First-line steroid treatment for spontaneous intracranial hypotension.

BACKGROUND: Spontaneous intracranial hypotension (SIH) is a syndrome characterized by low cerebrospinal fluid (CSF) pressure and postural headaches, and affects 1 per 20,000 individuals every year. CASE REPORT: We report an otherwise healthy 38-year-old man admitted to the hospital with orthostatic headache that developed 48 h after a short-haul flight during which he sustained a neck injury due to turbulence. Neurological examination, blood analysis and computed tomography scan performed at the emergency service were normal. Brain and spine magnetic resonance imaging (MRI) showed diffuse pachymeningeal enhancement and contrast medium egress from the subarachnoid space into the epidural space at the level of C2. The patient was treated with bed rest, hydration and 1 mg/kg/day oral prednisone for 5 days, with a gradual withdrawal in the following 7 days. Complete symptomatic relief was observed after 16 days, with resolution of the pathological findings on brain and spinal MRI after 1 month, except for localized pachymeningeal enhancement. Clinical relief was maintained over time until last follow-up visit 9 months later. CONCLUSION: Successful conservative treatment barely exceeds one quarter of cases of SIH. The clinical benefits of steroids may result from several mechanisms of action, for example, improving brain oedema and inflammation, determining fluid retention, and facilitating reabsorption of the CSF from extradural space. Notwithstanding that epidural blood patch remains the most successful treatment for SIH, future studies should explore the effectiveness of steroids as first-line therapy in addition to the most commonly suggested measures of bed rest and hydration.

Headache in spontaneous intracranial hypotension: an overview with indications for differential diagnosis in the clinical practice.

Headache is the most common symptom of spontaneous intracranial hypotension (SIH). The present review focuses on data regarding headache features reported in the most relevant published articles and summarizes the main SIH headache features, namely, orthostatic headache, headache triggered by Valsalva maneuver, pattern of onset of headache, and location and quality of headache. Published data indicate that the clinical suspect of this disorder may be challenging, due to its protean presentation. Among the main implications for clinical practice, we suggest to suspect SIH in all patients with a new onset headache, as different forms of primary and secondary headache should be considered in the differential diagnosis of SIH, particularly cervicogenic headache, new daily persistent headache, and headaches precipitated by Valsalva maneuver. The clinical interview must include specific questions on the possible orthostatic feature of headache, although its absence should not make clinicians to reject the SIH hypothesis as headache cannot be orthostatic in each patient and in all periods of the natural history of the disease. Other disorders with orthostatic symptoms, such as in postural tachycardia syndrome (POTS) and persistent postural-perceptual dizziness (PPPD), should be considered in the differential diagnosis. Awareness that SIH can present with acute, sudden onset requires that clinicians working in the emergency settings should consider SIH in the range of diagnoses of thunderclap headache.

MRI Findings of Spontaneous Intracranial Hypotension: Usefulness of Straight Sinus Distention.

OBJECTIVE. Spontaneous intracranial hypotension (SIH) shows various characteristic MRI findings. We evaluated the usefulness of straight sinus distention compared with transverse sinus distention and also evaluated other MRI findings of SIH. MATERIALS AND METHODS. Forty-three consecutive patients (28 female and 15 male patients) treated for SIH and 43 age- and sex-matched control subjects at two institutions from 2012 through 2014 were included in this study. Two reviewers determined whether the transverse sinus distention sign and straight sinus distention sign were present on MRI. Diagnostic performance values and interobserver agreement were calculated. Reviewers also assessed MRI examinations in consensus for the presence of the following findings: pachymeningeal enhancement, subdural effusion or hematoma, enlargement of the pituitary gland, and downward displacement of the brainstem and tonsils. RESULTS. The sensitivity, specificity, and diagnostic accuracy of the transverse sinus distention sign for SIH were 76.7%, 83.7%, and 80.2%, whereas those of the straight sinus distention sign were 79.1%, 95.4%, and 87.2%, respectively. The specificity of the straight sinus distention sign for SIH was significantly higher (p = 0.025) than that of the transverse sinus distention sign. In addition, the straight sinus distention sign showed substantial agreement (κ = 0.79), whereas the transverse sinus distention sign showed moderate agreement (κ = 0.60). The diagnostic accuracy of the presence of either transverse or straight sinus distention (83.7%) was significantly higher than that of pachymeningeal enhancement (80.2%, p = 0.032). CONCLUSION. The straight sinus distention sign could be helpful for the diagnosis of SIH because it has sensitivity comparable to other imaging findings and higher specificity and higher level of interobserver agreement than other imaging findings.

