Infratentorial Superficial Siderosis and Spontaneous Intracranial Hypotension.

OBJECTIVE: Spontaneous spinal cerebrospinal fluid (CSF) leaks cause intracranial hypotension (SIH) and also may cause infratentorial superficial siderosis (iSS) but the rate of development among different CSF leak types and outcome of treatment are not known. We determined the time interval from SIH onset to iSS and the outcome of treatment. METHODS: A total of 1,589 patients with SIH underwent neuroimaging and iSS was detected in 57 (23 men and 34 women, mean age = 41.3 years [3.6%]). We examined the type of underlying CSF leak by various imaging modalities. Percutaneous and surgical procedures were used to treat the CSF leaks. RESULTS: The iSS was detected in 46 (10.3%) of 447 patients with ventral CSF leaks, in 2 (3.9%) of 51 patients with dural ectasia, in 5 (2.6%) of 194 patients with CSF-venous fistulas, in 4 (0.9%) of 457 patients with simple meningeal diverticula, and in none of the 101 patients with lateral CSF leaks or the 339 patients with leaks of indeterminate origin (p < 0.001). The estimated median latency period from SIH onset to iSS was 126 months. Ventral CSF leaks could not be eliminated with percutaneous procedures in any patient and surgical repair was associated with low risk (<5%) and resulted in resolution of the CSF leak in all patients in whom the exact site of the CSF leak could be determined. Other types of CSF leak were treated with percutaneous or surgical procedures. INTERPRETATION: The iSS can develop in most types of spinal CSF leak, including CSF-venous fistulas, but mainly in chronic ventral CSF leaks, which require surgical repair. ANN NEUROL 2023;93:64-75.

Progressive superficial siderosis despite complete remission of intracranial hypotension symptoms following epidural patching: Case report.

Spontaneous intracranial hypotension (SIH) commonly results from ventral spinal cerebrospinal fluid (CSF) leaks and epidural patches are advocated as first-line treatment. Complications such as superficial siderosis can arise but have previously been reported only in the context of long-term persistent, ongoing, CSF leak and SIH. We report a case of a patient with SIH from a ventral spinal CSF leak that was treated with epidural patching and experienced complete resolution of SIH. Four years later SIH symptoms recurred, and brain magnetic resonance imaging unexpectedly showed the interval accumulation of hemosiderin pigmentation on the cerebellum and brainstem during the period when the patient was without symptoms of SIH. This case uniquely demonstrates the progression of superficial siderosis despite the apparent resolution of SIH. Our findings suggest two divergent pathophysiological outcomes from spinal ventral dural tear: (1) CSF loss causing SIH; and (2) persistent low-level bleeding arising from the spinal dural tear leading to superficial siderosis. These divergent pathophysiologies had a discordant response to epidural patching. Epidural patching successfully treated the SIH but did not prevent the progression of superficial siderosis, indicating that some patients may require more than epidural patching despite symptom resolution. This case highlights the need for post-treatment monitoring protocols in patients with ventral spinal CSF leaks and SIH and raises important questions about the adequacy of epidural patching in certain SIH cases arising from ventral spinal CSF leak.

Pediatric post-dural puncture headache and paraplegia.

A cerebrospinal fluid (CSF) leak developed in a 14-year-old girl and a 12-year-old boy following a diagnostic lumbar puncture. Two days and sixteen years later, respectively, paraplegia developed due to a functional disorder. Imaging revealed an extensive extradural CSF collection in both patients and digital subtraction myelography was required to pinpoint the exact site of a ventral dural puncture hole where the lumbar spinal needle had gone "through and through" the dural sac. The CSF leak was complicated by cortical vein thrombosis in one patient. Both patients underwent uneventful surgical repair of the ventral dural puncture hole with prompt resolution of the paraplegia. Iatrogenic ventral CSF leaks may become exceptionally long standing and may be complicated by paraplegia on a functional basis both in the acute and chronic phases.

Management of Tarlov cysts: an uncommon but potentially serious spinal column disease-review of the literature and experience with over 1000 referrals.

