Surgical closure of spinal cerebrospinal fluid leaks improves symptoms in patients with superficial siderosis.

BACKGROUND AND PURPOSE: Spinal cerebrospinal fluid (CSF) leaks may cause a myriad of symptoms, most common being orthostatic headache. In addition, ventral spinal CSF leaks are a possible etiology of superficial siderosis (SS), a rare condition characterized by hemosiderin deposits in the central nervous system (CNS). The classical presentation of SS involves ataxia, bilateral hearing loss, and myelopathy. Unfortunately, treatment options are scarce. This study was undertaken to evaluate whether microsurgical closure of CSF leaks can prevent further clinical deterioration or improve symptoms of SS. METHODS: This cohort study was conducted using data from a prospectively maintained database in two large spontaneous intracranial hypotension (SIH) referral centers in Germany and Switzerland of patients who meet the modified International Classification of Headache Disorders, 3rd edition criteria for SIH. Patients with spinal CSF leaks were screened for the presence of idiopathic infratentorial symmetric SS of the CNS. RESULTS: Twelve patients were included. The median latency between the onset of orthostatic headaches and symptoms attributed to SS was 9.5 years. After surgical closure of the underlying spinal CSF leak, symptoms attributed to SS improved in seven patients and remained stable in three. Patients who presented within 1 year after the onset of SS symptoms improved, but those who presented in 8-12 years did not improve. We could show a significant association between patients with spinal longitudinal extrathecal collections and SS. CONCLUSIONS: Long-standing untreated ventral spinal CSF leaks can lead to SS of the CNS, and microsurgical sealing of spinal CSF leaks might stop progression and improve symptoms in patients with SS in a time-dependent manner.

Myelography Using Energy-Integrating Detector CT Versus Photon-Counting Detector CT for Detection of CSF-Venous Fistulas in Patients With Spontaneous Intracranial Hypotension.

BACKGROUND. CSF-venous fistulas (CVFs), which are an increasingly recognized cause of spontaneous intracranial hypotension (SIH), are often diminutive in size and exceedingly difficult to detect by conventional imaging. OBJECTIVE. This purpose of this study was to compare energy-integrating detector (EID) CT myelography and photon-counting detector (PCD) CT myelography in terms of image quality and diagnostic performance for detecting CVFs in patients with SIH. METHODS. This retrospective study included 38 patients (15 men and 23 women; mean age, 55 ± 10 [SD] years) with SIH who underwent both clinically indicated EID CT myelography (slice thickness, 0.625 mm) and PCD CT myelography (slice thickness, 0.2 mm; performed in ultrahigh-resolution mode) to assess for CSF leak. Three blinded radiologists reviewed examinations in random order, assessing image noise, discernibility of spinal nerve root sleeves, and overall image quality (each assessed using a scale of 0-100, with 100 denoting highest quality) and recording locations of the CVFs. Definite CVFs were defined as CVFs described in CT myelography reports using unequivocal language and having an attenuation value greater than 70 HU. RESULTS. For all readers, PCD CT myelography, in comparison with EID CT myelography, showed higher mean image noise (reader 1: 69.9 ± 18.5 [SD] vs 37.6 ± 15.2; reader 2: 59.5 ± 8.7 vs 49.3 ± 12.7; and reader 3: 57.6 ± 13.2 vs 42.1 ± 16.6), higher mean nerve root sleeve discernibility (reader 1: 81.6 ± 21.7 [SD] vs 30.4 ± 13.6; reader 2: 83.6 ± 10 vs 70.1 ± 18.9; and reader 3: 59.6 ± 13.5 vs 50.5 ± 14.4), and higher mean overall image quality (reader 1: 83.2 ± 20.0 [SD] vs 38.1 ± 13.5; reader 2: 80.1 ± 10.1 vs 72.4 ± 19.8; and reader 3: 57.8 ± 11.2 vs 51.9 ± 13.6) (all p < .05). Eleven patients had a definite CVF. Sensitivity and specificity of EID CT myelography and PCD CT myelography for the detection of definite CVF were 45% and 96% versus 64% and 85%, respectively, for reader 1; 36% and 100% versus 55% and 96%, respectively, for reader 2; and 57% and 100% versus 55% and 93%, respectively, for reader 3. The sensitivity was significantly higher for PCD CT myelography than for EID CT myelography for reader 1 and reader 2 (both p < .05) and was not significantly different between the two techniques for reader 3 (p = .45); for all three readers, specificity was not significantly different between the two modalities (all p > .05). CONCLUSION. In comparison with EID CT myelography, PCD CT myelography yielded significantly improved image quality with significantly higher sensitivity for CVFs (for two of three readers), without significant loss of specificity. CLINICAL IMPACT. The findings support a potential role for PCD CT myelography in facilitating earlier diagnosis and targeted treatment of SIH, avoiding high morbidity during potentially prolonged diagnostic workups.

