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A 34-year-old man with a 2-week history of orthostatic headaches and a'dry tap' at lumbar puncture was found to have a lumbar intradural mass on magnetic resonance imaging (MRI) examination. A myxopapillary ependymoma was resected and the patient's headache completely resolved. The combination of spontaneous orthostatic headaches and a'dry tap' at the time of lumbar puncture does not always indicate the presence of a spontaneous cerebrospinal fluid (CSF) leak and intracranial hypotension. [ABSTRACT FROM AUTHOR]

Cerebrospinal fluid leaks are important to recognize because they can cause debilitating symptoms for patients and have life-threatening complications. Leakage of cerebrospinal fluid (CSF) from the subarachnoid space can occur at the cranial or spinal level, with distinct clinical presentations, diagnostic evaluations, and treatment modalities depending on the type and location of the leak. Spontaneous, traumatic, and iatrogenic spinal CSF leaks cause reduced intracranial CSF volume and the clinicoradiologic syndrome commonly called "intracranial hypotension". This review discusses the clinical presentations, etiologies, and risk factors of spinal and cranial CSF leaks., Competing Interests: Disclosure No authors report any disclosures., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Background and Purpose: Symptoms indistinguishable from behavioral-variant frontotemporal dementia (bvFTD) can develop in patients with spontaneous intracranial hypotension associated with severe brain sagging. An underlying spinal CSF leak can be identified in only a minority of these patients and the success rate of nondirected treatments, such as epidural blood patching and dural reduction surgery, is low. The disability associated with bvFTD sagging brain syndrome is high and, because of the importance of the venous system in the pathophysiology of CSF leaks in general, we have investigated the systemic venous circulation in those patients with recalcitrant symptoms., Materials and Methods: We reviewed the medical records and imaging studies of 21 consecutive patients with bvFTD sagging brain syndrome in whom no spinal CSF leak could be found and who underwent imaging of the systemic venous circulation (MR- or CT-venography). An SIH Disability Assessment Score (SIHDAS) questionnaire was completed to assess the severity of the symptoms., Results: The mean age of the 3 women and 18 men was 50 years (range, 26-68 years). Seven patients were found to have venous stenosis. Endovascular stent placement of moderate to high-grade azygos vein stenosis in 3 patients resulted in prompt and remarkable improvement of symptoms in 2 patients (SIHDAS: very severe disability to no or mild disability) and mild improvement in 1 patient (SIHDAS: very severe disability to severe disability). Treatment of internal jugular vein and inferior vena cava stenosis in 2 patients each did not result in any improvement of symptoms. Endovascular (5 patients) or surgical (2 patients) interruption of multiple epidural spinal venous pathways did not result in any clinical improvement., Conclusions: The azygos vein is the main conduit between the spinal CSF space and the systemic venous circulation and this study demonstrates that isolated azygos vein stenosis may be a cause of spinal CSF loss and sagging brain syndrome. In this study, the yield of finding a clinically important treatable venous lesion among patients with recalcitrant bvFTD sagging brain syndrome was relatively low (15%). However, high quality and safe noninvasive imaging of the systemic venous system is available, eg, MR-venography, and should be considered for those patients who have exhausted treatments for this devastating condition, focusing on the azygos system., (© 2025 by American Journal of Neuroradiology.)

Objective: Frontotemporal dementia (FTD) sagging brain syndrome is a disabling condition. An underlying spinal Cerebrospinal fluid leak can be identified in only a minority of patients and the success rate of non-directed treatments is low. Some of these patients have a remote history of craniectomy/cranioplasty and we report a positive response to custom implant cranioplasty revision many years after their initial cranioplasty., Methods: We reviewed medical records and imaging studies of 61 consecutive patients with FTD sagging brain syndrome. A SIH Disability Assessment Score (SIHDAS) questionnaire was completed to assess the severity of the symptoms before and after custom implant cranioplasty. Pre- and post-operative brain MRI was obtained to assess degree of brain sagging., Results: Eight (13.1%) of the 61 patients had a history of craniectomy/cranioplasty 1.5-13.5 years prior to onset of symptoms of FTD sagging brain syndrome. The mean age of the one woman and seven men at the time of presentation to our medical center was 50 years (range, 26-68 years). None had sinking scalp flap syndrome. Prior treatments included epidural blood patching and dural reduction surgery. Custom cranial implant surgery was performed in four patients and resulted in prompt and remarkable improvement of symptoms in three patients (SIHDAS: very severe disability to no or mild disability) and mild improvement in one patient. Brain MRI showed improvement of brain sagging., Interpretation: A disproportionate number of patients with FTD sagging brain syndrome have a remote history of supratentorial craniectomy/cranioplasty and revision cranioplasty should be considered., (© 2024 The Author(s). Annals of Clinical and Translational Neurology published by Wiley Periodicals LLC on behalf of American Neurological Association.)