Tarlov cysts were thought to be anatomic variants of uncertain etiology and clinical significance when initially described over 80 years ago. They are often detected in routine lumbosacral imaging and generally not reported in a differential diagnosis. There is increasing evidence that at least some Tarlov cysts are symptomatic and can have a significant adverse impact on patients' health and well-being. Women are disproportionately affected with this condition, often presenting with long-standing pain and neurological dysfunctions. Significant gender bias has been a concern in the management of these patients. Unfortunately, there is no consensus on patient selection or management approaches for symptomatic Tarlov cysts. This review article updates information on the prevalence, diagnosis, clinical significance, and treatments of these cysts. Based on these findings and experience with over 1000 patient referrals, a treatment decision algorithm for symptomatic Tarlov cysts was constructed to provide guidance for appropriate management of patients with these complex cysts.

Multidisciplinary consensus guideline for the diagnosis and management of spontaneous intracranial hypotension.

BACKGROUND: We aimed to create a multidisciplinary consensus clinical guideline for best practice in the diagnosis, investigation and management of spontaneous intracranial hypotension (SIH) due to cerebrospinal fluid leak based on current evidence and consensus from a multidisciplinary specialist interest group (SIG). METHODS: A 29-member SIG was established, with members from neurology, neuroradiology, anaesthetics, neurosurgery and patient representatives. The scope and purpose of the guideline were agreed by the SIG by consensus. The SIG then developed guideline statements for a series of question topics using a modified Delphi process. This process was supported by a systematic literature review, surveys of patients and healthcare professionals and review by several international experts on SIH. RESULTS: SIH and its differential diagnoses should be considered in any patient presenting with orthostatic headache. First-line imaging should be MRI of the brain with contrast and the whole spine. First-line treatment is non-targeted epidural blood patch (EBP), which should be performed as early as possible. We provide criteria for performing myelography depending on the spine MRI result and response to EBP, and we outline principles of treatments. Recommendations for conservative management, symptomatic treatment of headache and management of complications of SIH are also provided. CONCLUSIONS: This multidisciplinary consensus clinical guideline has the potential to increase awareness of SIH among healthcare professionals, produce greater consistency in care, improve diagnostic accuracy, promote effective investigations and treatments and reduce disability attributable to SIH.

Incidence of spontaneous intracranial hypotension in a community: Beverly Hills, California, 2006-2020.

BACKGROUND: Spontaneous intracranial hypotension is diagnosed with an increasing frequency, but epidemiologic data are scarce. The aim of this study was to determine the incidence rate of spontaneous intracranial hypotension in a defined population. METHODS: Using a prospectively maintained registry, all patients with spontaneous intracranial hypotension residing in Beverly Hills, California, evaluated at our Medical Center between 2006 and 2020 were identified in this population-based incidence study. Our Medical Center is a quaternary referral center for spontaneous intracranial hypotension and is located within 1.5 miles from downtown Beverly Hills. RESULTS: A total of 19 patients with spontaneous intracranial hypotension were identified. There were 12 women and seven men with a mean age of 54.5 years (range, 28 to 88 years). The average annual incidence rate for all ages was 3.7 per 100,000 population (95% confidence interval [CI]: 2.0 to 5.3), 4.3 per 100,000 for women (95% CI, 1.9 to 6.7) and 2.9 per 100,000 population for men (95% CI, 0.8 to 5.1). CONCLUSION: This study, for the first time, provides incidence rates for spontaneous intracranial hypotension in a defined population.

Spontaneous spinal cerebrospinal fluid-venous fistulas in patients with orthostatic headaches and normal conventional brain and spine imaging.

