Spontaneous Intracranial Hypotension Without CSF Leakage—Concept of a Pathological Cranial to Spinal Fluid Shift

Goldberg, Johannes; Häni, Levin; Jesse, Christopher Marvin; Zubak, Irena; Piechowiak, Eike Immo; Gralla, Jan; Dobrocky, Tomas; Beck, Jürgen; Raabe, Andreas

doi:10.3389/fneur.2021.760081

Cited by 15 publications

(31 citation statements)

References 39 publications

(50 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Although the exact mechanism behind this can only be speculated at this moment, it is presumably multifactorial, involving both the compensatory mechanism and cranial to spinal fluid shift concept that has been proposed in patients with SIH ( 10 , 11 ). In our patient, we believe that the decompensation was preceded by CSF depletion, causing the brain sagging, responsible for patients' initial BSD signs and symptoms, as described in great detail in a narrative review that we recently published ( 3 ).…”

Section: Discussionmentioning

confidence: 99%

The first report on brain sagging dementia caused by a cranial leak: A case report

Lashkarivand

Eide²

2022

Front. Neurol.

View full text Add to dashboard Cite

ObjectiveBrain Sagging Dementia (BSD) is an increasingly recognized syndrome for which diagnostic criteria recently were proposed. There have been no reports on BSD caused by a cranial leak. Here we present the first report on a patient with BSD caused by a cranial leak.Case descriptionA 60-year old male patient was admitted with a 2-year history of orthostatic headache and gradually progressive cognitive and behavioral changes. Traditional treatments for spontaneous intracranial hypotension, including repeated epidural blood patches, failed. Brain imaging showed severe brain sagging, and intracranial pressure monitoring demonstrated intracranial hypotension. No leakage site was found. His past medical history revealed an accident where a ski pole struck his head at age ten. Due to progressive clinical decline, surgery was pursued. A cranial defect with an accompanying cerebrospinal fluid leak site representing the trauma from his childhood was found and repaired. He also was in need of a ventriculoperitoneal shunt. Following surgery, he improved and recovered completely.DiscussionThis case report illustrates that a cranial leak may cause BSD, even with a “lucid interval” between trauma and symptom debut spanning many years. Moreover, this report validates well the recently proposed BSD diagnostic criteria.

show abstract

Section: Discussionmentioning

confidence: 99%

The first report on brain sagging dementia caused by a cranial leak: A case report

Lashkarivand

Eide²

2022

Front. Neurol.

View full text Add to dashboard Cite

show abstract

“…Finally, another possibility might be the development of CSF–venous fistulas in cases where imaging shows no leakage, suggesting that in those cases CSF may be drained into the epidural veins [ 17 ]. In rare cases of patients with SIH symptoms and classical cranial imaging findings, with no evidence, however, of CSF leak in spite of repeated spinal neuroimaging, it has been hypothesized that a pathological increased compliance of the spinal compartment, perhaps due to a prolapsing arachnoid or increased distensibility of the dura mater, can cause orthostatic CSF shift from the cranial to the spinal region and the characteristic symptoms of intracranial hypotension and brain sagging [ 3 ]. Coexisting predisposing factors might be an overall low total CSF volume, smaller intracranial CSF volume, decreased CSF outflow resistance and decreased intracranial venous pressure, leading to increased CSF reabsorption [ 3 ].…”

Section: Discussionmentioning

confidence: 99%

“…Occasionally, in patients with well recognized SIH symptoms and typical cranial imaging findings, there is no evidence of CSF leak despite a thorough and targeted diagnostic workup. In these cases, a pathological cranial-to-spinal shift without overt CSF leak has been advocated [ 3 ]. This may be due to increased spinal compliance, leading to downward displacement of cranial structures and to the clinical manifestations of SIH.…”

Section: Introductionmentioning

confidence: 99%

Treating Spontaneous Intracranial Hypotension with an Anesthetic Modality: The Role of the Epidural Blood Patch

