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Table of Contents    
Year : 2014  |  Volume : 62  |  Issue : 1  |  Page : 107-110

Rapidly progressive dementia, Parkinsonism and myoclonus: An unusual presentation of dural arteriovenous fistula

1 Department of Neurology, Sree Chitra Tirunal Institute of Medical Sciences and Technology, Trivandrum, Kerala, India
2 Department of Imaging Sciences and Intervention Radiology, Sree Chitra Tirunal Institute of Medical Sciences and Technology, Trivandrum, Kerala, India

Date of Submission29-Oct-2013
Date of Decision18-Dec-2013
Date of Acceptance02-Feb-2014
Date of Web Publication7-Mar-2014

Correspondence Address:
Sujit Abajirao Jagtap
Department of Neurology, Sree Chitra Tirunal Institute of Medical Sciences and Technology, Trivandrum, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.128360

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How to cite this article:
Jagtap SA, Nair SS, Jain N, Nair M D. Rapidly progressive dementia, Parkinsonism and myoclonus: An unusual presentation of dural arteriovenous fistula. Neurol India 2014;62:107-10

How to cite this URL:
Jagtap SA, Nair SS, Jain N, Nair M D. Rapidly progressive dementia, Parkinsonism and myoclonus: An unusual presentation of dural arteriovenous fistula. Neurol India [serial online] 2014 [cited 2022 Sep 28];62:107-10. Available from: https://www.neurologyindia.com/text.asp?2014/62/1/107/128360


Dural arteriovenous fistulas (DAVFs) consist of arteriovenous shunts between meningeal arteries and veins within the dura and account for 10-15% of cerebrovascular malformations. [1],[2],[3] The clinical presentation is heterogeneous headache , pulsatile tinnitus, proptosis, dementia, parkinsonism and intracranial hemorrhage. [3],[4],[5],[6],[7] The presenting feature of DAVFs is related to the location and pattern of venous drainage. [3] Untreated aggressive DAVF have an annual event rate of 15% and an annual mortality rate of 10.4%. [8] We report an untreated aggressive DAVF presenting with dementia, parkinsonism and myoclonus.

A 73-year-old female patient presented with 3 months history of progressive cognitive decline, gait difficulty and myoclonic jerks. Her initial symptom was cognitive decline in the form of memory impairment, disorientation to time and visuospatial disorientation which rapidly progressed over 2 months to complete inability to comprehend and attend to her routine activities. About 3 weeks into her illness, she developed tremulousness of the hands and stiffness of all limbs with slowness of gait. She also developed frequent jerking movements of her limbs after 1 month. Her illness progressed rapidly and she became bedridden and dependent for all activities of daily living by 2½ months. She had a past history of long standing diabetes mellitus and systemic hypertension. Neurological examination at presentation revealed significant and diffuse cognitive impairment with impaired comprehension and poor attention. She had moderate rigidity of all limbs with normal deep tendon reflexes and flexor plantar responses. She had stimulus sensitive multifocal myoclonus of upper limbs. Computed tomography (CT) brain showed prominent and hyperdense transverse sigmoid junction with a few calcific foci raising the suspicion of a DAVF fistula [Figure 1]. Magnetic resonance imaging (MRI) brain showed bilateral DAVFs involving transverse sinus-sigmoid sinus junctions with prominent draining vein and hypertrophied feeders from extracranial arteries bilaterally [Figure 2]. Ill-defined white matter hyperintensities were also seen in bilateral cerebral hemispheres. During the course of evaluation, she had worsening of lower respiratory tract infection, developed sepsis which progressed to multiorgan failure with hypotension, because of which confirmatory digital substraction angiography could not be done. She expired within 1 week of admission.
Figure 1: Non contrast computed tomography head (a) prominent right transverse-sigmoid sinus junction with focal calcifi cation. (b) Prominent left transverse-sigmoid sinus junction. (c) Right parahippocampal calcifi cation

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Figure 2: T2-weighted axial images (a-c) and susceptibility weighted imaging images (d) and (e) reveal presence of dural arteriovenous fistula at bilateral transverse sinus – sigmoid sinus junction (thick arrow) with prominent cortical veins in that region (thin arrow) with hypertrophied feeders from extracranial arteries bilaterally (hallow arrow)

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DAVFs most commonly develop in the region of the transverse and sigmoid sinuses [3],[4] and comprise more than 60% of all DAVFs. [2] The exact pathogenesis of DAVF is unknown and it has been postulated that thrombosis of the dural venous sinuses may be the initial triggering event in the development of DAVFs. [9] As a sequelae to sinus thrombosis or large amounts of flow through the DAVF there is an increase in pressure within the dural sinuses, resulting in venous hypertension. The retrograde transmission of venous pressure may occur into the superior sagittal sinus, straight sinus and cortical veins. [4],[9] The major risk factor for aggressive behavior of DAVFs, including intracranial hemorrhage is the retrograde transmission of pressure causing enlargement of cortical veins. [10],[11] The increased venous pressure results in venous hypertension, congestion and impaired parenchymal venous drainage resulting in ischemia, leading to progressive encephalopathy with dementia. [11],[12] In addition to cognitive deterioration, nonspecific clinical picture may delay detection of the responsible lesion, permitting further deterioration in the absence of treatment, as in our patient. Progressive headache and cranial bruit in a patient with dementia should raise suspicion of an underlying vascular lesion. In a large series of DAVFs, 3-12% of patients with DAVFs presented with predominantly progressive cognitive deficits with or without decreased level of consciousness or lateralizing findings. [11],[13]

