The role of computerized tomography-myelography in a patient with spontaneous intracranial hypotension from multiple cerebrospinal fluid leaks

OBJECTIVES: To describe a patient with spontaneous intracranial hypotension (SIH) secondary to multiple sites of cerebrospinal fluid (CSF) leaks, a rarely described phenomenon. To compare computerized tomography-myelography with radionuclide cisternography as confirmatory diagnostic aids in SIH. METHODS: A patient with SIH had transient or no response to 2 thoracic epidural blood patches. A computerized tomography-myelography showed bilateral CSF leaks at T11-T12 and T12-L1 levels and on the left side of T10-T11. RESULTS: A left paramedian epidural blood patch was performed at T12-L1 under fluoroscopy guidance. Injection of 1 mL dye showed confinement of the dye at the left T11-L1 epidural space. The patient had 90% to 95% relief of her headache and complete relief at 4 months. She was asymptomatic 1 year after the last epidural blood patch. CONCLUSIONS: In a patient with SIH, a computerized tomography-myelography should be performed if an initial epidural blood patch is ineffective. This is to show the vertebral level and site(s) of CSF leak and to guide the physician to the ideal vertebral level for the epidural blood patch.     

Paradoxical postural headaches in cerebrospinal fluid leaks

Two patients with cerebrospinal fluid (CSF) leak, one at the level of fourth thoracic spine and another with undetermined level of leak, presented with paradoxical postural headaches in that the headaches were present when in a horizontal position and resolved if the patients were upright. One patient improved spontaneously and the other responded to a targeted epidural blood patch. Paradoxical postural headache is yet another headache type that can be associated with CSF leak and CSF volume depletion. Its mechanism is uncertain, but it could be related to congestion and dilatation of cerebral venous sinuses and large veins.     

Spontaneous intracranial hypotension successfully treated by epidural patching with fibrin glue

We report a case of spontaneous intracranial hypotension due to a cerebrospinal fluid leak at the C2 level, which was successfully treated by epidural fibrin glue patching. Epidural blood patching was performed twice, first with 6 mL of autologous blood and then with 10 mL, but the intracranial hypotension was unresponsive. Although successful treatment of postdural puncture headache and persistent leak after intrathecal catheterization by epidural patching with fibrin glue has been reported, fibrin glue has not been previously applied in spontaneous intracranial hypotension. Our observation suggests that epidural patching with fibrin glue should be considered in patients with spontaneous intracranial hypotension, if epidural blood patching fails to resolve the symptoms.     

Development of spontaneous intracranial hypotension concurrent with grade IV mobilization of the cervical and thoracic spine: a case report

Donovan JS, Kerber CW, Donovan WH, Marshall LF. Development of spontaneous intracranial hypotension concurrent with grade IV mobilization of the cervical and thoracic spine: a case report.Spontaneous intracranial hypotension (SIH) has been clinically defined as the development of severe orthostatic headaches caused by an acute cerebrospinal fluid (CSF) leak. Typically, intracranial hypotension occurs as a complication of lumbar puncture, but recent reports have identified cases caused by minor trauma. We report a case of SIH secondary to a dural tear caused by a cervical and thoracic spine mobilization. A 32-year-old woman with SIH presented with severe positional headaches with associated hearing loss and C6-8 nerve root distribution weakness. CSF opening pressure was less than 5cmH₂O and showed no abnormalities in white blood cell count. Cranial, cervical, and thoracic magnetic resonance imaging revealed epidural and subdural collections of CSF with associated meningeal enhancement. Repeated computed tomography myelograms localized the leak to multiple levels of the lower cervical and upper thoracic spine. A conservative management approach of bedrest and increased caffeine intake had no effect on the dural tear. The headache, hearing loss, and arm symptoms resolved completely after 2 epidural blood patches were performed. Practitioners performing manual therapy should be aware of this rare, yet potential complication of spinal mobilizations and manipulations.     

