Posture-related headaches and pachymeningeal enhancement in CSF leaks from craniotomy site

Cerebrospinal fluid (CSF) leak is recognized to cause headaches that typically but not invariably have orthostatic features (present in upright posture, relieved by recumbency). Head magnetic resonance imaging (MRI) typically shows diffuse pachymeningeal enhancement. A 24-year-old woman, after resection of a right temporoparietal glioma, developed CSF leak from the craniotomy site, resulting in subgaleal fluid collection and associated with diffuse pachymeningeal gadolinium enhancement as well as posture-related headaches. The headaches, however, were present in a recumbent position and relieved after several minutes of being in an upright position. It is postulated that CSF leak took place when the patient was recumbent and ceased when she was upright, a position in which there is decrease in intracranial pressure. After cessation of the leak, along with disappearance of subgaleal collection of CSF, both the headaches and the pachymeningeal enhancement resolved.     

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.     

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.     

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 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.     

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.     

Trendelenburg position: a tool to screen for the presence of a low CSF pressure syndrome in daily headache patients

Objective.— To test the hypothesis that the Trendelenburg position is an accurate screening investigation for the presence of a low cerebrospinal fluid (CSF) pressure syndrome in patients with daily headache. Background.— The Trendelenburg position causes a rapid increase in intracranial CSF pressure. In a patient with a known CSF leak who overtime had less improvement in the supine position, being placed in Trendelenburg rapidly alleviated her daily headache. This suggested that the Trendelenburg position might be a good screening tool for low CSF pressure syndromes. Methods.— Case reports. All patients were placed in the Trendelenburg position (10°‐20° head‐down tilt) for 5 minutes. A patient was considered to have a positive Trendelenburg test if they experienced complete pain freedom or substantial improvement in baseline head pain in the Trendelenburg position. Results.— Case patients are presented for 3 clinical scenarios: Scenario 1: Daily headache with or without a positional component with a positive response to the Trendelenburg position and subsequent evidence of an underlying low CSF pressure syndrome. Scenario 2: Daily headache with a strong positional component but no improvement in the Trendelenburg position and a negative evaluation for a low CSF pressure syndrome. Scenario 3: Trendelenburg position proves the existence of a post‐lumbar puncture headache in patients with near‐daily headaches. Conclusion.— The Trendelenburg position appears potentially useful as a clinical tool to screen for the presence of a low CSF pressure syndrome in patients with daily headache.     

Complete External Ophthalmoplegia in a Case of Pseudotumor Cerebri

SYNOPSIS
A 28 year old woman was admitted to the hospital with a 4 month history of headaches and blurred vision and a 2 week progressive loss of visual acuity. On examination she was found to have complete external ophthalmoplegia, florid papilledema and an otherwise normal neurologic examination. Cerebrospinal fluid pressure and protein supported the diagnosis of pseudotumor cerebri. A normal cerebral angiogram and "slit-like" ventricles on brain computed tomography supported the diagnosis. She was treated with cortico-steroids, and acetazolamide and underwent optic nerve fenestration bilaterally. This resulted in complete resolution of the ophthalmoplegia and papilledema.     

Reversible segmental cerebral vasoconstriction (Call-Fleming syndrome): are calcium channel inhibitors a potential treatment option?

The Call-Fleming syndrome is characterized by sudden onset of thunderclap-like headache and focal neurological deficits. The pathophysiological correlate is a reversible segmental cerebral vasoconstriction frequently associated with focal cerebral ischaemia. The syndrome has been described in a variety of clinical conditions, and recently an association between the syndrome and exposure to vasoactive drugs was observed. Effective treatment options are not known. A 63-year-old female developed sudden 'worst ever' headache. Initial neurological examination, laboratory blood tests, CSF examination and brain magnetic resonance imaging (MRI) were normal. Previous medical history was unremarkable and she did not take vasoactive drugs. Eleven days after the onset of headache she developed visual field impairment and a right-sided hemiparesis. Brain MRI revealed bilateral posterior and left parietal ischaemic strokes. Cerebral catheter angiography showed segmental arterial vasoconstriction. A vasodilative therapy with calcium channel inhibitors was started and serial transcranial Doppler ultrasonography demonstrated resolution of cerebral arterial vasoconstriction. The present case illustrates that calcium channel inhibitors may be an effective therapy for segmental cerebral arterial vasoconstriction. However, more clinical data are needed to prove this observation.     