Background: Spontaneous skull base cerebrospinal fluid leaks (CSFLs) are associated with increased intracranial pressure in idiopathic intracranial hypertension (IIH) and hypothesized to relate to skull base erosions due to increased CSF pressure. Given the increasing recognition of internal jugular venous stenosis (IJVS) as a cause of intracranial hypertension (IH), we evaluated the relationship between spinal CSFL and venous causes of IH., Methods: The spinal CSFL database at a single institution was assessed to identify 12 consecutive spontaneous, non-traumatic spinal CSFL patients with CTV data. Exclusion criteria included documented IIH and iatrogenic CSFL. Demographics, clinical parameters, imaging characteristics, and IJV manometry results were recorded. Internal jugular venous stenosis was graded as: none (0-10%), mild (10-50%), moderate (50-80%), severe (>80-99%), and occluded (100%). Twelve consecutive patients who presented with cerebrovascular accidents without CSFL, matched by age and sex, were similarly analyzed as a control group. STROBE guidelines were used in reporting results., Results: All CSFL patients had IJVS (83.3% bilateral, 33.3% severe) compared to 41.7% of the control group (33.3% bilateral, 16.7% severe-occluded); p  = 0.04. All CSFL patients with available venogram manometry data had at least unilateral IJV gradients. Most patients presented with modified Rankin score (mRS) of 1 (66.7%), but in those with higher mRS, medical and/or surgical interventions were associated with decreased morbidity., Conclusion: Spontaneous spinal CSFL was associated with IJVS in patients not meeting IIH criteria. Persistently high CSF pressure resulting in CSFL may cause opening pressure to be falsely normal or low. Internal jugular venous stenosis may be a viable target in recurrent CSFL management and improve morbidity., Competing Interests: Author contributionsFH and CP contributed to the design of the research and collection of data. SM contributed to the collection of data, analysis of the results, and writing of the manuscript, with input/contribution from KF, MA, LG, WS, ML, and FH. Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

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., (© 2024 American Headache Society.)

Background and Purpose: CSF-venous fistulas are a common cause of spontaneous intracranial hypotension. The diagnosis and precise localization of these fistulas hinges on specialized myelographic techniques, which mainly include decubitus digital subtraction myelography and decubitus CT myelography (using either energy integrating or photon counting detector CT). A previous case series showed that cone beam CT myelography, performed as an adjunctive tool with digital subtraction myelography, increased the detection of CSF-venous fistulas. Here, we sought to determine the additive yield of cone beam CT myelography for CSF-venous fistula detection in a consecutive series of patients with spontaneous intracranial hypotension who underwent concurrent decubitus digital subtraction myelography and cone beam CT myelography., Materials and Methods: We retrospectively searched our institutional database for all consecutive patients who underwent decubitus digital subtraction myelography with adjunctive cone beam CT myelography between 8/5/2021 and 8/5/2024. We excluded any patients harboring extradural CSF on spine imaging, not meeting International Classification of Headache Disorders (3 rd edition) criteria for spontaneous intracranial hypotension, or not having undergone technically successful cone beam CT myelography in combination with digital subtraction myelography. All myelographic images were independently reviewed by two neuroradiologists. We calculated the diagnostic yield of both myelographic tests for localizing a CSF-venous fistula., Results: We identified 100 patients who underwent decubitus digital subtraction myelography with adjunctive cone beam CT. We excluded 15 patients based on above criteria. 59/85 patients had a single definitive CSF-venous fistula. Among positive cases, the fistula was visible on digital subtraction myelography in 38/59 patients and visible on cone beam CT myelography in 59/59 patients. In 26/85 patients, no definitive fistula was identified on either modality., Conclusions: Cone beam CT myelography increased the diagnostic yield for CSF-venous fistula detection and may be a useful addition to digital subtraction myelography., Abbreviations: CB-CTM = cone beam CT myelography; CVF = CSF-venous fistula; DSM = digital subtraction myelography; EID-CTM = energy integrating detector CT myelography; PCD CTM = photon counting detector CT myelography; SIH = spontaneous intracranial hypotension., Competing Interests: The authors declare no conflicts of interest related to the content of this article., (© 2024 by American Journal of Neuroradiology.)