Spontaneous intracranial hypotension: two steroid-responsive cases.

PURPOSE: Spontaneous intracranial hypotension (SIH) is characterised by orthostatic headache, low cerebrospinal fluid pressure and diffuse pachymeningeal enhancement after intravenous gadolinium contrast administration. Magnetic resonance imaging (MRI) often plays a crucial role for correct diagnosis. CASE DESCRIPTION: We described two similar cases of SIH, whose clinical and imaging features are typical for this pathology. At MRI brain scan, both patients showed diffuse and intense pachymeningeal enhancement and moderate venous distension and epidural vein engorgement. The two patients were treated with bed rest and oral steroid therapy, with complete and long-lasting symptomatic relief. CONCLUSIONS: Orthostatic nature of headache is the most indicative clinical feature suggesting SIH; contrast-enhanced MRI provides definite imaging diagnostic findings. Conservative treatment coupled to steroid therapy is often sufficient to obtain complete disappearance of symptoms.

Usefulness of phase-contrast magnetic resonance imaging for diagnosis and treatment evaluation in patients with SIH.

BACKGROUND: Most diagnostic tools for spontaneous intracranial hypotension (SIH) are either invasive or occasionally inconsistent with the clinical condition. In this study, we examined the cerebrospinal fluid (CSF) dynamics in SIH using phase-contrast magnetic resonance (PC-MR) imaging. MATERIALS AND METHOD: Seventeen SIH patients and 32 healthy individuals, matched by sex and age, were recruited. Each person underwent brain and PC-MR imaging using 3-Tesla MRI. We evaluated the differences in image parameters among patients during the initial and recovery stages against the status of the control group. RESULTS: SIH patients had lower CSF flow-volume, flux, peak velocity, and higher systolic-to-diastolic time ratio, as well as systolic-to-diastolic volume ratio compared to the control group and the conditions when they recovered. The flow time and volume of the diastolic phase markedly increased after treatment. The discriminating power of PC-MR for SIH was good. Diffuse pachymeningeal enhancement and venous engorgement were present when their PC-MR values were lower than the cut-off values for SIH diagnosis. The headache scores correlated with the peak velocity and pituitary volume. CONCLUSION: Noninvasive PC-MR could provide valid parameters for diagnosis and treatment follow-up in SIH patients. It may be more sensitive than conventional brain MRI.

Recurrent Spontaneous Intracranial Hypotension (SIH) and the Durability of Repeat Epidural Blood Patch (EBP).