Fibrin Glue to Treat Spinal Fluid Leaks Associated with Intrathecal Drug Systems

Intrathecal drug delivery systems (IDDSs) are used to treat resistant pain states as well as intractable spasticity via medication delivery into the spinal fluid. Risks associated with implantation of these devices include infection, bleeding, intrathecal granuloma formation, and neurologic sequelae similar to other neuraxial procedures. Intrathecal catheter placement creates the additional risk of persistent spinal fluid leak, which can lead to postdural puncture headaches as well as seroma formation and may require subsequent surgical exploration or explantation. This retrospective case series examines 3 patients at a single institution with persistent spinal fluid leak after IDDS placement (and explantation in one case) resulting in headache and/or seroma formation that were treated with epidural fibrin glue. Three patients underwent IDDS implantation with baclofen for spasticity. In 1 patient, a cerebral spinal fluid leak developed at 1‐week postoperatively. After several unsuccessful epidural blood patches and surgical exploration with a catheter revision, she was ultimately treated successfully with a fibrin glue patch. The second patient received an IDDS and did well until a seroma developed 1 year later. He was likewise treated with an epidural fibrin glue patch after 2 failed blood patches. In a third patient, a spinal fluid leak developed after explantation of an IDDS and was treated with an epidural fibrin glue patch as initial therapy.     

Intracranial hypertension after treatment of spontaneous cerebrospinal fluid leaks

Four patients, aged 10 to 44 years, with spontaneous cerebrospinal fluid (CSF) leaks and intracranial hypotension developed intracranial hypertension after treatment of their CSF leaks. The leak was at the spinal level in all patients (thoracic level, 2; lumbar level, 1; and undetermined, 1). One patient responded to an epidural blood patch. Three patients responded to surgery, of whom 2 had not responded to prior epidural blood patches. Treatment resulted in complete resolution of symptoms, including orthostatic headaches and disappearance of magnetic resonance imaging abnormalities. However, all patients later developed steady headaches different from their previous headaches. None had recurrence of magnetic resonance imaging abnormalities or any evidence of occlusion of cerebral venous sinuses. All had increased CSF opening pressures. One had bilateral papilledema, and another had no venous pulsations on examination of fundi. Follow-up was possible in 2 patients. One responded well to treatment with acetazolamide, and the other improved gradually and was asymptomatic within several months.     

Spontaneous intracranial hypotension presenting as mental deterioration

A 55-year-old woman had new onset of postural headache followed by change of mental status 3 weeks later. Magnetic resonance imaging (MRI) of the brain and whole spine showed typical spontaneous intracranial hypotension (SIH) findings, bilateral subdural hematoma, and cerebrospinal fluid leakage over the T7-T9. Her headache and mentality improved after epidural blood patches. Early recognition and correct diagnosis are crucial for successful treatment in patients with SIH presenting with mental confusion.     

Spontaneous intracranial hypotension--a case report

Positional or orthostatic headache symptoms secondary to intracranial hypotension are usually seen as complications in patients after medical procedures, such as lumbar puncture. We describe a case of spontaneous intracranial hypotension (SIH) in a 43-year-old man without a previous history of headaches. He presented to the Emergency Department (ED) with a new-onset orthostatic headache without any history of recent medical procedure, such as lumbar puncture (LP). The ED evaluation included a normal neurologic examination and normal computed tomography (CT) scan of the brain. An LP showed low opening pressure, elevated protein, 46 RBCs and one lymphocyte. Subsequent evaluation with radionuclide cisternography confirmed a cerebrospinal fluid (CSF) leak in the area of the upper thoracic spine, and treatment with an epidural blood patch dramatically improved his symptoms. SIH is due to CSF leakage, usually in the area of the cervical or upper thoracic spine, often without a clear etiology. Conservative medical management including bed rest, oral hydration and caffeine intake is the usual first line treatment. An epidural blood patch is often a dramatically effective treatment that can be done once the presence and location of the leak is identified. Although mostly benign, this condition occasionally can be associated with the formation of clinically significant subdural fluid collections or hematomas. SIH is an increasingly recognized cause of headache.     