Sex differences in the clinical manifestations and treatment outcomes in a large cohort of spontaneous intracranial hypotension.

OBJECTIVE: To determine sex differences in clinical profiles and treatment outcomes in patients with spontaneous intracranial hypotension. BACKGROUND: Spontaneous intracranial hypotension is associated with considerable functional disability and potentially fatal complications, and it is uncertain whether males and females should be managed differently. METHODS: This was a cohort study of consecutive patients with spontaneous intracranial hypotension enrolled from a medical center. Medical records and imaging findings were reviewed. The outcome of treatment responses to epidural blood patches and risks of subdural hematoma were measured. RESULTS: In total, 442 patients with spontaneous intracranial hypotension (165 males, 277 females) were included in the analysis. Males were more likely to have a delayed (>30 days) initial presentation than females (32.1% [53/165] vs. 19.9% [55/277], p = 0.004), and males were less likely to have nausea (55.8% [92/165] vs. 67.1% [186/277], p = 0.016), vomiting (43.0% [71/165] vs. 54.2% [150/277], p = 0.024), photophobia (9.7% [16/165] vs. 17.0% [47/277], p = 0.034), and tinnitus (26.7% [44/165] vs. 39.7% [110/277], p = 0.005) compared with females despite comparable radiologic findings. Among the 374 patients treated with epidural blood patches, males were more likely to be nonresponders to the first epidural blood patch (58.0% [80/138] vs. 39.0% [92/236], OR = 2.2, 95% CI = 1.4-3.3, p < 0.001). Males were at a higher risk of having subdural hematoma (29.7% [49/165] vs. 10.8% [30/277], OR = 3.5, 95% CI = 2.1-5.8, p < 0.001). Among patients with subdural hematoma, males had greater thickness (12.8 ± 4.3 vs. 8.1 ± 5.9 mm, p < 0.001) and were more likely to receive surgical drainage (55.1% [27/49] vs. 10.0% [3/30], OR = 11.0, 95% CI = 3.0-41.3, p < 0.001) than females. CONCLUSION: In the present study, spontaneous intracranial hypotension in males was characterized by a delayed presentation, poorer response to the first epidural blood patch, and a higher risk of subdural hematoma. Caution should be exercised in the management of males with spontaneous intracranial hypotension. The generalizability of the findings needs to be further confirmed.

Spontaneous intracranial hypotension mimicking iatrogenic spinal cerebrospinal fluid leaks.

OBJECTIVE: To raise awareness that patients with persistent post-dural puncture headache should be considered for evaluation of spontaneous cerebrospinal fluid (CSF) leak. BACKGROUND: Spontaneous intracranial hypotension (SIH) due to a spinal CSF leak may occur following more-or-less trivial traumatic events. We report our experience with spontaneous spinal CSF leaks that occur following percutaneous or open spine procedures, a potential source of diagnostic confusion. METHODS: In a retrospective cohort study, using a prospectively maintained database of patients with SIH, we identified all new patients evaluated between January 1, 2022, and June 30, 2023, who were referred for evaluation of an iatrogenic spinal CSF leak but were found to have a spontaneous spinal CSF leak. RESULTS: Nine (4%) of the 248 patients with SIH were originally referred for evaluation of an iatrogenic spinal CSF leak. The spinal procedures included epidural steroid injections, laminectomies, epidural anesthesia, and lumbar puncture. Brain magnetic resonance imaging (MRI) showed changes in intracranial hypotension in seven of the nine patients (78%). The spontaneous CSF leak was found to be at least five levels removed from the spinal procedure in all patients. CONCLUSIONS: A spontaneous spinal CSF leak should be suspected in patients with recalcitrant orthostatic headaches following a spinal procedure, even if symptoms of the leak occur within hours of the spinal procedure and especially if brain MRI is abnormal.