OBJECTIVE: Spontaneous intracranial hypotension (SIH) remains a rare and difficult clinical entity to diagnose and treat. Epidural blood patch (EBP) of the dural sac is the mainstay definitive treatment for refractory cases and has mixed efficacy. We sought to evaluate the recent efficacy and outcomes of EBP for SIH at our institution. METHODS: Twenty-three patients (14 women, 9 men, mean age 49) were seen and treated for SIH between Summer 2009 and Spring 2018 at the same institution. All patients underwent brain MRI with and without gadolinium contrast and T2-weighted spine MRI. Targeted EBP was placed one or two vertebral levels below areas of suspected leak, while the patient was positioned in the lateral decubitus position. Patients were seen in the outpatient setting within a week following initial EBP and repeat EBP was offered to patients with persistent symptoms. Patients were followed if symptoms persisted or for 6 months following clinical relief of symptoms. RESULTS: 22/23 (95.7%) patients presented with complaints of orthostatic headache, and 3 (13%) patients presented with altered mental status (AMS) or focal neurologic deficit. Brain MRI demonstrated pachymeningeal enhancement in 16/23 (69.6%) patients, and 5/23 (21.7%) patients had a subdural hematoma (SDH) present. Dural leaks were successfully identified in 18/23 (78.3%) patients. 12/23 (52.2%) patients had symptomatic relief with initial EBP, and 5/23 (21.7%) patients received further EBPs for persistent disease with all achieving relief after repeat EBP. 5/12 (41.7%) of patients had recurrent symptoms after initial relief with EBP, and 4/5 (80%) were successfully treated with a second EBP. The mean initial EBP volume and number of EBPs per patient were 21.7 mL (median 20 mL, 7-40 mL) and 3.54 (median 1, 1-13) respectively. There was one complication from initial EBP (cervical dural tear requiring operative closure) treated with open surgical management successfully. In total, 18/23 (78.2%) patients are currently asymptomatic with regard to their SIH. The mean follow-up in this cohort was 2.6 years (median 1.8 years, 1.8 months-9.27 years). CONCLUSIONS: EBP is a viable and effective option for the treatment of recurrent SIH caused by cerebrospinal fluid (CSF) leaks.

Overview of Spontaneous Intracranial Hypotension and Differential Diagnosis with Chiari I Malformation.

Spontaneous intracranial hypotension (SIH) occurs due to a leakage of the cerebrospinal fluid (CSF) lowering the pressure of subarachnoid space, mostly caused by a dural breach or discogenic microspur. As a result of less support provided by CSF pressure, intracranial structures are stretched downward, leading to a constellation of more or less typical MRI findings, including venous congestion, subdural effusions, brainstem sagging and low-lying cerebellar tonsils. Clinic examination and an MRI are usually enough to allow for the diagnosis; however, finding the location of the dural tear is challenging. SIH shares some MRI features with Chiari malformation type I (CM1), especially low-lying cerebellar tonsils. Since SIH is likely underdiagnosed, these findings could be interpreted as signs of CM1, leading to a misdiagnosis and an incorrect treatment pathway. Medical treatment, including steroids, bed rest, hydration caffeine, and a blind epidural blood patch, have been used in this condition with variable success rates. For some years, CSF venous fistulas have been described as the cause of SIH, and a specific diagnostic and therapeutic pathway have been proposed. The current literature on SIH with a focus on diagnosis, treatment, and differential diagnosis with CM1, is reviewed and discussed.

Closing the Gap: A Case Report on a Blood Patch Solution for Cerebrospinal Fluid Leak.

Spontaneous intracranial hypotension is a condition resulting from cerebrospinal fluid leaks at the spinal level that disrupt the regulation of intracranial pressure. This disorder is an uncommon cause of debilitating headaches but can have variable clinical manifestations, which contributes to delayed diagnosis and potentially severe consequences. The standard treatment consists of conservative measures such as bed rest, hydration, and a pharmacological approach with paracetamol, caffeine, ergotamine, and dexamethasone. When conservative measures fail, an epidural blood patch is the gold standard treatment, where a small amount of blood is injected into the epidural space to form a clot to seal any existing leak. Recent studies showed a success rate of 64% without the need for further intervention. The authors report a case of a 55-year-old woman with a three-month history of daily severe headaches. Imaging exams showed subdural collections, suggesting the hypothesis of cerebrospinal fluid hypotension. After the failure of conservative measures, an epidural blood patch was performed with progressive clinical improvement.  This case demonstrates the potential effectiveness of an epidural blood patch in the management of spontaneous intracranial hypotension and its complications, offering an encouraging option for those unresponsive to conservative measures. It also highlights the importance of a multidisciplinary approach involving neurologists and anesthesiologists.

