Factors to predict recurrence after epidural blood patch in patients with spontaneous intracranial hypotension.

OBJECTIVES: This study aimed to identify predictors for the recurrence of spontaneous intracranial hypotension (SIH) after epidural blood patch (EBP). BACKGROUND: Epidural blood patch is the main treatment option for SIH; however, the characteristics of patients who experience relapse after successful EBP treatment for SIH remain understudied. METHODS: In this exploratory, retrospective, case-control study, we included 19 patients with SIH recurrence after EBP and 36 age- and sex-matched patients without recurrence from a single tertiary medical institution. We analyzed clinical characteristics, neuroimaging findings, and volume changes in intracranial structures after EBP treatment. Machine learning methods were utilized to predict the recurrence of SIH after EBP treatment. RESULTS: There were no significant differences in clinical features between the recurrence and no-recurrence groups. Among brain magnetic resonance imaging signs, diffuse pachymeningeal enhancement and cerebral venous dilatation were more prominent in the recurrence group than no-recurrence group after EBP (14/19 [73%] vs. eight of 36 [22%] patients, p = 0.001; 11/19 [57%] vs. seven of 36 [19%] patients, p = 0.010, respectively). The midbrain-pons angle decreased in the recurrence group compared to the no-recurrence group after EBP, at a mean (standard deviation [SD]) of -12.0 [16.7] vs. +1.8[18.3]° (p = 0.048). In volumetric analysis, volume changes after EBP were smaller in the recurrence group than in the no-recurrence group in intracranial cerebrospinal fluid (mean [SD] -11.6 [15.3] vs. +4.8 [17.1] mL, p = 0.001) and ventricles (mean [SD] +1.0 [2.0] vs. +2.0 [2.5] mL, p = 0.003). Notably, the random forest classifier indicated that the model constructed with brain volumetry was more accurate in discriminating SIH recurrence (area under the curve = 0.80 vs. 0.52). CONCLUSION: Our study suggests that volumetric analysis of intracranial structures may aid in predicting recurrence after EBP treatment in patients with SIH.

Patterns of clinical and imaging presentations in patients with spontaneous intracranial hypotension due to spinal cerebrospinal fluid venous fistula: A single-center retrospective cross-sectional study.

OBJECTIVE: To identify distinct clinical or imaging subtypes of spontaneous intracranial hypotension (SIH) due to spinal cerebrospinal fluid (CSF) venous fistula (CVF). BACKGROUND: Spontaneous intracranial hypotension is classically understood to present clinically with an orthostatic headache and stereotyped brain magnetic resonance imaging (MRI) findings; however, most prior literature examining clinical and brain MRI features of SIH has focused on all types of spinal CSF leaks concurrently. This study aimed to evaluate whether data support the possibility of internally consistent subtypes based on brain imaging features and clinical symptoms analogous to those seen in primary headache syndromes. METHODS: This retrospective cross-sectional single-institution study included 48 consecutive patients meeting the International Classification of Headache Disorders, 3rd edition criteria for SIH due to CVF. Clinical symptoms, pre-treatment brain MRI, and symptom duration were analyzed. Clinical and MRI data were analyzed to identify patterns and associations between symptoms and imaging findings. RESULTS: A total of 20 males and 28 females were evaluated, with a mean (standard deviation) age of 61 (10) years. In all, 44/48 (92%) patients experienced headaches, though 18/48 (40%) did not endorse relief when flat, including six of the 48 (13%) with worsening symptoms when flat. In all, 19/48 (40%) patients reported at least one migraine symptom, and six of the 48 (13%) presented with at least one migraine symptom and had no relief when flat. Clinical symptoms clustered primarily into a "classic" presentation consisting of relief when flat, occipital head pain, comorbid neck pain, a pressure/throbbing headache quality, and an "atypical" presentation that was characterized by having several differences: less relief when flat (nine of 22 (41%) vs. 20/23 (87.0%), p = 0.002; odds ratio [OR] 0.110, 95% confidence interval [CI] 0.016-0.53), more frontal head pain (14/22 (64%) vs. one of 23 (4%), p < 0.001; OR 35.0, 95% CI 4.2-1681.0), less neck pain (two of 21 (4.5%) vs. nine of 13 (69.6%), p < 0.001; OR 0.023, 95% CI 0.0005-0.196), and more stabbing/sharp headache quality (nine of 22 (41%) vs. two of 23 (9%), p = 0.017; OR 7.0, 95% CI 1.18-75.9). Brain MRI findings clustered into three groups: those presenting with most imaging findings of SIH concurrently, those with brain sag but less pachymeningeal/venous engorgement, and those with pachymeningeal/venous engorgement but less brain sag. CONCLUSION: This study highlights the clinical and imaging diversity among patients with SIH due to CVF, challenging the reliance on classic orthostatic headache alone for diagnosis. The findings suggest the existence of distinct SIH subtypes based on clinical and imaging presentations, underscoring the need for comprehensive evaluation in patients with suspected CVF. Future research should further elucidate the relationship between clinical symptoms and imaging findings, aiming to refine diagnostic criteria and enhance understanding of SIH's pathophysiology.

