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Objective: To determine sex differences in clinical profiles and treatment outcomes in patients with spontaneous intracranial hypotension., Background: Spontaneous intracranial hypotension is associated with considerable functional disability and potentially fatal complications, and it is uncertain whether males and females should be managed differently., Methods: This was a cohort study of consecutive patients with spontaneous intracranial hypotension enrolled from a medical center. Medical records and imaging findings were reviewed. The outcome of treatment responses to epidural blood patches and risks of subdural hematoma were measured., Results: In total, 442 patients with spontaneous intracranial hypotension (165 males, 277 females) were included in the analysis. Males were more likely to have a delayed (>30 days) initial presentation than females (32.1% [53/165] vs. 19.9% [55/277], p = 0.004), and males were less likely to have nausea (55.8% [92/165] vs. 67.1% [186/277], p = 0.016), vomiting (43.0% [71/165] vs. 54.2% [150/277], p = 0.024), photophobia (9.7% [16/165] vs. 17.0% [47/277], p = 0.034), and tinnitus (26.7% [44/165] vs. 39.7% [110/277], p = 0.005) compared with females despite comparable radiologic findings. Among the 374 patients treated with epidural blood patches, males were more likely to be nonresponders to the first epidural blood patch (58.0% [80/138] vs. 39.0% [92/236], OR = 2.2, 95% CI = 1.4-3.3, p < 0.001). Males were at a higher risk of having subdural hematoma (29.7% [49/165] vs. 10.8% [30/277], OR = 3.5, 95% CI = 2.1-5.8, p < 0.001). Among patients with subdural hematoma, males had greater thickness (12.8 ± 4.3 vs. 8.1 ± 5.9 mm, p < 0.001) and were more likely to receive surgical drainage (55.1% [27/49] vs. 10.0% [3/30], OR = 11.0, 95% CI = 3.0-41.3, p < 0.001) than females., Conclusion: In the present study, spontaneous intracranial hypotension in males was characterized by a delayed presentation, poorer response to the first epidural blood patch, and a higher risk of subdural hematoma. Caution should be exercised in the management of males with spontaneous intracranial hypotension. The generalizability of the findings needs to be further confirmed., (© 2024 The Author(s). Headache: The Journal of Head and Face Pain published by Wiley Periodicals LLC on behalf of American Headache Society.)

Background: To develop and validate an easy-to-use scoring system to predict the response to the first epidural blood patching in patients with spontaneous intracranial hypotension., Methods: This study recruited consecutive patients with spontaneous intracranial hypotension receiving epidural blood patching in a tertiary medical center, which were chronologically divided into a derivation cohort and a validation cohort. In the derivation cohort, factors associated with the first epidural blood patching response were identified by using multivariable logistic regression modeling. A scoring system was developed, and the cutoff score was determined by using the receiver operating characteristic curve. The findings were verified in an independent validation cohort., Results: The study involved 280 patients in the derivation cohort and 78 patients in the validation cohort. The spontaneous intracranial hypotension-epidural blood patching score (range 0-5) included two clinical variables (sex and age) and two radiological variables (midbrain-pons angle and anterior epidural cerebrospinal fluid collections). A score of ≥3 was predictive of the first epidural blood patching response, which was consistent in the validation cohort. Overall, patients who scored ≥3 were more likely to respond to the first epidural blood patching (odds ratio = 10.3)., Conclusion: For patients with spontaneous intracranial hypotension-epidural blood patching score ≥3, it is prudent to attempt at least one targeted epidural blood patching before considering more invasive interventions.