Factors to predict recurrence after epidural blood patch in patients with spontaneous intracranial hypotension.

OBJECTIVES: This study aimed to identify predictors for the recurrence of spontaneous intracranial hypotension (SIH) after epidural blood patch (EBP). BACKGROUND: Epidural blood patch is the main treatment option for SIH; however, the characteristics of patients who experience relapse after successful EBP treatment for SIH remain understudied. METHODS: In this exploratory, retrospective, case-control study, we included 19 patients with SIH recurrence after EBP and 36 age- and sex-matched patients without recurrence from a single tertiary medical institution. We analyzed clinical characteristics, neuroimaging findings, and volume changes in intracranial structures after EBP treatment. Machine learning methods were utilized to predict the recurrence of SIH after EBP treatment. RESULTS: There were no significant differences in clinical features between the recurrence and no-recurrence groups. Among brain magnetic resonance imaging signs, diffuse pachymeningeal enhancement and cerebral venous dilatation were more prominent in the recurrence group than no-recurrence group after EBP (14/19 [73%] vs. eight of 36 [22%] patients, p = 0.001; 11/19 [57%] vs. seven of 36 [19%] patients, p = 0.010, respectively). The midbrain-pons angle decreased in the recurrence group compared to the no-recurrence group after EBP, at a mean (standard deviation [SD]) of -12.0 [16.7] vs. +1.8[18.3]° (p = 0.048). In volumetric analysis, volume changes after EBP were smaller in the recurrence group than in the no-recurrence group in intracranial cerebrospinal fluid (mean [SD] -11.6 [15.3] vs. +4.8 [17.1] mL, p = 0.001) and ventricles (mean [SD] +1.0 [2.0] vs. +2.0 [2.5] mL, p = 0.003). Notably, the random forest classifier indicated that the model constructed with brain volumetry was more accurate in discriminating SIH recurrence (area under the curve = 0.80 vs. 0.52). CONCLUSION: Our study suggests that volumetric analysis of intracranial structures may aid in predicting recurrence after EBP treatment in patients with SIH.

Patterns of clinical and imaging presentations in patients with spontaneous intracranial hypotension due to spinal cerebrospinal fluid venous fistula: A single-center retrospective cross-sectional study.

