Myelographic Techniques for the Localization of CSF-Venous Fistulas: Updates in 2024.

CSF-venous fistulas (CVFs) are a common cause of spontaneous intracranial hypotension. Despite their relatively frequent occurrence, they can be exceedingly difficult to detect on imaging. Since the initial description of CVFs in 2014, the recognition and diagnosis of this type of CSF leak has continually increased. As a result of multi-institutional efforts, a wide spectrum of imaging modalities and specialized techniques for CVF detection is now available. It is important for radiologists to be familiar with the multitude of available techniques, because each has unique advantages and drawbacks. In this article, we review the spectrum of imaging modalities available for the detection of CVFs, explain the advantages and disadvantages of each, provide typical imaging examples, and discuss provocative maneuvers that may improve the conspicuity of CVFs. Discussed modalities include conventional CT myelography, dynamic myelography, digital subtraction myelography, conebeam CT myelography, decubitus CT myelography by using conventional energy-integrating detector scanners, decubitus photon counting CT myelography, and intrathecal gadolinium MR myelography. Additional topics to be discussed include optimal patient positioning, respiratory techniques, and intrathecal pressure augmentation.

Diagnostic Performance of Decubitus Photon-Counting Detector CT Myelography for the Detection of CSF-Venous Fistulas.

BACKGROUND AND PURPOSE: CSF-venous fistulas are a common cause of spontaneous intracranial hypotension. Lateral decubitus digital subtraction myelography and CT myelography are the diagnostic imaging standards to identify these fistulas. Photon-counting CT myelography has technological advantages that might improve CSF-venous fistula detection, though no large studies have yet assessed its diagnostic performance. We sought to determine the diagnostic yield of photon-counting detector CT myelography for detection of CSF-venous fistulas in patients with spontaneous intracranial hypotension. MATERIALS AND METHODS: We retrospectively searched our database for all decubitus photon-counting detector CT myelograms performed at our institution since the introduction of the technique in our practice. Per our institutional workflow, all patients had prior contrast-enhanced brain MR imaging and spine MR imaging showing no extradural CSF. Two neuroradiologists reviewed preprocedural brain MRIs, assessing previously described findings of intracranial hypotension (Bern score). Additionally, 2 different neuroradiologists assessed each myelogram for a definitive or equivocal CSF-venous fistula. The yield of photon-counting detector CT myelography was calculated and stratified by the Bern score using low-, intermediate-, and high-probability tiers. RESULTS: Fifty-seven consecutive photon-counting detector CT myelograms in 57 patients were included. A single CSF-venous fistula was definitively present in 38/57 patients. After we stratified by the Bern score, a definitive fistula was seen in 56.0%, 73.3%, and 76.5% of patients with low-, intermediate-, and high-probability brain MR imaging, respectively. CONCLUSIONS: Decubitus photon-counting detector CT myelography has an excellent diagnostic performance for the detection of CSF-venous fistulas. The yield for patients with intermediate- and high-probability Bern scores is at least as high as previously reported yields of decubitus digital subtraction myelography and CT myelography using energy-integrating detector scanners. The yield for patients with low-probability Bern scores appears to be greater compared with other modalities. Due to the retrospective nature of this study, future prospective work will be needed to compare the sensitivity of photon-counting detector CT myelography with other modalities.

Clinical and imaging outcomes of 100 patients with cerebrospinal fluid-venous fistulas treated by transvenous embolization.