We investigated whether idiopathic spinal cord herniation is a congenital or acquired condition and undertook a study to determine the risk of developing iSCH in patients with persistent ventral spinal CSF leaks. De novo formation of iSCH was established among all 6 patients with iSCH who had undergone prior spinal imaging for symptoms unrelated to iSCH. Among 51 patients with persistent ventral spinal CSF leaks, iSCH developed in 2 patients (probability increased from 0% at 5 years to 9.4% at 10 years). This study shows that iSCH is an acquired condition, and early treatment of ventral CSF leaks offers a unique opportunity to prevent neurologic disability., (© 2024 by American Journal of Neuroradiology.)

Background and Purpose: Spinal CSF leaks cause spontaneous intracranial hypotension. Several types of leaks have been identified, and one of these types is the lateral dural tear. Performing myelography with the patient in the decubitus position allows precise characterization of these leaks. The purpose of the current study was to describe the different variants of spontaneous lateral CSF leaks., Materials and Methods: This retrospective cohort study included a consecutive group of patients with spontaneous intracranial hypotension and lateral CSF leaks who underwent digital subtraction myelography in the decubitus position and underwent surgery to repair the CSF leak between July 2018 and June 2023., Results: The mean age of the 53 patients (37 women and 16 men) was 35.5 years. Three different variants of lateral CSF leak could be identified. Forty-nine patients (92.5%) had a lateral dural tear associated with the nerve root sleeve. The dural tear was at the axilla of the nerve root sleeve in 36 patients (67.9%) and at the shoulder in 13 patients (24.5%). Four patients (7.5%) had a lateral dural tear at the level of the pedicle that was not associated with the nerve root sleeve. Findings on digital subtraction myelography were concordant with intraoperative findings in all patients. An extradural CSF collection was seen in all patients with a lateral dural tear associated with the nerve root sleeve but in only 2 of the 4 patients with the pedicular variant of a lateral dural tear., Conclusions: We identified 3 variants of spontaneous lateral dural tears. Most lateral dural tears are associated with extradural CSF collections and arise from either the axilla (67.9%) or the shoulder (24.5%) of the nerve root sleeve. Lateral dural tears at the level of the pedicle (7.5%) not associated with the nerve root sleeve are uncommon and may require specialized imaging for their detection., (© 2024 by American Journal of Neuroradiology.)

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., (© 2024 American Headache Society.)

Purpose of Review: This review focuses on the challenges of diagnosing and treating spontaneous intracranial hypotension (SIH), a condition caused by spinal CSF leakage. It emphasizes the need for increased awareness and advocates for early and thoughtful use of empirical epidural blood patches (EBPs) in suspected cases., Recent Findings: SIH diagnosis is hindered by variable symptoms and inconsistent imaging results, including normal brain MRI and unreliable spinal opening pressures. It is crucial to consider SIH in differential diagnoses, especially in patients with connective tissue disorders. Early EBP intervention is shown to improve outcomes., Summary: SIH remains underdiagnosed and undertreated, requiring heightened awareness and understanding. This review promotes proactive EBP use in managing suspected SIH and calls for continued research to advance diagnostic and treatment methods, emphasizing the need for innovative imaging techniques for accurate diagnosis and timely intervention., Competing Interests: The authors report no relevant disclosures. Full disclosure form information provided by the authors is available with the full text of this article at Neurology.org/cp., (Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.)

