Abstracts 2011-12

This is a collection of selected publication abstracts about spinal CSF leak / intracranial hypotension from 2011-2012.
* Abstract links are included. (click on the PMID number)
* Note that links to full-text are provided for open access papers (line below PMID).


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.
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.
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.
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.
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.
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.
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.
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.
Demographic, clinical, and radiological data were collected from a consecutive group of patients evaluated for spontaneous intracranial hypotension during a 9-year period.
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.
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.
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.
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.
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).
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: 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.
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.
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.
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%).
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: 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.
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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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.
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.

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.
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.
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.
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).
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.
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.
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).
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.
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.
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.
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.
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).
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.
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).
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.
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.
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