
The frequencies of joint hypermobility according to Beighton scores >4/9 (SIH 23.6% vs controls 16.4%, P = 0.48) and revised benign joint hypermobility syndrome criteria (SIH 23.6% vs controls 34.5%, P = 0.29) did not differ between SIH patients and controls. Sixteen patients and 16 controls had one or more skin features of EDS (P = 1.0). Nine SIH patients (16.4%) demonstrated the skeletal features of Marfan syndrome; this frequency did not differ from that of the control group (9.1%; P = 0.262). Only dolichostenomelia (disproportionately long limbs) was more prominent in SIH patients than in controls (34.5% vs 9.1%; P = 0.002).
CONCLUSION:
Compared with Western studies, the frequencies of connective tissue disorders were higher in our SIH patients. However, these frequencies did not differ between SIH patients and control individuals, except for dolichostenomelia.
PMID: 21220378

Frontotemporal brain sagging syndrome: an SIH-like presentation mimicking FTD.
Wicklund MR, Mokri B, Drubach DA, Boeve BF, Parisi JE, Josephs KA.
Neurology. 2011 Apr 19;76(16):1377-82.
doi: 10.1212/WNL.0b013e3182166e42.
Abstract
BACKGROUND:
Behavioral variant frontotemporal dementia (bvFTD) is a relatively well-defined clinical syndrome. It is associated with frontal and temporal lobe structural/metabolic changes and pathologic findings of a neurodegenerative disease. We have been evaluating patients with clinical and imaging features partially consistent with bvFTD but with evidence also suggestive of brain sagging, which we refer to as frontotemporal brain sagging syndrome (FBSS).
METHODS:
Retrospective medical chart review to identify all patients seen at our institution between 1996 and 2010, who had a clinical diagnosis of FTD and imaging evidence of brain sag.
RESULTS:
Eight patients, 7 male and 1 female, were diagnosed with FBSS. The median age at symptom onset was 53 years. All patients had insidious onset and slow progression of behavioral and cognitive dysfunction accompanied by daytime somnolence and headache. Of the 5 patients with functional imaging, all showed evidence of hypometabolism of the frontotemporal regions. On brain MRI, all patients had evidence of brain sagging with distortion of the brainstem; 3 patients had diffuse pachymeningeal enhancement. CSF opening pressure was varied and CSF protein was mildly elevated. A definite site of CSF leak was not identified by myelogram or cisternography, except in one patient with a site highly suggestive of leak who subsequently underwent surgery confirming a CSF leak. In 2 patients with a neuropathologic examination, there was no evidence of a neurodegenerative disease.
CONCLUSIONS:
This case series demonstrates that FBSS may mimic typical bvFTD but should be recognized as an unusual presentation that is potentially treatable.
PMID: 21502595
Full text: PMC3087405

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2010

Spontaneous spinal cerebrospinal fluid leaks as the cause of subdural hematomas in elderly patients on anticoagulation.
Schievink WI, Maya MM, Pikul BK, Louy C.
J Neurosurg. 2010 Feb;112(2):295-9.
doi: 10.3171/2008.10.JNS08428.
Abstract
Subdural hematoma is a relatively common complication of long-term anticoagulation, particularly in the elderly. The combination of anticoagulation and cerebral cortical atrophy is believed to be sufficient to explain the subdural bleeding. The authors report a series of elderly patients who were on a regimen of anticoagulation and developed chronic subdural hematomas (SDHs) due to a spontaneous spinal CSF leak. They reviewed the medical records and imaging studies of a consecutive group of patients with spontaneous intracranial hypotension who were evaluated at Cedars-Sinai Medical Center. Among 141 patients with spontaneous spinal CSF leaks and spontaneous intracranial hypotension, 3 (2%) were taking anticoagulants at the time of onset of symptoms. The mean age of the 3 patients (1 woman and 2 men) was 74 years (range 68-86 years). All 3 patients had chronic SDHs measuring between 12 and 23 mm in maximal diameter. The SDHs resolved after treatment of the underlying spontaneous spinal CSF leak, and there was no need for hematoma evacuation. Epidural blood patches were used in 2 patients, and percutaneous placement of a fibrin sealant was used in 1 patient. The presence of an underlying spontaneous spinal CSF leak should be considered in patients with chronic SDHs, even among the elderly taking anticoagulants.
PMID:
19199465