Background and Purpose: Spontaneous spinal CSF leaks typically cause orthostatic headache, but their detection may require specialized and invasive spinal imaging. We undertook a study to determine the value of simple optic nerve sheath MR imaging measurements in predicting the likelihood of finding a CSF-venous fistula, a type of leak that cannot be detected with routine spine MR imaging or CT myelography, among patients with orthostatic headache and normal conventional brain and spine imaging findings., Materials and Methods: This cohort study included a consecutive group of patients with orthostatic headache and normal conventional brain and spine imaging findings who underwent digital subtraction myelography under general anesthesia to look for spinal CSF-venous fistulas., Results: The study group consisted of 93 patients (71 women and 22 men; mean age, 47.5 years; range, 17-84 years). Digital subtraction myelography demonstrated a CSF-venous fistula in 15 patients. The mean age of these 8 women and 7 men was 56 years (range, 23-83 years). The mean optic nerve sheath diameter was 4.0 mm, and the mean perioptic subarachnoid space was 0.5 mm in patients with a CSF-venous fistula compared with 4.9 and 1.2 mm, respectively, in patients without a fistula ( P  < .001). Optimal cutoff values were found at 4.4 mm for optic nerve sheath diameter and 1.0 mm for the perioptic subarachnoid space. Fistulas were detected in about 50% of patients with optic nerve sheath diameter or perioptic subarachnoid space measurements below these cutoff values compared with <2% of patients with optic nerve sheath diameter or perioptic subarachnoid space measurements above these cutoff values. Following surgical ligation of the fistula, optic nerve sheath diameter increased from 4.0 to 5.3 mm and the perioptic subarachnoid space increased from 0.5 to 1.2 mm ( P  < .001)., Conclusions: Concerns about a spinal CSF leak should not be dismissed in patients with orthostatic headache when conventional imaging findings are normal, and simple optic nerve sheath MR imaging measurements can help decide if more imaging needs to be performed in this patient population., (© 2024 by American Journal of Neuroradiology.)

Spontaneous intracranial hypotension (SIH) is associated with cerebrospinal fluid (CSF) hypovolemia, often from a traumatic dural tear from a calcified spinal osteophyte. Visualizing osteophytes on CT imaging can guide decision making on candidate leak sites. We report the atypical case of a 41-year-old woman whose ventral CSF leak was associated with an osteophyte that resorbed over an 18-month period. Full workup and treatment were delayed due to unexpected pregnancy and completion of gestational cycle with delivery of a healthy term infant. The patient initially presented with persistent orthostatic headaches with nausea and blurred vision. Initial MRI suggested brain sagging among other findings consistent with SIH. CT myelogram showed an extensive thoracic CSF leak with a prominent ventral T11-T12 osteophyte and multiple small disc herniations. The patient did not respond to epidural blood patches and deferred additional imaging due to her pregnancy. CT myelography performed 5 months post-partum showed an absence of the osteophyte; a follow-up digital subtraction myelogram performed 10 months post-partum showed evidence of source leak at T11-T12 level. T11-T12 laminectomy visualized and repaired a 5 mm ventral dural defect with symptom resolution. This report highlights the potential for a resorbed osteophyte to be the causative agent for long-standing dural tears that do not show visible calcifications on myelography., Competing Interests: Declaration of conflicting interestsThe author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: Dr Robbins serves on the Board of Directors of the American Headache Society and the New York State Neurological Society, the editorial boards of Continuum and Current Pain and Headache Reports, and receives book royalties from Wiley.

Background and Objective: Spontaneous intracranial hypotension (SIH) from CSF leak commonly produces headache. It also may produce sagging brain syndrome (SBS), often with neurocognitive symptoms indistinguishable from behavioral-variant frontotemporal dementia (bvFTD). The authors describe a new clinical sign that appears to be pathognomonic of SBS., Methods: We reviewed medical records and brain imaging in patients seen at our 2 centers who presented with SIH, SBS, and bvFTD symptoms., Results: There were 51 patients (12 women, 39 men) with mean age 55.5 years (range, 26-70 years). MRI showed severe brain sagging in all. Thirteen patients displayed repetitive flexion with breath-holding at the time of clinical presentation. Five patients had repetitive flexion with breath-holding, which resolved before presenting for evaluation. Thus, 35.3% (18) of 51 patients with SBS displayed seemingly compulsive repetitive flexion with breath-holding., Discussion: Compulsive repetitive flexion with breath-holding appears to be pathognomonic of SBS, deserving the acronym CoRFBiS (compulsive repetitive flexion with breath-holding in SBS). CoRFBiS should alert the clinician to SBS with SIH as the proximate cause of the clinical constellation, rather than bvFTD., Competing Interests: The authors report no relevant disclosures. Full disclosure form information provided by the authors is available with the full text of this article at Neurology.org/cp., (© 2024 American Academy of Neurology.)