Spontaneous intracranial hypotension: Exploring the viability of non-contrast FLAIR as a substitute for contrast-enhanced T1WI in assessing pachymeningeal thickening.

PURPOSE: To avoid contrast administration in spontaneous intracranial hypotension (SIH), some studies suggest accepting diffuse pachymeningeal hyperintensity (DPMH) on non-contrast fluid-attenuated inversion recovery (FLAIR) as an equivalent sign to diffuse pachymeningeal enhancement (DPME) on contrast-enhanced T1WI (T1ce), despite lacking thorough performance metrics. This study aimed to comprehensively explore its feasibility. METHODS: In this single-center retrospective study, between April 2021 and November 2023, brain MRI examinations of 43 patients clinically diagnosed with SIH were assessed using 1.5 and 3.0 Tesla MRI scanners. Two radiologists independently assessed the presence or absence of DPMH on FLAIR and DPME on T1ce, with T1ce serving as a gold-standard for pachymeningeal thickening. The contribution of the subdural fluid collections to DPMH was investigated with quantitative measurements. Using Cohen's kappa statistics, interobserver agreement was assessed. RESULTS: In 39 out of 43 patients (90.7%), pachymeningeal thickening was observed on T1ce. FLAIR sequence produced an accuracy, sensitivity, specificity, positive predictive value, and negative predictive value of 72.1%, 71.8%, 75.0%, 96.6%, and 21.4% respectively, for determining pachymeningeal thickening. FLAIR identified pachymeningeal thickening in 28 cases; however, among these, 21 cases (75%) revealed that the pachymeningeal hyperintense signal was influenced by subdural fluid collections. False-negative rate for FLAIR was 28.2% (11/39). CONCLUSION: The lack of complete correlation between FLAIR and T1ce in identifying pachymeningeal thickening highlights the need for caution in removing contrast agent administration from the MRI protocol of SIH patients, as it reveals a major criterion (i.e., pachymeningeal enhancement) of Bern score.

Real-life study of the use of oto-acoustic emissions in the diagnosis of intracranial hypotension.

BACKGROUND: The diagnosis of spontaneous or post-traumatic intracranial hypotension (IH) mainly relies on clinical features and neuro-imaging. However, the results of brain and spine magnetic resonance imaging are not always contributive. There is an interest for other non-invasive procedures, able to confirm or refute the diagnosis. The use of oto-acoustic emissions (OAE) was previously reported on isolated cases of IH associated with endolymphatic hydrops (ELH). The aim of this study was to assess the real-life utilization of this electrophysiological method in a larger population of suspected IH. METHODS: A retro-prospective cohort study was conducted from November 2013 to July 2022 in patients with a suspected or doubtful diagnosis of IH. They were assessed for ELH by recording bilateral distortion product of oto-acoustic emissions (DPOAE) in sitting then in supine position. RESULTS: Among the 32 patients assessed, the diagnostic of IH was confirmed in 18 patients. An ELH was shown in 15 of them (83%), but also in seven other patients. They had several differential diagnoses: chronic migraine, Chiari malformation, rebound intracranial hypertension and perilymph fistula. CONCLUSIONS: This procedure seems to be insufficient to exclude differential diagnosis when intracranial hypotension is suspected.

Headache associated with intracranial hypotension: diagnostic challenges and difficulties in everyday neurological practice.

Low pressure of cerebrospinal fluid (CSF) is a rare cause of headache, except when the patient undergoes a lumbar puncture. Headache associated with a low CSF pressure i.e. intracranial hypotension causes diagnostic difficulties. Headaches related to spontaneous intracranial hypotension (SIH) pose a significant diagnostic challenge in everyday neurological practice. Patients with headaches due to SIH are usually diagnosed only after a long delay. Diagnostic problems may result in unnecessary invasive diagnostic procedures, or even neurosurgical operations. Diagnosing headaches attributed to SIH requires the consideration of several clinical scenarios, and the disease's features causing primary or secondary disturbances. In this review, we discuss the differential diagnosis of SIH-related headaches with reference to accumulated knowledge, including meta-analyses, guidelines, casuistry, and the applicable criteria of the International Classification of Headache Disorders. In addition, we discuss head and spine magnetic resonance imaging abnormalities, which may indicate intracranial hypotension.