OBJECTIVE: To identify distinct clinical or imaging subtypes of spontaneous intracranial hypotension (SIH) due to spinal cerebrospinal fluid (CSF) venous fistula (CVF). BACKGROUND: Spontaneous intracranial hypotension is classically understood to present clinically with an orthostatic headache and stereotyped brain magnetic resonance imaging (MRI) findings; however, most prior literature examining clinical and brain MRI features of SIH has focused on all types of spinal CSF leaks concurrently. This study aimed to evaluate whether data support the possibility of internally consistent subtypes based on brain imaging features and clinical symptoms analogous to those seen in primary headache syndromes. METHODS: This retrospective cross-sectional single-institution study included 48 consecutive patients meeting the International Classification of Headache Disorders, 3rd edition criteria for SIH due to CVF. Clinical symptoms, pre-treatment brain MRI, and symptom duration were analyzed. Clinical and MRI data were analyzed to identify patterns and associations between symptoms and imaging findings. RESULTS: A total of 20 males and 28 females were evaluated, with a mean (standard deviation) age of 61 (10) years. In all, 44/48 (92%) patients experienced headaches, though 18/48 (40%) did not endorse relief when flat, including six of the 48 (13%) with worsening symptoms when flat. In all, 19/48 (40%) patients reported at least one migraine symptom, and six of the 48 (13%) presented with at least one migraine symptom and had no relief when flat. Clinical symptoms clustered primarily into a "classic" presentation consisting of relief when flat, occipital head pain, comorbid neck pain, a pressure/throbbing headache quality, and an "atypical" presentation that was characterized by having several differences: less relief when flat (nine of 22 (41%) vs. 20/23 (87.0%), p = 0.002; odds ratio [OR] 0.110, 95% confidence interval [CI] 0.016-0.53), more frontal head pain (14/22 (64%) vs. one of 23 (4%), p < 0.001; OR 35.0, 95% CI 4.2-1681.0), less neck pain (two of 21 (4.5%) vs. nine of 13 (69.6%), p < 0.001; OR 0.023, 95% CI 0.0005-0.196), and more stabbing/sharp headache quality (nine of 22 (41%) vs. two of 23 (9%), p = 0.017; OR 7.0, 95% CI 1.18-75.9). Brain MRI findings clustered into three groups: those presenting with most imaging findings of SIH concurrently, those with brain sag but less pachymeningeal/venous engorgement, and those with pachymeningeal/venous engorgement but less brain sag. CONCLUSION: This study highlights the clinical and imaging diversity among patients with SIH due to CVF, challenging the reliance on classic orthostatic headache alone for diagnosis. The findings suggest the existence of distinct SIH subtypes based on clinical and imaging presentations, underscoring the need for comprehensive evaluation in patients with suspected CVF. Future research should further elucidate the relationship between clinical symptoms and imaging findings, aiming to refine diagnostic criteria and enhance understanding of SIH's pathophysiology.

Pediatric post-dural puncture headache and paraplegia.

A cerebrospinal fluid (CSF) leak developed in a 14-year-old girl and a 12-year-old boy following a diagnostic lumbar puncture. Two days and sixteen years later, respectively, paraplegia developed due to a functional disorder. Imaging revealed an extensive extradural CSF collection in both patients and digital subtraction myelography was required to pinpoint the exact site of a ventral dural puncture hole where the lumbar spinal needle had gone "through and through" the dural sac. The CSF leak was complicated by cortical vein thrombosis in one patient. Both patients underwent uneventful surgical repair of the ventral dural puncture hole with prompt resolution of the paraplegia. Iatrogenic ventral CSF leaks may become exceptionally long standing and may be complicated by paraplegia on a functional basis both in the acute and chronic phases.

Progressive superficial siderosis despite complete remission of intracranial hypotension symptoms following epidural patching: Case report.

Spontaneous intracranial hypotension (SIH) commonly results from ventral spinal cerebrospinal fluid (CSF) leaks and epidural patches are advocated as first-line treatment. Complications such as superficial siderosis can arise but have previously been reported only in the context of long-term persistent, ongoing, CSF leak and SIH. We report a case of a patient with SIH from a ventral spinal CSF leak that was treated with epidural patching and experienced complete resolution of SIH. Four years later SIH symptoms recurred, and brain magnetic resonance imaging unexpectedly showed the interval accumulation of hemosiderin pigmentation on the cerebellum and brainstem during the period when the patient was without symptoms of SIH. This case uniquely demonstrates the progression of superficial siderosis despite the apparent resolution of SIH. Our findings suggest two divergent pathophysiological outcomes from spinal ventral dural tear: (1) CSF loss causing SIH; and (2) persistent low-level bleeding arising from the spinal dural tear leading to superficial siderosis. These divergent pathophysiologies had a discordant response to epidural patching. Epidural patching successfully treated the SIH but did not prevent the progression of superficial siderosis, indicating that some patients may require more than epidural patching despite symptom resolution. This case highlights the need for post-treatment monitoring protocols in patients with ventral spinal CSF leaks and SIH and raises important questions about the adequacy of epidural patching in certain SIH cases arising from ventral spinal CSF leak.