OBJECTIVE: To determine the occurrence of cerebrospinal fluid (CSF)-venous fistulas, a type of spinal CSF leak that cannot be detected with routine computerized tomography myelography, among patients with orthostatic headaches but normal brain and spine magnetic resonance imaging. BACKGROUND: Spontaneous spinal CSF leaks cause orthostatic headaches but their detection may require sophisticated spinal imaging techniques. METHODS: A prospective cohort study of patients with orthostatic headaches and normal brain and conventional spine imaging who underwent digital subtraction myelography (DSM) to look for CSF-venous fistulas, between May 2018 and May 2020, at a quaternary referral center for spontaneous intracranial hypotension. RESULTS: The mean age of the 60 consecutive patients (46 women and 14 men) was 46 years (range, 13-83 years), who had been suffering from orthostatic headaches between 1 and 180 months (mean, 43 months). DSM demonstrated a spinal CSF-venous fistula in 6 (10.0%; 95% confidence interval [CI]: 3.8-20.5%) of the 60 patients. The mean age of these five women and one man was 50 years (range, 41-59 years). Spinal CSF-venous fistulas were identified in 6 (19.4%; 95% CI: 7.5-37.5%) of 31 patients with spinal meningeal diverticula but in none (0%; 95% CI: 0-11.9%) of the 29 patients without spinal meningeal diverticula (p = 0.024). All CSF-venous fistulas were located in the thoracic spine. All patients underwent uneventful surgical ligation of the fistula. Complete and sustained resolution of symptoms was obtained in five patients, while in one patient, partial recurrence of symptoms was noted 3 months postoperatively. CONCLUSION: Concerns about a spinal CSF leak should not be dismissed in patients suffering from orthostatic headaches when conventional imaging turns out to be normal, even though the yield of identifying a CSF-venous fistula is low.

Diffuse non-aneurysmal SAH in spontaneous intracranial hypotension: Sequela of ventral CSF leak?

BACKGROUND: Spontaneous intracranial hypotension due to a spinal cerebrospinal fluid (CSF) leak has become a well-recognized cause of headaches. Recently, various unusual neurological syndromes have been described in such patients with chronic ventral CSF leaks, including superficial siderosis and an amyotrophic lateral sclerosis-like syndrome. The authors now report two patients with spontaneous intracranial hypotension due to a chronic ventral CSF leak who suffered a diffuse non-aneurysmal subarachnoid hemorrhage (SAH). DESCRIPTION OF CASES: A 62-year-old woman underwent uneventful microsurgical repair of a ventral thoracic CSF leak that had been present for 13 years. Seventeen months after surgery, she was found unresponsive and CT showed a diffuse intracranial SAH. Cerebral angiography and spine and brain MRI did not reveal a source of the SAH. A 73-year-old woman was found unresponsive and CT showed a diffuse intracranial SAH. Cerebral angiography and brain MRI did not reveal a source of the SAH, although superficial siderosis was detected. Spine MRI showed a ventral thoracic CSF leak that by history had been present for 41 years. She underwent uneventful microsurgical repair of the CSF leak. DISCUSSION: The authors suggest that patients with a ventral spinal CSF leak of long duration may be at risk of diffuse non-aneurysmal SAH.

Vanishing calcification associated with a spontaneous ventral spinal cerebrospinal fluid leak.

BACKGROUND: Some patients with spontaneous intracranial hypotension have a ventral spinal cerebrospinal fluid (CSF) leak and these CSF leaks may be associated with calcified disk herniations. Identifying these calcifications is helpful in directing treatment. We report here the unusual case of a patient with a ventral CSF leak in whom the associated calcification absorbed over a five-month period. CASE REPORT: A 42-year-old woman developed orthostatic headaches and bilateral abducens nerve palsies. Magnetic resonance imaging of her brain showed typical findings of spontaneous intracranial hypotension. Magnetic resonance imaging of her spine showed an extensive cervicothoracic CSF leak. Computed tomographic myelography showed calcification at the Th1-2 disk space. Three epidural blood patches were performed, but her symptoms persisted. Digital subtraction myelography performed five months later showed an upper thoracic ventral CSF, but the calcification was no longer present. A dural tear, found at surgery at the Th1-2 level, was repaired and the patient made an uneventful recovery. DISCUSSION: The resorption of calcifications at the level of a ventral spinal CSF leak could explain the absence of any calcifications in at least some patients with such leaks and demonstrates the usefulness of reviewing previous imaging in patients with ventral CSF leaks if the exact site of the leak remains unknown.

Dural Reduction Surgery: A Treatment Option for Frontotemporal Brain Sagging Syndrome.