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 cerebrospinal fluid leaks: from intracranial hypotension to cerebrospinal fluid hypovolemia--evolution of a concept

Intracranial hypotension causes orthostatic headaches and diffuse pachymeningeal gadolinium enhancement on magnetic resonance imaging with or without subdural fluid collections or imaging evidence of descent of the brain. A review of the literature and my experience and investigations in the past decade reveal a broadening clinical and imaging spectrum of the syndrome. Besides the classic clinical-imaging syndrome of orthostatic headaches, diffuse pachymeningeal gadolinium enhancement, and low cerebrospinal fluid (CSF) pressure, several modes of presentation are recognized, including (1) the typical clinical-imaging syndrome with CSF pressures consistently within normal limits, (2) absence of diffuse pachymeningeal gadolinium enhancement with presence of low CSF pressures and typical clinical manifestations, and (3) absence of headaches despite low CSF pressures and presence of diffuse pachymeningeal gadolinium enhancement. Furthermore, in some patients with headaches, the orthostatic headaches may evolve into lingering chronic daily headaches, although they may be more prominent in upright positions. What determines the various clinical and imaging features of this syndrome seems to be the loss of CSF volume as the independent variable, while other manifestations, including clinical features, CSF pressures, and imaging abnormalities, are variables dependent on the CSF volume. The term CSF hypovolemia is proposed for this syndrome because the term intracranial hypotension no longer seems adequate to embrace all the variations that have emerged.     

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.     

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.     

Assessment of CSF flow dynamics using PC-MRI in spontaneous intracranial hypotension

(Headache 2012;52:808‐819) Aim.— 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. Materials and Methods.— 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. Results.— 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. Conclusions.— 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.     

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.     

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.     

Management of an intracranial hypotension patient with diplopia as the primary symptom: A case report

BACKGROUNDIntracranial hypotension (IH) is a disorder involving cerebrospinal fluid (CSF) hypovolemia due to spontaneous or traumatic spinal CSF leakage and is easily being misdiagnosed or missed, especially in these patients without the prototypical manifestation of an orthostatic headache. At present, the management of IH with both cranial nerve VI palsy and bilateral subdural hematomas (SDHs) is still unclear.CASE SUMMARYA 67-year-old male Chinese patient complained of diplopia on the left side for one and a half mo. Computed tomography revealed bilateral SDHs and a midline shift. However, neurotrophic drugs were not effective, and 3 d after admission, he developed a non-orthostatic headache and neck stiffness. Enhanced magnetic resonance imaging revealed dural enhancement as an additional feature, and IH was suspected. Magnetic resonance myelography was then adopted and showed CSF leakage at multiple sites in the spine, confirming the diagnosis of having IH. The patient fully recovered following multiple targeted epidural blood patch (EBP) procedures.CONCLUSIONIH is a rare disease, and to the best of our knowledge, IH with diplopia as its initial and primary symptom has never been reported. In this study, we also elucidated that it could be safe and effective to treat IH patients with associated cranial nerve VI palsy and bilateral SDHs using repeated EBP therapy.     

Cerebral Vasomotor Changes in the Transient Syndrome of Headache With Neurologic Deficits and CSF Lymphocytosis (HaNDL)

We report two patients with the recently described transient syndrome of headache with neurologic deficits and CSF lymphocytosis (HaNDL). Transcranial Doppler sonography performed during and after attacks of HaNDL showed asymmetrical decreases or increases in blood flow velocity of the middle cerebral artery, accompanied by increases or decreases in pulsatility suggesting fluctuations of arteriolar tone. The findings demonstrate focal vasomotor disturbances that link the transient headaches and deficits of HaNDL with attacks of migraine.     

Cisternographic pattern of spontaneous liquoral hypotension

We describe five cases of headache due to spontaneous liquoral hypotension, the syndrome comprising sudden, intense and oppressive orthostatic holocranial headache. The headache improved in the recumbent position and was accompanied by nausea and sometimes vomiting. There was no history of lumbar puncture or previous trauma. CSF tension was low or negative. The CSF showed a raised protein content and increased red and white cell counts. CT scan was normal or showed a slit ventricular system. Improvement was complete three to eight weeks from onset. The treatment consisted of bed rest and oral and parenteral fluid replacement. An isotope cisternography carried out in all patients while the headache was present showed a cisternographic pattern characterized by a combination of premature elimination and failure to detect the isotope at the cerebral convexity. Scan images did not show CSF leakage at any site. This stereotyped reaction pattern suggests that CSF hyperabsorption is the most likely pathophysiological mechanism of this entity.     

Spontaneous intracranial hypotension: case report

Spontaneous intracranial hypotension is a well defined entity, however it is characterised by a relevant clinical heterogeneity. The main feature is orthostatic headache, often accompanied by other symptoms. The diagnosis is easily made in the presence of orthostatic headache, excluding an obvious aetiology of low cerebrospinal fluid (CSF) pressure, and often there is no evidence of direct or indirect CSF leak.