Clinical utility of CT myelography renal collecting system density in the evaluation of spinal CSF leak or CSF-venous connection in patients with spontaneous intracranial hypotension.

INTRODUCTION: Identifying spontaneous CSF leaks can be difficult on CT myelography (CTM) in patients with suspected spontaneous intracranial hypotension (SIH). The current study compared renal collecting system (RCS) density during CTM in patients with suspected SIH relative to non-SIH controls and evaluated the overall utility as an indirect sign of spontaneous CSF leak in patients with SIH. METHODS: All CTM performed over an 8-year period (n = 392) were retrospectively reviewed and included cases (n = 295) were divided into groups consisting of SIH patients with (n = 35) or without (n = 77) confirmed CSF leak on CTM and non-SIH controls (n = 183). Average and relative average (relative to systemic contrast density) RCS densities were compared with multivariate analysis adjusting for patient characteristics and CTM technical differences. RESULTS: Average RCS densities were greater for confirmed versus non-confirmed SIH groups and greater for both SIH groups relative to non-SIH controls. Correlations between RCS density and time from injection to CTM were higher within SIH patient groups compared to controls. Measured RCS density had a higher negative predictive value for excluding CSF leak both within the SIH patient groups as well as the overall cohort (84% and 96%, respectively), with optimized thresholds yielding 80% sensitivity and 70% specificity for the presence of leak in the overall cohort. CONCLUSION: Accounting for RCS density may provide reliable additional indirect diagnostic value about the suspicion of a CSF leak in patients undergoing CTM for evaluation of SIH symptoms.

Spinal dementia: Don't miss it, it's treatable.

BACKGROUND & PURPOSE: Around 5% of dementia patients have a treatable cause. To estimate the prevalence of two rare diseases, in which the treatable cause is at the spinal level. METHODS: A radiology information system was searched using the terms CT myelography and the operation and classification system (OPS) code 3-241. The clinical charts of these patients were reviewed to identify patients with a significant cognitive decline. RESULTS: Among 205 patients with spontaneous intracranial hypotension (SIH) and proven CSF leaks we identified five patients with a so-called frontotemporal brain sagging syndrome: Four of those had CSF venous fistulas and significantly improved by occluding them either by surgery or transvenous embolization. Another 11 patients had infratentorial hemosiderosis and hearing problems and ataxia as guiding symptoms. Some cognitive decline was present in at least two of them. Ten patients had ventral dural tears in the thoracic spine and one patient a lateral dural tear at C2/3 respectively. Eight patients showed some improvement after surgery. DISCUSSION: It is mandatory to study the (thoracic) spine in cognitively impaired patients with brain sagging and/ or infratentorial hemosiderosis on MRI. We propose the term spinal dementia to draw attention to this region, which in turn is evaluated with dynamic digital subtraction and CT myelography.

Unruptured giant lateral thoracic meningocele: extremely rare cause of cerebrospinal fluid (CSF) hypotension in neurofibromatosis type 1.

Neurofibromatosis type 1 (NF1) is a multisystem neurocutaneous disorder. Scoliosis and dural ectasia are features of the associated mesodermal dysplasia. Lateral thoracic meningoceles can develop in NF1 and progressively enlarge due to cerebrospinal fluid (CSF) pulsations. Large meningoceles can cause compressive symptoms in the thorax. We are reporting a case of a NF1 presenting with acute onset respiratory distress, who also had chronic orthostatic headaches. CT chest showed unruptured enlarging bilateral lateral thoracic meningoceles causing lung compression. MRI of the brain and spine showed features of CSF hypotension, explaining the headaches. CSF hypotension with unruptured meningoceles is extremely rare. Management of the condition is challenging since surgical removal is prone to complications due to underlying mesodermal abnormalities. Cystoperitoneal shunting to relieve lung compression may worsen CSF hypotension. A shunt with a programmable valve allowed controlled drainage and successfully relieved lung compression without worsening of orthostatic headaches in our case.

