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.     

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.     

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.     

Intrathecal gadolinium-enhanced MR-cisternography in spontaneous intracranial hypotension associated with Behcet's syndrome

We report a 32-year-old woman diagnosed with spontaneous intracranial hypotension (SIH) and Behcet's Syndrome. Suspicion of SIH was based on cervical MRI findings, and magnetic resonance (MR)-cisternography revealed dural leaks bilaterally at lumbar region. MR-cisternography is a recently performed technique and gaining importance in demonstration of CSF fistula.     

Spontaneous Intracranial Hypotension‐Hypovolemia Associated With Tacrolimus

There is little precedent for a medication‐induced spontaneous intracranial hypotension/cerebrospinal fluid (CSF) hypovolemia (SIH). This case history of a woman with low CSF pressure, orthostatic headache, and radiographic findings consistent with SIH but without a detectable leak was notable for its association, both onset and resolution, with the use of the calcineurin inhibitor tacrolimus (FK506). A literature review for potential causes of a tacrolimus‐induced CSF hypotension suggests many potential mechanisms of action, including effects on blood brain barrier and dural compliance, and supports further vigilance for this condition in the medically complex setting of tacrolimus use.     

Transforaminal Epidural Blood Patches for the Treatment of Postsurgical Dural Leaks: Two Case Reports

Unintended dural punctures with leakage of cerebrospinal fluid (CSF) are recognized as a frequent complication of spinal surgery. Although conservative or invasive options may be used to treat postoperative CSF leaks, the existing literature does not define either an algorithmic treatment approach or a universally accepted standard of care. We believe that a transforaminal epidural blood patch (EBP) can serve as a minimally invasive, cost-effective option to treat postsurgical CSF leaks that do not resolve with conservative management. We have performed an EBP via the transforaminal route to treat postsurgical CSF leaks in both the cervical and lumbar spine. The first case describes a patient who underwent an anterior cervical diskectomy and fusion with a complication of profuse CSF leakage. The application of a cervical transforaminal EBP at the levels of surgical repair was effective in stopping the dural leak. The second case involves a patient who experienced classic positional spinal headaches after a lumbar hemilaminectomy and diskectomy. After utilization of lumbar transforaminal EBPs, his symptoms revolved. This article presents the potential use of an EBP via the transforaminal route to treat postsurgical dural leaks in both the cervical and lumbar region.

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.     

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 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.     

Unintentional Cervical Dural Tap Treated with Lumbar Blood Patch

Cervical radicular pain presents itself as pain radiating from the neck to the arm. If conservative treatment fails, a cervical epidural steroid injection can be considered. A rare but possible complication resulting from the interlaminar approach is unintentional cervical dural puncture that may result in post‐dural puncture headache (PDPH). Dural puncture from an interlaminar cervical epidural injection reportedly range from 0.25% to 2.65%. An epidural blood patch is a possible treatment option when conservative treatment fails. Relief could be secondary to ‘sealing’ of the dural tear from the clotted blood and reestablishment of physiological intracranial pressure. Another theory is an increase in the subarachnoid pressure from the injected blood. The increased pressure may restore normal intracranial pressure. We describe 2 cases of cervical PDPH treated with lumbar epidural blood patch. In 1 case, there was complete resolution of the symptoms and in the other case, there was great improvement of symptoms and a high thoracic blood patch was performed to resolve the remaining headache.     

A Novel Technique for Treatment of Intractable Spontaneous Intracranial Hypotension: Lumbar Dural Reduction Surgery

Background and Objective.— Spontaneous intracranial hypotension has become a well-described cause of headache particularly among young and middle-aged individuals. Treatment of the underlying spinal cerebrospinal fluid (CSF) leak is effective in relieving symptoms in the vast majority of patients but symptoms may become refractory. The author describes a novel surgical technique to treat intractable spontaneous intracranial hypotension.
Methods.— A lumbar laminectomy is performed, a strip of dura is resected, and the dural defect is closed. The resulting decrease in lumbar CSF volume is believed to increase intracranial CSF volume and pressure.
Results.— The technique was utilized in a patient who suffered with intractable positional headaches because of a spinal CSF leak for 6 years in spite of numerous surgical and nonsurgical therapies. Significant improvement of symptoms was sustained during a 1-year period of follow-up.
Conclusion.— Dural reduction surgery may be considered in carefully selected patients with intracranial hypotension.     

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 syndrome: report of twelve cases

PURPOSE: To investigate clinical, MRI, and radioisotope findings and therapeutic outcome of the syndrome of spontaneous intracranial hypotension (SIH). BACKGROUND: Spontaneous intracranial hypotension is characterized by orthostatic headache, low CSF pressure, and MRI findings of diffuse pachymeningeal gadolinium enhancement without previous history of head trauma or lumbar puncture. Spontaneous CSF leakage from a spinal dural tear has been suggested as the underlying pathogenic mechanism of SIH. Most patients recover without sequelae, but subdural collections have been described in a few. METHODS: Twelve consecutive patients (10 females, 2 males, mean age 39 years) with headache related to the syndrome of spontaneous intracranial hypotension were investigated. RESULTS: Eleven patients presented orthostatic headache, one patient had continuous nonpostural headache. Additional clinical symptoms included nausea, vomiting, tinnitus, diplopia, and back pain. All the patients had low CSF opening pressure, seven had increased CSF albumin, and four had pleocytosis. Brain MRI showed diffuse pachymeningeal gadolinium enhancement. Other features included subdural fluid collections (hematoma/hygroma) in four patients, downward displacement of the brain in four patients, and enlargement of the pituitary gland in one patient. Radioisotope cisternography results indicated, in two patients, a CSF leakage site in the cervico-thoracic region, and in one patient showed limited ascent of the tracer to the cerebral convexity and early appearance of radioisotope in the bladder. All the patients had complete resolution of headache with conservative treatment. CONCLUSIONS: Patients with SIH have distinct MRI and sometimes radioisotope cisternographic abnormalities and generally respond favorably to conservative management.     

