Cortical Veins Research Articles

The dilated cortical veins found in multiple sclerosis can explain the reduction in glymphatic flow

The dilated cortical veins found in multiple sclerosis can explain the reduction in glymphatic flow

Vascular Repair for Iatrogenic Injury during Microsurgical Procedures: Clinical Investigation and Review of 18 Cases at a Single Institution.

Intracranial vascular injury (VI) due to surgery is a critical complication that can lead to serious neurologic deficits. To our knowledge, only a few review articles on VI during an operation have been published so far. We retrospectively investigated the type, cause, and measurement of VI during surgery at our institution. Unexpected VI cases occurred in 18 of 2,228 craniotomy procedures, including 794 aneurysm clippings and 357 tumor resections. We investigated the causes and coping techniques of the VI cases, as well as their full details. There were six cases of aneurysm neck tear, one case of sylvian vein injury, and one case of superior trunk perforation during direct clipping. Regarding tumor resection procedures, nine cases of arterial injury and one case of cortical vein injury were extracted. Almost all VIs were caused by carelessness or basic manipulation mistakes. We repaired all these cases with simple placement of suture threads with or without pinch clips, flow alteration using bypass techniques, and in 16 cases no neurologic deficit or deterioration on imaging occurred; however, 3 patients were verified to have ischemic changes on postoperative imaging. Most VIs were directly caused by a simple error and carelessness of an operator or an assistant. Many of these injuries can be avoided if a basic set of rules are followed and remembered during the surgical procedure. However, the surgical procedure involves human work, and errors cannot be eradicated even upon maximum concentration levels. Neurosurgeons should be prepared for an eventual quick repair of an unexpected cerebral VI.

Decompressive Craniectomy in Postpartum Cortical venous Thrombosis Masquerading as Postspinal Headache: What the Obstetrician should know - A Case Report

Introduction: Cerebral venous sinus thrombosis is commonly seen during pregnancy or in the postpartum period due to multiple physiological changes resulting in an altered state of homeostasis and a hypercoagulable state. Cortical vein thrombosis is a subgroup of cerebral venous thrombosis where there is an involvement of the superficial cerebral veins and may occur with or without coexisting sinus venous thrombosis. Early identification of the diagnosis is of utmost importance to reduce morbidity and mortality. Differentiation from postspinal headache may be difficult until the occurrence of neurological features and a high level of suspicion for this condition is required. Contrast magnetic resonance imaging venography or computed tomography venography can confirm the diagnosis. Methods: We report a case of postpartum cortical venous thrombosis with malignant cerebral sinus venous thrombosis (CSVT). Results: The patient was managed with a lifesaving decompressive craniectomy (DC) procedure and anticoagulants with a favorable response. Conclusion: Obstetricians should be aware of this medical complication of pregnancy and of its management. Medical treatment with anticoagulants is the mainstay of therapy. Patients with malignant CSVT presenting with rapid and severe neurological decline carry a high mortality, and early DC in this subgroup can be lifesaving. Contraceptive agents containing estrogens should not be administered in patients with a previous history of CSVT, and prophylactic low-molecular-weight heparin should be administered throughout a subsequent pregnancy and continued into the puerperium.

Mastoid fluid signal in acute cerebral venous thrombosis is is associated with increased clot burden