Objectives: Several brain and spinal magnetic resonance imaging signs have been described in spontaneous intracranial hypotension. Their correlations are not fully studied. This study aimed to explore potential mechanisms underlying cerebral neuroimaging findings and to examine associations among spinal and brain magnetic resonance imaging signs., Methods: We conducted a retrospective review of magnetic resonance myelography and brain magnetic resonance imaging records of patients with spontaneous intracranial hypotension. Categorical principal component analysis was employed to cluster brain neuroimaging findings. Spearman correlation was employed to analyze associations among different brain neuroimaging findings and between brain and spinal neuroimaging findings., Results: In patients with spontaneous intracranial hypotension (n = 148), categorical principal component analysis of brain neuroimaging signs revealed two clusters: Cerebral venous dilation and brain descent. Among all brain magnetic resonance imaging signs examined, only midbrain-pons angle associated with anterior epidural cerebrospinal fluid collection length (surrogate spinal cerebrospinal fluid leak severity) (n = 148, Spearman's ρ = -0.38, p < .001). Subgroup analyses showed that the association between midbrain-pons angle (within brain descent cluster) and spinal cerebrospinal fluid leak severity was presented in patients with convex margins of the transverse sinuses (n = 122, Spearman's ρ = -0.43, p < .001), but not in patients without convex margins (n = 26, Spearman's ρ = -0.19, p = .348). The association between severity of transverse sinus distension and spinal cerebrospinal fluid leak severity was only presented in patients without convex margins (n = 26, Spearman's ρ = 0.52, p = .006)., Conclusion: This study indicates that there are two factors behind the brain neuroimaging findings in spontaneous intracranial hypotension: Cerebral venous dilation and brain descent. Certain brain neuroimaging signs correlate with spinal cerebrospinal fluid leakage severity, depending on different circumstances.

Spontaneous intracranial hypotension results from cerebrospinal fluid leakage. Currently, the treatment of choice for spontaneous intracranial hypotension is the epidural blood patch, which has a variable response rate and no clear outcome predictors. This study aimed to identify predictors for response rate of a first targeted epidural blood patch in patients with spontaneous intracranial hypotension. We reviewed cases of patients with spontaneous intracranial hypotension who received targeted epidural blood patch at our hospital between 1 January 2007 and 1 July 2014. The outcome measure was first epidural blood patch response. We analysed demographics, clinical manifestations, neuroimaging findings (non-contrast heavily T 2 -weighted magnetic resonance myelography and brain magnetic resonance imaging), and blood volume as potential outcome predictors. Significant predictors were tested and a decision tree was used to construct a predictive model. In total, 150 patients with spontaneous intracranial hypotension were included for final analyses. Their overall first targeted epidural blood patch response rate was 58.7%. Among patients with a greater injected blood volume (≥22.5 versus <22.5 ml), the response rate was higher (67.9% versus 47.0%, P = 0.01). In brain and spinal magnetic resonance imaging studies, significant predictors included anterior epidural cerebrospinal fluid collection length (<8 versus ≥8 segments; 72.5% versus 37.3%, odds ratio = 4.4, 95% confidence interval: 2.2-8.9, P < 0.001) and midbrain-pons angle (≥40° versus <40°; 71.3% versus 37.5%, odds ratio = 4.1, 95% confidence interval 2.1-8.3, P < 0.001). Decision tree analyses showed that patients with anterior epidural CSF collection involving <8 segments and an injected blood volume ≥22.5 ml had an 80.0% response rate. Patients with anterior epidural cerebrospinal fluid collection involving ≥8 segments and a midbrain-pons angle <40° had a 21.2% response rate. These three variables predicted first epidural blood patch response in 71.3% of patients. Brain and spinal neuroimaging findings and epidural blood patch blood volume can be used to predict targeted first epidural blood patch response in patients with spontaneous intracranial hypotension., (© The Author (2016). Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved. For Permissions, please email: journals.permissions@oup.com.)

Purpose: Spontaneous intracranial hypotension (SIH) is a rare type of headache. The association of SIH with malignancy and disseminated intravascular coagulation (DIC) has not previously been reported., Case Report: A 60-year-old woman had orthostatic headache for more than one month before admission. MRI of brain showed diffuse pachymeningeal enhancement with bilateral subdural hematoma. MR myelography revealed epidural fluid collection and possible CSF leakage at the level of C5 to C6. DIC due to carcinoma of unknown origin was found based on evidence of malignant pleural effusion and multiple bone metastases. After correction of coagulopathy, the patient received an epidural blood patch. Unfortunately, follow-up brain MRI showed disease progression. The patient died of acute respiratory failure four weeks after admission., Conclusion: This is the first case report of an association between SIH and DIC due to malignancy. Further case studies are needed to provide further support of this association.