Recovery and long-term outcome after neurosurgical closure of spinal CSF leaks in patients with spontaneous intracranial hypotension.

INTRODUCTION: Spontaneous intracranial hypotension due to a spinal cerebrospinal fluid leak causes orthostatic headaches and impacts quality of life. Successful closure rates are often reported, whereas data on long-term outcome are still scarce. METHODS: Between April 2020 and December 2022 surgically treated patients completed the Headache Impact Test-6 prior to surgery and at 14 days, three months, six months, and 12 months postoperatively. In addition to the Headache Impact Test-6 score, we extracted data related to orthostatic symptoms. RESULTS: Eighty patients were included. Median Headache Impact Test-6 score preoperatively was 65 (IQR 61-69), indicating severe and disabling impact of headaches. At three months headache impact significantly improved to 49 (IQR 44-58) (p < 0.001) and remained stable up to 12 months (48, IQR 40-56), indicating little to no impact of headaches on quality of life. The need to lie down "always" or "very often" was reduced from 79% to 23% three months postoperatively (p < 0.001). CONCLUSIONS: Surgical closure of spinal CSF leaks significantly improves the impact of headaches in the long term. At least three months should be expected for recovery. Despite permanent closure of the CSF-leak, a quarter of patients still have relevant long-term impairment, indicating the need for further research on its cause and possible treatment.

Spontaneous intracranial hypotension: updates from diagnosis to treatment.

Spontaneous intracranial hypotension (SIH) is caused by spinal cerebrospinal fluid (CSF) leaks, which result in continued loss of CSF volume and multiple debilitating clinical manifestations. The estimated annual incidence of SIH is 5/100,000. Diagnostic methods have evolved in recent years due to improved understanding of pathophysiology and implementation of advanced myelographic techniques. Here, we synthesize recent updates and contextualize them in an algorithm for diagnosis and treatment of SIH, highlighting basic principles and points of practice variability or continued debate. This discussion includes finer points of SIH diagnosis, CSF leak classification systems, less common types and variants of CSF leaks, brain MRI Bern scoring, potential SIH complications, key technical considerations, and positioning strategies for different types of dynamic myelography. The roles of conservative measures, non-targeted or targeted blood patches, surgery, and recently developed endovascular techniques are presented.

Spontaneous Intracranial Hypotension in Pregnancy.

PURPOSE OF REVIEW: Spontaneous intracranial hypotension (SIH) is a debilitating disease typically featuring orthostatic headache and caused by a spinal CSF leak. This review will describe the characteristics of SIH in pregnant patients and the associated unique management and treatment considerations. RECENT FINDINGS: Herein, a novel case is reported of a 41-year-old woman who presented with SIH pre-conception but saw marked improvement of symptoms after 5 weeks antepartum and symptom recurrence 2 months post-partum. A literature review of SIH in pregnancy revealed 14 reported patients across 10 studies since 2000. All the reported cases resulted in delivery of healthy infants and symptomatic improvement with conservative management or a variety of treatment modalities including non-targeted epidural blood patch (EBP). Clinical and imaging features of SIH in pregnancy are reviewed. We hypothesize an antenatal protective mechanism against SIH symptoms through cephalad redistribution of CSF volume from the spinal to intracranial compartments related to uterine growth and decreased CSF volume within the lumbar cistern. Treatment recommendations are discussed including duration of bed rest and decision for non-targeted multi-site EBPs. When required, non-invasive diagnostic spine MRI using fat-suppressed axial T2-weighted imaging may be helpful.

Surgical management of spontaneous intracranial hypotension syndrome: a literature review.

