Spontaneous Intracranial Hypotension Patients Research Articles

Intrathecal Saline Infusion: An Emergency Procedure in a Patient with Spontaneous Intracranial Hypotension

Spontaneous intracranial hypotension (SIH) is a neurologic condition with the prototypical symptom of orthostatic headache. We report a dramatic case of SIH with life-threatening bilateral hygroma and uncal herniation. Case report. A 44-year-old male patient presenting with orthostatic headache and double vision was diagnosed with SIH. Diagnostic imaging showed meningeal enhancement and bilateral hygroma. A conservative treatment regime was initiated. The patient's condition rapidly deteriorated with progressive loss of consciousness. Cranial MRI showed beginning uncal herniation. As an emergency treatment measure, an intracranial pressure (ICP) probe was inserted and intrathecal lumbal saline infusion was initiated. This led to a stabilization of ICP and allowed further diagnostics and treatment. Intrathecal lumbal saline infusion in combination with ICP monitoring can be a life-saving treatment option in unstable SIH patients.

Comparison of the radioisotope cisternography findings of spontaneous intracranial hypotension and iatrogenic cerebrospinal fluid leakage focusing on chronological changes

Radioisotope cisternography (RICG) is useful for detecting cerebrospinal fluid (CSF) leakage in spontaneous intracranial hypotension (SIH) patients. However, RICG can cause iatrogenic CSF leakage (ICSFL) due to a lumbar puncture. To compare the RICG findings of SIH and ICSFL. The presence of direct findings suggesting CSF leakage and indirect findings including early visualization of the bladder and absence of radioactivity over the brain convexities were evaluated in seven SIH and six ICSFL patients. Radioisotope clearance was assessed semi-quantitatively. In contrast to the variety of anatomical levels at which direct findings were detected in the SIH patients, the ICSFL patients only displayed direct findings at the lumbosacral level. None of the ICSFL patients displayed direct findings at 1 hour after the tracer injection. Although early visualization of the bladder was depicted in all patients, no activity was visualized over the brain convexities in the SIH patients. In the semi-quantitative analysis, the tracer retention index at 24 hours was lower in the SIH patients than the ICSFL patients. The early appearance of direct findings above the lumbosacral level, the absence of radioactivity over the brain convexities and a low tracer retention index are suggestive of SIH.

Assessment of CSF Flow Dynamics Using PC‐MRI in Spontaneous Intracranial Hypotension

Spontaneous intracranial hypotension (SIH) is caused by spontaneous cerebrospinal fluid (CSF) leaks and is known to cause orthostatic headaches. Phase-contrast magnetic resonance imaging (PC-MRI) is a non-invasive technique that can be used to quantify variation in CSF flow. The aim of this study was to assess CSF flow dynamics using PC-MRI in SIH. Twenty-five patients with a definitive diagnosis of SIH and 25 healthy subjects were evaluated with PC-MRI. Magnetic resonance (MR) images were acquired using a 1.5-T unit with an 8-channel head coil. Differences between SIH patients and control subjects were assessed statistically using Wilcoxon's rank sum test, Spearman's rho test, or Pearson's chi-square test, as appropriate. CSF flow volumes toward the third ventricle, CSF flow volumes toward the fourth ventricle, the absolute stroke volume, the peak systolic velocity, and the peak diastolic velocity in SIH patients were significantly smaller than those in control subjects (P < .0001). On the other hand, the net CSF flow volume (P = .9227) and the net CSF flow direction (P = .2472) for SIH patients and control subjects were not significantly different. The results obtained by CSF flow analysis were directly related to values of CSF opening pressure, determined by lumbar puncture, and clinical findings, such as headache scores. Thus, CSF flow analysis with PC-MRI, which has a short performance time and is non-invasive, may contribute to assessment of SIH patients.