Objective: The objective of this article is to elucidate the outcome, prognostic predictors and timing of surgical intervention for subdural hematoma (SDH) in patients with spontaneous intracranial hypotension (SIH)., Methods: Patients with SDH were identified retrospectively from 227 consecutive SIH patients. Data were collected on demographics, clinical courses, neuroimaging findings, and treatment of SDH, which was later divided into conservative treatment, epidural blood patches (EBP), and surgical intervention. Poor outcome was defined as severe neurological sequelae or death., Results: Forty-five patients (20%) with SDH (mean maximal thickness 11.9 ± 6.2 mm) were recruited. All 15 patients with SDH <10 mm achieved good outcomes by either conservative treatment or EBP. Of 30 patients with SDH ≥10 mm, patients with uncal herniation (n = 3) had poor outcomes, even after emergent surgical evacuation (n = 2), compared to those without (n = 27) (100% vs. 0%, p < 0.001). Fourteen patients underwent surgical evacuation, resulting in good outcomes in all 12 who received early intervention and poor outcomes in the remaining two who received delayed intervention after Glasgow Coma Scale (GCS) score ≤8 (100% vs. 0%, p = 0.01)., Conclusions: Uncal herniation results in poor outcomes in patients with SIH complicated with SDH. In individuals with SDH ≥10 mm and decreased GCS scores, early surgical evacuation might prevent uncal herniation., (© International Headache Society 2015.)

Objective: Spontaneous intracranial hypotension (SIH) is caused by spinal cerebrospinal fluid (CSF) leakage. An underlying connective tissue disorder has been hypothesized to cause dural weakness and predisposition to CSF leak. We conducted a case-controlled study to investigate the role of connective tissue disorders in SIH patients., Methods: We recruited 55 consecutive SIH patients (38 F, 17 M; mean age, 40.8 ± 9.8 years) and 55 age- and sex-matched control individuals (mean age, 38.0 ± 8.9 years) for this study. The connective tissue disorders were evaluated by: (i) Beighton hypermobility scores and revised diagnostic criteria for benign joint hypermobility syndrome; (ii) skin and skeletal manifestations of Ehlers-Danlos syndrome (EDS); and (iii) skeletal features of Marfan syndrome., Results: The frequencies of joint hypermobility according to Beighton scores >4/9 (SIH 23.6% vs controls 16.4%, P = 0.48) and revised benign joint hypermobility syndrome criteria (SIH 23.6% vs controls 34.5%, P = 0.29) did not differ between SIH patients and controls. Sixteen patients and 16 controls had one or more skin features of EDS (P = 1.0). Nine SIH patients (16.4%) demonstrated the skeletal features of Marfan syndrome; this frequency did not differ from that of the control group (9.1%; P = 0.262). Only dolichostenomelia (disproportionately long limbs) was more prominent in SIH patients than in controls (34.5% vs 9.1%; P = 0.002)., Conclusion: Compared with Western studies, the frequencies of connective tissue disorders were higher in our SIH patients. However, these frequencies did not differ between SIH patients and control individuals, except for dolichostenomelia.

Objective: To assess the diagnostic accuracy of heavily T2-weighted magnetic resonance myelography (MRM) in patients with spontaneous intracranial hypotension (SIH)., Methods: Patients with SIH were recruited prospectively, and first underwent MRM and then computed tomographic myelography (CTM). The results of MRM were validated with the gold standard, CTM, focusing on 1) CSF leaks along the nerve roots, 2) epidural CSF collections, and 3) high-cervical (C1-3) retrospinal CSF collections. Comparisons of these 3 findings between the 2 studies were made by kappa statistics and agreement rates. Targeted epidural blood patches (EBPs) were placed at the levels of CSF leaks if supportive treatment failed., Results: Nineteen patients (6 men and 13 women, mean age 37.9 +/- 8.6 years) with SIH completed the study. MRM did not differ from CTM in the detection rates of CSF leaks along the nerve roots (84% vs 74%, p = 0.23), high-cervical retrospinal CSF collections (32% vs 16%, p = 0.13), and epidural CSF collections (89% vs 79%, p = 0.20). MRM demonstrated more spinal levels of CSF leaks (2.2 +/- 1.7 vs 1.5 +/- 1.5, p = 0.011) and epidural collections (12.2 +/- 5.9 vs 7.1 +/- 5.8, p < 0.001) than CTM. The overall level-by-level concordance was substantial for CSF leaks along the nerve roots (C1-L3) (kappa = 0.71, p < 0.001, agreement = 95%) and high-cervical retrospinal CSF collections (C1-3) (kappa = 0.73, p < 0.001, agreement = 92%), and moderate for epidural CSF collections (C1-L3) (kappa = 0.47, p < 0.001, agreement = 72%). Ten of the 14 patients (71%) receiving targeted EBPs experienced sustained symptomatic relief after a single attempt., Conclusions: Heavily T2-weighted magnetic resonance myelography was accurate in localizing CSF leaks for patients with spontaneous intracranial hypotension. This noninvasive technique may be an alternative to computed tomographic myelography before targeted epidural blood patches.