INTRODUCTION: Spontaneous intracranial hypotension (SIH) is a highly disabling but often misdiagnosed disorder. The optimal management options for patients with SIH remain uncertain. The aim of this study was to review studies reporting the management of SIH with a special emphasis on the surgical treatment of SIH including clinical trials, case series and case reports related to the issue of various neurosurgical procedures performed for SIH treatment. OBJECTIVE: The clinical outcomes of patients diagnosed with SIH treated with either only surgery or with surgery as the primary method of treatment were analysed. MATERIAL AND METHODS: The PubMed, Scopus and Google Scholar databases were searched according to the established criteria. RESULTS: The literature search revealed seven clinical trials, five case series and eight case reports regarding surgical treatment of patients diagnosed with SIH. Manuscripts reporting at least five individuals treated surgically for SIH were considered as case series. In most published articles, surgery provided clinical benefit, resulting in a success rate of 82.6-100% for complete relief of SIH symptoms. CONCLUSIONS: Our literature review has revealed that SIH can be diagnosed reliably by MRI and cisternography. The identification of the location of SIH is mandatory for its successful surgical treatment. The clinical outcome is related to the location of SIH in the spinal canal. Most often, cerebrospinal fluid leakage occurs in the thoracic region. Surgical treatment is very effective and the obtained treatment results are complete and permanent. of the location of SIH is mandatory for its successful surgical treatment. The clinical outcome is related to the location of SIH in the spinal canal. Most often, cerebrospinal fluid leakage occurs in the thoracic region. Surgical treatment is very effective and the obtained treatment results are complete and permanent.

Update on "Treatment of orthostatic headache without intracranial hypotension: A case report" - nine years later.

BACKGROUND: In 2013, one of the authors described a 36-year-old female with orthostatic headache without documented intracranial hypotension or evidence of cerebrospinal fluid leak, despite extensive workup. Headache was unresponsive to conservative treatment since 2010, showed only transient benefit after repeated epidural blood patches while vitamin A supplementation resulted in progressive improvement. CASE: Since 2013, the patient followed a relapsing and remitting course yet relapse control became difficult after a drug induced liver injury required vitamin A discontinuation in 2017, when her headache became chronic. Greater occipital nerve blocks provided pain relief as alternative but were stopped due to the pandemic and her latest severe relapse, in late 2020, required not only restarting anaesthetic blocks and aggressive medication management, but also reassessing and treating comorbidities (obstructive sleep apnoea and major depressive disorder) with modest benefit. CONCLUSION: Orthostatic headache without intracranial hypotension is rare, with only 28 cases reported so far, all treated empirically and all treatment options revealing to be mostly ineffective. Vitamin A anecdotally appeared to be useful in our case but had to be stopped for severe side effects, so unfavourable long-term prognosis, in ours and 2/3 of the reported cases, seems to be the rule in this intriguing entity.

Headache and Autonomic Dysfunction: a Review.

PURPOSE OF REVIEW: We explore the anatomy of the central and peripheral autonomic pathways involved in primary headache as well as the mechanisms for secondary headache associated with disorders of the autonomic nervous system. The prevalence and clinical presentation of cranial and systemic autonomic symptoms in these conditions will be discussed, with a focus on recent studies. RECENT FINDINGS: Several small studies have utilized the relationship between headache and the autonomic nervous system to identify potential biomarkers to aid in diagnosis of migraine and cluster headache. Headache in postural orthostatic tachycardia syndrome (POTS) has also been further characterized, particularly in its association with orthostatic headache and spontaneous intracranial hypotension (SIH). This review examines the pathophysiology of primary and secondary headache disorders in the context of the autonomic nervous system. Mechanisms of headache associated with systemic autonomic disorders are also reviewed.

Magnetic resonance imaging findings of intracranial hypotension.

PURPOSE: This study aims to evaluate the differences in the sizes and configurations of various structures on brain MRIs of patients with intracranial hypotension (ICH) compared to normal individuals. METHODS: The present study consisted of two study groups as 21 patients with intracranial hypotension and 21 healthy individuals. Cranial MRI findings of patients with intracranial hypotension were compared retrospectively with MRI findings of patients without any pathology. Pachymeningeal enhancement, mamillopontine distance, venous sinus diameters, transverse and straight sinus distension, pituitary gland enlargement, tonsillar herniation, bleeding (subdural, epidural), pontomesencephalic angle, lateral ventricular angle, and pituitary infundibular angle were evaluated on MRI. RESULTS: Intracranial hypotension developed spontaneously in 6 cases and secondary in 15 patients. Diffuse pachymeningeal enhancement was observed in all intracranial hypotension cases. In addition, transverse sinus distension was observed in 19 cases, straight sinus distension in 17 cases, subdural effusion in 7 cases, spinal epidural effusion in 3 cases, tonsillar herniation in 2 cases, and thrombosis in dural sinuses in 2 cases. The intracranial hypotension group vs control group had dominant transverse sinus diameter 10 ± 1.75 vs 7.52 ± 1.2 mm, straight sinus diameter 4.76 ± 0.92 vs 3.69 ± 0.57 mm, superior sagittal sinus diameter 8.35 ± 1.57 vs 6.37 ± 0.71 mm, pontomesencephalic angle 46.67 ± 9.73° vs 56.27° ± 8.9°, mamillopontine distance 5.83 ± 1.5 vs 6.85 ± 1.1 mm, lateral ventricular angle 131.13° ± 6.17° vs 135.19° ± 5.28°, pituitary infundibular angle 44.42° ± 12.09° vs 63.3° ± 11.56°, and pituitary gland height 8.5 ± 1.83 vs 5.5 ± 1.27 mm, respectively. CONCLUSION: In cases with clinically suspected intracranial hypotension, MRI findings may contribute to the diagnosis of intracranial hypotension with quantitative evaluations.