BACKGROUND: Cerebrospinal fluid-venous fistulas (CSFVF) are a common cause of spontaneous intracranial hypotension (SIH). Transvenous embolization has emerged as a reliable treatment option. We review the clinical presentation, imaging, and clinical outcomes of 100 consecutive CSFVF patients who underwent embolization over 2 years. METHODS: Baseline clinical characteristics, imaging findings (including Bern SIH score), technical outcomes, and long-term imaging and clinical outcomes were collected. All patients had at least 3 months of clinical follow-up and had baseline MRI. 99/100 patients underwent follow-up imaging at ≥3 months post-treatment. RESULTS: 100 patients were included. Mean imaging and clinical follow-up duration was 8.3±7.7 months and 15.0±6.8 months, respectively. The mean duration of symptoms before embolization was 40.9±52 months. Mean baseline Bern SIH score was 5.9±3.3. The most common baseline symptoms were headache (96 patients), tinnitus (55 patients), and cognitive dysfunction (44 patients). Technical success rate was 100%. Mean post-treatment Bern SIH score was 0.9±1.6 (P<0.0001). Following treatment, 95% of patients reported significant improvement or resolution in symptoms (58 patients reporting resolution and 37 reporting improvement). 5 patients reported no improvement. There were no major procedural or periprocedural complications. 10 patients had minor procedural complications that did not result in any change in management (Onyx emboli, venous perforation). 19 patients had rebound intracranial hypertension requiring acetazolamide therapy. 7 patients had recurrent fistula at the initially treated level. CONCLUSIONS: Transvenous embolization of CSFVF in SIH patients is safe and effective with a 95% treatment response, significant improvement in imaging outcomes, and a very low rate of complications.

Timing and Predictors of T2-Lesion Resolution in Patients With Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease.

OBJECTIVES: To determine the timing and predictors of T2-lesion resolution in myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). METHODS: This retrospective observational study using standard-of-care data had inclusion criteria of MOGAD diagnosis, ≥2 MRIs 12 months apart, and ≥1 brain/spinal cord T2-lesion. The median (interquartile range [IQR]) number of MRIs (82% at disease onset) per-patient were: brain, 5 (2-8); spine, 4 (2-8). Predictors of T2-lesion resolution were assessed with age- and sex-adjusted generalized estimating equations and stratified by T2-lesion size (small <1 cm; large ≥1 cm). RESULTS: We studied 583 T2-lesions (brain, 512 [88%]; spinal cord, 71 [12%]) from 55 patients. At last MRI (median follow-up 54 months [IQR 7-74]) 455 T2-lesions (78%) resolved. The median (IQR) time to resolution was 3 months (1.4-7.0). Small T2-lesions resolved more frequently and faster than large T2-lesions. Acute T1-hypointensity decreased the likelihood (odds ratio [95% CI]) of T2-lesion resolution independent of size (small: 0.23 [0.09-0.60], p = 0.002; large: 0.30 [0.16-0.55], p < 0.001), whereas acute steroids favored resolution of large T2-lesions (1.75 [1.01-3.03], p = 0.046). Notably, 32/55 (58%) T2-lesions resolved without treatment. DISCUSSION: The high frequency of spontaneous T2-lesion resolution suggests that this represents MOGAD's natural history. The speed of T2-lesion resolution and influence of size, corticosteroids, and T1-hypointensity on this phenomenon gives insight into MOGAD pathogenesis.

Incremental diagnostic yield and clinical outcomes of lateral decubitus CT myelogram immediately following negative lateral decubitus digital subtraction myelogram.

INTRODUCTION: Spontaneous intracranial hypotension (SIH) caused by a spinal cerebrospinal fluid (CSF) leak classically presents with orthostatic headache. Digital subtraction myelography (DSM) has a well-established diagnostic yield in the absence of extradural spinal collection. At our institution, DSM is followed by lateral decubitus CT myelogram (LDCTM) in the same decubitus position to increase diagnostic yield of the combined study. We evaluated the incremental diagnostic yield of LDCTM following negative DSM and reviewed patient outcomes. METHODS: Retrospective review of consecutive DSMs with subsequent LDCTM from April 2019 to March 2021 was performed. Combined reports were reviewed, and studies with positive DSMs were excluded. Of the exams with negative DSM, only studies with LDCTM reports identifying potential leak site were included. Interventions and follow-up clinical notes were reviewed to assess symptoms improvement following treatment. RESULTS: Of the 83 patients with negative DSMs, 11 (13.2%) had positive leak findings on LDCTMs, and 21 (25.3%) were equivocal. Of 11 positive LDCTMs, 6 leaks were nerve sheath tears (NSTs) and 5 were CSF-venous fistulas (CVFs). 10/11 (90.9%) had intervention and follow-up, with 9/10 (90%) having positive clinical outcome. Of the 21 equivocal LDCTM patients (19 CVFs and 2 NSTs), 15 (71.4%) had interventions and follow-up, with 3/15 (20.0%) with positive clinical outcomes. CONCLUSION: LDCTM following negative DSM has an incremental diagnostic yield up to 38.6%, with up to 14.5% of positive patient outcomes following treatment. LDCTM should be considered after DSM to maximize diagnostic yield of the combined exam.