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2009

Heavily T2-weighted MR myelography vs CT myelography in spontaneous intracranial hypotension.
Wang YF, Lirng JF, Fuh JL, Hseu SS, Wang SJ.
Neurology. 2009 Dec 1;73(22):1892-8.
doi: 10.1212/WNL.0b013e3181c3fd99.
Abstract
OBJECTIVE:
To assess the diagnostic accuracy of heavily T2-weighted magnetic resonance myelography (MRM) in patients with spontaneous intracranial hypotension (SIH).
METHODS:
Patients with SIH were recruited prospectively, and first underwent MRM and then computed tomographic myelography (CTM). The results of MRM were validated with the gold standard, CTM, focusing on 1) CSF leaks along the nerve roots, 2) epidural CSF collections, and 3) high-cervical (C1-3) retrospinal CSF collections. Comparisons of these 3 findings between the 2 studies were made by kappa statistics and agreement rates. Targeted epidural blood patches (EBPs) were placed at the levels of CSF leaks if supportive treatment failed.
RESULTS:
Nineteen patients (6 men and 13 women, mean age 37.9 +/- 8.6 years) with SIH completed the study. MRM did not differ from CTM in the detection rates of CSF leaks along the nerve roots (84% vs 74%, p = 0.23), high-cervical retrospinal CSF collections (32% vs 16%, p = 0.13), and epidural CSF collections (89% vs 79%, p = 0.20). MRM demonstrated more spinal levels of CSF leaks (2.2 +/- 1.7 vs 1.5 +/- 1.5, p = 0.011) and epidural collections (12.2 +/- 5.9 vs 7.1 +/- 5.8, p < 0.001) than CTM. The overall level-by-level concordance was substantial for CSF leaks along the nerve roots (C1-L3) (kappa = 0.71, p < 0.001, agreement = 95%) and high-cervical retrospinal CSF collections (C1-3) (kappa = 0.73, p < 0.001, agreement = 92%), and moderate for epidural CSF collections (C1-L3) (kappa = 0.47, p < 0.001, agreement = 72%). Ten of the 14 patients (71%) receiving targeted EBPs experienced sustained symptomatic relief after a single attempt.
CONCLUSIONS:
Heavily T2-weighted magnetic resonance myelography was accurate in localizing CSF leaks for patients with spontaneous intracranial hypotension. This noninvasive technique may be an alternative to computed tomographic myelography before targeted epidural blood patches.
PMID:
19949036

A novel technique for treatment of intractable spontaneous intracranial hypotension: lumbar dural reduction surgery.
Schievink WI.
Headache. 2009 Jul;49(7):1047-51.
doi: 10.1111/j.1526-4610.2009.01450.x.
Abstract
BACKGROUND AND OBJECTIVE:
Spontaneous intracranial hypotension has become a well-described cause of headache particularly among young and middle-aged individuals. Treatment of the underlying spinal cerebrospinal fluid (CSF) leak is effective in relieving symptoms in the vast majority of patients but symptoms may become refractory. The author describes a novel surgical technique to treat intractable spontaneous intracranial hypotension.
METHODS:
A lumbar laminectomy is performed, a strip of dura is resected, and the dural defect is closed. The resulting decrease in lumbar CSF volume is believed to increase intracranial CSF volume and pressure.
RESULTS:
The technique was utilized in a patient who suffered with intractable positional headaches because of a spinal CSF leak for 6 years in spite of numerous surgical and nonsurgical therapies. Significant improvement of symptoms was sustained during a 1-year period of follow-up.
CONCLUSION:
Dural reduction surgery may be considered in carefully selected patients with intracranial hypotension.
PMID: 19473279

Clinical features and outcomes in spontaneous intracranial hypotension: a survey of 90 consecutive patients.
Mea E, Chiapparini L, Savoiardo M, Franzini A, Bussone G, Leone M.
Neurol Sci. 2009 May;30 Suppl 1:S11-3.
doi: 10.1007/s10072-009-0060-8.
Abstract
Spontaneous intracranial hypotension (SIH) is a rare disabling condition whose main clinical manifestation is orthostatic headache. We analysed clinical characteristics in relation to time to resolution in 90 consecutive patients diagnosed with SIH at our centre between 1993 and 2006. After excluding 7 patients lost to follow-up, the remaining 83 cases were divided into four groups: Group A (53 cases) with progressively worsening orthostatic headache; Group B (3 cases) with severe acute-onset orthostatic headache; Group C (9 cases) with fluctuating non-continuous headache, of mild severity, that, in 33% of cases, did not worsen on standing; Group D (18 cases), 5 with a previous history of headache, 14 with orthostatic headache, and 10 with altered neurological examination. Complete symptoms and neuroradiological resolution occurred during follow-up in Groups A, B and D, but was longer in Group D probably in relation to more severe clinical picture with altered neurological examination. However, after a mean of 52 months (range 24-108), none of the nine Group C patients had MRI indicating complete resolution. The main characteristic of Group C related to incomplete resolution was delayed diagnosis. These preliminary findings suggest that early diagnosis of SIH correlates with better outcome, further suggesting that patients with a new headache that may worsen on standing or sitting should undergo MRI with contrast to expedite a possible SIH diagnosis, even if the pain is relatively mild.
PMID: 19415418