Frontotemporal brain sagging syndrome is a dementia associated with hypersomnolence, personality changes, and features of intracranial hypotension on magnetic resonance imaging. The literature is sparse with respect to treatment options; many patients simply worsen. We present a case in which this syndrome responded to lumbar dural reduction surgery. Postoperative magnetic resonance imaging indicated normalization of brain sagging and lumbar intrathecal pressure. Although no evidence of cerebrospinal leak was found, extremely thin dura was noted intraoperatively, suggesting that a thin and incompetent dura could result in this low-pressure syndrome. Clinicians who encounter this syndrome should consider dural reduction surgery as a treatment strategy.

Cervical arterial dissections and association with cervical manipulative therapy: a statement for healthcare professionals from the american heart association/american stroke association.

PURPOSE: Cervical artery dissections (CDs) are among the most common causes of stroke in young and middle-aged adults. The aim of this scientific statement is to review the current state of evidence on the diagnosis and management of CDs and their statistical association with cervical manipulative therapy (CMT). In some forms of CMT, a high or low amplitude thrust is applied to the cervical spine by a healthcare professional. METHODS: Members of the writing group were appointed by the American Heart Association Stroke Council's Scientific Statements Oversight Committee and the American Heart Association's Manuscript Oversight Committee. Members were assigned topics relevant to their areas of expertise and reviewed appropriate literature, references to published clinical and epidemiology studies, morbidity and mortality reports, clinical and public health guidelines, authoritative statements, personal files, and expert opinion to summarize existing evidence and to indicate gaps in current knowledge. RESULTS: Patients with CD may present with unilateral headaches, posterior cervical pain, or cerebral or retinal ischemia (transient ischemic or strokes) attributable mainly to artery-artery embolism, CD cranial nerve palsies, oculosympathetic palsy, or pulsatile tinnitus. Diagnosis of CD depends on a thorough history, physical examination, and targeted ancillary investigations. Although the role of trivial trauma is debatable, mechanical forces can lead to intimal injuries of the vertebral arteries and internal carotid arteries and result in CD. Disability levels vary among CD patients with many having good outcomes, but serious neurological sequelae can occur. No evidence-based guidelines are currently available to endorse best management strategies for CDs. Antiplatelet and anticoagulant treatments are both used for prevention of local thrombus and secondary embolism. Case-control and other articles have suggested an epidemiologic association between CD, particularly vertebral artery dissection, and CMT. It is unclear whether this is due to lack of recognition of preexisting CD in these patients or due to trauma caused by CMT. Ultrasonography, computed tomographic angiography, and magnetic resonance imaging with magnetic resonance angiography are useful in the diagnosis of CD. Follow-up neuroimaging is preferentially done with noninvasive modalities, but we suggest that no single test should be seen as the gold standard. CONCLUSIONS: CD is an important cause of ischemic stroke in young and middle-aged patients. CD is most prevalent in the upper cervical spine and can involve the internal carotid artery or vertebral artery. Although current biomechanical evidence is insufficient to establish the claim that CMT causes CD, clinical reports suggest that mechanical forces play a role in a considerable number of CDs and most population controlled studies have found an association between CMT and VAD stroke in young patients. Although the incidence of CMT-associated CD in patients who have previously received CMT is not well established, and probably low, practitioners should strongly consider the possibility of CD as a presenting symptom, and patients should be informed of the statistical association between CD and CMT prior to undergoing manipulation of the cervical spine.

High-pressure headaches, low-pressure syndromes, and CSF leaks: diagnosis and management.

BACKGROUND: Headache resulting from idiopathic intracranial hypertension (IIH) in a population of moderately to obese women of childbearing age. The causes overall remain unclear. With this review, we provide an overview of clinical treatment and management strategies. RESULTS: IIH management is dependent on the signs and symptoms presented. Symptomatic treatment should attempt to lower intracranial pressure, reduce pain, and protect the optic nerves. Consideration for lumbar puncture and draining fluid as an option for reducing pressure may be helpful; however, repeated treatment is not usually favored by patients. Traditional prophylactic medications used in migraine may help reduce the primary headache often induced by raised intracranial pressure. We suggested surgical intervention for patients experiencing visual loss or impending visual loss and not responding to medication therapy. CONCLUSION: In this review, we discuss headache associated with IIH and spontaneous intracranial hypotension. Much needs to be learned about treatment options for patients with cerebrospinal fluid leaks including methods to strengthen the dura.

