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Year : 2020  |  Volume : 68  |  Issue : 4  |  Page : 939--940

Rapidly Progressive Dementia as a Clinical Feature of Multiple Dural Arteriovenous Fistulas

Agustin Pappolla, Federico Sosa Albacete, Ariel Luzzi, Maria Cristina Zurru, Marcelo Rugiero 
 Neurology Department, Italian Hospital of Buenos Aires, Buenos Aires, Argentina

Correspondence Address:
Dr. Agustin Pappolla
Peron 4190 Street, Buenos Aires C1199ABB

How to cite this article:
Pappolla A, Albacete FS, Luzzi A, Zurru MC, Rugiero M. Rapidly Progressive Dementia as a Clinical Feature of Multiple Dural Arteriovenous Fistulas.Neurol India 2020;68:939-940

How to cite this URL:
Pappolla A, Albacete FS, Luzzi A, Zurru MC, Rugiero M. Rapidly Progressive Dementia as a Clinical Feature of Multiple Dural Arteriovenous Fistulas. Neurol India [serial online] 2020 [cited 2020 Nov 30 ];68:939-940
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A 69-year-old male with a personal history of arterial hypertension and dyslipidemia complained of headache, pulsatile tinnitus, vision disturbances, and a hearing loss that started 2 months earlier. His physical examination revealed bilateral papilledema, reduced visual acuity, and hypoacusia without other abnormalities. Brain magnetic resonance imaging (MRI) showed mild dilation of both external carotid arteries on T2-weighted sequences without other abnormalities [Figure 1]. Lumbar puncture (LP) revealed an elevated opening pressure. He was diagnosed with idiopathic intracranial hypertension, and acetazolamide treatment was started. Nevertheless, his visual symptoms continued to worsen and a lumboperitoneal shunt was placed. An initial improvement was obtained, but during the following 4 months, he continued to develop recurrent confusional episodes, global bradykinesia, gait apraxia, and generalized myoclonus. A neuropsychological profile disclosed a severe cognitive impairment, which was observed particularly in trail making tests A and B and digit symbol test. Electroencephalogram was negative for epileptic discharges. A new MRI revealed bilateral subdural effusions with supra- and infratentorial engorged pial vessels and a second LP-disclosed persistent elevated opening pressure. No hydrocephalus was observed. Shunt parameters were adjusted, obtaining a transient improvement of motor symptoms; however, the patient's cognitive status declined throughout the following year, adding apathy, visual hallucinations, and dysphagia. MRI was repeated, showing absence of flow in multiple dural sinuses, widespread cortical vessel dilations, and cortical bleedings. A digital subtraction angiography (DSA) revealed multiple dural arteriovenous fistulas (DAVFs) between both external carotid arteries and the superior sagittal sinus. In addition, signs of thrombosis were observed in both sigmoid sinuses and superior sagittal sinus. Bilateral embolization was performed, and oral anticoagulation was started. Postembolization images showed adequate DAVF occlusion, but cortical vessels remained dilated and venous sinuses were occluded to date. His motor symptoms markedly improved, but comparative neuropsychological assessment reveals that he presents a residual mild subcortical cognitive impairment to date.{Figure 1}

Although no clear consensus exists for the time frame of a rapidly progressive dementia (RPD), we usually refer to conditions that progress from the onset of first symptom to dementia in <1–2 years.[1] The most common causes are primary dementias (such as Alzheimer disease), prions, autoimmune/antibody-related conditions, and encephalitis.[2],[3] Vascular conditions are a relatively unusual cause of RPD, being most frequently associated with stroke or vascular dementia in acute or chronic clinical scenarios, respectively. However, real RPDs are scarce in cerebrovascular disease, and only a few disorders, such as DAVFs, may cause them.

DAVFs are abnormal connections of vessels in which one or more arteries are directly connected to one or more veins, venous sinuses, or cortical veins. They represent 10%–15% of the arteriovenous malformations of the central nervous system. Males are more commonly affected, usually at 50–70 years.[4],[5] Congenital and acquired conditions, such as persistence of fetal vessels, head trauma, surgery, tumors, infections, coagulopathies, pregnancy, and oral contraceptives, may cause or predispose DAVFs. Dural sinus thrombosis and the consequent high venous pressure appear to be another important cause of DAVF formation. In such cases, vascular growth factors such as vascular endothelial growth factor and hypoxia-inducible factor-1 are secreted, inducing vascular remodeling and angiogenesis.[6] As a final result, communication of dural sinuses and arteries is initiated, which perpetuates the increased venous pressure, favoring surface vein reflux, hemorrhages, and cortical bleeding/ischemia.[6],[7]

Most patients with DAVFs complain of headache, fluctuating level of consciousness, memory loss, seizures, and gait instability. Others complain of focal neurologic deficits or hearing bruit over the skull. Most symptoms are related to cortical and white matter damage, while Parkinsonism might be related to basal ganglia dysfunction. Duration from symptom onset to RPD usually ranges from about 2 to 12 months.[6] Common MRI findings include diffuse hyperintensities in the cerebral white matter, enlarged vessels over the hemispheric surface on T2-weighted images, or engorged pial vessels in DSA. Basal ganglia hyperintensities have been rarely reported.[7],[8],[9]

It is critical to consider a DAVF in a patient presenting with RPD, particularly with movement disorders and MRI showing surface vessel engorgement on T2 sequences, diffuse white matter lesions, and cortical ischemia/hemorrhages. Given that symptoms are often reversible, a DSA must be performed promptly in these patients in order to avoid misdiagnosis and achieve an effective embolization.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.


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