Diagnostic value of spinal MR imaging in spontaneous intracranial hypotension syndrome.
Watanabe A, Horikoshi T, Uchida M, Koizumi H, Yagishita T, Kinouchi H.
Am J Neuroradiol. 2009 Jan;30(1):147-51.
doi: 10.3174/ajnr.A1277. Epub 2008 Sep 3.
Abstract
BACKGROUND AND PURPOSE:
Spontaneous intracranial hypotension (SIH) presents with orthostatic headache, and the diagnosis is made on the basis of low CSF pressure and brain MR imaging findings characteristic of the disorder. However, a broad spectrum of symptoms and MR imaging findings of SIH is recognized, and some cases have no typical MR imaging abnormalities. SIH is believed to be caused by CSF leakage from the spinal dural sac, whereas the usefulness of MR imaging of the spine remains unclear. Our aim was to elucidate the diagnostic value of brain and spinal MR imaging
MATERIALS AND METHODS:
The sensitivities for the detection of SIH were retrospectively evaluated in 18 patients with SIH treated in our institutions between January 1998 and August 2007.
RESULTS:
Brain MR imaging detected abnormalities in 15 of the 18 patients (83%): diffuse pachymeningeal enhancement in 15 (83%), descent of the cerebellar tonsil in 13 (72%), brain stem sagging in 13 (72%), enlargement of the pituitary gland in 12 (67%), and subdural fluid collection in 13 (72%). Spinal MR imaging detected abnormalities in 17 of the 18 patients (94%): distention of the epidural veins in 14 (78%), epidural fluid collection on fat-saturated T2-weighted images in 16 (89%), and abnormal visualization of the nerve root sleeve in only 1 (6%). The sensitivity for SIH was 83% for brain MR imaging and 94% for spinal MR imaging.
CONCLUSIONS:
Spinal MR imaging is useful for the diagnosis of SIH, especially in the early stage.
PMID: 18768717
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2008

** good review article
Spontaneous spinal cerebrospinal fluid leaks.
Schievink WI.
Cephalalgia. 2008 Dec;28(12):1345-56.
doi: 10.1111/j.1468-2982.2008.01776.x.
Abstract
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.
PMID:
19037970

Cerebral venous thrombosis in spontaneous intracranial hypotension.
Schievink WI, Maya MM.
Headache. 2008 Nov-Dec;48(10):1511-9.
doi: 10.1111/j.1526-4610.2008.01251.x.
Abstract
BACKGROUND AND OBJECTIVE:
The occurrence of cerebral venous thrombosis has been reported among patients with spontaneous intracranial hypotension, but a causal relationship has not been clearly established. We reviewed our experience with spontaneous intracranial hypotension and cerebral venous thrombosis and we reviewed the relevant literature to evaluate the relationship between these 2 entities.
METHODS:
We reviewed the medical records and imaging studies of a consecutive group of patients with spontaneous intracranial hypotension evaluated at a tertiary care center between 1/1/2001 and 12/31/2007. The main search strategy was a systemic review of journal articles in MEDLINE (1966 to January 2008).
RESULTS:
Among 141 patients with spontaneous intracranial hypotension, 3 (2.1%) were also diagnosed with cerebral venous thrombosis. Among these 3 patients and the 17 reported in the literature there were 11 men and 9 women with a mean age of 39.5 years. Radiographic or clinical evidence for spontaneous intracranial hypotension preceding cerebral venous thrombosis was found in most patients, while there was no evidence for cerebral venous thrombosis preceding spontaneous intracranial hypotension in any patient. Eight (40%) of the 20 patients were found to have a change in their headache pattern believed to be due to the development of cerebral venous thrombosis. Complications of cerebral venous thrombosis, eg, cerebral venous infarction, occurred in 8 patients (40%).
CONCLUSIONS:
Spontaneous intracranial hypotension is a risk factor for cerebral venous thrombosis, but cerebral venous thrombosis is found in only about 2% of patients with spontaneous intracranial hypotension. A change in headache pattern is not a reliable predictor of the development of cerebral venous thrombosis in patients with spontaneous intracranial hypotension.
PMID: 19076649

