Figure 2 - uploaded by Anne Ducros
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Photographs of the ocular fundus showing bilateral papilloedema The right eye is shown on the left and the left eye is on the right. Photograph taken at an emergency department with a non-mydriatic digital fundus camera. The patient presented with new-onset headaches. Bilateral severe papilloedema is present, consistent with intracranial hypertension. The optic nerves are elevated and the margins are blurry. The veins are dilated and tortuous.
Source publication
New onset of sudden or progressive headache can have various causes, including disorders of intracranial pressure (ICP). Headache is the most common-and often the presenting-symptom of both intracranial hypertension and intracranial hypotension syndromes, which can be symptomatic or idiopathic. Despite the widespread availability of diagnostic test...
Citations
... Conservative therapy includes bed rest to relieve the orthostatic headache and create conditions for spontaneous closure of the CSF leak, as well as vigorous hydration [56]. The efficacy of corticosteroids is uncertain and carries the risk of side effects [5,6]. ...
Spontaneous intracranial hypotension (SIH) is an important cause of daily headaches that occur in young and middle-aged, active persons and is often misdiagnosed, leading to prolonged inactivity and rather high healthcare expenditures. Its diagnosis requires a high degree of clinical suspicion and careful interpretation of imaging studies. We present a case of SIH, which was successfully treated but which posed serious diagnostic challenges, ranging from cerebro-vascular disease and meningitis to granulomatous diseases, and for whom every therapeutic attempt just worsened the patient’s condition until we finally reached the correct diagnosis. To raise awareness of this condition, we also present an updated overview of the clinical picture, evaluation, and treatment options for SIH.
... [5] is downstream occlusion impairs venous outflow, thus disrupting CSF absorption. [3] is can lead to an abnormally increased ICP, shown to be associated with nontraumatic spontaneous CSF rhinorrhea. [4] Surgical endoscopic endonasal repair is the preferred treatment method with good outcomes for most causes of CSF rhinorrhea. ...
Background
Antiphospholipid syndrome is a complex autoimmune condition associated with the formation of recurrent thrombosis in any vascular bed throughout the body. Jugular vein thrombosis is very rare with only a 0.9% occurrence and is not typically associated with cerebrospinal rhinorrhea as a result of raised intracranial pressure.
Case Description
A 54-year-old patient presented with a 9-month history of cerebrospinal fluid (CSF) rhinorrhea and headache on a background of antiphospholipid syndrome. Investigations showed a superior vena cava (SVC) and right internal jugular vein (IJV) obstruction with moderately elevated intracranial venous pressures. Her magnetic resonance imaging (MRI) brain was consistent with a CSF leak. The patient underwent successful endovascular stenting of her obstructed SVC and right IJV followed by surgical repair of a herniating meningocele in the posterior left ethmoid air cells.
Conclusion
CSF rhinorrhea is uncommon and never previously reported associated with SVC thrombosis induced by antiphospholipid syndrome. A combination of endovascular techniques and surgical repair is recommended for this challenging presentation.
... HIT-6 scores at baseline were significantly higher in patients with shorter symptom duration, representing a more severe impact of headaches. This observation aligns with the commonly reported phenomenon where orthostatic headaches, characteristic at the onset of the disease, may become less typical and recede into the background during a prolonged course [1,20,[32][33][34]. The subgroup with shorter symptom duration demonstrated a higher improvement in HIT-6 scores of 22.5 points 6 months after surgery (baseline 68.5 to 46 at 6 months). ...
Objective
Microsurgical sealing of spinal cerebrospinal fluid (CSF) leaks is a viable treatment option in spontaneous intracranial hypotension (SIH). Several factors may influence the outcome, with symptom duration probably the most modifiable variable.
Methods
Patients with closure of spinal CSF leaks between September 2020 and March 2023 and a follow-up period of 6 months were included in this retrospective single-center study. Pre- and postoperative scores for impact of headaches (Headache Impact Test, HIT-6) and quality of life (QoL, EQ-5D-5L) were systematically collected. Multiple regression modelling and subgroup analyses for different symptom durations and comorbidities were performed for these outcomes.
Results
One hundred patients (61% female, median age 43.5 years) were included. Six months postoperatively, there was significant improvement in headache impact (HIT-6: 66 (IQR 62–69) to 52 (IQR 40–61, p < 0.001) and QoL (EQ-5D-5L VAS: 40 (IQR 30–60) to 79 (IQR 60–90); EQ-5D-5L Index: 0.67 (IQR 0.35–0.8) to 0.91 (IQR 0.8–0.94, p < 0.001, respectively). Subgroup analysis for a symptom duration above (74%) and below 90 days (26%) and comorbidity, as well as multiple regression analysis, revealed a trend in favor of early treatment and lower comorbidity. However, even after a prolonged symptom duration, improvements were significant.
