Clinical Presentation, Investigation Findings, and Treatment Outcomes of Spontaneous Intracranial Hypotension Syndrome

Abstract

IMPORTANCE Spontaneous intracranial hypotension (SIH) is a highly disabling but often misdiagnosed disorder. The best management options for patients with SIH are still uncertain.OBJECTIVE To provide an objective summary of the available evidence on the clinical presentation, investigations findings, and treatment outcomes for SIH.DATA SOURCES Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) reporting guideline-compliant systematic review and meta-analysis of the literature on SIH. Three databases were searched from inception to April 30, 2020: PubMed/MEDLINE, Embase, and Cochrane. The following search terms were used in each database: spontaneous intracranial hypotension, low CSF syndrome, low CSF pressure syndrome, low CSF volume syndrome, intracranial hypotension, low CSF pressure, low CSF volume, CSF hypovolemia, CSF hypovolaemia, spontaneous spinal CSF leak, spinal CSF leak, and CSF leak syndrome. STUDY SELECTION Original studies in English language reporting 10 or more patients with SIH were selected by consensus.DATA EXTRACTION AND SYNTHESIS Data on clinical presentation, investigations findings, and treatment outcomes were collected and summarized by multiple observers. Random-effect meta-analyses were used to calculate pooled estimates of means and proportions. MAIN OUTCOMES AND MEASURESThe predetermined main outcomes were the pooled estimate proportions of symptoms of SIH, imaging findings (brain and spinal imaging), and treatment outcomes (conservative, epidural blood patches, and surgical). RESULTSOf 6878 articles, 144 met the selection criteria and reported on average 53 patients with SIH each (range, 10-568 patients). The most common symptoms were orthostatic headache (92% [95% CI, 87%-96%]), nausea (54% [95% CI, 46%-62%]), and neck pain/stiffness (43% [95% CI, 32%-53%]). Brain magnetic resonance imaging was the most sensitive investigation, with diffuse pachymeningeal enhancement identified in 73% (95% CI, 67%-80%) of patients. Brain magnetic resonance imaging findings were normal in 19% (95% CI, 13%-24%) of patients. Spinal neuroimaging identified extradural cerebrospinal fluid in 48% to 76% of patients. Digital subtraction myelography and magnetic resonance myelography with intrathecal gadolinium had high sensitivity in identifying the exact leak site. Lumbar puncture opening pressures were low, normal (60-200 mm H 2 O), and high in 67% (95% CI, 54%-80%), 32% (95% CI, 20%-44%), and 3% (95% CI, 1%-6%), respectively. Conservative treatment was effective in 28% (95% CI, 18%-37%) of patients and a single epidural blood patch was successful in 64% (95% CI, 56%-72%). Large epidural blood patches (>20 mL) had better success rates than small epidural blood patches (77% [95% CI, 63%-91%] and 66% [95% CI, 55%-77%], respectively).CONCLUSIONS AND RELEVANCE Spontaneous intracranial hypotension should not be excluded on the basis of a nonorthostatic headache, normal neuroimaging findings, or normal lumbar puncture opening pressure. Despite the heterogeneous nature of the studies...

The effectiveness of a blind blood patch at the L1-2 level for the treatment of spontaneous intracranial hypotension

“…There are no guidelines widely acknowledged for the treatment of SIH yet. According to meta-analysis about SIH, conservative treatment including bed rest, hydration, analgesia, steroids, caffeine was effective in about 24.5% of 881 patients for a period ranging from 7 to 9 weeks [7,17]. EBP is commonly used treatment to patients showed no response to conservative treatment.…”

“…Even though EBP is commonly used for the treatment of SIH, the process how it acts has not been determined, but may involve a temporary increase in epidural pressure, the sealing of a tear by coagulating injected blood, or the initiation of inflammatory reactions that promote dural tear healing [8,[10][11][12]. According to previous studies, the therapeutic effect of EBP showed various improvement rate in 64-93% of patients after first treatment [7,13,[18][19][20]. There are two methods of EBP, targeted and non-targeted one.…”

“…SIH is not uncommon with an annual incidence of 5 per 100,000 individuals per year and typically encountered in middle age and showed female preponderance [2,3].Diagnostic exams including lumbar puncture, radioisotope cisternography, computed tomography myelography (CTM), and magnetic resonance myelography are performed for suspected patients. Although various neuroimaging features of SIH has been suggested, such diagnostic findings are not always noticed [4][5][6].The treatment of SIH includes conservative treatments such as bed rest and fluid infusion or surgical repair of dural defect, but recently, when conservative treatment is ineffective, epidural blood patch (EBP) is a treatment of choice [3,[7][8][9]. The mechanism of EBP has not been determined, but may involve a tempo-…”

“…The treatment of SIH includes conservative treatments such as bed rest and fluid infusion or surgical repair of dural defect, but recently, when conservative treatment is ineffective, epidural blood patch (EBP) is a treatment of choice [3,[7][8][9]. The mechanism of EBP has not been determined, but may involve a tempo-…”

“…EBP is divided into a blind EBP, which is performed regardless of the location of CSF leakage, and a targeted EBP, which is performed to the location of CSF leakage [4]. There is debate which one is better [5,7,8,13,14].…”