Comparisons between heavily T2-weighted MR and CT myelography studies in two patients with spontaneous intracranial hypotension

Our previous study demonstrated that heavily T2-weighted magnetic resonance (MR) myelography could successfully detect abnormal cerebrospinal fluid (CSF) collections in patients with spontaneous intracranial hypotension (SIH); however, its ability to demonstrate ongoing CSF leakage sites is uncertain. Currently, computed tomographic (CT) myelography is still considered the study of choice for such a purpose. In this study, we compared the results of the two imaging modalities in two patients with SIH. CSF leakages, such as C1-2 extraspinal collections, CSF along nerve root sleeves, and epidural fluid collections were noted on both studies with minor discrepancies. CSF collection along nerve root sleeves demonstrated by MR myelography was the most likely ongoing leakage site in comparison with CT myelography. Targeted epidural blood patches in both patients resulted in complete headache resolution. Non-invasive, non-contrasted and time-saving MR myelography is a promising study for patients with SIH. Further large-scale validation studies are needed.     

Spontaneous intracranial hypotension

Spontaneous intracranial hypotension (SIH) is typically manifested by orthostatic headaches that may be associated with one or more of several other symptoms, including pain or stiffness of the neck, nausea, emesis, horizontal diplopia, dizziness, change in hearing, visual blurring or visual field cuts, photophobia, interscapular pain, and occasionally face numbness or weakness or radicular upper-limb symptoms. Cerebrospinal fluid (CSF) pressures, by definition, are quite low. SIH almost invariably results from a spontaneous CSF leak. Only very infrequently is this leak at the skull base (cribriform plate). In the overwhelming majority of patients, the leak is at the level of the spine, particularly the thoracic spine and cervicothoracic junction. Sometimes, documented leaks and typical clinical and imaging findings of SIH are associated with CSF pressures that are consistently within limits of normal. Magnetic resonance imaging of the head typically shows diffuse pachymeningeal gadolinium enhancement, often with imaging evidence of sinking of the brain, and less frequently with subdural fluid collections, engorged cerebral venous sinuses, enlarged pituitary gland, or decreased size of the ventricles. Radioisotope cisternography typically shows absence of activity over the cerebral convexities, even at 24 or 48 hours, and early appearance of activity in the kidneys and urinary bladder, and may sometimes reveal the level of the leak. Although various treatment modalities have been implemented, epidural blood patch is probably the treatment of choice in patients who have failed an initial trial of conservative management. When adequate trials of epidural blood patches fail, surgery can offer encouraging results in selected cases in which the site of the leak has been identified. Some of the spontaneous CSF leaks are related to weakness of the meningeal sac, likely in connection with a connective tissue abnormality.     

An MRI Study of Lumbar Puncture Headaches

We studied 11 patients undergoing a routine lumbar puncture to determine if there were cerebrospinal fluid leaks at the puncture site and whether the maximum volume of leakage correlates with a lumbar puncture headache. Patients completed a headache questionnaire before and after the lumbar puncture. Limited magnetic resonance imaging of the lumbar spine was obtained 8 to 36 hours after the lumbar puncture and two patients also had later imaging. In a blinded fashion, the largest diameter of cerebrospinal fluid leakage into the paraspinous area was determined from T2 weighted magnetic resonance images and the maximum possible fluid volume was calculated. Six patients had a small cerebrospinal fluid leakage (< 10 mL), two had a medium leakage (10 to 110 mL), and three had a large leakage (> 110 mL). The volume of cerebrospinal fluid leakage did not corre late with occurrence of a lumbar puncture headache. The study demonstrates that cerebrospinal fluid usually leaks into the paraspinous area after a lumbar puncture, but the volume of escaped fluid does not correlate with a lumbar puncture headache.     

Spontaneous intracranial hypotension

Spontaneous intracranial hypotension (SIH) is caused by leakage of cerebrospinal fluid (CSF), with resultant CSF hypovolemia and intracranial hypotension. Although in some patients SIH may be preceded by minor trauma, it often occurs in the absence of any identifiable initiating event. Orthostatic headache is the primary clinical manifestation, usually accompanied by one or more other symptoms of SIH. Diagnosis can be reached by a combination of imaging studies, which may include MRI with gadolinium, nuclear cisternography, and myelography. Treatment ranges from conservative management, such as bed rest and hydration, to invasive procedures, such as lumbar puncture with autologous blood patch, CT-guided fibrin glue injection at the site of the leak, and open surgical intervention. Outcomes vary from complete resolution of CSF leak with alleviation of symptoms to continued and/or recurrent leaks with chronic unremitting symptomatology.     

