This is a collection of selected publication abstracts about spinal CSF leak / intracranial hypotension from 2002-2010.
* 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 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.
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.
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.
To assess the diagnostic accuracy of heavily T2-weighted magnetic resonance myelography (MRM) in patients with spontaneous intracranial hypotension (SIH).
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.
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.
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.
A novel technique for treatment of intractable spontaneous intracranial hypotension: lumbar dural reduction surgery.
Headache. 2009 Jul;49(7):1047-51.
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.
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.
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.
Dural reduction surgery may be considered in carefully selected patients with intracranial hypotension.
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.
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.
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.
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.
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.
Spinal MR imaging is useful for the diagnosis of SIH, especially in the early stage.
Spontaneous spinal cerebrospinal fluid leaks.
Cephalalgia. 2008 Dec;28(12):1345-56.
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.
Cerebral venous thrombosis in spontaneous intracranial hypotension.
Schievink WI, Maya MM.
Headache. 2008 Nov-Dec;48(10):1511-9.
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.
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).
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%).
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.
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.
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.
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.
A new diagnostic scheme is presented reflecting the wide spectrum of clinical and radiographic manifestations of spontaneous spinal CSF leaks and intracranial hypotension.
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.
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.
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.
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.
full text: PMC3476180
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.
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.
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.
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.
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.
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.
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.
Spontaneous spinal cerebrospinal fluid leaks and intracranial hypotension.
JAMA. 2006 May 17;295(19):2286-96.
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.
To summarize existing evidence regarding the epidemiology, pathophysiology, diagnosis, and management of spontaneous spinal CSF leaks and intracranial hypotension.
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.
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.
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.
full text: jama.jamanetwork.com/article.aspx?articleid=202849
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.
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.
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.
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.
Cranial MRI predicts outcome of spontaneous intracranial hypotension.
Schievink WI, Maya MM, Louy C.
Neurology. 2005 Apr 12;64(7):1282-4.
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.
Spontaneous intracranial hypotension presenting as mental deterioration.
Tsai PH, Wang SJ, Lirng JF, Fuh JL.
Headache. 2005 Jan;45(1):76-80.
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.
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.
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.
We present a case of a rare presentation of SIH.
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.
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.
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.
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.
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.
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.
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.
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.
We examined a group of 18 consecutive patients with spontaneous spinal CSF leaks for features of a connective tissue disorder.
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.
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.
Orthostatic headaches without CSF leak in postural tachycardia syndrome.
Mokri B, Low PA.
Neurology. 2003 Oct 14;61(7):980-2.
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.
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.
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.
full text: www.ajnr.org/content/24/8/1711.long
Intracranial hypertension after treatment of spontaneous cerebrospinal fluid leaks.
Mayo Clin Proc. 2002 Nov;77(11):1241-6.
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.
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.
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.
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.
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.
Spontaneous CSF leaks: underlying disorder of connective tissue.
Mokri B, Maher CO, Sencakova D.
Neurology. 2002 Mar 12;58(5):814-6.
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.