PurposeMastoid air cell abnormalities in the form of hyperintense T2 fluid signal have been reported in cases of acute Cerebral Venous Thrombosis (CVT) without otologic infection and have been hypothesized to be a result of venous congestion rather than infectious mastoiditis. The aim of this study was to investigate a link between the spectrum of mastoid abnormalities and clot burden in patients with acute CVT. MethodsA retrospective study of adult patients admitted to the National Institute of Mental Health and Neurosciences between 2016 and 2023 who were diagnosed with acute CVT and had no clinical evidence of active or recent ear infections was conducted. Pre- and post-contrast MR Images were analyzed to identify the dural sinuses and/or cerebral veins involved and the presence of fluid signal in the mastoid. Fluid signal in the mastoid was graded from 0 to 3 as described by Shah et al- no fluid signal (grade 0), thin curvilinear hyperintensities (grade 1), thick crescenteric hyperintensities (grade 2), and complete hyperintensity (grade 3). Clot Burden Score (CBS) was calculated by assigning one point for each sinus involved, one point for extension of thrombus into the intracranial Internal Jugular Vein (IJV), one point for thrombosis of cortical veins and one point for thrombosis of deep cerebral veins. ResultsA total of 89 patients with acute CVT were included in the final analysis. Median time from presentation to MRI was 2 days (range 0–13). 51 patients (57.3%) had fluid signal in the mastoid air cells on T2-weighted images, of whom 33 showed mucosal contrast enhancement. Higher grade of fluid signal in the mastoid was present ipsilateral to the side of venous thrombosis in 59 out of 60 patients with posterior fossa CVT. CBS was significantly different between patients with different grades of fluid signal (p = 0.002). Grade 2–3 fluid signal was associated with higher clot burden (CBS > 3) in both the entire study population (n = 89) – OR = 8.281, 95 %CI: 2.758–24.866 (p < 0.001) and among patients with posterior fossa CVT – OR = 4.375, 95 %CI: 1.320–14.504 (p = 0.016). Among patients with posterior fossa CVT, grade 2–3 fluid signal was associated with left sided transverse and/or sigmoid sinus thrombosis – OR = 5.600, 95 %CI: 1.413–22.188 (p = 0.014), and extension of thrombosis into the IJV – OR = 4.606, 95 %CI: 1.162–18.262 (p = 0.030). ConclusionT2 fluid signal in the mastoid is associated with venous congestion in adults with acute CVT without evidence of otologic infection. Moderate-to-severe T2 fluid signal in the mastoid air cells is associated with increased clot burden.

Prognostic Value of Venous Outflow Profiles on Multiphase CT Angiography for the Patients with Acute Ischemic Stroke After Endovascular Thrombectomy.

To evaluate the prognostic value of venous outflow (VO) profiles evaluated on multiphase CTA (mCTA) for the patients with acute ischemic stroke (AIS) after endovascular thrombectomy (EVT). We retrospectively collected 150 patients with AIS who underwent pre-treatment CT perfusion (CTP) evaluation and subsequent EVT from April 2018 to April 2022. Three-phases (peak arterial phase, peak venous phase, late venous phase) CTA was reconstructed from CTP raw data, and VO was evaluated on three-phases CTA, respectively. Favorable VO was regarded as a cortical vein opacification score of 3-6, and unfavorable VO as a score of 0-2. Good outcome was defined as modified Rankin Scale score of 0-2 at 90 days after EVT. Multivariate logistic regression analysis was performed to explore the predictors of good outcome. Prognostic value was assessed and compared using receiver operating characteristic (ROC) curves and Delong test. We found that good outcome was achieved in 85 (56.7%) patients. Among the mCTA-derived VO profiles, only favorable peak venous phase VO was found to be independently associated with good outcome (P < 0.001). After integrating favorable peak venous phase VO with lower post-treatment National Institute of Health Stroke Scale score at 24 hours, successful recanalization and favorable hypoperfusion intensity ratio, the predictive ability for a good outcome was significantly improved than before (area under the ROC curve; 0.947 vs 0.881; P = 0.002). This study supports that favorable peak venous VO profiles on mCTA might be a promising biomarker in predicting the good outcome in patients with AIS after EVT.

Synchronous Superficial Middle Cerebral Vein Outflow Correlates Favorable Tissue Fate After Mechanical Thrombectomy for Acute Ischemic Stroke

The purpose of this study was to determine the association between hemispheric synchrony in venous outflow at baseline and tissue fate after mechanical thrombectomy (MT) for acute ischemic stroke (AIS). A two-center retrospective analysis involving AIS patients who underwent MT was performed. The four cortical veins of interest include the superficial middle cerebral vein (SMCV), sphenoparietal sinus (SS), vein of Labbé (VOL), and vein of Trolard (VOT). Baseline computed tomography perfusion data were used to compare the following outflow parameters between the hemispheres: first filling time (△FFT), time to peak (△TTP) and total filling time (△TFT). Synchronous venous outflow was defined as △FFT=0. Multivariable regression analyses were performed to evaluate the association of venous outflow synchrony with penumbral salvage, infarct growth, and intracranial hemorrhage (ICH) after MT. A total of 151 patients (71.4±13.2 years, 65.6% women) were evaluated. Patients with synchronous SMCV outflow demonstrated significantly greater penumbral salvage (41.3 mL vs. 33.1 mL, P=0.005) and lower infarct growth (9.0 mL vs. 14.4 mL, P=0.015) compared to those with delayed SMCV outflow. Higher △FFTSMCV (β=-1.44, P=0.013) and △TTPSMCV (β=-0.996, P=0.003) significantly associated with lower penumbral salvage, while higher △FFTSMCV significantly associated with larger infarct growth (β=1.09, P=0.005) and increased risk of ICH (odds ratio [OR]=1.519, P=0.047). Synchronous SMCV outflow is an independent predictor of favorable tissue outcome and low ICH risk, and thereby carries the potential as an auxiliary radiological marker aiding the treatment planning of AIS patients.