Spontaneous intracranial hypotension: Exploring the viability of non-contrast FLAIR as a substitute for contrast-enhanced T1WI in assessing pachymeningeal thickening.

PURPOSE: To avoid contrast administration in spontaneous intracranial hypotension (SIH), some studies suggest accepting diffuse pachymeningeal hyperintensity (DPMH) on non-contrast fluid-attenuated inversion recovery (FLAIR) as an equivalent sign to diffuse pachymeningeal enhancement (DPME) on contrast-enhanced T1WI (T1ce), despite lacking thorough performance metrics. This study aimed to comprehensively explore its feasibility. METHODS: In this single-center retrospective study, between April 2021 and November 2023, brain MRI examinations of 43 patients clinically diagnosed with SIH were assessed using 1.5 and 3.0 Tesla MRI scanners. Two radiologists independently assessed the presence or absence of DPMH on FLAIR and DPME on T1ce, with T1ce serving as a gold-standard for pachymeningeal thickening. The contribution of the subdural fluid collections to DPMH was investigated with quantitative measurements. Using Cohen's kappa statistics, interobserver agreement was assessed. RESULTS: In 39 out of 43 patients (90.7%), pachymeningeal thickening was observed on T1ce. FLAIR sequence produced an accuracy, sensitivity, specificity, positive predictive value, and negative predictive value of 72.1%, 71.8%, 75.0%, 96.6%, and 21.4% respectively, for determining pachymeningeal thickening. FLAIR identified pachymeningeal thickening in 28 cases; however, among these, 21 cases (75%) revealed that the pachymeningeal hyperintense signal was influenced by subdural fluid collections. False-negative rate for FLAIR was 28.2% (11/39). CONCLUSION: The lack of complete correlation between FLAIR and T1ce in identifying pachymeningeal thickening highlights the need for caution in removing contrast agent administration from the MRI protocol of SIH patients, as it reveals a major criterion (i.e., pachymeningeal enhancement) of Bern score.

Bibrachial amyotrophy as a rare manifestation of intraspinal fluid collection: a case report and systematic review.

INTRODUCTION: Intraspinal cerebrospinal fluid (CSF) collection has been reported as a rare cause of lower motor neuron (LMN) disorder. We report a case of bibrachial diplegia associated with intraspinal CSF collection and perform a systematic literature review. PATIENT AND METHODS: A 52-year-old man developed a bibrachial amyotrophy over 6 years, confirmed by the presence of cervical subacute neurogenic changes at electromyography (EMG). Brain magnetic resonance imaging (MRI) revealed cerebral siderosis, while spine MRI showed a ventral longitudinal intraspinal fluid collection (VLISFC) from C2 to L2. No CSF leakage was localized at myelography; a conservative treatment was chosen. We searched for all published cases until 30th April 2023 and extrapolated data of 44 patients reported in 27 publications. RESULTS: We observed a male predominance, a younger disease onset compared to amyotrophic lateral sclerosis, and a quite long disease duration, highlighting a slow disease progression. LMN signs were more frequently bilateral, mostly involving C5-C6 myotomes. Around 61% of patients presented additional symptoms, but only three referred to a history of headache. Accordingly, CSF opening pressure was mostly normal. Spinal MRI revealed the presence of VLISFC and in some cases myelomalacia. EMG patterns displayed both chronic and subacute neurogenic change in the cervical region. The disease course mainly depended on the treatment choice, which was mostly represented by a surgical approach when a specific dural defect was detected by imaging. CONCLUSION: Bibrachial diplegia due to VLISFC can be a treatable cause of focal amyotrophy and presents some clinical and radiological "red flags" which cannot be missed by a clinical neurologist.

Cerebrospinal fluid-lymphatic fistula in a child with generalized lymphatic anomaly treated with targeted blood patch - a rare case report and review of the literature.