Connective tissue disorders in patients with spontaneous intracranial hypotension

Spontaneous intracranial hypotension (SIH) is caused by spinal cerebrospinal fluid (CSF) leakage. An underlying connective tissue disorder has been hypothesized to cause dural weakness and predisposition to CSF leak. We conducted a case-controlled study to investigate the role of connective tissue disorders in SIH patients. We recruited 55 consecutive SIH patients (38 F, 17 M; mean age, 40.8 ± 9.8 years) and 55 age- and sex-matched control individuals (mean age, 38.0 ± 8.9 years) for this study. The connective tissue disorders were evaluated by: (i) Beighton hypermobility scores and revised diagnostic criteria for benign joint hypermobility syndrome; (ii) skin and skeletal manifestations of Ehlers-Danlos syndrome (EDS); and (iii) skeletal features of Marfan syndrome. The frequencies of joint hypermobility according to Beighton scores >4/9 (SIH 23.6% vs controls 16.4%, P = 0.48) and revised benign joint hypermobility syndrome criteria (SIH 23.6% vs controls 34.5%, P = 0.29) did not differ between SIH patients and controls. Sixteen patients and 16 controls had one or more skin features of EDS (P = 1.0). Nine SIH patients (16.4%) demonstrated the skeletal features of Marfan syndrome; this frequency did not differ from that of the control group (9.1%; P = 0.262). Only dolichostenomelia (disproportionately long limbs) was more prominent in SIH patients than in controls (34.5% vs 9.1%; P = 0.002). Compared with Western studies, the frequencies of connective tissue disorders were higher in our SIH patients. However, these frequencies did not differ between SIH patients and control individuals, except for dolichostenomelia.

Spontaneous Intracranial Hypotension Presenting Without Orthostatic Headache Complicated by Acute Subdural Hematoma After Drainage for Chronic Subdural Hematoma-Case Report-

A 28-year-old man presented with a case of spontaneous intracranial hypotension (SIH) manifesting as a bilateral chronic subdural hematoma (CSDH) without orthostatic headache. He developed life-threatening acute SDH as a complication of CSDH drainage. Neurosurgeons should be aware that SIH patients do not always present with orthostatic headache. Brain magnetic resonance imaging with gadolinium may be recommended for young adults with non-traumatic CSDH before drainage to exclude SIH, even if they do not present with orthostatic headache.

Spontaneous Intracranial Hypotension: Clinical Presentation, Imaging Features and Treatment

In the present study, the authors investigated the clinical and imaging features as well as the therapeutic outcomes of SIH (spontaneous intracranial hypotension) patients. A retrospective review of 12 SIH patients was carried out. The diagnostic work-up included lumbar tapping and measurement of CSF opening pressure, radioisotope cisternography, brain and spinal magnetic resonance imaging (MRI), and computed tomography (CT) myelography. Autologous epidural blood patching was performed in patients who did not respond to conservative therapies, including analgesics, steroids, hydration and rest. Typical postural headache was found in 11 (91%) patients. Nine (75%) patients showed pachymeningeal enhancement on their initial T1-weighted MR images. The CSF opening pressure was less than 60 mmH(2)O in 9 of 11 patients. Autologous epidural blood patching was performed in 7 patients, and all of them showed good responses. SIH can present with various clinical presentations and neuroimaging findings. Autologous epidural blood patching is thought to be the treatment of choice for patients with SIH.

Spontaneous Intracranial Hypotension Treated by Epidural Blood Patches

We present a case of spontaneous intracranial hypotension (SIH) diagnosed from the clinical symptoms and magnetic resonance imaging brain scans. After failure of conservative treatment, and lack of identification of the cerebrospinal fluid leak site, the headache was managed successfully and simply with two applications of an epidural blood patch (EBP). The strategy of our management for the patient was as follows: (1) application of an EBP to the lumbar epidural space initially and manipulating it into the proximity of the possible leak site; (2) a greater volume of autologous blood was injected at the second attempt of EBP; and (3) the patient was required to lie flat for at least 2 hours after the procedure. Better management of SIH is still developing. Traditionally, SIH is initially managed by conservative treatment. However, recent studies have shown that the success rate in arresting SIH after weeks or months of conservative treatment is not quite satisfactory. Hence, the EBP has proven to be more effective in treating SIH patients. Early EBP application may offer immediate relief of clinical symptoms. The role of the EBP in treating SIH patients should have greater emphasis and its application is worth recommendation.