Efficacy and safety of cervicothoracic epidural blood patch for patients with spontaneous intracranial hypotension.

BACKGROUND: Epidural blood patch (EBP) is a generally effective treatment for spontaneous intracranial hypotension (SIH) caused by cerebrospinal fluid (CSF) leakage through the spinal dura mater. It is still unclear; however, whether application near the leakage site (targeted EBP) is more effective than distal application (untargeted EBP). Further, EBP targeted to high thoracic or cervical spine levels is infrequent due to greater technical requirements and potential complications. Here, we examined the safety and efficacy of EBP applied to high thoracic or cervical spine levels. METHODS: We retrospectively reviewed the clinical and outcome data of 13 patients receiving cervical or high thoracic EBP for SIH. All patients were referred by neurologists following poor response to conservative treatment and presented with persistent headache aggravated by orthostatic changes. RESULTS: Neuroimaging confirmed CSF leakage and targeted EBP resulted in immediate pain improvement. Repeated injections provided additional improvement for patients with recurrent headache. No serious adverse events were documented during follow-up. CONCLUSION: Based on recent studies and our clinical experience, we conclude that EBP targeted to the high thoracic and cervical spine is safe and effective for early-stage SIH.

Surgical treatment of spontaneous intracranial hypotension syndrome secondary to the cervical cerebrospinal leak - a case report.

Spontaneous Intracranial Hypotension (SIH) is formed as a result of the leakage of cerebrospinal fluid (CSF) into the extradural space. The most common symptom caused by SIH is headache associated with changes in body position. Imaging, especially magnetic resonance imaging (MRI) of the head and spine with contrast, is the most important examination method in the diagnosis of SIH. In the case of no improvement after symptomatic treatment, surgery is very effective, especially in the case of finding the site of CSF leakage within the spinal canal. A CASE REPORT: We present a case of a 28-year-old patient with 2- month history of severe increasing headaches. The patient denied any head injuries, nasal CSF leakage or lumbar puncture in the past. The neurological examination showed no abnormalities. MRI of the head showed typical features of the intracranial hypotension syndrome. MRI of the cervical spine revealed potential sites for leakage of the cerebrospinal fluid at the C1-C2 level. The patient was qualified for surgical treatment - dural plastic surgery in the cervical section. After treatment the symptoms of intracranial hypotension resolved, and subsequent control MRI examinations showed regression of typical changes of the spontaneous intracranial hypotension. CONCLUSIONS: The authors would like to take notice that imaging diagnostics due to headaches should include MRI of the head and spine, as it is the most important test in diagnosing SIH. Moreover, surgical treatment is a quick and very effective method of treatment.

Spontaneous spinal cerebrospinal fluid-venous fistulas in patients with orthostatic headaches and normal conventional brain and spine imaging.

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.

Synergistic but separable sensory changes in postural tachycardia syndrome and chronic migraine.

PURPOSE: Up to 90% of patients with postural tachycardia syndrome (PoTS) report headaches, and comorbid migraine headaches are common. Given this, pathophysiological interaction is possible, which may reveal key aspects of disease expression and treatment opportunities. We hypothesized that PoTS subjects-both with and without migraine-would show features of central sensitization, including allodynia and photophobia. METHODS: Eighty participants were evaluated, including 30 PoTS, 30 chronic migraine (CM), and 20 non-headache healthy controls (NH), using tilt table testing, psychophysical assessment of sensory sensitivity thresholds, and an online questionnaire to assess measures of headache burden and associated symptoms. Clinical characteristics and sensory thresholds were compared between disease groups and controls, as well as in a subgroup analysis within the PoTS group, based on headache phenotype. RESULTS: Sensory sensitivity thresholds were significantly lower and symptom scores were higher in both the PoTS and CM groups compared to controls. However, the patterns of expression differed between PoTS and CM, with pain threshold reductions in the forearm only of PoTS subjects (non-trigeminal sensory sensitization), compared to both periorbital and forearm sites in CM. Unexpectedly, light sensitivity thresholds were significantly lower in PoTS than in both CM and NH. CONCLUSIONS: These findings reveal an underappreciated aspect of disease burden in PoTS, and suggest network sensitization similar to, but separable from, that of migraine. The presence of both photophobia and allodynia in PoTS is reflective of exteroceptive rather than strictly interoceptive disruption, and expands our fundamental understanding of the disorder.