Spontaneous intracranial hypotension may result in debilitating postural headaches and severe neurological symptoms due to secondary cerebellar sagging. The most common cause is the cerebrospinal fluid (CSF) leak within the spinal canal. Although previously reported in only a few cases, also paraspinal lymphatic malformations causing vertebral bone destruction may occasionally result in CSF leak to these pathological formations. Here, we present a case of a 9-year-old girl with generalized lymphatic anomaly (GLA) presenting with severe postural headache. Radiological imaging revealed a typical feature of cerebellar sagging. Myelography localized the CSF leakage into vertebral bodies of C7 and Th1, which both were partly involved in pathological paravertebral masses of known lymphatic anomaly, and from there along the right C8 nerve root sleeve into the anomaly. As the C8-nerve root could not be ligated due to the risk of significant neurological injury, we attempted image-guided targeted percutaneous epidural placement of a blood patch directly into the foramen at the affected level. The procedure resulted in obliteration of the fistula and regression of cerebellar sagging, with significant relief of symptoms. Although it is an extremely rare coincidence, patients with paraspinal lymphatic malformations may develop intraspinal CSF leak into these pathological formations. The present case report suggests that besides a direct surgical obliteration of the fistula and sacrificing the nerve root, a targeted percutaneous epidural blood patch may be a possible alternative in the case of a functionally important nerve root.

Endovascular embolization of cerebrospinal fluid-venous fistula: a comprehensive systematic review on its efficacy and safety for the management of spontaneous intracranial hypotension.

Cerebrospinal fluid (CSF)-venous fistula can cause spontaneous intracranial hypotension (SIH) and poses a significant diagnostic and management challenge. This study aims to provide a comprehensive overview of the clinical and radiological outcomes of endovascular embolization as a novel treatment approach for CSF-venous fistula in patients with SIH. This systematic review adheres to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses 2020 statement. The primary outcome was the efficacy of the embolization procedure in occlusion of the CSF-venous fistula, and secondary outcomes included procedural complications and improvement of clinical symptoms and radiological findings. A total of nine studies consisting of 77 patients met the inclusion criteria. Orthostatic and/or Valsalva headache was the most common symptom. The mean age of the patients was 57 ± 8.9 years, and females accounted for 59.7% (46/77) of the cases. Sixty-five (84.4%) patients reported complete resolution or significant improvement in symptoms. The Bern score, Headache Impact Test-6, and the Patient Global Impression of Change scales demonstrated significant improvements in radiological findings and patients' quality of life. Following the procedure, 22 patients (28.6%) experienced rebound intracranial hypertension and 27 patients (35.1%) had transient local pain at the site of the embolization. Our study showed that endovascular embolization is a safe and effective treatment for CSF-venous fistula in patients with SIH, providing complete resolution or significant improvement of clinical symptoms and radiological findings, and positive impacts on patients' quality of life.

Management of cervical CSF-venous fistula causing acute cognitive impairment and coma.

CSF-venous fistulas (CVFs) are increasingly recognised as a cause of spontaneous intracranial hypotension. They may present atypically including with brain sagging pseudo-dementia. Cervical CVFs are rare and their management can be difficult due to associated eloquent nerve roots. We report the case of a 49-year-old woman who presented with cognitive decline progressing to coma. Brain imaging showed features of spontaneous intracranial hypotension and a right C7 CVF was identified at digital subtraction and CT myelography. Initial treatment with CT-guided injection of fibrin sealant produced temporary improvement in symptoms before surgical treatment resulted in total clinical remission and radiological resolution.

Real-life study of the use of oto-acoustic emissions in the diagnosis of intracranial hypotension.

