Spontaneous intracranial hypotension syndrome: a novel speculative physiopathological hypothesis and a novel patch method in a series of 28 consecutive patients

2010 ◽  
Vol 112 (2) ◽  
pp. 300-306 ◽  
Author(s):  
Angelo Franzini ◽  
Giuseppe Messina ◽  
Vittoria Nazzi ◽  
Eliana Mea ◽  
Massimo Leone ◽  
...  

Object Spontaneous intracranial hypotension (SIH) is a potentially serious pathological syndrome consisting of specific symptoms and neuroradiological signs that can sometimes be used to assess the efficacy of the treatment. In this paper the authors report a series of 28 patients with this syndrome who were all treated with an epidural blood patch at the authors' institution. The authors propose a novel physiopathological theory of SIH based on some anatomical considerations about the spinal venous drainage system. Methods Between January 1993 and January 2007, the authors treated 28 patients in whom SIH had been diagnosed. Twenty-seven of the 28 patients presented with the typical findings of SIH on brain MR imaging (dural enhancement and thickening subdural collections, caudal displacement of cerebellar tonsils, and reduction in height of suprachiasmatic cisterns). The sites of the patients' neuroradiologically suspected CSF leakage were different, but the blood patch procedure was performed at the lumbar level in all patients. The patients were then assessed at 3-month and 1- and 3-year follow-up visits. At the last visit (although only available for 11 patients) 83.3% of patients were completely free from clinical symptoms and 8.3% complained of sporadic orthostatic headache. Results The authors think that in the so-called SIH syndrome, the dural leak, even in those cases in which it can be clearly identified on neuroradiological examinations, is not the cause of the disease but the effect of the epidural hypotension maintained by the inferior cava vein outflow to the heart. The goal of their blood patch procedure (a sort of epidural block obtained using autologous blood and fibrin glue at the L1–2 level) is not to seal CSF leaks, but instead to help in reversing the CSF-blood gradient within the epidural space along the entire cord. Conclusions The authors' procedure seems to lead to good and long-lasting clinical results.

Author(s):  
Magdalena Anitescu ◽  
David Arnolds

Spontaneous intracranial hypotension is a condition that affects young and middle-aged individuals. Women are more frequently affected than men. It is associated with severe positional headache without previous dural puncture and is often confused with other common headache conditions. Delay in diagnosis of the condition may predispose patients to severe complications. Many radiodiagnostic tools carry important risks to patients, including nerve injury and iatrogenic spinal cord injury. Imaging studies must be correlated with a detailed medical history and a thorough physical examination. Epidural blood patch, the mainstay of treatment, may require multiple attempts with increasing amounts of autologous blood. Increased awareness of spontaneous intracranial hypotension will likely contribute to its proper diagnosis and treatment.


2015 ◽  
Vol 7 (1) ◽  
pp. 71-77 ◽  
Author(s):  
Joji Inamasu ◽  
Shigeta Moriya ◽  
Junpei Shibata ◽  
Tadashi Kumai ◽  
Yuichi Hirose

Spontaneous intracranial hypotension (SIH) is a syndrome in which hypovolemia of the cerebrospinal fluid (CSF) results in various symptoms. Although its prognosis is usually benign, cases with a rapid neurologic deterioration resulting in an altered mental status have been reported. One of the characteristic radiographic findings in such cases is the presence of bilateral accumulation of subdural fluid (hematoma/hygroma). When SIH-related subdural hematoma is present only unilaterally with a concomitant midline shift, making an accurate diagnosis may be challenging, and inadvertent hematoma evacuation may result in further neurologic deterioration. We report a 58-year-old woman with an altered mental status who had visited a local hospital and in whom a brain CT showed a unilateral subdural hematoma with a marked midline shift. She was referred to our department because of her neurologic deterioration after hematoma evacuation. A CT myelography revealed a massive CSF leakage in the entire thoracic epidural space. She made a full neurologic recovery following blood patch therapy. Our case is unique and educational because the suspicion for SIH as an underlying cause of subdural hematoma is warranted in nongeriatric patients not only with bilateral but also unilateral lesions. An immediate search for CSF leakage may be important in cases with failed hematoma evacuation surgery.