An adolescent male presented with orthostatic headaches following head trauma. MRI showed cerebellar tonsil displacement and a bony defect in the clival skull base. Digital subtraction myelography (DSM) confirmed a cerebrospinal fluid-venous fistula (CVF). This was repaired endoscopically. CVFs cause uncontrolled flow of CSF into the venous system resulting in symptoms of intracranial hypotension. They're often difficult to identify on initial imaging. This is the first reported CVF originating in the central skull base, and the first treated via endoscopic trans-nasal approach. CVFs may elude initial imaging, making DSM crucial for unexplained spontaneous intracranial hypotension. Laryngoscope, 134:645-647, 2024., (© 2023 The American Laryngological, Rhinological and Otological Society, Inc.)

Positioned along the ventral surface of the pons, proximal superior cerebellar artery (SCA) aneurysms account for only 1.7% of all intracranial aneurysms [1]. Unlike more commonly encountered basilar artery aneurysms, patients often experience good outcomes when treated via endovascular coiling or surgical clipping [1,2]. These lesions frequently have a lateral projection and paucity of perforator arteries [2]. With further development of endoscopic endonasal techniques, access to this region is possible via a direct frontal exposure to the ventral brainstem, basilar artery and branching vessels. To date, there are only a limited number of reports describing an endoscopic endonasal transclival (EETC) approach for surgical clipping [3-5]. In this operative video, we detail the surgical clipping of a cerebellar arteriovenous malformation feeding vessel and an associated aneurysm using the EETC approach in a 59-year-old woman who presented with sudden onset of a severe headache. The feeding vessel and aneurysm's midline location, just below the take-off of the SCA made it a good candidate for this surgery. Major steps included in this video include 1) transsphenoidal exposure of and subsequent drilling of the clivus, 2) dural opening into the pre-pontine cistern and dissection of the aneurysm, 3) clipping of the aneurysm, and 4) multi-layered closure of the skull base defect. Overall, the patient tolerated the procedure well and was found to have no residual filling of the aneurysm or the AVM feeding vessel at 2-year follow-up. EETC is a viable surgical option for the treatment of aneurysm located along the midline of the pre-pontine cistern., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 Elsevier Ltd. All rights reserved.)

Background: Cranial and spinal cerebrospinal fluid (CSF) leaks are associated with opposite CSF fluid dynamics. The differing pathophysiology between spontaneous cranial and spinal CSF leaks are, therefore, mutually exclusive in theory., Observations: A 66-year-old female presented with tension pneumocephalus. The patient underwent computed tomography (CT) scanning, which demonstrated left-sided tension pneumocephalus, with an expanding volume of air directly above a bony defect of the tegmen tympani and mastoideum. The patient underwent a left middle fossa craniotomy for repair of the tegmen CSF leak. In the week after discharge, she developed a recurrence of positional headaches and underwent head CT. Further magnetic resonance imaging of the brain and thoracic spine showed bilateral subdural hematomas and multiple meningeal diverticula., Lessons: Cranial CSF leaks are caused by intracranial hypertension and are not associated with subdural hematomas. Clinicians should maintain a high index of suspicion for intracranial hypotension due to spinal CSF leak whenever "otogenic" pneumocephalus is found. Close postoperative follow-up and clinical monitoring for symptoms of intracranial hypotension in any patients who undergo repair of a tegmen defect for otogenic pneumocephalus is recommended.