Dilatation of the bridging cerebral veins in multiple sclerosis correlates with fatigue and suggests an increase in pressure.

There is a significant increase in the parenchymal microvessel blood volume in the earliest forms of multiple sclerosis (MS) which may be due to venular dilatation. Increased cortical venous pressure could account for this finding. Venous pressure is also implicated in the physiology of fatigue. The purpose of this study is to discover if there is dilatation of the veins within the subarachnoid space in multiple sclerosis and to estimate the pressures required to maintain any enlargement found. These findings will be correlated with the fatigue symptoms found in MS. 103 patients with MS were compared with a control group of 50 patients. Post contrast 3DT1 images were used. The cross-sectional area of the bridging cortical veins and the vein of Galen were measured. In MS, the superficial territory cortical veins were 29% larger and the veins of Galen were 25% larger than the controls. There is evidence of a significant increase in the bridging vein transmural pressure in MS, estimated to be approximately 6.5 mmHg in the superficial cortical veins. MS patients with significant fatigue have larger cortical veins than those who are not significantly fatigued.

Retrospective Observational Study Amidst Myriad Conundrums and Myths of Pediatric Headaches: A Critique on Diagnostics and Effectiveness of Interventions.

Objective To study the etiological profile of pediatric headaches (PH) in a tertiary child neurology clinic and to determine the utility of diagnostics, interventions, and long-term prognosis. Methods Children (ages 4-15) observed over four years were recruited retrospectively. In primary headaches, the headache frequency and impact on quality of life (QOL) parameters at pre-treatment (T1) were compared post-treatment at follow-up (T2). Results Of the 311 eligible patients, 285 had primary headaches (Tension-Type Headache {TTH}: 156; Migraine: 129), and 26 had secondary headaches. The mean (±SD) onset age was 10 (±3) years with a male-to-female ratio of 2.3:1. Migraine was more common in children aged less than seven years (17/28) and TTH in older patients (146/283). The most common causes of secondary headache were intracranial hypertension (ICH) in 11/26 patients (fouridiopathic intracranial hypertension (IIH), four following aseptic meningitis, three with cortical vein thrombosis), and ophthalmologic causes in 7/26 (of these five had convergence insufficiency). Hypertension was a rare cause of secondary headaches (2/26 patients). Neuroimaging was performed in 173/311 (56%), primarily for parental anxiety (160/173; 92%), and was abnormal in only four. At T2 (Median time to follow-up: 29 months; Interquartile range: 22-37 months), data were collected in 207/285 patients with primary headaches (TTH: 109; Migraine: 98). In both migraine and TTH groups, there were statistically significant reductions (p-value <0.0001) in headache frequency and QOL parameters. Conclusion In our study, TTH wasthe most common cause of PH. Neuroimaging was normal in most cases. Psychological interventions were effective but underutilized. The symptoms of primary headaches improved significantly over time, despite poor adherence to prophylactic medications.

Cortical vein involvement and its influence in a cohort of adolescents with cerebral venous thrombosis