Diagnostic criteria for spontaneous spinal CSF leaks and intracranial hypotension.
Schievink WI, Maya MM, Louy C, Moser FG, Tourje J.
AJNR Am J Neuroradiol. 2008 May;29(5):853-6.
doi: 10.3174/ajnr.A0956.
Abstract
BACKGROUND AND PURPOSE:
Comprehensive diagnostic criteria encompassing the varied clinical and radiographic manifestations of spontaneous intracranial hypotension are not available. Therefore, we propose a new set of diagnostic criteria.
MATERIALS AND METHODS:
The diagnostic criteria are based on results of brain and spine imaging, clinical manifestations, results of lumbar puncture, and response to epidural blood patching. The diagnostic criteria include criterion A, the demonstration of extrathecal CSF on spinal imaging. If criterion A is not met, criterion B, which is cranial MR imaging findings of spontaneous intracranial hypotension, follows, with at least one of the following: 1) low opening pressure, 2) spinal meningeal diverticulum, or 3) improvement of symptoms after epidural blood patch. If criteria A and B are not met, there is criterion C, 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, and 3) improvement of symptoms after epidural blood patch. These criteria were applied to a group of 107 consecutive patients evaluated for spontaneous spinal CSF leaks and intracranial hypotension.
RESULTS:
The diagnosis was confirmed in 94 patients, with use of criterion A in 78 patients, criterion B in 11 patients, and criterion C in 5 patients.
CONCLUSIONS:
A new diagnostic scheme is presented reflecting the wide spectrum of clinical and radiographic manifestations of spontaneous spinal CSF leaks and intracranial hypotension.
PMID: 18258706
full text: www.ajnr.org/content/29/5/853.long

The timing of MRI determines the presence or absence of diffuse pachymeningeal enhancement in patients with spontaneous intracranial hypotension.
Fuh JL, Wang SJ, Lai TH, Hseu SS.
Cephalalgia. 2008 Apr;28(4):318-22.
doi: 10.1111/j.1468-2982.2007.01498.x.
Abstract
The timing and clinical relevance of diffuse pachymeningeal enhancement (DPE) in the magnetic resonance imaging (MRI) examination of patients with spontaneous intracranial hypotension (SIH) remain undetermined. We reviewed 53 consecutive SIH patients (30 F/23 M, mean age of onset 41.7 +/- 11.3 years) in a tertiary hospital. Thirteen (24.5%) patients did not have DPE on their initial cranial MRIs. They had significantly shorter latency between the time of MRI examinations and the time of headache onset compared with those with DPE (6.5 +/- 4.4 vs. 20.4 +/- 16.3 days, t-test, P < 0.001). Eight of these 13 patients received a follow-up MRI (mean duration 30.3 +/- 16.6 days, range 6-59 days) and six of them revealed DPE. Among patients with DPE, the enhancement disappeared as early as 25 days after headache onset. The outcome did not differ between patients with and without DPE. The presence of DPE was associated with the timing of the MRI examination.
PMID: 18284422

Absence of TGFBR2 mutations in patients with spontaneous spinal CSF leaks and intracranial hypotension.
Schievink WI, Gordon OK, Hyland JC, Ala-Kokko L.
J Headache Pain. 2008 Apr;9(2):99-102.
doi: 10.1007/s10194-008-0017-y.
Abstract
A heritable connective-tissue-disorder often is suspected in patients with spontaneous spinal CSF leaks and intracranial hypotension, but the nature of the disorder remains unknown in most patients. The aim of this study was to assess the gene encoding TGF-beta receptor-2 (TGFBR2) as a candidate gene for spinal CSF leaks. We searched the TGFBR2 gene for mutations in eight patients with spontaneous spinal CSF leaks who also had other features associated with TGFBR2 mutations, i.e., skeletal features of Marfan syndrome, arterial tortuosity, and(or) thoracic aortic aneurysm. The mean age of these 7 women and 1 man was 38 years (range 14-60 years). We detected no TGFBR2 mutations and conclude that TGFBR2 mutations are not a major factor in spontaneous spinal CSF leaks.
PMID: 18264665
full text: PMC3476180