The timing and clinical relevance of diffuse pachymeningeal enhancement (DPE) in the magnetic resonance imaging (MRI) examination of patients with spontaneous intracranial hypotension (SIH) remain undetermined. We reviewed 53 consecutive SIH patients (30 F/23 M, mean age of onset 41.7 +/- 11.3 years) in a tertiary hospital. Thirteen (24.5%) patients did not have DPE on their initial cranial MRIs. They had significantly shorter latency between the time of MRI examinations and the time of headache onset compared with those with DPE (6.5 +/- 4.4 vs. 20.4 +/- 16.3 days, t-test, P < 0.001). Eight of these 13 patients received a follow-up MRI (mean duration 30.3 +/- 16.6 days, range 6-59 days) and six of them revealed DPE. Among patients with DPE, the enhancement disappeared as early as 25 days after headache onset. The outcome did not differ between patients with and without DPE. The presence of DPE was associated with the timing of the MRI examination.

Spontaneous intracranial hypotension (SIH) associated with subarachnoid haemorrhage (SAH) has never been reported. Here, we report on a case of a 33-year-old woman with SIH, who developed simple partial sensory seizures 3 weeks later. Neuroimaging studies, including brain computed tomography and angiography, were initially normal, but revealed an isolated cortical venous thrombosis at 3 weeks. One week later, brain magnetic resonance imaging showed SAH around the thrombosed cortical vein. We postulate that the decline in the venous blood flow velocity due to SIH may have resulted in cortical venous thrombosis, which in turn led to rupture of the vessel wall and SAH in this patient.

We performed whole-spine heavily T2-weighted magnetic resonance (MR) myelography using a single-shot fast spin-echo pulse sequence in 17 patients (8 M/9 F) with spontaneous intracranial hypotension (SIH) to detect abnormal cerebrospinal fluid (CSF) collections. In addition, a group of age- and sex-matched controls were recruited. Follow-up MR myelography was also done at 3 weeks. MR myelography showed three kinds of abnormal CSF collections in 15 patients with SIH (88%): epidural fluid collection (n = 15, 88%), C1-2 extraspinal collections (n = 6, 35%) and CSF collections along nerve roots in the lower cervical or upper thoracic spines (n = 6, 35%). One patient (6%) showed a meningeal diverticulum. In contrast, none of the controls showed these findings. Overall, MR myelography results helped in early diagnosis of SIH in four (24%) patients whose initial brain MRIs failed to show typical SIH findings. Follow-up MR myelography results were compatible with the clinical changes with kappa statistics of 0.52 and an agreement rate of 76%. Our study showed heavily T2-weighted MR myelography provided a rapid, non-invasive and high yield method to diagnose and follow-up patients with SIH. Whether the CSF collections along the nerve roots represent the ongoing leakage sites warrants further study.

The incidence and clinical relevance of subdural haematoma (SDH) in patients with spontaneous intracranial hypotension (SIH) remain undetermined. We reviewed 40 consecutive SIH patients (18 female, 22 male) in a tertiary hospital. Eight (20%) of them had SDH and nine (23%), non-haemorrhagic subdural collections. The presence of SDH was associated with higher frequencies of male gender, recurrence of severe headache and neurological deficits. Outcomes were satisfactory after supportive care or epidural blood patches except for one SDH patient, who developed transtentorial herniation resulting in Duret haemorrhage and infarctions of bilateral posterior cerebral artery territories. In conclusion, subdural fluid collections were common in patients with SIH. SDH was associated with headache worsening or neurological deficits. Patients with SDH generally recovered well; however, serious sequela might occur.