2015 ◽  
Vol 18;4 (4;18) ◽  
pp. 343-348
Author(s):  
Paolo Feltracco

Background: Spontaneous intracranial hypotension (SIH) results from leaks developing in the dura mater. The major symptom is orthostatic headache which gradually disappears after lying down. Lumbar epidural blood patches (EBPs) can be effective in relieving headaches, however, thoracic and cervical EBPs have also been applied to alleviate the symptoms. Objective and Methods: Retrospective collection of the main characteristics of SIH, site and amount of blood injection, and clinical outcomes of 18 patients who underwent thoracic EBPs for intractable SIH. { Study Design: Retrospective case series Results: All thoracic autologous EBPs except 3 were performed in the sitting position. Patients undergoing epidural puncture at lower thoracic levels (T10-T12) received 25 mL of autologous blood, 15 mL and 18 mL were injected at spinal segments T5-T7 (mid-thoracic) and T2-T4 (upperthoracic), respectively. Thoracic EBPs did not lead to immediate resolution of symptoms in 3 of 18 patients; one of them underwent early repetition with complete headache relief, one refused a second EBP, and one experienced partial resolution, followed by a recurrence, and then satisfactory improvement with a second high thoracic EBP. In long-term follow-up only 2 patients complained of symptoms or relapses. Limitations: Retrospective nature of the case series, single center experience. Conclusions: Performing thoracic-targeted EBPs as the preferred approach theoretically improves results with respect to those observed with lumbar EBPs. The immediate response was comparable with that of other reports, but the long-term success rate (90%) turned out to be very effective in terms of both quality of headache relief and very low incidence of recurrence. Key words: Central pain, cerebrospinal fluid leak, headache disorders, low intracranial pressure syndrome, epidural blood patch, occipital headache


2021 ◽  
Vol 14 (6) ◽  
pp. e243179
Author(s):  
Pushpendra Nath Renjen ◽  
Dinesh Mohan Chaudhari ◽  
Nidhi Goyal ◽  
Kamal Ahmed

The most common cause of spontaneous intracranial hypotension headache is a cerebrospinal fluid (CSF) leakage, but the underlying mechanisms remain unknown. Intracranial hypotension is characterised by diffuse pachymeningeal enhancement on cranial MRI features, low CSF pressure and orthostatic headaches mostly caused by the dural puncture. We report a 31-year-old woman who presented to our services with reports of continuous severe bifrontal headache, which increased on sitting up and resolved on lying down. MRI of the cervical and lumbosacral spine showed signs of CSF leak; hence, patient was diagnosed with spontaneous intracranial hypotension headache. A CT-guided epidural blood patch was done at L4–5 with fibrin glue injected at the site of leak. The patient’s signs and symptoms improved after the procedure.


Author(s):  
Farnaz Amoozegar ◽  
Esma Dilli ◽  
Rashmi B. Halker ◽  
Amaal J. Starling

Spontaneous intracranial hypotension (SIH) caused by a spontaneous cerebrospinal fluid (CSF) leak, results in CSF hypovolaemia. Owing to the variety of clinical presentations and numerous possible diagnostic investigations, diagnosis and appropriate treatment remains challenging in many patients. Although the typical presentation of SIH is an orthostatic headache, the clinical spectrum includes a variety of headache types, focal neurological symptoms, and even spinal manifestations. The underlying pathophysiology of SIH varies depending on the clinical scenario. However, a deeper understanding of the pathophysiology has led to the recognition of risk factors and an explanation for clinical symptoms and abnormalities on diagnostic investigations. Multiple diagnostic investigations can be used to determine if a CSF leak is present or not. Magnetic resonance imaging of the head with and without contrast is sensitive and non-invasive. However, computed tomography myelography remains the study of choice to locate the site of the leak. The rate of flow of the CSF leak can pose a challenge to standard diagnostic investigations. To date, a large-volume blind epidural blood patch is the mainstay of treatment, although more targeted approaches are used for more refractory cases.


2021 ◽  
Vol 36 (1) ◽  
Author(s):  
Efthalia Angelopoulou ◽  
Eirini Pantou ◽  
Georgios Zacharis ◽  
Michail Rentzos ◽  
Leonidas Stefanis ◽  
...  

Abstract Background Spontaneous intracranial hypotension (SIH) is characterized by positional headache caused by low CSF pressure, without any major traumatic event. Optimal treatment is still debated; epidural blood patch (EBP) is usually used after unsuccessful conservative treatment with variable efficacy and potentially severe complications. Although steroids have been reported to be beneficial, their effectiveness is still controversial, and more clinical evidence is needed. Case presentation A 37-year-old woman was admitted to the neurology department due to severe orthostatic headache with nausea over the last 5 days. No trauma history or spinal manipulation were mentioned. On arrival, neurological examination, brain CT, and laboratory investigation were normal. Intracranial hypotension was clinically suspected, and lumbar puncture revealed low opening pressure. Brain MRI demonstrated pachymeningeal gadolinium enhancement and distended and rounded dural venous sinuses, while cervicothoracic spine MRI revealed thoracic CSF leakage, leading to SIH diagnosis. The patient was treated with high-dose intravenous methylprednisolone, with complete clinical resolution within 24 h. Conclusions Our case, combined with literature evidence, supports the high-dose intravenous corticosteroids as a reasonable treatment option in selected cases, before trying EBP or surgical repair. Randomized clinical trials are needed, in order to optimize SIH patients’ outcomes.