The MRI abnormalities in our patients arose as a consequence of venous outflow obstruction and correlate well with the potential for aggressive behavior from these high-flow DAVFs. Gradient or susceptibility weighted imaging may be helpful in the diagnosis of DAVF even before angiogram. Angiography is imaging of choice for the diagnosis of a DAVF and also provides a route for endovascular therapy. [11] CT or MR studies most often show minimal or no abnormalities in the case of DAVFs venous outflow obstruction, and and a high degree of suspicion is required to make the diagnosis. [14] MRI may show diffuse white matter hyperintensities, hemorrhage, dilated vessels, vascular enhancement and flow-voids. MR angiograms may identify DAVF with sufficiently high flow with widespread venous hypertension and generalized cognitive dysfunction. [14] The presence of non-specific parenchymal abnormalities in patients with DAVFs helps in excluding other more common causes of cognitive decline. [11] Arterial embolization is usually successful in decreasing flow through a DAVF, with cognitive improvement in most of the cases. [13] Additional arterial embolization may be required if there is recurrence of cognitive decline, in order to decrease flow and venous hypertension. A combined endovascular - surgical approach is considered in many centers. The potential for recurrent and possibly permanent ischemic damage emphasizes the need for an aggressive approach to closure of these lesions to relieve excessive venous pressure. Our patient presented with rapidly progressive dementia, Parkinsonism and myoclonus and succumbed to her illness before definitive treatment could be administered underlining the need for maintaining a high index of clinical suspicion for this condition.

In patients with rapidly progressive dementia, Parkinsonism and myoclonus; venous hypertensive encephalopathy secondary to DAVFs should be considered as a rare but reversible differential diagnosis. Delay in diagnosis and treatment will result in irreversible neuronal damage due to venous ischemia.

 » References Top

1.Obrador S, Soto M, Silvela J. Clinical syndromes of arteriovenous malformations of the transverse-sigmoid sinus. J Neurol Neurosurg Psychiatry 1975;38:436-51.  Back to cited text no. 1
2.Awad IA, Little JR, Akarawi WP, Ahl J. Intracranial dural arteriovenous malformations: Factors predisposing to an aggressive neurological course. J Neurosurg 1990;72:839-50.  Back to cited text no. 2
3.Lasjaunias P, Chiu M, ter Brugge K, Tolia A, Hurth M, Bernstein M. Neurological manifestations of intracranial dural arteriovenous malformations. J Neurosurg 1986;64:724-30.  Back to cited text no. 3
4.Viñuela F, Fox AJ, Pelz DM, Drake CG. Unusual clinical manifestations of dural arteriovenous malformations. J Neurosurg 1986;64:554-8.  Back to cited text no. 4
5.Malik GM, Pearce JE, Ausman JI, Mehta B. Dural arteriovenous malformations and intracranial hemorrhage. Neurosurgery 1984;15:332-9.  Back to cited text no. 5
6.Lee PH, Lee JS, Shin DH, Kim BM, Huh K. Parkinsonism as an initial manifestation of dural arteriovenous fistula. Eur J Neurol 2005;12:403-6.  Back to cited text no. 6
7.Kajitani M, Yagura H, Kawahara M, Hirano M, Ueno S, Fujimoto K, et al. Treatable fluctuating Parkinsonism and dementia in a patient with a dural arteriovenous fistula. Mov Disord 2007;22:437-9.  Back to cited text no. 7
8.van Dijk JM, terBrugge KG, Willinsky RA, Wallace MC. Clinical course of cranial dural arteriovenous fistulas with long-term persistent cortical venous reflux. Stroke 2002;33:1233-6.  Back to cited text no. 8
9.Chaudhary MY, Sachdev VP, Cho SH, Weitzner I Jr, Puljic S, Huang YP. Dural arteriovenous malformation of the major venous sinuses: An acquired lesion. AJNR Am J Neuroradiol 1982;3:13-9.  Back to cited text no. 9
10.Cognard C, Gobin YP, Pierot L, Bailly AL, Houdart E, Casasco A, et al. Cerebral dural arteriovenous fistulas: Clinical and angiographic correlation with a revised classification of venous drainage. Radiology 1995;194:671-80.  Back to cited text no. 10
11.Hurst RW, Bagley LJ, Galetta S, Glosser G, Lieberman AP, Trojanowski J, et al. Dementia resulting from dural arteriovenous fistulas: The pathologic findings of venous hypertensive encephalopathy. AJNR Am J Neuroradiol 1998;19:1267-73.  Back to cited text no. 11
12.Hirono N, Yamadori A, Komiyama M. Dural arteriovenous fistula: A cause of hypoperfusion-induced intellectual impairment. Eur Neurol 1993;33:5-8.  Back to cited text no. 12
13.Dehdashti AR, Matouk CC, Terbrugge K, Wallace MC, Willinsky RA. Clues to dural arteriovenous fistulas in patients with progressive dementia. Can J Neurol Sci 2010;37:532-4.  Back to cited text no. 13
14.De Marco JK, Dillon WP, Halback VV, Tsuruda JS. Dural arteriovenous fistulas: Evaluation with MR imaging. Radiology 1990;175:193-9.  Back to cited text no. 14


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