Spontaneous Low Pressure,Low CSF Volume Headaches: Spontaneous CSF Leaks

Spontaneous intracranial hypotension typically results from spontaneous cerebrospinal fluid (CSF) leak, often at spine level and only rarely from skull base. Once considered rare, it is now diagnosed far more commonly than before and is recognized as an important cause of headaches. CSF leak leads to loss of CSF volume. Considering that the skull is a rigid noncollapsible container, loss of CSF volume is typically compensated by subdural fluid collections and by increase in intracranial venous blood which, in turn, causes pachymeningeal thickening, enlarged pituitary, and engorgement of cerebral venous sinuses on magnetic resonance imaging (MRI). Another consequence of CSF hypovolemia is sinking of the brain, with descent of the cerebellar tonsils and brainstem as well as crowding of the posterior fossa noted on head MRI. The clinical consequences of these changes include headaches that are often but not always orthostatic, nausea, occasional emesis, neck and interscapular pain, cochleovestibular manifestations, cranial nerve palsies, and several other manifestations attributed to pressure upon or stretching of the cranial nerves or brain or brainstem structures. CSF lymphocytic pleocytosis or increase in CSF protein concentration is not uncommon. CSF opening pressure is often low but can be within normal limits. Stigmata of disorders of connective tissue matrix are seen in some of the patients. An epidural blood patch, once or more, targeted or distant, at one site or bilevel, has emerged as the treatment of choice for those who have failed the conservative measures. Epidural injection of fibrin glue of both blood and fibrin glue can be considered in selected cases. Surgery to stop the leak is considered when the exact site of the leak has been determined by neurodiagnostic studies and when less invasive measures have failed. Subdural hematomas sometimes complicate the CSF leaks; a rebound intracranial hypertension after successful treatment of a leak is not rare. Cerebral venous sinus thrombosis as a complication is fortunately less common, and superficial siderosis and bibrachial amyotrophy are rare. Short‐term recurrences are not uncommon, and long‐term recurrences are not rare.     

Treatment Outcomes in Spontaneous Intracranial Hypotension: Do Epidural Blood Patches Stop the Leaks?

Abstract:   Spontaneous intracranial hypotension (SIH) is a postural headache syndrome unrelated to dural puncture. Because of the increasing failure of epidural blood patch (EBP) to relieve headache in SIH, we retrospectively investigated the epidemiological features and treatment outcomes in 55 cases of SIH. The study population was stratified by age and sex; continuous variables were compared for differences by t -tests; categorical variables were compared by Chi-squared analysis or Fisher exact tests. Significant differences were identified by P values of 0.05 or less. The mean age of the study population was 44 ± 12 years with a female to male ratio of 1.3:1.0. Men presented with subdural hematomas ( P  = 0.001) more often than women. Meningeal enhancement on contrast magnetic resonance imaging (MRI) was the most consistent radiographic finding. Radionuclide cisternography (RC) demonstrated thoracolumbar dural leaks in 16 of 22 patients. EBP failures were more common in patients aged 40 and younger than in older patients ( P  = 0.003). Postural headache from SIH was not uniformly responsive to EBP, and had significant comorbidities, especially in men. The management of postural headache in SIH by other techniques to restore brain position and cerebrospinal fluid dynamics should be investigated.     

Intracranial Hypotension Caused by Anterior Cervical CSF Alleviated by an Epidural Blood Patch

We present a case in which a thoracocervical epidural blood patch was used to treat an anteriorly situated cerebrospinal fluid leak following 2 failed blood patches in the lumbar region. The challenge in identifying the source of the leak, deteriorating health of the patient, and risks from the procedure, contributes to the uniqueness of this case.     