T2-Sampling Perfection With Application-Optimized Contrasts by Using Flip Angle Evolution (SPACE) Protocol MRI: A Safe, Minimally Invasive Screening Tool for Spinal CSF Leak Causing Spontaneous Intracranial Hypotension.

Spontaneous intracranial hypotension (SIH) due to a spinal cerebrospinal fluid (CSF) leak is secondary cause of headache with potentially devastating consequences. Its diagnosis is complicated owing to the lack of a reasonable, minimally invasive screening test. This results in many patients remaining undiagnosed for years after the headache onset. Current testing approaches are either overly invasive, namely the CSF infusion protocol or both invasive and insensitive viz. lumbar puncture (LP) with an opening pressure (OP) or computed tomography myelogram (CTM). These diagnostic methods are frequently employed in a clinical setting since they require access to the thecal space; they unfortunately have a dearth of sensitivity. CTM will not document a leak if it is intermittent or very slow and in the setting of a spinal CSF leak, the OP on LP may be high, low, or normal. A potential remedy for this state is the T2-sampling perfection with application-optimized contrasts by using flip angle evolution (SPACE) protocol spinal magnetic resonance imaging (MRI). We present two cases that demonstrate its potential value as a screening tool. It is well known for its high sensitivity for identifying spinal pathology and is minimally invasive, making it a good choice for a screening modality when diagnosing possible SIH cases.

Spontaneous Intracranial Hypotension Manifesting Orthostatic Headache Worsened by Playing the Saxophone and Treated by Japanese Herbal Kampo Medicine Goreisan.

We present a 15-year-old Japanese girl with no previous medical history who presented with a gradually worsening series of orthostatic headaches. We diagnosed spontaneous intracranial hypotension, worsened by playing the saxophone and its Valsalva maneuver effect. She was treated with Japanese herbal Kampo medicine Goreisan 7.5 g/day in three divided doses, and her symptoms gradually improved. Her headache has never recurred for a year when she played the saxophone. Our case's headache may have been further exacerbated by cerebrospinal fluid (CSF) leakage due to CSF pressure increase by Valsalva maneuvers while playing the saxophone. Our case suggested that the Japanese herbal Kampo medicine Goreisan can facilitate the glymphatic system and adjust the CSF pressure appropriately.

Cerebrospinal fluid hypovolemia syndrome mimicking ocular myasthenia gravis: A case report.

Purpose: Cerebrospinal fluid hypovolemia syndrome (CHS) is a rare clinical entity that can be caused by spontaneous cerebrospinal fluid (CSF) leakage. The aim of this study is to report a rare case of CHS after a traffic accident in a patient who presented with diplopia and ptosis with fluctuation and was initially diagnosed with ocular myasthenia gravis. Observeations: A 29-year-old man exhibited fluctuating left ptosis and diplopia after a traffic accident. Although he was suspected of having myasthenia gravis and was treated using oral pyridostigmine bromide, his symptoms did not improve. He also had orthostatic headaches and malaise after the accident. His symptoms were suspected to be associated with traumatic cerebrospinal fluid hypovolemia. After 1000-mL fluid replacement, his diplopia and ptosis improved, and orbital T2-weghted MRI detected a high-signal zone around the optic nerve. We diagnosed him with oculomotor nerve paresis associated with cerebrospinal fluid hypovolemia. The symptoms, including ptosis, diplopia, orthostatic headaches, and malaise, disappeared after epidural blood patch therapy. Conclusions and Importance: When treating patients with fluctuating ocular symptoms, such as diplopia and ptosis, who have a history of trauma and orthostatic headaches, the possibility of CHS should be considered in the differential diagnosis.

Total transferrin in cerebrospinal fluid is a novel biomarker for spontaneous intracranial hypotension.

