Cerebrospinal Venous Fistula Presenting with Cognitive Decline: Systematic Literature Review and Report of Two Cases

Stuebe, Caren M.; Jones, Breck; Syal, Arjun; Rahme, Rudy J.; Turcotte, Evelyn L.; Toussaint, L. Gerard; Ross, Jeffrey S.; Bendok, Bernard R.

doi:10.1016/j.wneu.2023.03.056

Cited by 3 publications

(7 citation statements)

References 25 publications

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“…Other frequently encountered symptoms of SIH include nausea, vomiting, dizziness, vertigo, hearing loss, distorted hearing, tinnitus, and cognitive impairment. 9,10 Many of these clinical manifestations correspond to dysfunction of the inner ear apparatus, which is the result of an abnormal pressure gradient across the vestibular membrane due to impaired CSF-derived perilymph production. 11 In extreme cases, SIH may present with diplopia, visual disturbances, frontotemporal dementia, or coma, all of which are the direct result of extreme brain sag and caudal displacement of brainstem structures or traction on cranial nerves.…”

Section: Clinical Presentation Of Sihmentioning

confidence: 99%

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Transvenous Embolization of Cerebrospinal Fluid-Venous Fistulas: Pearls, Pitfalls, and Lessons Learned

2023

Contemporary Neurosurgery

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Section: Clinical Presentation Of Sihmentioning

confidence: 99%

“…Additionally, some groups have had success using MR myelography for detecting CSFVF whereas others have questioned the utility of MR myelography in CSFVF detection. 10,17,18 Thus, further investigation is needed to elucidate the role for these imaging modalities in CSFVF detection.…”

Section: Diagnosis Of Sih and Identification Of Csfvfmentioning

confidence: 99%

Transvenous Embolization of Cerebrospinal Fluid-Venous Fistulas: Pearls, Pitfalls, and Lessons Learned

2023

Contemporary Neurosurgery

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“…The annual incidence of SIH is estimated at 5 cases per 100,000 individuals [ 21 ]. The occurrence of BSD remains unknown, but recent evidence suggests the disease to be more prevalent than previously recognized [ 1 ••, 3 , 4 ••, 5 – 7 , 8 •]. This increased awareness and recognition of BSD mirror the trajectory of SIH, which was also initially considered a rare disorder [ 22 ].…”

Section: Epidemiologymentioning

confidence: 99%

“…Spinal diverticula ( type 2 CSF leak ) [ 38 ], spondylotic spurs, calcified disc herniations, and connective tissue abnormalities can all cause dural tear and CSF leak leading to BSD. In recent years, advanced diagnostic techniques such as MRI myelography and digital subtraction myelography (DSM) have identified an increasing number of CSF-venous fistulas ( type 3 CSF leak ) [ 38 ] in BSD patients [ 4 ••, 8 •]. However, despite these advancements, a CSF leak site is only localized in a minority (13%) of cases [ 2 •].…”

Section: Etiology and Pathogenesismentioning

confidence: 99%

“…Brain sagging dementia (BSD) is a rare however devastating disorder that has recently gained increasing attention and recognition [ 1 ••, 2 •, 3 , 4 ••, 5 – 7 , 8 •, 9 ]. This disease is considered an extension of the better-recognized condition known as spontaneous intracranial hypotension (SIH) which is caused by cerebrospinal fluid (CSF) leak and hypovolemia.…”

Section: Introductionmentioning

confidence: 99%

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Brain Sagging Dementia

Lashkarivand,

Eide

2023

Curr Neurol Neurosci Rep

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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.

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Transvenous Embolization of Cerebrospinal Fluid-Venous Fistulas: Pearls, Pitfalls, and Lessons Learned

Naylor,

Madhavan,

Cutsforth-Gregory

et al. 2023

Contemporary Neurosurgery

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Cerebrospinal fluid-venous fistulas (CSFVFs) are increasingly recognized as a cause of spontaneous intracranial hypotension (SIH), a debilitating neurologic condition that may present with postural headaches, hearing loss, and/or cognitive impairment. Curative treatment is available for patients whose fistulas are localized with myelography. Transvenous endovascular embolization has emerged in recent years as a safe, effective, and durable treatment option for patients with CSFVF. Here, we briefly review the clinical presentation of SIH, present diagnostic algorithm by which CSFVFs are identified, and discuss a stepwise treatment paradigm. We then detail endovascular treatment strategies of CSFVF with particular emphasis on direct versus indirect approaches to the fistula, embolization principles, and techniques to minimize complications. With increasing awareness of SIH and enhanced detection of CSFVF, it is important for endovascular neurosurgeons and neurointerventionalists to be familiar with transvenous CSFVF embolization strategies to meet the needs of this patient population.

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Cerebrospinal Venous Fistula Presenting with Cognitive Decline: Systematic Literature Review and Report of Two Cases

Cited by 3 publications

References 25 publications

Transvenous Embolization of Cerebrospinal Fluid-Venous Fistulas: Pearls, Pitfalls, and Lessons Learned

Transvenous Embolization of Cerebrospinal Fluid-Venous Fistulas: Pearls, Pitfalls, and Lessons Learned

Brain Sagging Dementia

Transvenous Embolization of Cerebrospinal Fluid-Venous Fistulas: Pearls, Pitfalls, and Lessons Learned

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