The domino effect of acephalgic spontaneous intracranial hypotension.

Spontaneous intracranial hypotension results from a spinal CSF leak and usually presents with orthostatic headache, although acephalgic presentations have anecdotally been reported. The underlying low CSF volume, rarely, leads to serious complications such as cerebral venous thrombosis and coma. We report a patient presenting with cerebral venous thrombosis secondary to acephalgic spontaneous intracranial hypotension. An epidural blood patch was performed; nonetheless, the patient intracracal condition deteriorated to coma and neuroimages showed a deep brain swelling with midbrain distortion, subsequently complicated by intracranial pontine hemorrhage.

Management of Spontaneous Intracranial Hypotension During Pregnancy: A Case Series.

BACKGROUND: Spontaneous intracranial hypotension (SIH) is a rare condition resulting from cerebrospinal fluid (CSF) volume depletion, nearly always from spontaneous CSF leaks. CSF pressure in SIH is usually normal; low CSF pressure is found in a substantial minority of patients. SIH is uncommonly described in pregnancy. CASE SERIES: Five women with SIH during pregnancy have been conservatively treated adopting bed rest and overhydration. After prolonged conservative treatment, only 1 patient showed complete symptoms resolution. A rare SIH complication as cerebral venous thrombosis has been reported in 1 case. All 4 remaining patients had lumbar epidural blood patch (EBP) with symptoms disappearance. CONCLUSIONS: EBP might be proposed to SIH patients also during pregnancy and after a brief period (~10 days) of ineffective conservative treatment, because it could allow faster symptoms improvement and complete recovery. Furthermore, EBP would avoid prolonged bed rest with the risk of SIH severe complications.

Spontaneous intracranial hypotension: key features for a frequently misdiagnosed disorder.

Spontaneous intracranial hypotension (SIH) is a rare neurological condition caused by low cerebrospinal fluid (CSF) volume, most commonly due to a CSF leak. The most common presenting symptom is an orthostatic headache, but some patients may present with atypical neurological manifestations such as cranial nerve palsies, an altered mental status, and movement disorders, which complicate the clinical diagnosis. Therefore, the diagnosis is based on the combination of clinical signs and symptoms, neuroimaging, and/or a low cerebrospinal fluid pressure. In this review, we describe the wide variety of neurological manifestations and complications seen in patients with SIH as well as the most common features described on imaging studies, including both subjective and objective measurements, in order to lead the clinician to a correct diagnosis. The prompt and correct management of patients with SIH will help prevent the development of life-threatening complications, such as subdural hematomas, cerebral venous thrombosis, and coma, and avoid unnecessary invasive procedures.

Ventral Displacement of Spinal Cord in Spontaneous Intracranial Hypotension: A Sign Easily Be Overlooked.

PURPOSE: Spontaneous intracranial hypotension (SIH) is suspected in patients presenting orthostatic headache and needs excluding structural or iatrogenic causes. Image studies are required to confirm the diagnosis and define exact locations of cerebrospinal fluid leakage, but currently there is no single study sensitive enough to make identifications among patients with various symptoms. CASE REPORT: We present a 24-year-old young woman having acute orthostatic headache. She denied having previous headache, head trauma, or neuraxial procedures like lumbar punctures. Brain magnetic resonance image (MRI) with gadolinium enhancement reported normal findings on arrival. She received conservative treatment including analgesics and aggressive intravenous hydration, but her headache improved little. Whole spine MRI with gadolinium enhancement revealed no obvious leakage of cerebrospinal fluid but typical dilated epidural veins with ventral displacement of her thoracic spinal cord. After autologous epidural blood patches therapy, her headache relieved completely. CONCLUSION: We review the typical and uncommon findings of spinal MRI in SIH, which is more sensitive than brain MRI in acute stages. Spinal MRI offers the diagnostic value in SIH especially when cranial images do not respond in time.