Tumefactive Demyelination in MOG Ab-Associated Disease, Multiple Sclerosis, and AQP-4-IgG-Positive Neuromyelitis Optica Spectrum Disorder.

BACKGROUND AND OBJECTIVES: Studies on tumefactive brain lesions in myelin oligodendrocyte glycoprotein-immunoglobulin G (IgG)-associated disease (MOGAD) are lacking. We sought to characterize the frequency clinical, laboratory, and MRI features of these lesions in MOGAD and compare them with those in multiple sclerosis (MS) and aquaporin-4-IgG-positive neuromyelitis optica spectrum disorder (AQP4+NMOSD). METHODS: We retrospectively searched 194 patients with MOGAD and 359 patients with AQP4+NMOSD with clinical/MRI details available from the Mayo Clinic databases and included those with ≥1 tumefactive brain lesion (maximum transverse diameter ≥2 cm) on MRI. Patients with tumefactive MS were identified using the Mayo Clinic medical record linkage system. Binary multivariable stepwise logistic regression identified independent predictors of MOGAD diagnosis; Cox proportional regression models were used to assess the risk of relapsing disease and gait aid in patients with tumefactive MOGAD vs those with nontumefactive MOGAD. RESULTS: We included 108 patients with tumefactive demyelination (MOGAD = 43; AQP4+NMOSD = 16; and MS = 49). Tumefactive lesions were more frequent among those with MOGAD (43/194 [22%]) than among those with AQP4+NMOSD (16/359 [5%], p < 0.001). Risk of relapse and need for gait aid were similar in tumefactive and nontumefactive MOGAD. Clinical features more frequent in MOGAD than in MS included headache (18/43 [42%] vs 10/49 [20%]; p = 0.03) and somnolence (12/43 [28%] vs 2/49 [4%]; p = 0.003), the latter also more frequent than in AQP4+NMOSD (0/16 [0%]; p = 0.02). The presence of peripheral T2-hypointense rim, T1-hypointensity, diffusion restriction (particularly an arc pattern), ring enhancement, and Baló-like or cystic appearance favored MS over MOGAD (p ≤ 0.001). MRI features were broadly similar in MOGAD and AQP4+NMOSD, except for more frequent diffusion restriction in AQP4+NMOSD (10/15 [67%]) than in MOGAD (11/42 [26%], p = 0.005). CSF analysis revealed less frequent positive oligoclonal bands in MOGAD (2/37 [5%]) than in MS (30/43 [70%], p < 0.001) and higher median white cell count in MOGAD than in MS (33 vs 6 cells/µL, p < 0.001). At baseline, independent predictors of MOGAD diagnosis were the presence of somnolence/headache, absence of T2-hypointense rim, lack of T1-hypointensity, and no diffusion restriction (Nagelkerke R 2 = 0.67). Tumefactive lesion resolution was more common in MOGAD than in MS or AQP4+NMOSD and improved model performance. DISCUSSION: Tumefactive lesions are frequent in MOGAD but not associated with a worse prognosis. The clinical, MRI, and CSF attributes of tumefactive MOGAD differ from those of tumefactive MS and are more similar to those of tumefactive AQP4+NMOSD with the exception of lesion resolution, which favors MOGAD.