Conclusion
As patients with shorter symptom duration show a trend for a better outcome, our results promote a timely diagnosis and treatment in SIH patients. However, a significant postoperative improvement can still be expected even after a prolonged symptom duration.
... Monro-Kellie's theory states that since the cranial vault is a rigid compartment, the volume within it remains constant, it contains different components: arterial and venous blood volume, encephalic mass, and CSF; when there is an alteration in the volume of one of these, it will cause a compensatory modification in another of the components [10]. Upon occurrence of a CSFL, a reduction in the intracranial pressure is produced, leading to a venous dilation as a compensatory mechanism [11]. ...
... Upon occurrence of a CSFL, a reduction in the intracranial pressure is produced, leading to a venous dilation as a compensatory mechanism [11]. These changes in intracranial pressure also result in a modification of the cerebral blood flow, subarachnoid space collapse, and epidural space dilation, causing headaches due to meningeal and vascular traction [10]. IHS has been linked to other causes such as spinal arachnoiditis, which is incurable and has a poor prognosis [12,13]. ...
... She remained asymptomatic at 2 weeks and one year after applying the EBP. than 6 cmH2O, with or without evidence of CSF leak in imaging studies; other etiology should be excluded [10] The imaging study of choice is Computed Tomography Myelography (CTM) or intrathecal gadolineum-contrasted magnetic resonance myelography, with high sensitivity for locatization of the leak site in up to 95.8% of cases, especially when slow leaks are suspected [14]. It is also very useful for the detection of meningeal diverticula and dural ectasia in patients without an identified leak site [1]. ...
Intracranial hypotension syndrome may occur secondary to Cerebrospinal Fluid Leak (CSFL). Its etiology can be iatrogenic, traumatic or spontaneous. The most common cause is related to incidental or intentional punctures during anesthetic approaches. It is a rare complication during lumbar spine surgery. The clinical manifestations of this syndrome are characterized by orthostatic headache as the most frequent symptom, accompanied by a wide variety of manifestations, which frequently result in a disability for daily life activities and a delayed recovery. Diagnosis is made through a detailed medical history, the presence of symptoms and signs, and additional imaging studies. Initial treatment is conservative; however, using a hematic patch is recommended for a significant number of patients as the next step, reserving surgical treatment for cases that are not resolved with these measures. This paper presents the clinical case of a 56-year-old female patient diagnosed with CSF fistula following a surgical procedure, which is treated by applying a blood patch with complete remission of symptoms; similarly, a therapeutic algorithm is proposed for the diagnosis of CSFL.
... Spine MRI has become the preferred imaging modality for identifying signs of CSF leak and should be the initial choice whenever possible [70]. A fat-saturated T2-weighted sequence can visualize CSF accumulation in the epidural space [71,72]. ...
Purpose of Review
Brain sagging dementia (BSD) is a rare but devastating form of early-onset dementia characterized by intracranial hypotension and behavioral changes resembling behavioral variant frontotemporal dementia. This review aims to provide a comprehensive overview of BSD, highlighting its pathomechanism, diagnostic tools, and available treatment options.
Recent Findings
BSD exhibits a complex clinical manifestation with insidious onset and gradual progression of behavioral disinhibition, apathy, inertia, and speech alterations. Additionally, patients may exhibit brainstem and cerebellar signs such as hypersomnolence and gait disturbance. Although headaches are common, they may not always demonstrate typical orthostatic features. Recent radiological advances have improved the detection of CSF leaks, enabling targeted treatment and favorable outcomes.
Summary
Understanding the pathomechanism and available diagnostic tools for BSD is crucial for a systematic approach to timely diagnosis and treatment of this reversible form of early-onset dementia, as patients often endure a complex and lengthy clinical course.
... According to previous literature, CSF fistulas at the skull base are not typically associated with the development of intracranial hypotension, and in the case of SIH, it is suggested to look for CSF leakage at the spinal level (Schievink et al., 2012;Rgen Beck et al., 2018;Ducros and Biousse, 2015). Rhinoliquorrhoea is often intermittent, positional, of low volume, secondary to low subarachnoid cistern pressure, and may not be expected to result in a CSF leak sufficient to cause SIH. ...
Background
Meningoencephalocele is defined as an abnormal sac of brain tissue and meninges extending beyond natural skull margins, often leading to cerebrospinal fluid (CSF) leakage. When this condition arises in the spheno-ethmoidal region, the diagnosis becomes more challenging as it can be mistaken for other nasal pathologies, such as mucocele.