Background: Postoperative spinal cerebrospinal fluid (CSF) leaks are common but rarely cause extensive CSF collections that require specialized imaging to detect the site of the dural breach., Objective: To investigate the use of digital subtraction myelography (DSM) for patients with extensive extradural CSF collections after spine surgery., Methods: A retrospective review was performed to identify a consecutive group of patients with extensive postoperative spinal CSF leaks who underwent DSM., Results: Twenty-one patients (9 men and 12 women) were identified. The mean age was 46.7 years (range, 17-75 years). The mean duration of the postoperative CSF leak was 3.3 years (range, 3 months to 21 years). MRI showed superficial siderosis in 6 patients. DSM showed the exact location of the CSF leak in 19 (90%) of the 21 patients. These 19 patients all underwent surgery to repair the CSF leak, and the location of the CSF leak could be confirmed intraoperatively in all 19 patients. In 4 (19%) of the 21 patients, DSM also showed a CSF-venous fistula at the same location as the postoperative dural tear., Conclusion: In this study, DSM had a 90% detection rate of visualizing the exact site of the dural breach in patients with extensive postoperative spinal CSF leaks. The coexistence of a CSF-venous fistula in addition to the primary dural tear was present in about one-fifth of patients. The presence of a CSF-venous fistula should be considered if CSF leak symptoms persist in spite of successful repair of a durotomy., (Copyright © Congress of Neurological Surgeons 2023. All rights reserved.)

Objective: Spontaneous spinal cerebrospinal fluid (CSF) leaks cause intracranial hypotension (SIH) and also may cause infratentorial superficial siderosis (iSS) but the rate of development among different CSF leak types and outcome of treatment are not known. We determined the time interval from SIH onset to iSS and the outcome of treatment., Methods: A total of 1,589 patients with SIH underwent neuroimaging and iSS was detected in 57 (23 men and 34 women, mean age = 41.3 years [3.6%]). We examined the type of underlying CSF leak by various imaging modalities. Percutaneous and surgical procedures were used to treat the CSF leaks., Results: The iSS was detected in 46 (10.3%) of 447 patients with ventral CSF leaks, in 2 (3.9%) of 51 patients with dural ectasia, in 5 (2.6%) of 194 patients with CSF-venous fistulas, in 4 (0.9%) of 457 patients with simple meningeal diverticula, and in none of the 101 patients with lateral CSF leaks or the 339 patients with leaks of indeterminate origin (p < 0.001). The estimated median latency period from SIH onset to iSS was 126 months. Ventral CSF leaks could not be eliminated with percutaneous procedures in any patient and surgical repair was associated with low risk (<5%) and resulted in resolution of the CSF leak in all patients in whom the exact site of the CSF leak could be determined. Other types of CSF leak were treated with percutaneous or surgical procedures., Interpretation: The iSS can develop in most types of spinal CSF leak, including CSF-venous fistulas, but mainly in chronic ventral CSF leaks, which require surgical repair. ANN NEUROL 2023;93:64-75., (© 2022 American Neurological Association.)

Introduction: Due to loss of brain buoyancy, spontaneous spinal cerebrospinal fluid (CSF) leaks cause orthostatic headaches but also can cause symptoms indistinguishable from behavioral variant frontotemporal dementia (bvFTD) due to severe brain sagging (including the frontal and temporal lobes), as visualized on brain magnetic resonance imaging. However, the detection of these CSF leaks may require specialized spinal imaging techniques, such as digital subtraction myelography (DSM)., Methods: We performed DSM in the lateral decubitus position under general anesthesia in 21 consecutive patients with frontotemporal dementia brain sagging syndrome (4 women and 17 men; mean age 56.2 years [range: 31-70 years])., Results: Nine patients (42.8%) were found to have a CSF-venous fistula, a recently discovered type of CSF leak that cannot be detected on conventional spinal imaging. All nine patients underwent uneventful surgical ligation of the fistula. Complete or near-complete and sustained resolution of bvFTD symptoms was obtained by all nine patients, accompanied by reversal of brain sagging, but in only three (25.0%) of the twelve patients in whom no CSF-venous fistula could be detected ( P = 0.0011), and who were treated with non-targeted therapies., Discussion: Concerns about a spinal CSF leak should not be dismissed in patients with frontotemporal brain sagging syndrome, even when conventional spinal imaging is normal. However, even with this specialized imaging the source of the loss of spinal CSF remains elusive in more than half of patients., Competing Interests: The authors report no disclosures. Author disclosures are available in the supporting information., (© 2022 The Authors. Alzheimer's & Dementia: Translational Research & Clinical Interventions published by Wiley Periodicals LLC on behalf of Alzheimer's Association.)