Heavily T2-weighted MR myelography in patients with spontaneous intracranial hypotension: a case-control study

We performed whole-spine heavily T2-weighted magnetic resonance (MR) myelography using a single-shot fast spin-echo pulse sequence in 17 patients (8 M/9 F) with spontaneous intracranial hypotension (SIH) to detect abnormal cerebrospinal fluid (CSF) collections. In addition, a group of age- and sex-matched controls were recruited. Follow-up MR myelography was also done at 3 weeks. MR myelography showed three kinds of abnormal CSF collections in 15 patients with SIH (88%): epidural fluid collection (n = 15, 88%), C1-2 extraspinal collections (n = 6, 35%) and CSF collections along nerve roots in the lower cervical or upper thoracic spines (n = 6, 35%). One patient (6%) showed a meningeal diverticulum. In contrast, none of the controls showed these findings. Overall, MR myelography results helped in early diagnosis of SIH in four (24%) patients whose initial brain MRIs failed to show typical SIH findings. Follow-up MR myelography results were compatible with the clinical changes with kappa statistics of 0.52 and an agreement rate of 76%. Our study showed heavily T2-weighted MR myelography provided a rapid, non-invasive and high yield method to diagnose and follow-up patients with SIH. Whether the CSF collections along the nerve roots represent the ongoing leakage sites warrants further study.     

Intracranial hypotension following chiropractic spinal manipulation

We report a case of intracranial hypotension caused by chiropractic manipulation which, in contrast to previously reported cases, documents the location of the cerebrospinal fluid leak by radionuclide cisternography. Cervical spinal manipulation produced a remote lumbar dural tear in our patient. Spinal magnetic resonance imaging may falsely localize the dural tear in this condition. Although conservative management is often sufficient, precise localization is required for more invasive therapies.     

Intracranial hypotension syndrome following chiropractic manipulation of the cervical spine

Cervical spine manipulation has been associated with several disorders such as cervical arteries dissection, but rarely has a relationship with intracranial hypotension been reported. We describe a patient showing intracranial hypotension syndrome following chiropractic cervical spine treatment. Magnetic resonance showed the presence of dural leakage at cervical level, suggesting the pathogenesis of the syndrome. We state that cervical spine manipulation should be considered a treatment with risk of neurological complications, including the occurrence of intracranial hypotension.     

Cranial Magnetic Resonance Imaging in Spontaneous Intracranial Hypotension after Epidural Blood Patch

▪ Abstract:   Spontaneous intracranial hypotension (SIH) is a syndrome characterized by orthostatic headache, nausea, vomiting, photophobia, and diplopia. Subdural effusion, diffuse dural enhancement, dilatation of epidural veins, and increased height of hypophysis are cranial magnetic resonance (MR) imaging findings in SIH. Epidural blood patch is reportedly one of the effective treatment options. We present the follow-up MR imaging findings in a case of SIH after a successful epidural blood patch treatment. We propose that cranial MR imaging as an objective test to evaluate the success of epidural blood patch treatment. ▪     

Patulous Eustachian tube in spontaneous intracranial hypotension syndrome

A 48-year-old slender woman presented with a rare case of spontaneous intracranial hypotension syndrome manifesting as patulous Eustachian tube. The patient complained of sudden onset of ear fullness and nasal voice as well as typical orthostatic headache. Patulous Eustachian tube was identified by observation of synchronous movement of the tympanic membrane with respiration and swallowing. The diagnosis of spontaneous intracranial hypotension was confirmed by negative cerebrospinal fluid pressure, and typical magnetic resonance imaging and radioisotope cisternography findings. All symptoms completely resolved within a few days after epidural blood patch treatment. Changes in the venous blood distribution led by collapse of the dural sac of the cervical spine in the standing position presumably caused decreased size of the pterygoid venous plexus around the Eustachian tube.     

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 CSF leaks mimicking benign exertional headaches

Spontaneous CSF leaks are increasingly recognized, and a broader clinical and imaging spectrum of the disorder is emerging. The headaches of CSF leaks are typically orthostatic, but sometimes especially with chronicity the orthostatic features are blurred into lingering chronic daily headaches. Additional types of headache are also increasingly recognized. Two patients with spontaneous CSF leaks presented with intermittent transient severe headaches provoked by Valsalva-type manoeuvres. Orthostatic features were absent and the patients were asymptomatic if they avoided the provoking manoeuvres. One patient had been treated for 6 years for benign exertional headaches and had failed many medical treatments, including courses of indomethacin. He was found to have a leak from cribriform plate. The second patient had been symptomatic for several months, had diffuse pachymeningeal gadolinium enhancement on head magnetic resonance imaging, spinal meningeal diverticula, and CSF leak at the thoracic spine level. Headaches that mimic benign exertional headaches are yet another mode of the still broadening clinical presentation of spontaneous CSF leaks.