Intracranial Dural Arteriovenous Fistula as a Reversible Cause of Dementia: Case Series and Literature Review

doi:10.1016/j.wneu.2018.09.161

World Neurosurgery

Volume 121, January 2019, Pages e543-e553

https://doi.org/10.1016/j.wneu.2018.09.161 Get rights and content

Highlights

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Dementia, cognitive decline, and parkinsonism are rare presentations of intracranial DAVFs.
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There are 2 potential mechanisms of DAVF-induced dementia: cortical dementia and thalamic dementia.
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Cortical dementia was present in all of our patients.
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Thalamic dementia is due to edema of deep gray and white matter structures.
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All of our patients had resolution or improvement of dementia-type symptoms after endovascular treatment.

Objective

Intracranial dural arteriovenous fistulas (DAVFs) can manifest with a reversible form of dementia and parkinsonism that may be related to medullary venous hypertension and impaired cortical venous drainage. We report a consecutive case series of patients with DAVFs who presented with cognitive decline and report angioarchitecture and outcomes of treatment.

Methods

From a prospectively collected database of 389 cranial DAVFs, we identified patients who presented with progressive dementia. Data collected included demographic characteristics, other presenting symptoms, fistula location and type, preoperative and postoperative imaging findings, and treatment outcomes. Descriptive statistics were reported.

Results

Of 389 patients with DAVF, 6 patients with progressive dementia (1.4%) were identified. All patients were male. Mean patient age was 68.6 years. Four patients had superior sagittal sinus DAVFs, 1 patient had a tentorial DAVF, and 1 patient had a transverse sinus DAVF. All patients had evidence of medullary venous hypertension on imaging with either reflux and arterialization of the transmedullary veins or venous congestion of the transmedullary veins. All patients underwent endovascular treatment and had reversal of dementia-related symptoms after a mean follow-up of 6.2 months.

Conclusions

Cognitive decline is a rare but reversible manifestation of DAVFs that have impaired venous drainage of the transmedullary veins and associated medullary venous hypertension.

Introduction

Intracranial dural arteriovenous fistulas (DAVFs) are acquired lesions that account for 10%–15% of intracranial vascular malformations.1, 2 These lesions are characterized by a shunt between dural arteries and a venous sinus and/or cortical vein.3, 4 DAVFs can have a wide variety of clinical manifestations depending on their location and angiographic features, including ocular symptoms, proptosis, pulsatile tinnitus, cranial nerve deficits, intracranial hemorrhage, or nonhemorrhagic neurologic deficits.4, 5 The natural history of DAVFs is dictated primarily by their venous drainage pattern. Patients with angiographic evidence of cortical venous reflux are at higher risk of intracranial hemorrhage and thus are believed to require more urgent treatment.5, 6 Patients with cortical venous reflux are have higher rates of nonhemorrhagic neurologic deficits secondary to venous congestion. It has been reported that certain DAVFs with deep venous drainage that manifest with bilateral thalamic and basal ganglia edema can result in cognitive and memory impairment, parkinsonism, and hypersomnolence.2, 3, 4, 7, 8, 9, 10, 11, 12, 13, 14, 15 Much of the literature on DAVF-induced dementia has focused on thalamic dementia resulting from fistulas affecting the deep venous system.2, 8, 9, 10, 16, 17 We report a consecutive case series of patients with DAVF-induced dementia from both deep and superficial fistulas that we believe is related to abnormal cerebrospinal fluid (CSF) reabsorption through transmedullary venous congestion.

Section snippets

Patient Population

This retrospective review was approved by our institutional review board. From our prospectively collected database of 389 patients with dural arteriovenous fistulas from 2008 to 2018, patients were selected who met the following inclusion criteria: 1) adult patients; 2) presentation with cognitive decline or dementia as determined by a neurologic specialist; 3) no other known cause of cognitive decline present (e.g., infection, stroke, paraneoplastic syndrome, intracranial mass,

Patient Population and Baseline Characteristics

Results data of our patients are summarized in Table 1. From 389 patients with DAVFs, we identified 6 patients (2%). All patients were male. Mean age was 69 years (range, 51–79 years). All patients presented with rapid-onset and progressive dementia, together with motor weakness in 1 patient (patient 1), and imbalance and incontinence in 1 patient (patient 4). None of our patients presented with parkinsonism.