Background and purposeCortical vein thrombosis (CVT) is a rare form of cerebral venous sinus thrombosis (CVST) in adolescent patients that has received little attention. We aimed to analyze the clinical and radiological features of adolescents with CVST and investigate the effects of CVT involvement.MethodsPatients aged ≥ 10 to ≤ 18 years and diagnosed with CVST were identified at Xuanwu Hospital, Capital Medical University between January 2015 and August 2022 and divided into two groups according to the presence or absence of cortical vein involvement. Additionally, the patients were also categorized based on their sex. Clinical features, radiological characteristics, and 12-month follow-up outcomes were compared between the two groups.ResultsFifty-three adolescents, including 21 with CVT, were included (mean age: 15.2 ± 1.8 years; females, 54.7%). The CVT group was more likely to experience seizures (P = 0.028) and deterioration (28.6% vs. 6.2%, P = 0.047) during hospitalization than the non-CVT group. Poor short-term outcomes, based on the modified Rankin Scale (mRS) score at discharge, were more common in adolescents with CVT (P = 0.007). The proportions of patients showing edema (42.9% vs. 6.2%, P = 0.004) and mass effect (P = 0.015) were significantly higher in the CVT group. Recanalization was observed in 61.9% and 82.1% of the patients in the CVT and non-CVT groups, respectively, during the first imaging review (median, 22 days). After a 12-month follow-up, female adolescents had more frequent resident secondary headaches than male adolescents (52.9% vs. 12.5%; P = 0.014).ConclusionsCortical vein involvement in adolescents with CVST was associated with a higher risk of epilepsy at presentation, deterioration during hospitalization, edema, and mass effect on acute imaging. Moreover, cortical vein involvement may lead to worse short-term outcomes. Sex differences require consideration in etiological analyses and prolonged follow-ups.

Anterior ischemic stroke: Analysis of the multivariable CT-based models for prediction of clinical outcome

ObjectiveTo determine the predictive value of multiple CT-based measurements, individually and collectively, including arterial collateral filling (AC), tissue perfusion parameters, as well as cortical venous (CV) and medullary venous (MV) outflow, in patients with acute ischemic stroke (AIS). MethodsWe retrospectively reviewed a database of patients with AIS in the middle cerebral artery distribution, who underwent evaluation by multiphase CT-angiography and perfusion. AC pial filling was evaluated using a multiphase CTA imaging. The CV status was scored using the adopted PRECISE system based on contrast opacification of the main cortical veins. The MV status was defined by the degree of contrast opacification of medullary veins in one cerebral hemisphere as compared to the contralateral hemisphere. The perfusion parameters were calculated using FDA-approved automated software. A good clinical outcome was defined as a Modified Rankin Scale of 0-2 at 90 days. ResultsA total of 64 patients were included. Each of the CT-based measurements could predict clinical outcomes independently (P<0.05). AC pial filling and perfusion core based models did slightly better compared to each of the other models (AUC = 0.66). Among models with two variables, the perfusion core combined with MV status had the highest AUC=0.73 followed by a combination of MV status and AC (AUC=0.72). Multivariable modeling with all four variables resulted in the highest predictive value (AUC=0.77). ConclusionThe combination of arterial collateral flow, tissue perfusion, and venous outflow provides a more accurate prediction of clinical outcome in AIS than each variable alone. This additive effect of these techniques suggests that the information collected by each of these methods only partially overlaps.

Intracranial Hypotension and Bilateral Isolated Cortical Vein Thrombosis: A Case Report and Literature Review

Background: Intracranial hypotension (IH) is a clinical manifestation of low cerebrospinal fluid pressure with reports of isolated cortical venous thrombosis (ICVT) rarely described. Case Presentation: A 23-year-old man presented with postural headache followed by blurry vision and horizontal binocular diplopia with extreme gaze. Patient workup showed diffuse thickening and enhancement of the dura suggestive of intracranial hypotension and biparietal superficial cortical vein thrombosis was noted on imaging. The patient was started on folate and therapeutic anticoagulation and his condition improved. Conclusion: Patients with IH are at risk for CVT due to the dilation of the venous sinuses of the brain. Changes in the characteristics of the headache of the patient or the onset of new neurological symptoms should warrant further investigation. Prophylactic systemic anticoagulation might cautiously be indicated in high-risk patients with IH to prevent ICVT.

Utility of 3D T1-weighted turbo spin echo black blood sequence for the diagnosis of cerebral venous thrombosis.

Accurate assessment of dural sinus, deep and cortical venous thrombosis on MR imaging is challenging. The aim of this study is to evaluate the accuracy of 3D-T1 turbo spin echo (T1S), sequences in detecting venous thrombosis and comparing it with susceptibility-weighted imaging (SWI), magnetic resonance venography (MRV) and post contrast T1 magnetization-prepared rapid acquisition gradient echo (T1C). A blinded retrospective observational analysis of 71 consecutive patients evaluated for cerebral venous thrombosis (CVT) and 30 control patients was performed. Multimodality reference standard adopted included T1C, SWI with MRV. Sub-analyses in superficial, deep and cortical venous segments were performed in addition to correlation of signal intensity of thrombus with the clinical stage. A total of 2222 segments in 101 complete MRI examinations were evaluated. Sensitivity/specificity/positive predictive value/negative predictive value/accuracy and precision of T1S for detection of cortical vein thrombosis was 0.994/1/1/0.967/0.995/1, 1/0.874/0.949/1/0.963/0.950 for detection of superficial venous sinus thrombosis and 1/1/1/1/1/1 for deep venous thrombosis. The AUC yield for T1S was 0.997 for cortical, 1 for deep and 0.988 for superficial venous segments. T1S paralleled the accuracy of conventional sequences in the overall detection of CVT but showed superior accuracy in the detection of cortical venous thrombosis. It makes a fitting addition to the CVT MRI protocol in scenarios demanding negation of gadolinium administration.

