Dural arteriovenous fistula-induced thalamic dementia: report of 4 cases

Abstract

Nonhemorrhagic neurological deficits are underrecognized symptoms of intracranial dural arteriovenous fistulas (dAVFs) having cortical venous drainage. These symptoms are the consequence of cortical venous hypertension and portend a clinical course with increased risk of neurological morbidity and mortality. One rarely documented and easily misinterpreted type of nonhemorrhagic neurological deficit is progressive dementia, which can result from venous hypertension in the cortex or in bilateral thalami. The latter, which is due to dAVF drainage into the deep venous system, is the less common of these 2 dementia syndromes. Herein, the authors report 4 cases of dAVF with venous drainage into the vein of Galen causing bithalamic edema and rapidly progressive dementia. Two patients were treated successfully with endovascular embolization, and the other 2 patients were treated successfully with endovascular embolization followed by surgery. The radiographic abnormalities and presenting symptoms rapidly resolved after dAVF obliteration in all 4 cases. Detailed descriptions of these 4 cases are presented along with a critical review of 15 previously reported cases. In our analysis of these 19 published cases, the following were emphasized: 1) the clinical and radiographic differences between dAVF-induced thalamic versus cortical dementia syndromes; 2) the differential diagnosis and necessary radiographic workup for patients presenting with a rapidly progressive thalamic dementia syndrome; 3) the frequency at which delays in diagnosis occurred and potentially dangerous and avoidable diagnostic procedures were used; and 4) the rapidity and completeness of symptom resolution following dAVF treatment.http://thejns.org/doi/abs/10.3171/2015.5.JNS15473Key words dural arteriovenous fistula; cortical venous hypertension; thalamic edema; nonhemorrhagic neurological deficit; thalamic dementia; vascular disorders ©AANS, 2016 davF-induced thalamic dementiaBorden-Shucart classification system-based not only on angiographic appearance but also mode of presentationto permit more accurate risk stratification and assist with clinical decision making for the type and timing of dAVF treatment (Table 1). 68,91One relatively underappreciated type of NHND is progressive dementia resulting from dAVF-induced cortical venous hypertension, though correct diagnosis and treatment is increasing, thanks to improved imaging techniques.18,79 Dural AVF-induced progressive dementia can be differentiated as either cortical or thalamic in origin-2 categories that have relatively distinct patterns of clinical symptomatology 18,54,62,80,85 and highly specific venous outflow patterns.18,80 Of the two, dAVFinduced thalamic dementia is less frequent, with only 15 published cases reported in the literature to date. All were individual case reports, and several lacked adequate clinical, radiographic, and/or treatment specifics to permit detailed assessment as to manner of presentation, underlying hemodynamic pathophysiology, and long-term outcome. 18,[21][22][23]...

Dural Arteriovenous Fistula Manifested as Rapid Progressive Dementia Successfully Treated by Endovascular Embolization Only

“…Venous congestion and ischemia of the thalamus occurred through venous hypertension followed by retrograde cerebral blood flow that caused thalamic dementia. As far as we know, a DAVF case with cognitive impairment and transient memory loss is uncommon [3678]. In our case, the patient had bilateral thalamic congestion due to a DAVF located in the posterior fossa and showed episodic memory loss with inattention and disorientation.…”

“…Clinical manifestations of intracranial DAVF are various including pulsatile tinnitus, orbital congestion, headache, intracranial hemorrhage or infarction [2]. However, there have been little reports about DAVFs manifested as rapidly progressive dementia [34]. Most offending DAVFs causing dementia are located at the tentorial edge, thereby a report about DAVFs located in the posterior fossa has meaning.…”

Intracranial Dural Arteriovenous Fistulae

“…symptoms related to cortical venous hypertension including ICH and NHND)—Type 2S and 3S. 6, 19 By integrating angiographic appearance (presence or absence of CVD) and mode of presentation (asymptomatic vs. symptomatic CVD) into a single scheme, this classification system more accurately predicts risk of future hemorrhagic and non-hemorrhagic neurological events and therefore affords improved risk stratification to guide appropriate timing and manner of dAVF treatment.…”

“…15, 16 Reported NHNDs related to dAVFs include seizures, focal cortical deficits, cranial nerve palsies, trigeminal neuralgia, thalamic or cortical dementia, Parkinsonism, cerebellar dysfunction, dysmetria, paresthesias, myelopathy, quadriparesis, aphasia or dysphasia, apraxia, apathy, failure to thrive, and symptoms related to increased pressure including headache, nausea, and vomiting. 1, 17, 19–23 …”

Progressive Neurological Decline with Deep Bilateral Imaging Changes: A Protean Presentation of Dural Arteriovenous Fistulae

“…Increasingly, deep cerebral DAVF are being recognized as a cause of these presentations and imaging findings. Case reports have emerged over the prior decade showing that this type of presentation has led to misdiagnoses that include Creutzfeldt-Jakob disease [1], progressive dementia [2,3], parkinsonism [4], brainstem glioma [5][6][7], and viral encephalitis [6].…”