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2007

Frequency of spontaneous intracranial hypotension in the emergency department.
Schievink WI, Maya MM, Moser F, Tourje J, Torbati S.
J Headache Pain. 2007 Dec;8(6):325-8.
Abstract
Spontaneous intracranial hypotension is considered a rare disorder. We conducted a study on the frequency of spontaneous intracranial hypotension in the emergency department (ED). We identified patients with spontaneous intracranial hypotension evaluated in the ED of a large urban hospital between 1 January 2003 and 31 December 2006. For comparison, we also identified all patients with spontaneous subarachnoid haemorrhage (SAH). Eleven patients with previously undiagnosed spontaneous intracranial hypotension were evaluated in the ED during the four-year time period. All patients presented with positional headaches and the duration of symptoms varied from one day to three months. None of the patients were correctly diagnosed with spontaneous intracranial hypotension in the ED. During the same time period, 23 patients with aneurysmal SAH were evaluated. Spontaneous intracranial hypotension is more common than previously appreciated and the diagnosis in the ED remains problematic.
PMID:
18071632
full text: PMC3476164

Precipitating factors of spontaneous spinal CSF leaks and intracranial hypotension.
Schievink WI, Louy C.
Neurology. 2007 Aug 14;69(7):700-2.
PMID: 17698794

Heavily T2-weighted MR myelography in patients with spontaneous intracranial hypotension: a case-control study.
Tsai PH, Fuh JL, Lirng JF, Wang SJ.
Cephalalgia. 2007 Aug;27(8):929-34.
Abstract
We performed whole-spine heavily T2-weighted magnetic resonance (MR) myelography using a single-shot fast spin-echo pulse sequence in 17 patients (8 M/9 F) with spontaneous intracranial hypotension (SIH) to detect abnormal cerebrospinal fluid (CSF) collections. In addition, a group of age- and sex-matched controls were recruited. Follow-up MR myelography was also done at 3 weeks. MR myelography showed three kinds of abnormal CSF collections in 15 patients with SIH (88%): epidural fluid collection (n = 15, 88%), C1-2 extraspinal collections (n = 6, 35%) and CSF collections along nerve roots in the lower cervical or upper thoracic spines (n = 6, 35%). One patient (6%) showed a meningeal diverticulum. In
contrast, none of the controls showed these findings. Overall, MR myelography results helped in early diagnosis of SIH in four (24%) patients whose initial brain MRIs failed to show typical SIH findings. Follow-up MR myelography results were compatible with the clinical changes with kappa statistics of 0.52 and an agreement rate of 76%. Our study showed heavily T2-weighted MR myelography provided a rapid, non-invasive and high yield method to diagnose and follow-up patients with SIH. Whether the CSF collections along the nerve roots represent the ongoing leakage sites warrants further study.
PMID: 17645756

Subdural haematoma in patients with spontaneous intracranial hypotension.
Lai TH, Fuh JL, Lirng JF, Tsai PH, Wang SJ.
Cephalalgia. 2007 Feb;27(2):133-8.
Abstract
The incidence and clinical relevance of subdural haematoma (SDH) in patients with spontaneous intracranial hypotension (SIH) remain undetermined. We reviewed 40 consecutive SIH patients (18 female, 22 male) in a tertiary hospital. Eight (20%) of them had SDH and nine (23%), non-haemorrhagic subdural collections. The presence of SDH was associated with higher frequencies of male gender, recurrence of severe headache and neurological deficits. Outcomes were satisfactory after supportive care or epidural blood patches except for one SDH patient, who developed transtentorial herniation resulting in Duret haemorrhage and infarctions of bilateral posterior cerebral artery territories. In conclusion, subdural fluid collections were common in patients with SIH. SDH was associated with headache worsening or neurological deficits. Patients with SDH generally recovered well; however, serious sequela might occur.
PMID: 17257233