MRI to differentiate multiple sclerosis, neuromyelitis optica, and myelin oligodendrocyte glycoprotein antibody disease.

Differentiating multiple sclerosis (MS) from other relapsing inflammatory autoimmune diseases of the central nervous system such as neuromyelitis optica spectrum disorder (NMOSD) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is crucial in clinical practice. The differential diagnosis may be challenging but making the correct ultimate diagnosis is critical, since prognosis and treatments differ, and inappropriate therapy may promote disability. In the last two decades, significant advances have been made in MS, NMOSD, and MOGAD including new diagnostic criteria with better characterization of typical clinical symptoms and suggestive imaging (magnetic resonance imaging [MRI]) lesions. MRI is invaluable in making the ultimate diagnosis. An increasing amount of new evidence with respect to the specificity of observed lesions as well as the associated dynamic changes in the acute and follow-up phase in each condition has been reported in distinct studies recently published. Additionally, differences in brain (including the optic nerve) and spinal cord lesion patterns between MS, aquaporin4-antibody-positive NMOSD, and MOGAD have been described. We therefore present a narrative review on the most relevant findings in brain, spinal cord, and optic nerve lesions on conventional MRI for distinguishing adult patients with MS from NMOSD and MOGAD in clinical practice. In this context, cortical and central vein sign lesions, brain and spinal cord lesions characteristic of MS, NMOSD, and MOGAD, optic nerve involvement, role of MRI at follow-up, and new proposed diagnostic criteria to differentiate MS from NMOSD and MOGAD were discussed.

A Study of Clinico-Radiological Profile and Outcomes of Cerebral Venous Thrombosis

ABSTRACT Background: Cerebral venous thrombosis (CVT) includes highly variable clinical presentations ranging from headache to coma. It often affects young to middle-aged patients. Early diagnosis and treatment improve outcomes. Outcome determinant factors are the extent of the disease, jugular occlusion, spread to deep or cortical veins, level of sensorium at onset, background factors, and presence of raised intracranial pressure. Objectives: This study aims to study the clinical profile, radiological features, and outcomes of CVT. Materials and Methods: Fifty-seven patients admitted to SSIMS&amp;RC, Davangere, clinically suspected of CVT, were subjected to neuroimaging techniques, patients with a confirmed diagnosis of CVT (radiologically) were recruited by simple random sampling, and data collected was analyzed by correlational studies. Follow-up was done until 6 months. Results: Of the 57 patients, 30 (53%) were females and 27 (47%) were males, with a maximum incidence of 84% in the age group 18–40 years. The most common symptoms were headache (86%), seizures (65%), focal deficits (58%), and altered sensorium (52.6%). Radiologically, the most common sinus involved was the superior sagittal sinus 72%. In total, out of 57 patients, 6 patients died during hospitalization. Out of the surviving patients, 39 patients (70%) had a complete recovery. Poor prognostic factors were stupor and coma on admission and evidence of hemorrhagic infarction in the primary computed tomography (CT) scan. Conclusion: Compared to the previous studies, the prevalence of CVT was higher in women. Clinical manifestations, prognostic factors, “commonly involved sinuses,” and “image findings of this study” were similar to those of other studies. The overall prognosis was good, but a small percentage of patients died.

Intracranial Dural Arteriovenous Fistulas.

Dural arteriovenous fistulas (DAVFs) are abnormal communications between meningeal arteries and dural venous sinuses and/or cortical veins. Although many fistulas are benign and do not require treatment, some may carry a significant risk of bleeding or cause symptoms and warrant treatment. This review provides a review of various aspects of intracranial DAVFs including epidemiology, pathophysiology, clinical presentation, imaging characteristics, classification, natural history, and management options. By exploring these topics, we aim to enhance understanding of this condition and facilitate patient care.