Research question
We show in this case report a non-congenital sphenoethmoidal meningoencephalocele causing rhinoliquoral fistula and spontaneous intracranial hypotension.
Results
this 65-year-old woman presented with sporadic rhinoliquorrhoea associated with orthostatic headache, nausea and dizziness. Brain MRI revealed a small lesion of an ethmoidal sinus, which was successfully treated with endoscopic endonasal surgery. Histology confirmed the presence of meningoencephalic tissue positive for S100 protein on immunohistochemistry.
Conclusions
When dealing with lesions of the paranasal sinuses in contact with the anterior skull base, rhinoliquorrhoea presence suggests meningoencephalocele. In dubious cases, a proper workup, including a thorough clinical history and neurological examination, specific imaging, and a direct search of CSF-like markers, is essential to support the differential diagnosis. In such cases, a transnasal endoscopic surgical approach is recommended to obtain a final histological diagnosis and to perform eventual dural plastic surgery.
... In patients with abnormally low ICP, headache may worsen when upright and improve in the supine position. 9 Invasive measurement of ICP in humans reported near-zero pressure (−2 to +4 mmHg) in standing or seated upright posture and 9-15 mmHg in a supine posture, with further increase in HDT. 10,11 On the other hand, IOP varies much less with posture than ICP; only a 1to 4-mmHg increase in IOP was observed in HDT compared to a seated upright posture. ...
Purpose:
Intracranial pressure increases in head-down tilt (HDT) body posture. This study evaluated the effect of HDT on the optic nerve sheath diameter (ONSD) in normal subjects.
Methods:
Twenty six healthy adults (age 28 [4.7] years) participated in seated and 6° HDT visits. For each visit, subjects presented at 11:00 h for baseline seated scans and then maintained a seated or 6° HDT posture from 12:00 to 15:00 h. Three horizontal axial and three vertical axial scans were obtained at 11:00, 12:00 and 15:00 h with a 10 MHz ultrasonography probe on the same eye, randomly chosen per subject. At each time point, horizontal and vertical ONSD (mm) were quantified by averaging three measures taken 3 mm behind the globe.
Results:
In the seated visit, ONSDs were similar across time (p > 0.05), with an overall mean (standard deviation) of 4.71 (0.48) horizontally and 5.08 (0.44) vertically. ONSD was larger vertically than horizontally at each time point (p < 0.001). In the HDT visit, ONSD was significantly enlarged from baseline at 12:00 and 15:00 h (p < 0.001 horizontal and p < 0.05 vertical). Mean (standard error) horizontal ONSD change from baseline was 0.37 (0.07) HDT versus 0.10 (0.05) seated at 12:00 h (p = 0.002) and 0.41 (0.09) HDT versus 0.12 (0.06) seated at 15:00 h (p = 0.002); mean vertical ONSD change was 0.14 (0.07) HDT versus -0.07 (0.04) seated at 12:00 h (p = 0.02) and 0.19 (0.06) HDT versus -0.03 (0.04) seated at 15:00 h (p = 0.01). ONSD change in HDT was similar between 12:00 and 15:00 h (p ≥ 0.30). Changes at 12:00 h correlated with those at 15:00 h for horizontal (r = 0.78, p < 0.001) and vertical ONSD (r = 0.73, p < 0.001).
Conclusion:
The ONSD increased when body posture transitioned from seated to HDT position without any further change at the end of the 3 h in HDT.
... Заболеваемость СВГ оценивается приблизительно 2-5 случая на 100 000 человек в странах Запада [26]. Это -одна из самых мимикрирующих патологий, и в настоящее время принято считать, что она очень плохо диагностируется [27]. ...
In people over 65 years of age, hearing disorders occur in 30% of cases. Hearing impairment affects the psychoemotional status of patients, exacerbating the progression of cognitive deficits. One of the variants of frontotemporal dementia (FTD) is pathogenetically caused by intracranial hypotension syndrome (IHS). The incidence of IHS is estimated to be approximately 2–5 cases per 100,000 inhabitants. One of the complications of SVH is dementia with sagging brain syndrome (BSD), clinically similar to FTD. IHS is regularly accompanied by cochleovestibular disorders, among which audiological deviations prevail. To confirm IHS, positional audiometry is of great importance. Unlike typical FTD, for which effective therapy is unknown, BSD treatment results are positive in 81% of cases. The authors of the publication note an improvement in concentration of attention, analytical ability, and daytime activity in their patients after the course of treatment of IHS. For earlier detection, or confirmation, of the mentioned pathology at the diagnostic stage, the authors propose an increase in the number of studies due to audiometric tests.