Headache secondary to intracranial hypotension.

Intracranial hypotension is known to occur as a result of spinal cerebrospinal fluid (CSF) leaking, which may be iatrogenic, traumatic, or spontaneous. Headache is usually, but not always, orthostatic. Spontaneous cases are recognized more readily than in previous decades as a result of a greater awareness of clinical presentations and typical cranial magnetic resonance imaging findings. An underlying disorder of connective tissue that predisposes to weakness of the dura is implicated in spontaneous spinal CSF leaks. CT, MR, and digital subtraction myelography are the imaging modalities of choice to identify spinal CSF leakage. Spinal imaging protocols continue to evolve with improved diagnostic sensitivity. Epidural blood patching is the most common initial intervention for those seeking medical attention, and may be repeated several times. Surgery is reserved for cases that fail to respond or relapse after simpler measures. While the prognosis is generally good with intervention, serious complications do occur. More research is needed to better understand the genetics and pathophysiology of dural weakness as well as physiologic compensatory mechanisms, to continue to refine imaging modalities and treatment approaches, and to evaluate short- and long-term clinical outcomes.

Compulsive Repetitive Flexion With Breath-Holding in Sagging Brain Syndrome.

Background and Objective: Spontaneous intracranial hypotension (SIH) from CSF leak commonly produces headache. It also may produce sagging brain syndrome (SBS), often with neurocognitive symptoms indistinguishable from behavioral-variant frontotemporal dementia (bvFTD). The authors describe a new clinical sign that appears to be pathognomonic of SBS. Methods: We reviewed medical records and brain imaging in patients seen at our 2 centers who presented with SIH, SBS, and bvFTD symptoms. Results: There were 51 patients (12 women, 39 men) with mean age 55.5 years (range, 26-70 years). MRI showed severe brain sagging in all. Thirteen patients displayed repetitive flexion with breath-holding at the time of clinical presentation. Five patients had repetitive flexion with breath-holding, which resolved before presenting for evaluation. Thus, 35.3% (18) of 51 patients with SBS displayed seemingly compulsive repetitive flexion with breath-holding. Discussion: Compulsive repetitive flexion with breath-holding appears to be pathognomonic of SBS, deserving the acronym CoRFBiS (compulsive repetitive flexion with breath-holding in SBS). CoRFBiS should alert the clinician to SBS with SIH as the proximate cause of the clinical constellation, rather than bvFTD.

Spontaneous absorption of osteophytic calcification associated with chronic dural tear and ventral spinal CSF leak.

Spontaneous intracranial hypotension (SIH) is associated with cerebrospinal fluid (CSF) hypovolemia, often from a traumatic dural tear from a calcified spinal osteophyte. Visualizing osteophytes on CT imaging can guide decision making on candidate leak sites. We report the atypical case of a 41-year-old woman whose ventral CSF leak was associated with an osteophyte that resorbed over an 18-month period. Full workup and treatment were delayed due to unexpected pregnancy and completion of gestational cycle with delivery of a healthy term infant. The patient initially presented with persistent orthostatic headaches with nausea and blurred vision. Initial MRI suggested brain sagging among other findings consistent with SIH. CT myelogram showed an extensive thoracic CSF leak with a prominent ventral T11-T12 osteophyte and multiple small disc herniations. The patient did not respond to epidural blood patches and deferred additional imaging due to her pregnancy. CT myelography performed 5 months post-partum showed an absence of the osteophyte; a follow-up digital subtraction myelogram performed 10 months post-partum showed evidence of source leak at T11-T12 level. T11-T12 laminectomy visualized and repaired a 5 mm ventral dural defect with symptom resolution. This report highlights the potential for a resorbed osteophyte to be the causative agent for long-standing dural tears that do not show visible calcifications on myelography.