Masourou

Papagiannakis

Mantzikopoulos

et al. 2022

Life

View full text Add to dashboard Cite

Background: Spontaneous intracranial hypotension (SIH) is a rare syndrome characterized by heterogeneity of presentation and prognosis, which can occasionally result in serious complications, such as the formation of subdural hematomas (SDHs). This case series aims to emphasize that SIH remains a diagnostic and therapeutic challenge; it can present with a broad clinical spectrum of symptoms, can lead to SDH and, if conservative treatment fails, an epidural blood patch (EBP) is a viable treatment option. Although the exact etiology of SIH is not known, it is believed to be due to cerebrospinal fluid (CSF) leak or a low CSF pressure. Case Series: Three patients (two males and one female) with ages ranging between 38 and 53 years old who presented with complaints of not only an orthostatic headache, but also a variety of symptoms of SIH, including the formation of two SDHs in one of them, were included in this series. These patients did not respond to conservative management and, subsequently, given the clinical and radiological evidence of SIH, were referred to the Anesthesiology Department for an EBP. Diagnostic workup was facilitated by imaging modalities, including magnetic resonance imaging (MRI) of the brain and spinal cord, prior to the EBP. All three patients were subjected to an EBP with an 18-gauge epidural needle. A total of between 30 and 43 mL of autologous blood was collected from the patients and was injected into the epidural space under strict aseptic conditions. Two lumbar (L1–L2, L2–L3) EBPs and one thoracic (T11–T12) EBP were performed on the three patients, respectively. All patients reported complete resolution of symptoms following the EBPs, while MRI improved substantially. Conclusions: This report describes three cases of SIH with CSF leak originating from the cervical, the thoracic and the lumbar level. The EBP restored CSF pressure and relieved the patients’ persistent symptoms. MRI helps in revealing indirect signs of a low volume of CSF, though it may not be possible to locate the actual site of the leak. In conclusion, EBP is a well-accepted and beneficial treatment modality for SIH when conventional measures fail.

show abstract

“…The first theory of how EBP works relates to a compression of the thecal sac as a result of blood being injected in the epidural space This implies a decreased thecal sac compliance, which results in a reduced CSF shift from the cranial to the spinal compartment after changing from the recumbent to the upright position (Fig. 4b; [1,5,21,22]).…”

Section: Discussionmentioning

confidence: 99%

“…After epidural injection of autologous blood, local compression of the thecal sac is demonstrated, leading to a decreased compliance of the CSF compartment. c Occlusion of the dural breach due to fibrous transformation of the coagulated blood inside the leak ment over time results in decreasing compression of the thecal sac, leading again to an increased compliance and may thus explain the decreasing effectiveness seen at longterm follow-up [22,23].…”

Section: Discussionmentioning

confidence: 99%

Epidural Blood Patching in Spontaneous Intracranial Hypotension—Do we Really Seal the Leak?

et al. 2022

Self Cite

View full text Add to dashboard Cite

Purpose Epidural blood patch (EBP) is a minimally invasive treatment for spontaneous intracranial hypotension (SIH). Follow-up after EBP primarily relies on clinical presentation and data demonstrating successful sealing of the underlying spinal cerebrospinal fluid (CSF) leak are lacking. Our aim was to evaluate the rate of successfully sealed spinal CSF leaks in SIH patients after non-targeted EBP. Methods Patients with SIH and a confirmed spinal CSF leak who had been treated with non-targeted EBP were retrospectively analyzed. Primary outcome was persistence of CSF leak on spine MRI or intraoperatively. Secondary outcome was change in clinical symptoms after EBP. Results In this study 51 SIH patients (mean age, 47 ± 13 years; 33/51, 65% female) treated with non-targeted EBP (mean, 1.3 EBPs per person; range, 1–4) were analyzed. Overall, 36/51 (71%) patients had a persistent spinal CSF leak after EBP on postinterventional imaging and/or intraoperatively. In a best-case scenario accounting for missing data, the success rate of sealing a spinal CSF leak with an EBP was 29%. Complete or substantial symptom improvement in the short term was reported in 45/51 (88%), and in the long term in 17/51 (33%) patients. Conclusion Non-targeted EBP is an effective symptomatic treatment providing short-term relief in a substantial number of SIH patients; however, successful sealing of the underlying spinal CSF leak by EBP is rare, which might explain the high rate of delayed symptom recurrence. The potentially irreversible and severe morbidity associated with long-standing intracranial hypotension supports permanent closure of the leak.

show abstract

Spontaneous Intracranial Hypotension Without CSF Leakage—Concept of a Pathological Cranial to Spinal Fluid Shift

Cited by 15 publications

References 39 publications

The first report on brain sagging dementia caused by a cranial leak: A case report

The first report on brain sagging dementia caused by a cranial leak: A case report

Treating Spontaneous Intracranial Hypotension with an Anesthetic Modality: The Role of the Epidural Blood Patch

Epidural Blood Patching in Spontaneous Intracranial Hypotension—Do we Really Seal the Leak?

Contact Info

Product

Resources

About