Neuroprotective effects of pravastatin in cerebral venous infarction in a rat model.

Pravastatin sodium is reported to have multiple beneficial effects in cerebral atherosclerosis and neuronal injury; however, the preventive effects on cerebral venous ischemia are still unknown. Herein, we aimed to examine the neuroprotective effects of transoral prior administration of pravastatin sodium against cerebral cortical venous ischemia with suppression of apoptosis. Thirty 8-week-old male Wistar rats were divided equally into two study groups (n=15 vs. n=15); the pravastatin group was fed 1% pravastatin sodium with their usual diet for 2 weeks, while the control group only received the usual diet. Two-vein occlusion (2VO) model was applied for this study, and two adjacent cortical veins in each animal were permanently occluded photochemically with rose bengal dye. During photo-thrombosis, regional changes of the cerebral blood flow (CBF) in area of the venous ischemia were recorded. At 48-h after 2VO, animals were euthanized using perfusion fixation, and we histologically measured ratios of infarcted area to contralateral hemisphere, and counted Bax- and Bcl-2-positive cells in the penumbra to investigate the implications for apoptosis. The ratio of infarcted area was significantly decreased in the pravastatin group compared to the control group (P<0.01). The number of Bax-positive cells also decreased significantly in the pravastatin group (P<0.01). In contrast, immunolabeling for Bcl-2 was essentially negative in all areas in both groups. There were also no significant differences in regional CBF changes after 2VO between the two groups (P=0.13). Pre-emptive administration of pravastatin sodium mixed in the food has neuroprotective effects against cerebral cortical venous ischemia with suppression of apoptosis associated with inhibition of Bax expression but has little influence on regional CBF.

Visualising and semi-quantitatively measuring brain fluid pathways, including meningeal lymphatics, in humans using widely available MRI techniques

Brain fluid dynamics remains poorly understood with central issues unresolved. In this study, we first review the literature regarding points of controversy, then pilot study if conventional MRI techniques can assess brain fluid outflow pathways and explore potential associations with small vessel disease (SVD). We assessed 19 subjects participating in the Mild Stroke Study 3 who had FLAIR imaging before and 20–30 minutes after intravenous Gadolinium (Gd)-based contrast. Signal intensity (SI) change was assessed semi-quantitatively by placing regions of interest, and qualitatively by a visual scoring system, along dorsal and basal fluid outflow routes. Following i.v. Gd, SI increased substantially along the anterior, middle, and posterior superior sagittal sinus (SSS) (82%, 104%, and 119%, respectively), at basal areas (cribriform plate, 67%; jugular foramina, 72%), and in narrow channels surrounding superficial cortical veins separated from surrounding cerebrospinal fluid (CSF) (96%) (all p < 0.001). The SI increase was associated with higher intraparenchymal perivascular spaces (PVS) scores (Std. Beta 0.71, p = 0.01). Our findings suggests that interstitial fluid drainage is visible on conventional MRI and drains from brain parenchyma via cortical perivenous spaces to dural meningeal lymphatics along the SSS remaining separate from the CSF. An association with parenchymal PVS requires further research, now feasible in humans.