A spinal CSF-venous fistula is an increasingly recognized type of CSF leak that causes spontaneous intracranial hypotension. The detection of these fistulas requires specialized imaging such as digital subtraction myelography or dynamic CT myelography, and several treatment options are available. A novel treatment for these CSF-venous fistulas consisting of transvenous embolization with the liquid embolic agent Onyx has been described recently, but some patients require further treatment if embolization fails. The purpose of this study was to evaluate the safety and effectiveness of surgery following transvenous embolization. In a series of 6 consecutive patients who underwent surgical ligation of the fistula after endovascular embolization, there were no surgical complications. Postoperatively, complete resolution of symptoms was reported by 5 of the 6 patients, and brain MR imaging findings of spontaneous intracranial hypotension resolved in all patients. This study suggests that surgical ligation of spontaneous spinal CSF-venous fistulas after endovascular embolization is effective and safe., (© 2022 by American Journal of Neuroradiology.)

Background: Spontaneous intracranial hypotension is diagnosed with an increasing frequency, but epidemiologic data are scarce. The aim of this study was to determine the incidence rate of spontaneous intracranial hypotension in a defined population., Methods: Using a prospectively maintained registry, all patients with spontaneous intracranial hypotension residing in Beverly Hills, California, evaluated at our Medical Center between 2006 and 2020 were identified in this population-based incidence study. Our Medical Center is a quaternary referral center for spontaneous intracranial hypotension and is located within 1.5 miles from downtown Beverly Hills., Results: A total of 19 patients with spontaneous intracranial hypotension were identified. There were 12 women and seven men with a mean age of 54.5 years (range, 28 to 88 years). The average annual incidence rate for all ages was 3.7 per 100,000 population (95% confidence interval [CI]: 2.0 to 5.3), 4.3 per 100,000 for women (95% CI, 1.9 to 6.7) and 2.9 per 100,000 population for men (95% CI, 0.8 to 5.1)., Conclusion: This study, for the first time, provides incidence rates for spontaneous intracranial hypotension in a defined population.

Background and Objectives: Superficial siderosis, bibrachial amyotrophy, and spinal cord herniation are unusual but serious long-term sequelae of persistent spontaneous spinal CSF leaks in patients with spontaneous intracranial hypotension (SIH), particularly ventral spinal CSF leaks. However, the risk of developing such sequelae has not been established in this population. We undertook this study to determine the risk of these serious complications of persistent ventral spinal CSF leaks., Methods: This cohort study was conducted using data from a prospectively maintained database of patients who meet the modified International Classification of Headache Disorders, 3rd edition criteria for SIH. The patient population consisted of a consecutive group of patients with SIH and persistent ventral spinal CSF leaks who were first seen within 1 year of onset of SIH symptoms and who had at least 1 year of follow-up., Results: Among 51 patients with SIH and a persistent ventral spinal CSF leak, superficial siderosis developed in 6 patients and bibrachial amyotrophy in 2 patients during 280 patient-years of follow-up. The probability of these complications increased from 0% at 48 months to 4.5% (95% confidence interval [CI] 1.0%-28.0%) at 56 months, 10.5% (95% CI 3.0%-36.4%) at 96 months, 32.7% (95% CI 15.0%-62.8%) at 144 months, and 57.9% (95% CI 30.2%-87.6%) at 192 months. None of the patients developed spinal cord herniation., Discussion: Among patients with SIH and a persistent ventral spinal CSF leak, the risk of developing serious long-term sequelae is considerable. This study shows that early treatment of a ventral spinal CSF leak offers a unique opportunity to prevent neurologic disability from superficial siderosis and bibrachial amyotrophy., (© 2021 American Academy of Neurology.)