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2006

*** best review article
Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension.
Schievink WI.
JAMA. 2006 May 17;295(19):2286-96.
Abstract
CONTEXT:
Spontaneous intracranial hypotension is caused by spontaneous spinal cerebrospinal fluid (CSF) leaks and is known for causing orthostatic headaches. It is an important cause of new headaches in young and middle-aged individuals, but initial misdiagnosis is common.
OBJECTIVE:
To summarize existing evidence regarding the epidemiology, pathophysiology, diagnosis, and management of spontaneous spinal CSF leaks and intracranial hypotension.
EVIDENCE ACQUISITION:
MEDLINE (1966-2005) and OLDMEDLINE (1950-1965) were searched using the terms intracranial hypotension, CSF leak, low pressure headache, and CSF hypovolemia. Reference lists of these articles and ongoing investigations in this area were used as well.
EVIDENCE SYNTHESIS:
Spontaneous intracranial hypotension is caused by single or multiple spinal CSF leaks. The incidence has been estimated at 5 per 100,000 per year, with a peak around age 40 years. Women are affected more commonly than men. Mechanical factors combine with an underlying connective tissue disorder to cause the CSF leaks. An orthostatic headache is the prototypical manifestation but other headache patterns occur as well, and associated symptoms are common. Typical magnetic resonance imaging findings include subdural fluid collections, enhancement of the pachymeninges, engorgement of venous structures, pituitary hyperemia, and sagging of the brain (mnemonic: SEEPS). Myelography is the study of choice to identify the spinal CSF leak. Treatments include bed rest, epidural blood patching, percutaneous placement of fibrin sealant, and surgical CSF leak repair, but outcomes have been poorly studied and no management strategies have been studied in properly controlled randomized trials.
CONCLUSIONS:
Spontaneous intracranial hypotension is not rare but it remains underdiagnosed. The spectrum of clinical and radiographic manifestations is varied, with diagnosis largely based on clinical suspicion, cranial magnetic resonance imaging, and myelography. Numerous treatment options are available, but much remains to be learned about this disorder.
PMID:
16705110
full text: jama.jamanetwork.com/article.aspx?articleid=202849

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2005

Spectrum of subdural fluid collections in spontaneous intracranial hypotension.
Schievink WI, Maya MM, Moser FG, Tourje J.
J Neurosurg. 2005 Oct;103(4):608-13.
Abstract
OBJECT:
Spontaneous intracranial hypotension is a noteworthy but commonly misdiagnosed cause of new daily persistent headaches. Subdural fluid collections are frequent radiographic findings, but they can be interpreted as primary rather than secondary pathological entities, and uncertainties exist regarding their optimal management. The authors therefore reviewed their experience with subdural fluid collections in 40 consecutive patients with spontaneous spinal cerebrospinal fluid (CSF) leaks and intracranial hypotension.
METHODS:
The mean age of the 26 female and 14 male patients was 43 years (range 13-72 years). Subdural fluid collections were present in 20 patients (50%); 12 of these patients (60%) had subdural hygromas alone, and eight (40%) had subacute to chronic subdural hematomas (SDHs) associated with significant mass effect. The subdural hygromas resolved within several days to weeks following treatment of the underlying CSF leak. Three patients with SDHs underwent evacuation of the hematoma prior to the establishment of the diagnosis of spontaneous intracranial hypotension, but the SDHs did not resolve until the underlying spinal CSF leak was treated. In the remaining five patients, the CSF leak was treated primarily and the SDHs resolved over a 1- to 3-month period without the need for evacuation.
CONCLUSIONS:
Subdural fluid collections are common in spontaneous intracranial hypotension, varying in appearance from thin subdural hygromas to large SDHs associated with significant mass effect. These collections can be safely managed by directing treatment at the underlying CSF leak without the need for hematoma evacuation.
PMID:
16266041

Cranial MRI predicts outcome of spontaneous intracranial hypotension.
Schievink WI, Maya MM, Louy C.
Neurology. 2005 Apr 12;64(7):1282-4.
Abstract
The outcome of spontaneous intracranial hypotension has been unpredictable. The results of initial MRI were correlated to outcome of treatment in 33 patients with spontaneous intracranial hypotension. A good outcome was obtained in 25 (97%) of 26 patients with an abnormal MRI vs only 1 (14%) of 7 patients with a normal MRI (p = 0.00004). These findings show that normal initial MRI is predictive of poor outcome in spontaneous intracranial hypotension.
PMID: 15824366

Spontaneous intracranial hypotension presenting as mental deterioration.
Tsai PH, Wang SJ, Lirng JF, Fuh JL.
Headache. 2005 Jan;45(1):76-80.
Abstract
A 55-year-old woman had new onset of postural headache followed by change of mental status 3 weeks later. Magnetic resonance imaging (MRI) of the brain and whole spine showed typical spontaneous intracranial hypotension (SIH) findings, bilateral subdural hematoma, and cerebrospinal fluid leakage over the T7-T9. Her headache and mentality improved after epidural blood patches. Early recognition and correct diagnosis are crucial for successful treatment in patients with SIH presenting with mental confusion.
PMID: 15663618