0992 Reversible Pediatric Central Sleep Apnea in Hyperhomocysteinemia and Cerebral Sinus Venous Thrombosis

Abstract Introduction Pediatric central sleep apnea (CSA) rarely initially presents in adolescent age groups, being more commonly diagnosed in infants. CSA may result from a multitude of neurological insults, such as stroke and brain masses. It may also less often present in metabolic and non-ischemic thrombotic disorders. Report of case(s) A 12-year-old female with history of global developmental delay of unclear etiology, chronic headaches, insulin resistance, and early morning tremors was referred for initial evaluation of possible narcolepsy. Severe daytime sleepiness had acutely worsened over the previous month. She was unable to stay awake during school, despite sleeping 13 hours per 24 hours. Central hypersomnia was suspected and polysomnogram with MSLT was scheduled. The patient presented to the Emergency Department 10 days later for worsening headaches and new oral aversion with 12-pound weight loss. Examination showed ataxic gait and dysarthria. Brain MRI revealed non-occlusive cerebral sinus venous thrombosis (CSVT) involving the superior sagittal sinus, right transverse sinus, right sigmoid sinus, right jugular bulb/vein, and cortical veins overlying both cerebral hemispheres with no stroke. Anticoagulation with enoxaparin was initiated and thrombophilia work-up commenced. Polysomnogram demonstrated a central AHI of 49.9/hr with significant periodic breathing and no obstructive sleep apnea. Oxygen titration sleep study failed to significantly improve CSA and BPAP was initiated. Thrombophilia work-up revealed severe hyperhomocysteinemia. Genetic analysis was consistent with autosomal recessive severe methylenetetrahydrofolate reductase (MTHFR) deficiency. The patient was begun on metabolic treatment with betaine, pyridoxine, hydroxocobalamin, and vitamin C, and transitioned to oral rivaroxaban. At 4 months, blood levels had normalized, and MRI demonstrated resolution of CSVT. Family endorsed BPAP compliance and complete resolution of all sleep symptoms. In addition, there was significant improvement in cognition, interpersonal skills, and abstract thought. Follow up polysomnogram 10 months after presentation demonstrated no CSA nor periodic breathing. Conclusion To our knowledge, this is the first report of MTHFR deficiency and CSVT resulting in severe CSA. While it is difficult to ascertain which entity ultimately caused the CSA, MTHFR deficiency can produce significant neurologic impairment through white matter disease and defective myelination, while CSVT can provoke venous hypertension, venous stasis, or hypoxia. Support (if any)

Doppler Ultrasound Flow Reversal in the Superior Sagittal Sinus to Detect Cerebral Venous Congestion in Vein of Galen Malformation.

Vein of Galen malformation is a rare congenital cerebrovascular malformation. In affected patients, increased cerebral venous pressure constitutes an important etiologic factor for the development of brain parenchymal damage. The aim of this study was to investigate the potential of serial cerebral venous Doppler measurements to detect and monitor increased cerebral venous pressure. This was a retrospective monocentric analysis of ultrasound examinations within the first 9 months of life in patients with vein of Galen malformation admitted at <28 days of life. Categorization of perfusion waveforms in the superficial cerebral sinus and veins into 6 patterns was based on antero- and retrograde flow components. We performed an analysis of flow profiles across time and correlation with disease severity, clinical interventions, and congestion damage on cerebral MR imaging. The study included 44 Doppler ultrasound examinations of the superior sagittal sinus and 36 examinations of the cortical veins from 7 patients. Doppler flow profiles before interventional therapy correlated with disease severity determined by the Bicêtre Neonatal Evaluation Score (Spearman ρ = -0.97, P = < .001). At this time, 4 of 7 patients (57.1%) showed a retrograde flow component in the superior sagittal sinus, whereas after embolization, none of the 6 treated patients presented with a retrograde flow component. Only patients with a high retrograde flow component (equal or more than one-third retrograde flow, n = 2) showed severe venous congestion damage on cerebral MR imaging. Flow profiles in the superficial cerebral sinus and veins appear to be a useful tool to noninvasively detect and monitor cerebral venous congestion in vein of Galen malformation.

Extravascular optical coherence tomography of cerebral vessel walls in vivo.

Evaluation of extravascular, microscope integrated OCT (iOCT) as an in vivo imaging modality of cerebral blood vessels and as an intraoperative imaging method. Microscope integrated optical coherence tomography of major cerebral arteries (n = 13) and superficial sylvian veins (n = 5) and one incidental cerebral vasospasm (n = 1) in (n = 10) patients. Post procedural analysis of OCT volume scans, microscopic images and videos during the time of scan as well as measurements of the diameter of vessel walls and its layers with an accuracy of 7.5 μm. iOCT was feasible during vascular microsurgical procedures. In all scanned arteries a clear delineation of the physiological three layered vessel wall composition could be achieved. Pathological arteriosclerotic alterations of cerebral artery walls could precisely be demonstrated. Major superficial cortical veins conversely presented a mono layered composition. First in vivo measurements of vascular mean diameters were possible. Cerebral artery walls showed a diameter of 296 μm, tunica externa 78 μm, media 134 μm and interna 84 μm. For the first time the microstructural composition of cerebral blood vessels could be illustrated in vivo. Due to an outstanding spatial resolution a clear definition of physiological and pathological characteristics was possible. Therefore, microscope integrated optical coherence tomography holds promise for basic research in the field of cerebrovascular arteriosclerotic diseases and for intraoperative guidance during microvascular surgery.