Headache attributed to spontaneous intracranial hypotension

Recent evidence suggests that spontaneous intracranial hypotension (SIH) is not as rare as previously thought. Orthostatic headache is the prototypical manifestation of SIH, but various headache syndromes have also been reported such as migraine-like headache, tension-type headache and non-specific headaches among the others. The International Headache Society (IHS) has recently proposed diagnostic criteria for headache attributed to SIH. Seventy patients consecutively seen at our institution between 1993 and 2005 and diagnosed with SIH were included in the study. SIH diagnosis was confirmed in all patients by brain-enhanced MRI: 23 were males (33%), 47 females (77%) and mean age was 45 years (range 18-69). median value 35 months (range: 8 months-14 years). Time between symptom onset and diagnosis was 4 months (median) (range 15 days-45 months). The IHS (2004) criteria for "Headache attributed to SIH" were applied. Typical brain imaging findings confirmed the diagnosis of SIH in all patients: criteria B and C were fulfilled in all patients. Criterion A of the IHS classification was not fulfilled in 34 (49%) patients. Sixty-two (89%) patients did not fulfil criterion D of the IHS classification; 28 (40%) did not fulfil both criterion A and D. So far, only 2 (3%) fulfilled all IHS criteria for headache attributed to SIH. The IHS criteria for headache attributed to SIH could not classify the headache in most of our SIH patients. A revision of the IHS criteria for headache attributed to SIH is necessary.

The Timing of MRI Determines the Presence or Absence of Diffuse Pachymeningeal Enhancement in Patients with Spontaneous Intracranial Hypotension

The timing and clinical relevance of diffuse pachymeningeal enhancement (DPE) in the magnetic resonance imaging (MRI) examination of patients with spontaneous intracranial hypotension (SIH) remain undetermined. We reviewed 53 consecutive SIH patients (30 F/23 M, mean age of onset 41.7 +/- 11.3 years) in a tertiary hospital. Thirteen (24.5%) patients did not have DPE on their initial cranial MRIs. They had significantly shorter latency between the time of MRI examinations and the time of headache onset compared with those with DPE (6.5 +/- 4.4 vs. 20.4 +/- 16.3 days, t-test, P < 0.001). Eight of these 13 patients received a follow-up MRI (mean duration 30.3 +/- 16.6 days, range 6-59 days) and six of them revealed DPE. Among patients with DPE, the enhancement disappeared as early as 25 days after headache onset. The outcome did not differ between patients with and without DPE. The presence of DPE was associated with the timing of the MRI examination.

Spontaneous intracranial hypotension with deep brain swelling

Spontaneous intracranial hypotension (SIH) is caused by leakage of CSF, and characterized on MRI by brain sagging, dilatation of veins and dural sinuses, subdural fluid collections and post-contrast enhancement of the thickened dura. A few cases may present a very severe brain sagging through the tentorial notch and swelling of the diencephalic-mesencephalic structures, with absent or scarce subdural collections and post-contrast enhancement. These patients may have surprisingly few neurological signs or may become drowsy and even lapse into coma due to central herniation. We retrospectively examined the diffusion studies obtained in five patients with these MRI findings, in seven patients with SIH without brain swellings and in ten controls. Mean diffusivity was increased in SIH patients with brain swelling in areas draining into the deep venous system, collected by the vein of Galen (vG) and straight sinus (SS). In the hypothesis that central herniation might be responsible for venous stagnation because of impaired flow of the vG into the SS, the vG/SS angle was measured. The angle formed by the vG entering the SS was not altered in patients without brain swelling (group E, 67.8 degrees +/- 10.3 degrees, mean +/- SD, range 49-80 degrees) when compared to controls (group C, 73.3 degrees +/- 12.3 degrees, mean +/- SD, range 56-95 degrees). It was, however, grossly decreased in patients with brain swelling (group D, 40.7 degrees +/- 12.8 degrees, mean +/- SD, range 22-61 degrees), P < 0.001 for comparison with groups E and C. As suggested by previous studies, downward stretching of the vG and narrowing of the vG/SS angle may cause a functional stenosis at the vG-SS junction. We suggest that in the application of the Monro-Kellie doctrine to SIH, the brain volume should not be considered as always invariable.

