This is a collection of selected publication abstracts about spinal CSF leak / intracranial hypotension from 2014.
* Abstract links are included. (click on the PMID number)
* Note that links to full-text are provided for open access papers (line below PMID).
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.
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.
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.
Movement disorders associated with spontaneous CSF leaks: A case series.
Cephalalgia. 2014 Dec;34(14):1134-41.
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.
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).
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.
Movement disorders are uncommon in spontaneous CSF leaks but occasionally can be one of the major components of the clinical presentation.
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.
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.
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.
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.
Headache secondary to intracranial hypotension.
Schievink WI, Deline CR.
Curr Pain Headache Rep. 2014 Nov;18(11):457.
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.
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.
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.
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.
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.
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.
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.
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.
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.
Radioisotope Cisternography in Spontaneous CSF Leaks: Interpretations and Misinterpretations.
Headache. 2014 Sep;54(8):1358-68. doi: 10.1111/head.12421. Epub 2014 Jul 4.
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.
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.
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.
CSF-venous fistula in spontaneous intracranial hypotension: demonstration by digital subtraction myelography.
Schievink W, Moser F, Maya M.
Neurology. 2014 Jul;83:472-473
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.
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.
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.
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.
Targeted epidural blood patch can result in rapid hematoma regression and good recovery in some patients with a combination of SDH and SIH.
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.
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.
Spontaneous CSF leaks: low CSF volume syndromes.
Neurol Clin. 2014 May;32(2):397-422.
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.