... 11 SIH is an increasingly recognized cause of headaches, but it may also cause a variety of other more or less serious neurologic manifestations such as hearing loss, diplopia, coma, brachial amyotrophy, and superficial siderosis. [12][13][14][15] The cause of SIH is a CSF leak at the level of the spine 16 and several types have been identified. 17 In 2002, Hong et al. were the first to report the association of symptoms of bvFTD and SIH in a patient who also had other features of SIH, including pachymeningeal enhancement and severe brain sagging on MRI and low CSF pressure. ...
Introduction:
Due to loss of brain buoyancy, spontaneous spinal cerebrospinal fluid (CSF) leaks cause orthostatic headaches but also can cause symptoms indistinguishable from behavioral variant frontotemporal dementia (bvFTD) due to severe brain sagging (including the frontal and temporal lobes), as visualized on brain magnetic resonance imaging. However, the detection of these CSF leaks may require specialized spinal imaging techniques, such as digital subtraction myelography (DSM).
Methods:
We performed DSM in the lateral decubitus position under general anesthesia in 21 consecutive patients with frontotemporal dementia brain sagging syndrome (4 women and 17 men; mean age 56.2 years [range: 31-70 years]).
Results:
Nine patients (42.8%) were found to have a CSF-venous fistula, a recently discovered type of CSF leak that cannot be detected on conventional spinal imaging. All nine patients underwent uneventful surgical ligation of the fistula. Complete or near-complete and sustained resolution of bvFTD symptoms was obtained by all nine patients, accompanied by reversal of brain sagging, but in only three (25.0%) of the twelve patients in whom no CSF-venous fistula could be detected (P = 0.0011), and who were treated with non-targeted therapies.
Discussion:
Concerns about a spinal CSF leak should not be dismissed in patients with frontotemporal brain sagging syndrome, even when conventional spinal imaging is normal. However, even with this specialized imaging the source of the loss of spinal CSF remains elusive in more than half of patients.
... It is also notable that CSF production and flow, along with intracranial pressure (ICP), are highly dependent upon gravity and vary at different altitudes and with spaceflight (e.g., astronauts in microgravity often develop new onset migraine headaches). 8,9 Although it remains speculative, the regional gravitational ischemia hypothesis implies a physiologic sequence that could potentially occur for some IBS patients, as follows: GI symptoms and comorbidities disrupt sleep, manifesting with less time spent in the horizontal position; lack of prolonged and restful sleep leads to diminished time reperfusing the gravity-dependent hypothalamus and pituitary; HPA function is undermined and cortisol cycles are dysregulated; abnormalities in cortisol directly impact GI physiology and alter gut microbiology; a dysregulated microbiome leads to increased production of bacterial metabolites which cause peripheral sensitization of afferent visceral nerves, ultimately leading to worsened IBS symptoms. Of course, stress itself can directly alter the HPA axis independent of sleep, but the gravity hypothesis offers an additional mechanism that seeks to accommodate the links between cerebrovascular blood flow, CSF flow, ICP, and gravity. ...
The pathogenesis of irritable bowel syndrome (IBS)-a disorder of gut-brain interaction that affects up to 10% of the world's population-remains uncertain. It is puzzling that a disorder so prevalent and archetypal among humans can be explained by disparate theories, respond to treatments with vastly different mechanisms of action, and present with a dazzling array of comorbidities. It is reasonable to question whether there is a unifying factor that binds these divergent theories and observations, and if so, what that factor might be. This article offers a testable hypothesis that seeks to accommodate the manifold theories, clinical symptoms, somatic comorbidities, neuropsychological features, and treatment outcomes of IBS by describing the syndrome in relation to a principal force of human evolution: gravity. In short, the hypothesis proposed here is that IBS may result from ineffective anatomical, physiological, and neuropsychological gravity management systems designed to optimize gastrointestinal form and function, protect somatic and visceral integrity, and maximize survival in a gravity-bound world. To explain this unconventional hypothesis of IBS pathogenesis, referred to herein as the gravity hypothesis, this article reviews the influence of gravity on human evolution; discusses how Homo sapiens imperfectly evolved to manage this universal force of attraction; and explores the mechanical, microbial, and neuropsychological consequences of gravity intolerance with a focus on explaining IBS. This article concludes by considering the diagnostic and therapeutic implications of this new hypothesis and proposes experiments to support or reject this line of inquiry. It is hoped that the ideas in this thought experiment may also help encourage new or different ways of thinking about this common disorder.
