Spontaneous intracranial hypotension (SIH) is a rare disorder characterized by continuous or intermittent cerebrospinal fluid (CSF) leakage from the CSF cavity, which causes symptoms such as headache or neck pain upon standing. However, no well-established measures concerning the type of delivery and anesthesia for pregnant women with a history of SIH have been reported. A woman had developed SIH 9 years earlier from lifting luggage into an overhead bin with stretching movements, which required continuous saline epidural infusion for recovery. Upon the patient\'s pregnancy at the age of 35 years, although an elective cesarean section (CS) under general anesthesia was planned to avoid SIH recurrence, the patient had an emergency CS at 36 weeks. Since there is no prescribed method of delivery and anesthetic management for patients with a history of SIH, it is important to plan and adapt a treatment strategy based on the patient\'s wishes and the institution\'s protocols. As a sidenote, we reviewed the available literature regarding the type of delivery and anesthesia for pregnant women with a history of SIH.

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

Pulsatile spinal cord and CSF velocities related to the cardiac cycle can be depicted by phase-contrast MRI. Among patients with spontaneous intracranial hypotension, we have recently described relevant differences compared with healthy controls in segment C2/C3. The method might be a promising tool to solve clinical and diagnostic ambiguities. Therefore, it is important to understand the physiological range and the effects of clinical and anatomical parameters in healthy volunteers. Within a prospective study, 3D T2-weighted MRI for spinal canal anatomy and cardiac-gated phase-contrast MRI adapted to CSF flow and spinal cord motion for time-resolved velocity data and derivatives were performed in 70 participants (age 20-79 years) in segments C2/C3 and C5/C6. Correlations were analyzed by multiple linear regression models; p < 0.01 was required to assume a significant impact of clinical or anatomical data quantified by the regression coefficient B. Data showed that in C2/C3, the CSF and spinal cord craniocaudal velocity ranges were 4.5 ± 0.9 and 0.55 ± 0.15 cm/s; the total displacements were 1.1 ± 0.3 and 0.07 ± 0.02 cm, respectively. The craniocaudal range of the CSF flow rate was 8.6 ± 2.4 mL/s; the CSF stroke volume was 2.1 ± 0.7 mL. In C5/C5, physiological narrowing of the spinal canal caused higher CSF velocity ranges and lower stroke volume (C5/C6 B = +1.64 cm/s, p < 0.001; B = -0.4 mL, p = 0.002, respectively). Aging correlated to lower spinal cord motion (e.g., B = -0.01 cm per 10 years of aging, p < 0.001). Increased diastolic blood pressure was associated with lower spinal cord motion and CSF flow parameters (e.g., C2/C3 CSF stroke volume B = -0.3 mL per 10 mmHg, p < 0.001). Males showed higher CSF flow and spinal cord motion (e.g., CSF stroke volume B = +0.5 mL, p < 0.001; total displacement spinal cord B = +0.016 cm, p = 0.002). We therefore propose to stratify data for age and sex and to adjust for diastolic blood pressure and segmental narrowing in future clinical studies.

A cerebrospinal fluid (CSF) venous fistula (CVF) is an aberrant connection between the subarachnoid space and a vein resulting in CSF loss. The presentation and management of CVF with cognitive decline is incompletely understood.
A systematic review was completed following the PRISMA guidelines. Articles that included at least 1 case of imaging-confirmed CVF with details on patient treatment were included. A separate review of cases of patients with spontaneous intracranial hypotension (SIH) with frontotemporal dementia (FTD) or dementia symptoms was also completed.
Ten CVF articles (69 patients; average age, 51.5 years) and 5 SIH with FTD or dementia articles (n = 41; average age, 55.9 years) were identified. Only 1 patients with CVF with cognitive abnormalities was identified. The most common symptom was headache in both reviews. Brain sag was identified in all patients, whereas CSF leak was identified in only 2 patients with SIH with FTD or dementia (4.9%). An epidural blood or fibrin glue patch was used in all patients with CVF and in 33 patients with SIH with FTD or dementia. Fifty-five patients with CVF (79.7%) and 27 patients with SIH with FTD or dementia (65.9%) had surgery.
The 2 cases and literature reviews show the difficulty in diagnosis and treatment of CVF with cognitive decline. Novel imaging techniques should be used in patients with cognitive decline in whom a CSF leak is suspected. Transvenous embolization or surgery should be considered before patching for treatment of CVF-induced brain sag and resulting dementia.