Atypical Spontaneous Intracranial Hypotension (SIH) With Nonorthostatic Headache

Some patients with spontaneous intracranial hypotension (SIH) often do not demonstrate typical orthostatic headache, which is contrary to the typical SIH syndrome. They usually have an obscure and intermittent headache, regardless of their positional change. The objective of this study is to investigate the clinical course of atypical SIH that manifests with diffuse pachymeningeal enhancement, but no orthostatic headaches. Between January 1997 and December 2005, we observed a total of 6 patients who revealed atypical presentations including nonpostural headaches and normal cerebrospinal fluid (CSF) pressure, despite the diffuse pachymeningeal enhancement seen on their MR images. For a comparison of the clinical features and the disease course, 13 other SIH patients with typical clinical manifestations were selected as a control group. Cerebrospinal fluid leakage sites were confirmed in only one patient through a variety of diagnostic tools; in contrast, definite focal CSF leakage sites were found in 12 of 13 patients with typical SIH. The 6 atypical SIH patients were treated with conservative treatment, including strict bed rest and intravenous hydration for 2 to 3 weeks. After a mean follow-up of 12 months, their headaches were gradually relieved after 2 to 3 weeks of conservative treatment only. All SIHs do not necessarily show the typical clinical manifestations. The atypical finding of SIH such as nonorthostatic headache or normal CSF pressure may be the result of a normal physiologic response to the typical SIH as a compensatory reaction. Therefore, when faced with patients showing findings compatible with SIH on the brain MR images, regardless of nonpostural headache, atypical SIH should be suspected.

Subdural Haematoma in Patients With Spontaneous Intracranial Hypotension

The incidence and clinical relevance of subdural haematoma (SDH) in patients with spontaneous intracranial hypotension (SIH) remain undetermined. We reviewed 40 consecutive SIH patients (18 female, 22 male) in a tertiary hospital. Eight (20%) of them had SDH and nine (23%), non-haemorrhagic subdural collections. The presence of SDH was associated with higher frequencies of male gender, recurrence of severe headache and neurological deficits. Outcomes were satisfactory after supportive care or epidural blood patches except for one SDH patient, who developed transtentorial herniation resulting in Duret haemorrhage and infarctions of bilateral posterior cerebral artery territories. In conclusion, subdural fluid collections were common in patients with SIH. SDH was associated with headache worsening or neurological deficits. Patients with SDH generally recovered well; however, serious sequela might occur.

Spontaneous CSF leak treated with percutaneous CT-guided fibrin glue

Gladstone et al.1 advocate sealing CSF leaks with CT-guided, targeted fibrin glue injections in patients with spontaneous intracranial hypotension (SIH). We agree that the injection should be made at the site of the leak as a distant epidural blood patch may often give only temporary relief. In our SIH patients, we obtained permanent sealing of the leak in 13 of 16 cases in which the CSF loss at a spinal root level was approached either by direct surgery (three successful cases) or by targeted percutaneous …

Abducens nerve paresis in spontaneous intracranial hypotension syndrome.