2017 ◽  
Vol 79 (03) ◽  
pp. 217-223 ◽  
Author(s):  
Fei-Fang He ◽  
Li Li ◽  
Min-Jun Liu ◽  
Tai-Di Zhong ◽  
Qiao-Wei Zhang ◽  
...  

Objective An epidural blood patch (EBP) is the mainstay of treatment for refractory spontaneous intracranial hypotension (SIH). We evaluated the treatment efficacy of targeted EBP in refractory SIH. Methods All patients underwent brain magnetic resonance imaging (MRI) with contrast and heavily T2-weighted spine MRI. Whole spine computed tomography (CT) myelography with non-ionic contrast was performed in 46 patients, and whole spine MR myelography with intrathecal gadolinium was performed in 119 patients. Targeted EBPs were placed in the prone position one or two vertebral levels below the cerebrospinal fluid (CSF) leaks. Repeat EBPs were offered at 1-week intervals to patients with persistent symptoms, continued CSF leakage, or with multiple leakage sites. Results Brain MRIs showed pachymeningeal enhancement in 127 patients and subdural hematomas in 32 patients. One hundred fifty-two patients had CSF leakages on heavily T2-weighted spine MRIs. CSF leaks were also detected on CT and MR myelography in 43 and 111 patients, respectively. Good recovery was achieved in all patients after targeted EBP. No serious complications occurred in patients treated with targeted EBP during the 1 to 7 years of follow-up. Conclusions Targeted and repeat EBPs are rational choices for treatment of refractory SIH caused by CSF leakage.


2021 ◽  
Author(s):  
Elham Jafari ◽  
Maryam Karaminia ◽  
Mansoure Togha

Abstract Background: Spontaneous intracranial hypotension (SIH) is a secondary headache that has been attributed to a cerebrospinal fluid (CSF) leak. It may resolve spontaneously or require conservative treatment. An epidural blood patch (EBP) with autologous blood is performed in cases exhibiting an inadequate response to conservative methods. Rebound intracranial hypertension (RIH) can develop following an EBP in up to 27% of patients. It is characterized by a change in the headache features and is often accompanied by nausea, blurred vision. and diplopia. Symptoms commonly begin within the first 36 hours, but could develop over days to weeks. It is important to differentiate this rebound phenomenon from unimproved SIH, as the treatment options differ. Case presentation: Here we present an interesting case of a patient with SIH who was treated with EBP and developed both immediate RIH after 24 hours and delayed RIH 3 weeks following EBP. Conclusions: Following EBP for treatment of SIH, new onset of a headache having a different pattern and location should always be monitored for the occurrence of a RIH. A lumbar puncture should be done if the symptoms of elevated CSF pressure become intolerable or if the diagnosis is uncertain. Lack of early diagnosis and treatment and differentiation from SIH can cause complications and could affect the optic nerves.


Cephalalgia ◽  
2012 ◽  
Vol 33 (1) ◽  
pp. 43-51 ◽  
Author(s):  
Amer A Ghavanini ◽  
Courtney A Scott ◽  
David K Chan ◽  
David F Tang-Wai

Background Decreased level of consciousness is a rare neurological manifestation of spontaneous intracranial hypotension (SIH), which typically presents with orthostatic headache. The optimal management of this uncommon presentation remains uncertain. Methods We analyzed the presentation, management and outcome of two patients in our institution and reviewed 22 patients reported in the literature with SIH and decreased level of consciousness, defined as any decrease in the patient’s Glasgow Coma Scale score. Results There were 20 male and four female patients (M:F ratio of 5:1) with an average age of 52 years (range 37 to 68 years). There was a variable time interval of up to many months between the initial presentation of SIH and changes in the level of consciousness. An epidural autologous blood patch was eventually successful in 79% of the patients, although up to three trials were necessary in seven of these patients. Intrathecal saline infusion used as a temporizing measure resulted in excellent response within hours in five out of six patients who received this treatment. Drainage of the subdural collection either did not result in any sustained improvement or resulted in clinical deterioration in 12 out of 12 patients who received this treatment. Conclusions In the absence of a clinical trial because of the rarity of this entity, the treatment of SIH complicated by decreased level of consciousness remained controversial in the past. However, current collective experience supports early treatment of patients with SIH and decreased level of consciousness with one or more epidural blood patches. Fibrin glue and surgical duroplasty are the next steps in the management of patients in whom epidural blood patches fail. Drainage of the subdural collections may be detrimental.


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