Frequency of New or Enlarging Lesions on MRI Outside of Clinical Attacks in Patients With MOG-Antibody-Associated Disease.

OBJECTIVE: To determine the frequency of new or enlarging T2-hyperintense or enhancing lesions outside of clinical attacks in myelin-oligodendrocyte-glycoprotein-antibody-associated-disease (MOGAD) versus multiple sclerosis (MS) and aquaporin-4 antibody-positive-neuromyelitis-optica-spectrum-disorder (AQP4+NMOSD). DESIGN/METHODS: We retrospectively included Mayo Clinic MOGAD patients with: 1) MOG-IgG positivity by live-cell-based-assay; 2) Fulfilling proposed MOGAD diagnostic criteria; 3) Baseline and follow-up paired MRIs without interval attacks. A neurologist and neuroradiologist reviewed MRIs (T2-FLAIR brain, T2 spine, and T1-post-gadolinium brain and spine) to identify new or enlarging lesions. A MOGAD subset was then compared to MS and AQP4+NMOSD patients, based on broadly similar inter-scan intervals. RESULTS: We included 105 MOGAD patients (median age, 31 years[range, 2-80]; 60% female) with 373 paired MRIs. In total, 10/105 (9.5%) patients and 13/373 (3%) scans had one or more new T2-lesions (brain, 12/213[6%]; spine, 1/160[0.6%]) and 8/367 (2%) had enhancing lesions. New brain lesions were less in MOGAD (1/25[4%]) than MS (14/26[54%], p<0.0001) but did not differ from AQP4+NMOSD (1/13[8%], p=1.0) in subgroup analysis. New spinal lesions were rare across groups (0-4%). CONCLUSIONS: New or enlarging MRI lesions rarely develop outside of clinical attacks in MOGAD differing from MS. Surveillance MRIs in MOGAD have limited utility with implications for clinical practice and trial design.

Frequency of Asymptomatic Optic Nerve Enhancement in a Large Retrospective Cohort of Patients With Aquaporin-4+ NMOSD.

BACKGROUND AND OBJECTIVES: Asymptomatic or persistent optic nerve enhancement in aquaporin-4 (AQP4)-immunoglobulin G (IgG)-positive neuromyelitis optica spectrum disorder (NMOSD) is thought to be rare. Improved understanding may have important implications for assessment of treatment efficacy in clinical trials and in clinical practice. Our objective was to characterize NMOSD interattack optic nerve enhancement. METHODS: This was a retrospective cohort study performed between 2000 and 2019 (median follow-up 5.5 [range 1-35] years) of patients with AQP4-IgG-positive optic neuritis (ON) evaluated at Mayo Clinic. MRI orbits were reviewed by a neuroradiologist, neuro-ophthalmologist, and neuroimmunologist blinded to the clinical history. Interattack optic nerve enhancement (>30 days after attack) was measured. The correlation between interattack enhancement and Snellen visual acuity (VA), converted to logarithm of the minimum angle of resolution (logMAR), at attack and at follow-up were assessed. RESULTS: A total of 198 MRI scans in 100 patients with AQP4-IgG+ NMOSD were identified, with 107 interattack MRIs from 78 unique patients reviewed. Seven scans were performed before any ON (median 61 days before attack [range 21-271 days]) and 100 after ON (median 400 days after attack [33-4,623 days]). Optic nerve enhancement was present on 18/107 (16.8%) interattack scans (median 192.5 days from attack [33-2,943]) of patients with preceding ON. On 15 scans, enhancement occurred at the site of prior attacks; the lesion location was unchanged, but the lesion length was shorter. Two scans (1.8%) demonstrated new asymptomatic lesions (prior scan demonstrated no enhancement). In a third patient with subjective blurry vision, MRI showed enhancement preceding detectable eye abnormalities on examination noted 15 days later. There was no difference in VA at preceding attack nadir (logMAR VA 1.7 vs 2.1; p = 0.79) or long-term VA (logMAR VA 0.4 vs 0.2, p = 0.56) between those with and without interattack optic nerve enhancement. DISCUSSION: Asymptomatic optic nerve enhancement occurred in 17% of patients with NMOSD predominantly at the site of prior ON attacks and may represent intermittent blood-brain barrier breakdown or subclinical ON. New asymptomatic enhancement was seen only in 2% of patients. Therapeutic clinical trials for NMOSD require blinded relapse adjudication when assessing treatment efficacy, and it is important to recognize that asymptomatic optic nerve enhancement can occur in patients with ON.