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2004

Reversible coma: a rare presentation of spontaneous intracranial hypotension.
Kashmere JL, Jacka MJ, Emery D, Gross DW.
Can J Neurol Sci. 2004 Nov;31(4):565-8.
Abstract
BACKGROUND:
Spontaneous intracranial hypotension (SIH) is a well-recognized neurologic disorder that typically presents with orthostatic headaches, low cerebral spinal fluid pressures and distinct abnormalities on magnetic resonance imaging.
METHODS:
We present a case of a rare presentation of SIH.
RESULTS:
A 49-year-old man presented with a two week history of orthostatic headaches that rapidly progressed to encephalopathy and coma, requiring intubation. Neuroimaging revealed abnormalities typical of SIH; diffusely enhancing pachymeninges, subdural fluid collections, and descent of the brain. Treatment with an epidural blood patch reversed his coma within minutes. Following a second blood patch, the patient became asymptomatic. No cerebral spinal leak could be identified on magnetic resonance imaging or on a nuclear medicine technetium cerebral spinal fluid flow study. At six month follow-up, he remained symptom free.
CONCLUSION:
The mechanism of coma in SIH is presumed to be compression of the diencephalon from downward displacement of the brain. Although it is very unusual for patients with SIH to present with coma, it is important to recognize since the coma may be reversible with epidural blood patches.
PMID:
15595268

False localizing sign of C1-2 cerebrospinal fluid leak in spontaneous intracranial hypotension.
Schievink WI, Maya MM, Tourje J.
J Neurosurg. 2004 Apr;100(4):639-44.
Abstract
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.
PMID: 15070118

Connective tissue disorders with spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension: a prospective study.
Schievink WI, Gordon OK, Tourje J.
Neurosurgery. 2004 Jan;54(1):65-70; discussion 70-1.
Abstract
OBJECTIVE:
Intracranial hypotension attributable to a spontaneous spinal cerebrospinal fluid (CSF) leak is an increasingly recognized cause of postural headaches. The cause of these leaks is poorly understood, but it is likely multifactorial and may involve a primary connective tissue disorder. We undertook a study to estimate the contribution of systemic connective tissue disorders to the development of spontaneous spinal CSF leaks.
METHODS:
We examined a group of 18 consecutive patients with spontaneous spinal CSF leaks for features of a connective tissue disorder.
RESULTS:
The mean age of the 15 female patients and 3 male patients was 38 years (range, 22-55 yr). Seven patients (38%) demonstrated stigmata of a systemic connective tissue disorder, and three distinct types of disorders could be identified, as follows. 1) The association of spontaneous spinal CSF leaks and minor skeletal features of Marfan syndrome was noted for three patients. 2) Ehlers-Danlos syndrome Type II was noted for two patients. 3) Joint hypermobility associated with marked attenuation of the dorsal muscular fascia, precluding proper wound closure, was noted for two patients. In addition, isolated small-joint hypermobility was observed for five patients (28%). Slit-lamp ocular examinations, echocardiographic evaluations, histopathological examinations of skin biopsy specimens, and renal scanning did not reveal any other features of a systemic connective tissue disorder.
CONCLUSION:
Findings suggesting connective tissue disorders are common among patients with spontaneous spinal CSF leaks, and manifestations may be subtle. A variety of disorders can be identified, probably reflecting genetic heterogeneity. Problems with wound healing may occur as a result of the systemic nature of the underlying connective tissue disorder.
PMID: 14683542

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2003

Orthostatic headaches without CSF leak in postural tachycardia syndrome.
Mokri B, Low PA.
Neurology. 2003 Oct 14;61(7):980-2.
Abstract
Four women age 17 to 28 years presented with orthostatic headaches as the most prominent feature of their symptom complex. None had CSF leak or intracranial hypotension. Autonomic studies showed evidence of orthostatic intolerance with tachycardia in all cases. Treatment of orthostatic intolerance, mainly with volume expansion, was only partially effective. Orthostatic headaches are not always caused by CSF leak or supine intracranial hypotension. Occasionally they may be the major clinical manifestation of postural tachycardia syndrome or orthostatic intolerance.
PMID:
14557573

