JAMA Neurol: The latest evidence on the clinical manifestations and therapeutic characteristics of spontaneous low craniofacial pressure

Spontaneous intracranial low pressure (SIH) defines a clinical symptom characterized by a debilitating positional headache that occurs from spontaneous spinal cord fluid (CSF) leakage and/or CSF hypotension.
According to the third edition of the International Classification of Headache Diseases (ICHD), SIH can be diagnosed when headaches occur spontaneously and are associated with cerebrospinal fluid leakage (imagingly obvious) and/or hypotension of cerebrospinal fluid (lumbar puncture pressure - lt;60 mm CSF).
spontaneous intracranial low pressure is a highly misdiagnosed and underdiagnosed disease.
data show that SIH is not uncommon, occurring in 5 cases per 100,000 people per year, which is half the incidence of celiac bleeding in the cobwebs.
despite the lack of objective evidence on the impact of SIH on the quality of life of patients, typical erect headaches in this case weaken SIH and affect the patient at the most active time.
exact pathogenesis of SIH is unclear, and this lack of knowledge has led to a series of misunderstandings.
addition, SIH's ICHD diagnostic standards have changed significantly over the past few decades and have introduced alternative diagnostic criteria that may lead to current uncertainties about how to reliably diagnose SIH and treat these patients effectively.
studies have summarized available evidence on the clinical manifestations, diagnostic investigations and treatment results of SIH.
include: (1) What are the signs and symptoms of SIH and how often does it occur? (2) How sensitive are brain magnetic resonance imaging (MRI), spinal imaging, and lumbar puncture perforation pressure to SIH signs? (3) What is the most sensitive spinal cord imaging technique for detecting cerebrospinal fluid leakage? (4) What are the results of conservative treatment and epidural blood supplementation (EBP) in SIH patients? (5) What is the most effective EBP technology in SIH? (Non-targeted vs targeted, small size vs large volume).
study was published in the journal JAMA Neurol.
clinical performance, findings and treatment results were collected and summarized by multiple observers.
use random effect meta-analysis to calculate a mixture of average and proportion estimates.
predetermined major outcomes are a comprehensive estimate of SIH symptoms, imaging performance (brain and spinal imaging) and treatment outcomes (conservative, epidural blood supplementation and surgery).
6,878 articles, 144 met the selection criteria, with an average of 53 SIH patients (ranging from 10-568).
most common symptoms are erect 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 survey, with 73% of patients seeing diffuse thick meninges enhancement (95% CI, 67%-80%).
19% (95% CI, 13%-24%) of patients had normal MRI results.
neuroimaging examination of the spinal cord found that 48% to 76% of patients had epidural cerebrospinal fluid.
digital reduced shadow spinal formation and epidural magnetic resonance spinal cord ocytomy are highly sensitive in determining the exact leak site.
lumbar puncture opening pressure is low, normal (60-200mm H2O), high is 67% (95% CI, 54%-80%), 32% (95% CI, 20%-44%) and 3% (95% CI, 1%-6%).
28% (95% CI, 18%-37%) of patients were treated conservatively effectively, and 64% (95% CI, 56%-72%) of patients were successful with a single epidural.
success rate of large epidural blood supplements is better than that of small epidural blood supplements, which are 77% (95% CI, 63% to 91%) and 66% (95% CI, 55% to 77% respectively).
The clinical manifestations, findings and treatment results of 144 documents were summarized in this systematic review and meta-analysis of the results of spontaneous low craniofacial pressure and spontaneous low craniofacial pressure brain magnetic resonance imaging (MRI) performance spinal cord imaging results.
no erect headache, normal imaging performance or normal lumbar puncture pressure should not rule out the diagnosis of SIH.
a single EBP success rate of 64%.
this meta-analysis shows that large EBPs are successful in a higher proportion of patients than smaller EBPs, this requires further validation.
large randomized clinical trials are needed to determine the best treatment for SIH.
: D'Antona L, Jaime Merchan MA, Vassiliou A, et al. Clinical Presentation, Investigation Findings, and Treatment Outcomes of Spontaneous Intracranial Hypotension Syndrome: A Systematic Review and Meta-analysis. JAMA Neurol. Published online January 04, 2021. doi:10.1001/jamaneurol.2020.4799MedSci Original Source: MedSci Original Copyright Notice: All notes on this website "Source: Met Medical" or "Source: MedSci Original" text, images and audio and video materials, copyrights are owned by Metz Medicine, without authorization, no media, website or individual may reproduce, authorized to reproduce with the words "Source: Mets Medicine".
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