Editor, Over the past decade the rare syndrome of spontaneous intracranial hypotension (SIH) has been recognized with increasing frequency. Most SIH patients are found to have spontaneous cerebrospinal fluid (CSF) leaks and have unique, clinically distinct imaging findings, which confirm the diagnosis (Lay 2002). Spontaneous intracranial hypotension is a benign and usually self-limiting syndrome of orthostatic headache in association with one or more of numerous symptoms, including nausea, vomiting, horizontal diplopia, unsteadiness or vertigo, altered hearing, neck pain or stiffness, interscapular pain and, occasionally, transient visual obscurations, blurred vision, visual field defects, photophobia and nystagmus (Mokri 2001; Lay 2002). The headache is relieved when the patient spends a few minutes in a supine position and becomes worse in an upright position. By definition, the opening CSF pressure is low; however, the pressure may be normal (Mokri 2001, 2003; Lay 2002). Spontaneous intracranial hypotension is more common in women than men (Diaz 2001). Stigmata of connective tissue abnormalities, especially abnormalities of fibrillin and elastin, are seen in a notable minority of patients, pointing to weakness of the dural sac as one of the aetiological factors (Mokri 2001, 2003; Schievink et al. 2004). The locations of the leaks are mainly cervical or at the cervicothoracal junction (Thoemke et al. 1999; Mokri 2001). Brain and spinal magnetic resonance imaging (MRI) studies show diffuse pachymeningeal gadolinium enhancement, frequently in association with sinking of the brain, and less frequently with subdural fluid collections, engorged cerebral venous sinuses, enlarged pituitary gland, or decreased size of the ventricles (Thoemke et al. 1999; Diaz 2001; Mokri 2001, 2003; Lay 2002). Although conservative measures are often undertaken first, epidural blood patch (EBP − injection of autologous blood in the epidural space) is the treatment of choice. For those who fail EBP, surgery may need to be undertaken in those cases with clearly identified leaks (Mokri 2001; Lay 2002). A 39-year-old woman had severe orthostatic headache, nausea, vertigo and tinnitus over a period of several weeks in November and December 2003. Lumbar puncture revealed a low− normal CSF pressure. Over the next weeks she noted neck stiffness and horizontal diplopia. On examination in January 2004, visual acuity was 20/20 in each eye with correction. No visual field defect was detected. There was a right abducens nerve paresis resulting in 7 degrees of esotropia in primary gaze. An MRI scan demonstrated diffuse pachymeningeal gadolinium enhancement and small bilateral subdural effusions (Fig. 1). The clinical history, ocular findings, and MRI features were so characteristic of SIH that a second lumbar puncture was deemed unnecessary. The patient was treated by EBP. Her headache and the right abducens nerve paresis resolved over the next 4 weeks. The MRI scan of a 39-year-old woman with SIH demonstrated diffuse pachymeningeal gadolinium enhancement and small bilateral subdural effusions. Spontaneous intracranial hypotension is a rare syndrome, characterized by orthostatic headaches, characteristic aspects on MRI, and, usually, low CSF pressure. It is thought that in SIH the brain sags and exerts pressure and traction on meningeal and vascular structures, causing headache, a stiff neck and nausea (Horton & Fishman 1994; Christoforidis et al. 1998). Vertigo and tinnitus probably result from changes in intralabyrinthine pressure (Christoforidis et al. 1998). Visual symptoms were reported in 17 of 40 patients (42.5%) with SIH. They included diplopia and occasionally transient visual obscurations, blurred vision, visual field defects, photophobia and nystagmus (Horton & Fishman 1994; Christoforidis et al. 1998; Mokri 2001; Lay 2002). Diplopia occurred in 30% of patients. Inferior displacement of the brain stem is thought to stretch the abducens nerve, which is anchored to Grubber's ligament, thus causing diplopia. Crowding of the optic nerves in the chiasmatic cistern is thought to cause the visual field defects (Horton & Fishman 1994; Christoforidis et al. 1998). Epidural blood patch is the treatment of choice (Mokri 2001; Lay 2002). In our patient all findings resolved after treatment.

Spontaneous Intracranial Hypotension and Epidural Blood Patch: A case report

Spontaneous intracranial hypotension (SIH), a syndrome of low CSF pressure, occurs without preceeding events such as lumbar puncture, back trauma, an operative procedure, or medical illness. Postural headache, the most characteristic symptom, usually resolves spontaneously or with bed rest, hydration, and medication. Autologous epidural blood patch is an effective management of headache in SIH patients, and here we describe a one case successfully treated 5 times with an epidural blood patch. The patient had a cardinal feature of a postural headache, low CSF pressure, and showed CSF leakages on RI cisternography. The first three epidural blood patches improved the patient's headache markedly, but the headache recurred in one month. On her second RI cisternography, we found other CSF leakage sites. After a fifth epidural blood patch the patient became asymptomatic and remained so throughout 4 months of follow up. Epidural blood patches are considered the treatment of choice in SIH patients.