A causative role for remote dural puncture and resultant arachnoid bleb in new daily persistent headache: A case report

A 24-year-old woman experienced a postdural puncture headache following a labor epidural, recovered following bedrest, and was then without headache for 12 years. She then experienced sudden onset of daily, holocephalic headache persisting for 6 years prior to presentation. Pain reduced with prolonged recumbency. MRI brain, MRI myelography, and later bilateral decubitus digital subtraction myelography showed no cerebrospinal fluid (CSF) leak or CSF venous fistula, and normal opening pressure. Review of an initial noncontrast MRI myelogram revealed a subcentimeter dural outpouching at L3–L4, suspicious for a posttraumatic arachnoid bleb. Targeted epidural fibrin patch at the bleb resulted in profound but temporary symptom relief, and the patient was offered surgical repair. Intraoperatively, an arachnoid bleb was discovered and repaired followed by remission of headache. We report that a distant dural puncture can play a causative role in the long delayed onset of new daily persistent headache.     

Suspected subarachnoid haemorrhage with a negative CT head scan: What next?

The current recommended ‘next investigation’ for patients with a suspected subarachnoid haemorrhage and a negative non‐contrast computed tomography head scan, is a lumbar puncture. The cerebrospinal fluid obtained at lumbar puncture may be examined for the presence of blood and xanthochromia. Xanthochromia may be assessed by macroscopic examination or by spectrophotometry. Total red cell count and the three‐tube test have poor sensitivity and specificity as investigations for diagnosing subarachnoid haemorrhage. Macroscopic examination for xanthochromia is unreliable, with a risk of false‐negative results. Spectrophotometric examination for xanthochromia is associated with false positive results. The use of cerebrospinal fluid derivative spectroscopy and D‐Dimer testing using an enzyme‐linked immunosorbent assay need further evaluation. In the future, computed tomography angiography or magnetic resonance angiography may change current practice. Until then, all patients should be advised to have a lumbar puncture. A cerebrospinal fluid specimen obtained at least 8 h after the onset of headache and analysed for xanthochromia via spectrophotometry has the best chance of placing the patient in the lowest possible risk category. The emergency physician must be aware of the methods of cerebrospinal fluid analysis used locally.     

Low-Pressure/Spinal Fluid Leak Headache

Low cerebrospinal fluid (CSF) pressure results in neurologic deficits, of which the most common manifestation is headache. Typically, the headache is postural – and specifically, orthostatic – in presentation. There are three hypotheses to explain the occurrence of headache associated with low CSF fluid. The first is traction on pain-sensitive intracranial and meningeal structures; the second is CSF hypovolemia; and the third is spinal loss of CSF resulting in increased compliance at the caudal end of the CSF space. Spontaneous intracranial hypotension (SIH), once believed to be rare, is now more commonly recognized. It is typically associated with orthostatic headache (although initially it may not be) and one or more other symptoms such as alterations in hearing, nausea, vomiting, neck stiffness, diplopia, and visual field cuts. Magnetic resonance imaging (MRI) of the brain with gadolinium is the first study of choice, which typically reveals diffuse pachymeningeal enhancement and, frequently, cerebellar tonsillar descent and posterior fossa crowding. Epidural blood patch (EBP) is the treatment of choice. Surgery and epidural fibrin glue injection are options for those who fail conservative therapy and/or EBP.     

Spontaneous Low Cerebrospinal Fluid Pressure Headache

A 69 year old man presented with headache on arising and relieved with recumbency. His clinical presentation, laboratory data, response to treatment and radiographic evaluation were consistent with spontaneous low cerebrospinal fluid pressure from CSF hyperabsorption. Cisternography showed rapid accumulation in the kidneys and urinary bladder, without evidence of CSF leak. His headache was eventually controlled with two blood patches and oral caffeine.     

Postural headache and cerebrospinal fluid leak: believing is seeing

A 33-year-old woman developed persistent postural headache following epidural anesthesia (L2-L3 level). Iohexol myelography (L5-S1 puncture) demonstrated no epidural extravasation of contrast material (Figure A), but subsequent computed tomography (CT) axial images (B, C) revealed leakage of dye through the needle track of the L5-S1 puncture (arrows). No leakage of contrast medium was found at the site of the L2-L3 epidural puncture.