Diagnostic Yield of Intrathecal Gadolinium MR Myelography for CSF Leak Localization.

PURPOSE: Intrathecal gadolinium magnetic resonance (MR) myelography can be used to localize various types of spinal cerebrospinal fluid (CSF) leaks; however, its diagnostic yield is not well known. We sought to determine the diagnostic yield of MR myelography in patients with spontaneous intracranial hypotension. METHODS: A retrospective review was performed on all patients who had undergone intrathecal gadolinium MR myelography at our institution from 2002 to 2020 for suspected spinal CSF leak. The MR myelography images were reviewed for the presence or absence of a spinal CSF leak site. Images were also evaluated for the presence an extradural fluid collection. RESULTS: A total of 97 patients were included in the final cohort. The average age was 52.6 years; 67.0% were female, 4 patients underwent 2 examinations each, yielding a total of 101 MR myelograms. The source of a spinal CSF leak was localized in 14 patients. The diagnostic yield for CSF leak localization on intrathecal gadolinium MR myelography was 14/101 (13.9%) per GdM examination and 14/97 (14.4%) per patient. Among the subset of patients without extradural fluid collections, the yield was 15.7% per examination. All detected leaks were either CSF-venous fistulas or distal nerve root sleeve tears. CONCLUSION: Intrathecal gadolinium MR myelography is capable of localizing CSF-venous fistulas and distal nerve root sleeve tears; however, our data show that it has a limited diagnostic yield. We suggest that other modalities may be a better first step before attempting intrathecal gadolinium MR myelography.

Utility of CT venography in monitoring stent patency in idiopathic intracranial hypertension: retrospective single-center study.

BACKGROUND: Cerebral venous sinus stenting is an established treatment for patients with idiopathic intracranial hypertension (IIH), refractory to medical management and with stenotic venous sinus on conventional cerebral venography. Currently, there are no clear guidelines on optimal noninvasive imaging modality for routine post-stenting follow-up. We investigated diagnostic yield of CT venography (CTV) for evaluation of stent patency. METHODS: We reviewed our clinical database of patients with a diagnosis of IIH, who underwent stenting of stenotic transverse or transverse/sigmoid sinus junction. Patients who had follow-up CTVs after more than 30 days were included in the final study group. All CTVs were reviewed by two readers for in-stent thrombosis, extrinsic stent compression, juxta-stent stenosis, and new contralateral venous sinus stenosis. Inter-observer agreement and association of stent patency with clinical outcomes (headache and papilledema) were assessed. RESULTS: In all 36 included patients with 38 cerebral venograms for stenting, follow-up CTVs demonstrated adequate opacifications of the venous sinuses and stents for confident evaluation for in-stent thrombosis, extrinsic stent compression, juxta-stent stenosis, and new contralateral venous sinus stenosis, with inter-observer agreement coefficient of 0.7, 1.0, 0.8, and 1.0 respectively. Association between abnormal CTV and higher rates of persistent headache and lower rates of headache improvement/resolution was statistically significant (P-value of 0.01). CONCLUSION: CTV is a reliable noninvasive imaging modality for evaluation of cerebral venous sinuses and stent patency following treatment of idiopathic intracranial hypertension with venous sinus stenting and could be used as a routine follow-up study.