Cerebrospinal fluid (CSF) hypovolemia is the core of spontaneous intracranial hypotension (SIH). More than 1000 magnetic resonance myelography (MRM) images are required to evaluate each subject. An effective spinal CSF quantification method is needed. In this study, we proposed a cascade artificial intelligence (AI) model to automatically segment spinal CSF. From January 2014 to December 2019, patients with SIH and 12 healthy volunteers (HVs) were recruited. We evaluated the performance of AI models which combined object detection (YOLO v3) and semantic segmentation (U-net or U-net++). The network of performance was evaluated using intersection over union (IoU). The best AI model was used to quantify spinal CSF in patients. We obtained 25,603 slices of MRM images from 13 patients and 12 HVs. We divided the images into training, validation, and test datasets with a ratio of 4:1:5. The IoU of Cascade YOLO v3 plus U-net++ (0.9374) was the highest. Applying YOLO v3 plus U-net++ to another 13 SIH patients showed a significant decrease in the volume of spinal CSF measured (59.32 ± 10.94 mL) at disease onset compared to during their recovery stage (70.61 ± 15.31 mL). The cascade AI model provided a satisfactory performance with regard to the fully automatic segmentation of spinal CSF from MRM images. The spinal CSF volume obtained through its measurements could reflect a patient\'s clinical status.

OBJECTIVE: Epidural blood patching (EBP) is the mainstay therapy for spontaneous intracranial hypotension (SIH). MRI is used for evaluating spinal CSF leakage. Post-EBP MRI has been. shown to be effective in predicting the efficacy of EBP. However, there are few reports on how post-EBP MRI findings may change with time. The aim of this study was to evaluate the relationship between post-EBP MRI findings at different time points and the corresponding effectiveness of EBP.
METHODS: We retrospectively reviewed 63 SIH patients who had received target EBP. All patients received an MRI follow-up within 10 days (post-EBP MRI) and at 3 months after EBP (3-month MRI). A sub-group analysis was performed at different post-EBP MRI time points (0-2, 3-6, and 7-10 days). The relationships between the post-EBP MRI findings and the EBP effectiveness were evaluated.
RESULTS: Thirty-five (55.56%) patients were assigned to the EBP-effective group, and 28 (44.44%) were assigned to the EBP non-effective group according to the 3-month MRI. Compared to the EBP non-effective group, the EBP-effective group had significantly lower numbers of spinal CSF leakage in the post-EBP MRI (4.49 vs. 11.71; p = 0.000) and greater numbers of leakage improvement (7.66 vs. 2.96; p = 0.003). For patients who received post-EBP MRI during periods of 0-10, 0-2, 3-6, and 7-10 days, the cutoff values of numbers of spinal CSF leakage for predicting EBP failure were 4, 6, 4, and 5, respectively, with an AUC above 0.77.
CONCLUSIONS: By using post-EBP MRI, which only takes approximately 20 min, predicting EBP efficacy became possible in SIH patients. This study provides cutoff values of numbers of spinal CSF leakage at different follow-up times to serve as clues of if further EBP is needed, which provides the novelty of the current study.

Cerebrospinal fluid (CSF)-venous fistula presents a pathologic connection between spinal subarachnoid space and adjacent epidural vein or veins. It is one of the 3 main causes of spontaneous intracranial hypotension along with dural defects and meningeal diverticulum. We performed a systematic review of the literature and analyzed individual participants\' data focusing on clinical outcomes after different treatment modalities of CSF-venous fistula.
Systematic review was conducted according to PRISMA recommendations. Literature search was performed in PubMed and Web of Science databases with following key phrases: \"CSF-venous fistula\", \"Spontaneous intracranial hypotension\". Overall, 97 articles were found during the initial search; 15 were included for the final analysis, with a total number of 137 patients.
Epidural blood patch (EBP) was performed as a first-line treatment in 37.1% of patients in individual data group, often not combined with fibrin glue (61.5%). Either partial (69.2%) or no resolution (30.8%) of symptoms was achieved after EBP injection. Nerve root ligation was the most common method of exclusion of CSF-venous fistula. Complete resolution of symptoms was achieved in 69.0% of patients, in 21.4% it was partial and in 9.5% no regress was found. Endovascular treatment was described only in 1 study.
Surgical ligation of fistula is a treatment of choice. In approximately 70% of patients complete long-term resolution of symptoms is achieved after surgery. Endovascular treatment and fibrin glue injections are prospective and evolving options, which require further investigation.

OBJECTIVE: 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.
CONCLUSIONS: 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.

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