Spontaneous intracranial hypotension (SIH) is caused by cerebrospinal fluid (CSF) leakage. Patients with SIH experience postural headaches, nausea, etc., due to CSF hypovolemia. Imaging studies and clinical examinations, such as radioisotope (RI) scintigraphy, are useful for diagnosing SIH. However, 20-30% of patients do not show typical morphology and clinical test results. We previously reported that CSF contains transferrin (Tf) isoforms:"brain-type" Tf derived from the choroid plexus and "serum-type" Tf derived from blood. We showed that both isoforms increased in the CSF of patients with SIH by Western blotting. In the present study, we demonstrate that conventional ELISA for quantifying total Tf is useful for diagnosing SIH more accurately than Western blotting. In addition, SIH with chronic subdural hematoma (CSDH) was also accurately diagnosed.　Total Tf in the CSF can serve as a useful biomarker for diagnosing SIH with or without CSDH.

An unusual postural headache: a case report.

BACKGROUND: This paper presents a case of an evolving unusual thunderclap headache that presented to a chiropractor. CASE PRESENTATION: The intense "migraine-like" headache was aggravated by standing up and relieved substantially when lying down. This low pressure, orthostatic headache was diagnosed as a spontaneous intracranial hypotension (SIH) secondary to a spontaneous tear of the dura. It was referred to the local hospital for management with autogolous blood injection to form an epidural blood patch of the defect. It resolved substantially within 3 days. CONCLUSIONS: The significance of key features in the history and examination and how if not recognised and subsequently treated with manual therapy, the dural tear could be attributed to the treatment of the chiropractor, a treatment that would typically involve cervical manipulation. Discussion is provided of the implications of a missed diagnosis and possible subsequent chiropractic management with the evolving SIH being attributed to the chiropractic intervention rather than its true "spontaneous" nature.

Penetrating Osseous Spicules Causing High-Flow Ventral CSF Leaks in the Setting of Relatively Low BMI : A Preliminary Study.

OBJECTIVE/BACKGROUND: We have anecdotally observed patients with high-flow ventral cerebrospinal fluid (CSF) leaks resulting from penetrating osseous spicules or calcified discs to be relatively thin. The purpose of this study was to explore the validity of this observation and determine if a potential association exists between low body mass index (BMI) and high-flow spinal ventral CSF leaks resulting from such dura-penetrating lesions. METHODS: Sixteen consecutive patients with precisely localized high-flow ventral spinal CSF leaks on dynamic myelography were identified. The cause of the CSF leak was determined. The BMI on the date nearest to and within 2 weeks of myelography was recorded. Utilizing exact sign test, the body mass index was compared to the average BMI from the National Health and Nutrition Examination Survey (Centers for Disease Control), matched to sex and age-range. RESULTS: The cohort consisted of 10 males (63%) and 6 females with a mean age of 54 years (range 37-72 years). In all patients, a spiculated osteophyte/calcified disc was identified at the site of the leak. Fourteen patients (88%) had a BMI below the matched national average, while only two patients (13%) had values above the national average (p = 0.004). CONCLUSIONS: Patients with high-flow ventral CSF leaks resulting from spiculated osteophyte or calcified disc as identified by dynamic myelography are more likely to have a BMI below the U.S. national average, matched for gender and age-range. This exploratory analysis requires confirmation as well as further characterization of potential pathophysiologic mechanisms and impact on radiographic and clinical assessments.

Spontaneous CSF leaks: low CSF volume syndromes.

Practically all cases of spontaneous intracranial hypotension results from spontaneous cerebral spinal fluid (CSF) leaks, often at the level of the spine and only rarely from the skull base. The triad of orthostatic headaches, diffuse pachymeningeal enhancement on head imaging and low CSF opening pressure is considered the hallmark of these leaks but substantial variability is noted in most aspects of this disorder including in features of the headaches, imaging and CSF findings, response to treatment and outcome.