BACKGROUND: The diagnosis of spontaneous or post-traumatic intracranial hypotension (IH) mainly relies on clinical features and neuro-imaging. However, the results of brain and spine magnetic resonance imaging are not always contributive. There is an interest for other non-invasive procedures, able to confirm or refute the diagnosis. The use of oto-acoustic emissions (OAE) was previously reported on isolated cases of IH associated with endolymphatic hydrops (ELH). The aim of this study was to assess the real-life utilization of this electrophysiological method in a larger population of suspected IH. METHODS: A retro-prospective cohort study was conducted from November 2013 to July 2022 in patients with a suspected or doubtful diagnosis of IH. They were assessed for ELH by recording bilateral distortion product of oto-acoustic emissions (DPOAE) in sitting then in supine position. RESULTS: Among the 32 patients assessed, the diagnostic of IH was confirmed in 18 patients. An ELH was shown in 15 of them (83%), but also in seven other patients. They had several differential diagnoses: chronic migraine, Chiari malformation, rebound intracranial hypertension and perilymph fistula. CONCLUSIONS: This procedure seems to be insufficient to exclude differential diagnosis when intracranial hypotension is suspected.

A new modified technique of dynamic CT myelography to detect dural tears in spontaneous intracranial hypotension.

We propose a modified dynamic CT-myelography technique for patients with fast CSF leaks caused by ventral dural tears in order to reduce radiation exposure and complications. A fluoroscopy-guided lumbar puncture using an epidural anesthesia kit replaces a CT-guided lumbar puncture, and a smaller volume of less concentrated contrast media is used. This approach has advantages, including speeding up the procedure, reduced radiation exposure, and elimination of the risk of contrast injection into the epidural space.

Value of CT in targeted CT-guided epidural blood patching: Predictors for successful epidural punctures.

BACKGROUND AND PURPOSE: Differentiating epidural from intrathecal punctures before computed tomography (CT)-guided epidural blood patching (EBP) is subjective, relying on operator experience. This study aimed to investigate CT findings for epidural and intrathecal punctures and identify reliable predictors for successful epidural punctures before targeted CT-guided EBP. MATERIALS AND METHODS: We included 65 patients with low-cerebrospinal fluid (CSF)-pressure headache receiving targeted CT-guided EBP between January 2021 and October 2022 in this retrospective study. We analyzed clinical data, technical information, and CT features before EBP. Fisher's exact test was used for discrete variables, while Mann-Whitney U test was used for continuous variables. Positive (PLR) and negative likelihood ratios (NLR) were calculated to identify predictors for confirming epidural punctures. RESULTS: We confirmed 43 patients as epidural punctures and 22 patients as intrathecal punctures. Before contrast injection, epidural fat at the needle tip in the epidural group was higher than the intrathecal group (37.2 % [16/43] vs. 4.5 % [1/22], p = 0.006). After contrast injection, the "contrast-needle tip connection" sign was mostly observed in the epidural group than the intrathecal group (95.3 % [41/43] vs. 9.1 % [2/22], p < 0.001). Additionally, the epidural group had significantly higher boomerang-shaped contrast morphology than the intrathecal group (65.1 % [28/43] vs. 9.1 % [2/22], p < 0.001). The "contrast-needle tip connection" sign had the highest PLR (10.49) and lowest NLR (0.05). CONCLUSION: Identifying epidural fat at the needle tip, "contrast-needle tip connection" sign, and boomerang-shaped contrast morphology on CT scans are useful for confirming proper placement of the needle tip within the epidural space.

Headache associated with intracranial hypotension: diagnostic challenges and difficulties in everyday neurological practice.

Low pressure of cerebrospinal fluid (CSF) is a rare cause of headache, except when the patient undergoes a lumbar puncture. Headache associated with a low CSF pressure i.e. intracranial hypotension causes diagnostic difficulties. Headaches related to spontaneous intracranial hypotension (SIH) pose a significant diagnostic challenge in everyday neurological practice. Patients with headaches due to SIH are usually diagnosed only after a long delay. Diagnostic problems may result in unnecessary invasive diagnostic procedures, or even neurosurgical operations. Diagnosing headaches attributed to SIH requires the consideration of several clinical scenarios, and the disease's features causing primary or secondary disturbances. In this review, we discuss the differential diagnosis of SIH-related headaches with reference to accumulated knowledge, including meta-analyses, guidelines, casuistry, and the applicable criteria of the International Classification of Headache Disorders. In addition, we discuss head and spine magnetic resonance imaging abnormalities, which may indicate intracranial hypotension.