Dynamic CT myelography: a technique for localizing high-flow spinal cerebrospinal fluid leaks.
Luetmer PH, Mokri B.
AJNR Am J Neuroradiol. 2003 Sep;24(8):1711-4.
Abstract
In some patients with spontaneous spinal CSF leaks, leaks are numerous or tears are so large that extrathecal myelographic contrast material is seen at multiple levels during CT, making identification of their source impossible. This study introduces a dynamic CT myelographic technique that provides high temporal and spatial resolution. In this technical note, we describe the utility of this technique in four patients with challenging high-flow spinal CSF leaks.
PMID: 13679297
full text: www.ajnr.org/content/24/8/1711.long

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2002

Intracranial hypertension after treatment of spontaneous cerebrospinal fluid leaks.
Mokri B.
Mayo Clin Proc. 2002 Nov;77(11):1241-6.
Abstract
Four patients, aged 10 to 44 years, with spontaneous cerebrospinal fluid (CSF) leaks and intracranial hypotension developed intracranial hypertension after treatment of their CSF leaks. The leak was at the spinal level in all patients (thoracic level, 2; lumbar level, 1; and undetermined, 1). One patient responded to an epidural blood patch. Three patients responded to surgery, of whom 2 had not responded to prior epidural blood patches. Treatment resulted in complete resolution of symptoms, including orthostatic headaches and disappearance of magnetic resonance imaging abnormalities. However, all patients later developed steady headaches different from their previous headaches. None had recurrence of magnetic resonance imaging abnormalities or any evidence of occlusion of cerebral venous sinuses. All had increased CSF opening pressures. One had bilateral papilledema, and another had no venous pulsations on examination of fundi. Follow-up was possible in 2 patients. One responded well to treatment with acetazolamide, and the other improved gradually and was asymptomatic within several months.
PMID:
12440561

Spontaneous spinal cerebrospinal fluid leaks and minor skeletal features of Marfan syndrome: a microfibrillopathy.
Schrijver I, Schievink WI, Godfrey M, Meyer FB, Francke U.
J Neurosurg. 2002 Mar;96(3):483-9.
Abstract
OBJECT:
Spontaneous spinal cerebrospinal fluid (CSF) leaks are increasingly recognized as a cause of postural headaches. The authors examined a group of patients suffering from spontaneous spinal CSF leaks who also had minor skeletal features of Marfan syndrome for abnormalities of fibrillin-containing microfibrils.
METHODS:
Patients with spontaneous CSF leaks were evaluated for the clinical characteristics of connective tissue disorders. Skin biopsies were obtained in three patients with skeletal manifestations that constitute part of the Marfan syndrome phenotype. Cultured fibroblasts were studied for fibrillin-1 synthesis and incorporation into the extracellular matrix (ECM) by performing quantitative metabolic labeling and immunohistochemical analysis. Among 20 consecutive patients found to have spinal CSF leaks, four (20%) exhibited minor skeletal features of Marfan syndrome, but lacked any ocular or cardiovascular abnormalities. The mean age of these patients (30 years) was lower than that of the 16 patients without skeletal abnormalities (44 years; p = 0.01). Abnormalities in fibrillin-1 metabolism and immunostaining were detected in all three patients with the skeletal abnormalities who underwent examination, but not in a control patient without these skeletal manifestations.
CONCLUSIONS:
Twenty percent of patients who experience spontaneous spinal CSF leaks have minor skeletal features of Marfan syndrome. The authors demonstrated abnormalities in fibrillin-1 protein deposition in all patients examined, but only one person was found to have a fibrillin-1 abnormality typically found in classic Marfan syndrome. The results indicate that there is a heterogeneous involvement of other components of ECM microfibrils at the basis of this cerebrospinal manifestation. In addition, the authors identified a connective-tissue etiological factor in a group of disorders not previously classified as such.
PMID: 11883832

Spontaneous CSF leaks: underlying disorder of connective tissue.
Mokri B, Maher CO, Sencakova D.
Neurology. 2002 Mar 12;58(5):814-6.
Abstract
Of 58 consecutive patients with spontaneous CSF leaks, nine exhibited features of connective tissue disorder. One had Marfan’s syndrome. Five additional patients had hyperflexible joints, of whom four had arachnodactyly, four were tall and slender, two had hyperextensible skin, and one had a strong family history of abdominal aorta aneurysms. Retinal detachment at a young age was noted in two. One patient had bilateral carotid dissections. A dural weakness may predispose patients to spontaneous CSF leak.
PMID: 11889250