Preoperative Imaging Findings

All patients had evidence of medullary venous congestion on either CT angiography or

Discussion

Our small case series of 6 patients with reversible DAVF-induced dementia demonstrated a number of interesting findings. All patients had rapid onset of dementia symptoms, and the most frequent sign identified on MRI was dilatation of medullary and transmedullary veins. Four patients developed reversible white matter hyperintensities, whereas 2 patients did not have any white matter signal changes. In 4 of the 6 cases, patients had widespread fistula sites over a larger area, impairment of

Conclusions

Our study demonstrates a potential relationship between intracranial venous hypertension from DAVF and dementia. All patients in our series had subacute progressive cognitive decline owing to chronic intracranial venous hypertension affecting the medullary veins. Endovascular treatment in these patients resulted in resolution of the dementia-type symptoms by restoring normal venous hemodynamics. Further studies and case series are needed to validate our results. Future studies should include

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Citation Excerpt :
Imaging changes and pathologic findings support the hypothesis that the clinical course results from the delivery of excessive volumes of blood flow into a venous system with outflow obstruction. Venous congestion, the result of hypertension with impaired parenchymal venous drainage [12], and ischemia occur from the downstream effect of arterialization of cortical venous drainage from cortical venous hypertension [16], leading to cerebral venous congestion [17]. There are two investigated mechanisms of DAVF-induced dementia: cortical dementia and thalamic dementia due to edema of deep gray and white matter structures [4].
Cerebrovascular malformations (CVMs) such as arteriovenous malformations (AVMs) or dural arteriovenous fistulas (DAVFs) represent a possible source of intracranial hemorrhage, but these malformations can also manifest with neurologic disorders secondary to ischemic penumbra from vascular steal. In the latter case, the clinical manifestations are less obvious and characteristic, and may include a varied clinical spectrum ranging from focal deficits to generalized malfunction of the brain parenchyma resulting in dementia. Dementias secondary to CVMs constitute a probably underestimated subpopulation of patients of great interest because they present with devastating but potentially reversible cognitive impairment. We examined the pertinent literature regarding the clinical manifestations of CVMs characterized by cognitive impairment and describe the distinctive clinical features. Our results confirm that cognitive impairment is one of the clinical manifestations of CVMs and is a frequently misrecognized and often late-diagnosed cause of reversible dementia.
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The large variety of clinical manifestations of DAVFs depends on its location, but this is not exactly valid for the onset of dementia. Some reports have suggested that patients with DAVFs involving the superior sagittal sinus (SSS) have a higher rate of presenting clinically with dementia [77], but some others reported that the involvement of SSS is infrequently [47,62]. On the other hand, dural fistulas of the transverse and sigmoid sinuses vary in both symptoms and prognosis [14].
Dementia is a chronic loss of neurocognitive function that is progressive and irreversible. Dural arteriovenous fistulas (DAVFs) are acquired lesions that account for 10–15% of intracranial vascular malformations that could present with a rapid decline in neurocognitive function with or without Parkinson-like symptoms and evolve in a rapidly progressive dementia (RPD). Often the DAVFs are not even included in the differential hypotheses of this type of dementia and are not present in any type of diagnostic algorithm for evaluating RPD.
We performed a systematic review of the international literature and adding the cases coming from our institutional experience and we have collected all the reported cases of DAVFs that debut with ROD identifying the most frequent forms in terms of location and type, reporting the neurological characteristics and the outcome of each patient.
The exact pathogenesis for developing dementia in patients with DAVFs remains largely unknown. The imaging changes and pathologic findings support the hypothesis that the clinical course results from the delivery of excessive volumes of blood flow into a venous system with outflow obstruction and venous congestion. The large variety of clinical manifestations of DAVFs depends on its location but this is not exactly valid for the onset of dementia. It supposed that the highly variable clinical manifestation of DAVFs has been convincingly related to the pattern of venous drainage more than location.
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: Conflict of interest statement: This study received support from the Andreae Holt Hornsby Vascular Dementia Research Unit of the Toronto Western Hospital.

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Original ArticleIntracranial Dural Arteriovenous Fistula as a Reversible Cause of Dementia: Case Series and Literature Review

Highlights

Objective

Methods

Results

Conclusions

Introduction

Section snippets

Patient Population

Patient Population and Baseline Characteristics

Preoperative Imaging Findings

Discussion

Conclusions

Neurosurg Clin N Am

Lancet

Clin Neurol Neurosurg

Neuroimaging Clin N Am

Surg Neurol

Dementia resulting from dural arteriovenous fistulas: the pathologic findings of venous hypertensive encephalopathy

AJNR Am J Neuroradiol