Objective: To determine the frequency of multiple spinal CSF leaks in a recent group of patients with spontaneous intracranial hypotension (SIH) who were investigated with digital subtraction myelography (DSM)., Methods: This observational study was conducted using data from a prospectively maintained data base of patients who meet the International Classification of Headache Disorders, third edition, criteria for SIH. The patient population consisted of a consecutive group of 745 patients with SIH who underwent DSM between March 2009 and February 2020. Based on the results of DSM, participants were classified according to the type and number of spinal CSF leaks., Results: Among 398 patients with SIH and extradural CSF on spinal imaging, multiplicity of CSF leaks was observed in none of 291 patients with type 1a ventral leaks and in 4 (6.2%) of 65 patients with type 1b (postero-) lateral leaks. Among 97 patients with SIH from spinal CSF-venous fistulas (type 3 leaks) who did not have extradural CSF on spinal imaging, 9 patients (9.3%) had multiple fistulas ( p < 0.0001 for comparison between groups). Type 3 and type 1a or 1b CSF leaks coexisted in an additional 5 patients., Conclusions: Among patients with SIH, multiplicity of CSF leaks was observed radiographically in none of the patients with ventral leaks, in 6% of patients with lateral leaks, and in 9% of patients with CSF-venous fistulas. These results suggest that patients with SIH can be reassured that the occurrence of multiple CSF leaks is negligible to uncommon at most, depending on the type of CSF leak., (© 2021 American Academy of Neurology.)

Background: Spontaneous intracranial hypotension has historically been a poorly understood pathology that is often unrecognized and undertreated. Even more rarely has it been described in pediatric patients with an otherwise benign past medical history., Observations: Herein the authors describe one of the youngest patients ever reported, a 2-year-old girl who developed severe headaches, nausea, and vomiting and experienced headache relief after lying down. Imaging revealed tonsillar herniation 14 mm below the foramen magnum, presumed to be a Chiari malformation, along with extensive dural cysts starting from thoracic level T2 down to the sacrum. She was found to have streaky skin pigmentary variation starting from the trunk down to her feet. Genetic analysis of skin biopsies revealed mosaicism for an isodicentric marker chromosome (10p15.3-10q11.2 tetrasomy) in 27%-50% of cells. After undergoing a suboccipital and cervical decompression at an outside institution, she continued to be symptomatic. She was referred to the authors' hospital, where she was diagnosed with spontaneous intracranial hypotension., Lessons: After receiving a series of epidural blood patches, the patient experienced almost complete relief of her symptoms. To the authors' knowledge, this is the first time this chromosomal anomaly has ever been reported in a living child, and this may represent a new genetic association with dural ectasia., Competing Interests: Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper., (© 2021 The authors.)

Objective: To determine the occurrence of cerebrospinal fluid (CSF)-venous fistulas, a type of spinal CSF leak that cannot be detected with routine computerized tomography myelography, among patients with orthostatic headaches but normal brain and spine magnetic resonance imaging., Background: Spontaneous spinal CSF leaks cause orthostatic headaches but their detection may require sophisticated spinal imaging techniques., Methods: A prospective cohort study of patients with orthostatic headaches and normal brain and conventional spine imaging who underwent digital subtraction myelography (DSM) to look for CSF-venous fistulas, between May 2018 and May 2020, at a quaternary referral center for spontaneous intracranial hypotension., Results: The mean age of the 60 consecutive patients (46 women and 14 men) was 46 years (range, 13-83 years), who had been suffering from orthostatic headaches between 1 and 180 months (mean, 43 months). DSM demonstrated a spinal CSF-venous fistula in 6 (10.0%; 95% confidence interval [CI]: 3.8-20.5%) of the 60 patients. The mean age of these five women and one man was 50 years (range, 41-59 years). Spinal CSF-venous fistulas were identified in 6 (19.4%; 95% CI: 7.5-37.5%) of 31 patients with spinal meningeal diverticula but in none (0%; 95% CI: 0-11.9%) of the 29 patients without spinal meningeal diverticula (p = 0.024). All CSF-venous fistulas were located in the thoracic spine. All patients underwent uneventful surgical ligation of the fistula. Complete and sustained resolution of symptoms was obtained in five patients, while in one patient, partial recurrence of symptoms was noted 3 months postoperatively., Conclusion: Concerns about a spinal CSF leak should not be dismissed in patients suffering from orthostatic headaches when conventional imaging turns out to be normal, even though the yield of identifying a CSF-venous fistula is low., (© 2021 American Headache Society.)