A 55-year-old woman had new onset of postural headache followed by change of mental status 3 weeks later. Magnetic resonance imaging (MRI) of the brain and whole spine showed typical spontaneous intracranial hypotension (SIH) findings, bilateral subdural hematoma, and cerebrospinal fluid leakage over the T7-T9. Her headache and mentality improved after epidural blood patches. Early recognition and correct diagnosis are crucial for successful treatment in patients with SIH presenting with mental confusion.

The authors measured the average diameter of bilateral superior ophthalmic veins (SOV) in 13 patients with spontaneous intracranial hypotension (SIH) on contrast-enhanced, coronal, T1-weighted MRI. Compared with sex- and age-matched neurology inpatients with normal CSF pressure, the SIH group had a smaller SOV diameter (0.90 vs 1.85 mm, p < 0.001), which partly reversed after treatment (1.09 vs 0.90 mm, p = 0.045, n = 7). Collapsed SOV might provide an additional MRI finding for SIH.

Purpose: To present the MR features of spontaneous intracranial hypotension (SIH) and to discuss the correlation of MR features with clinical manifestations., Material and Methods: Between 1997 and 2000, 15 patients with SIH underwent brain MR investigation. Lumbar puncture for the measurement of cerebrospinal fluid (CSF) pressure was performed in 6 patients. Follow-up MR was obtained in 8 patients after the clinical symptoms had improved. We analyzed the MR findings of SIH, and discuss them in relation to CSF pressure and clinical manifestations., Results: Fourteen of the 15 patients with SIH had abnormal MR findings. The imaging findings included: diffuse pachymeningeal enhancement in 13 patients; descent of the brain in 6 patients; and subdural effusion/hematoma in 2 patients. Low CSF pressure was shown at lumbar puncture in 5 out of 6 patients. Follow-up MR examination in the symptoms-relieved status demonstrated a recovery of the abnormal MR findings in 6 of 8 patients., Conclusion: Our data revealed that MR is sensitive in diagnosing SIH. The presence of characteristic clinical manifestations and MR features are virtually diagnostic for SIH syndrome. Invasive lumbar puncture is thus reserved for the equivocal cases.

Background: Measurement of CSF pressure is the only known way to confirm the diagnosis of intracranial hypotension. We aimed to assess colour doppler flow imaging (CDFI) for measurement of blood flow of the superior ophthalmic vein for the diagnosis of intracranial hypotension., Methods: We enrolled 25 consecutive patients with orthostatic headache who had clinical features of intracranial hypotension. We defined low-pressure headache as cerebrospinal-fluid pressure below 60 mm H2O. We used CDFI to measure the diameter and maximum flow velocity of the superior ophthalmic vein in all patients. Magnetic resonance imaging of the brain and lumbar puncture with measurement of cerebrospinal-fluid pressure within 24 h were also done after sonographic examination. The control group comprised 13 healthy individuals of a similar age; in addition, those patients who had orthostatic headache without low pressure served as a control group for the patients., Findings: Of the 25 patients recruited for this study, 13 satisfied the criteria for low-pressure headache. The remaining 12 patients with normal cerebrospinal-fluid pressure had transformed migraine (five patients) or chronic tension-type headache (seven patients), and therefore served as the control group for the patients. The mean diameter of the superior ophthalmic vein was substantially larger in the patients with intracranial hypotension (3.9 [SD 0.2] mm) than in the healthy controls (2.6 [0.4] mm) and the controls from the patients' group (2.7 [0.2] mm) (p<0.0001). The mean maximum flow velocity was significantly higher in the intracranial-hypotension group (17.0 [SD 3.4] cm/s) than in the healthy controls (7.9 [1.1] cm/s) and the other patients (7.3 [1.7] cm/s) (p<0.0001). Seven patients with intracranial hypotension were reassessed after treatment with epidural blood patch. After this treatment the clinical symptoms were relieved and there was a striking reversal of the superior ophthalmic vein flow., Interpretation: CDFI to measure blood flow of the superior ophthalmic vein provides a practical, simple, and non-invasive diagnostic method for suspected intracranial hypotension.