Journal of Clinical Neuroscience xxx (2014) xxx–xxx

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Case Report

Rapidly progressive cognitive impairment in a patient with high flow dural arteriovenous fistulas, cerebral sinus thrombosis and protein S deficiency M. Pasi a, S. Nappini b, E. Salvadori a, S. Mangiafico b, N. Limbucci b, L. Pantoni c,⇑ a b c

NEUROFARBA Department, Neuroscience Section, University of Florence, Italy Interventional Neuroradiology Unit, Azienda Ospedaliero Universitaria Careggi, Florence, Italy Stroke Unit and Neurology, Azienda Ospedaliero Universitara Careggi, Largo Brambilla 3, 50134 Firenze, Italy

a r t i c l e

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Article history: Received 26 October 2013 Accepted 19 December 2013 Available online xxxx Keywords: Angiographic procedure Cognitive impairment Dural arteriovenous fistula Neuropsychological evaluation Vascular dementia

a b s t r a c t Dural arteriovenous fistula (DAVF) may present with a variety of neurological symptoms, ranging from tinnitus to fatal hemorrhage. We report a case of rapidly progressive cognitive impairment due to cerebral venous engorgement that reversed after endovascular treatment in a patient with DAVF, cerebral sinus thrombosis and protein S deficiency. DAVF may be a cause of vascular cognitive impairment and should be considered particularly in cases with a rapidly progressive course because they are potentially treatable. Ó 2014 Elsevier Ltd. All rights reserved.

1. Introduction Dural arteriovenous fistulas (DAVF) may present with a variety of neurological symptoms, depending on localization, size and clinical stage, ranging from tinnitus to fatal hemorrhage. We report a case of DAVF presenting as rapidly progressive cognitive impairment and acute confusional state.

2. Case report A 71-year-old man with a history of deep vein thrombosis and pulmonary embolism was admitted to our department because of rapidly progressive memory deficit, difficulties in writing and acute mental confusion. Cognitive disturbances had started some weeks before. Brain CT scan revealed left transverse sinus occlusion, left hemispheric venous engorgement and an occipital hemorrhagic lesion with faint contrast enhancement, raising the suspicion of a venous infarction (Fig. 1E). A cerebral MRI performed a few days later showed extensive hyperintense signal abnormality on T2weighted, fluid attenuated inversion recovery (FLAIR) and diffusion weighted imaging (DWI) sequences extending through the whole left hemisphere white matter, and confirmed thrombosis of the left ⇑ Corresponding author. Tel.: +39 055 7945 519; fax: +39 055 4298 461. E-mail address: [email protected]fi.it (L. Pantoni).

transverse, torcular and superior sagittal sinuses on venous MR angiogram (Fig. 1F, G). Digital subtraction cerebral angiography (DSA) revealed occlusion of the deep venous draining system (internal cerebral veins, vein of Galen, straight sinus), of the left transverse sinus and of the torcular, arterialization and flow inversion of the sagittal superior sinus, and bi-hemispheric venous engorgement. This was supported by three DAVF: two high flow Cognard type IIa + IIb fistulas, one on the left transverse sinus, fed by branches of the middle meningeal artery, and one on the torcular, fed by branches of the posterior occipital artery; and one low flow type IIa fistula on the sagittal superior sinus, supplied by the left middle meningeal artery (Fig. 1A, C). A neuropsychological evaluation revealed a marked impairment in all the investigated domains (Table 1). Looking for possible causes of hypercoagulability, we found a protein S deficiency confirmed by a chromogenic assay. Endovascular treatment was planned and the two fistulas were treated with embolization with Onyx 18 (ev3 Endovascular, Plymouth, MN, USA) in two steps, obtaining the complete exclusion of the left transverse sinus fistula and a 90% flow reduction of the torcular fistula. The patient was treated with intravenous heparin and oral coagulation therapy was started at discharge. After the procedures the patient showed progressive clinical improvement and the neuropsychological examination performed 3 months after discharge showed a significant improvement in cognitive performance (Table 1). Furthermore, cerebral

http://dx.doi.org/10.1016/j.jocn.2013.12.025 0967-5868/Ó 2014 Elsevier Ltd. All rights reserved.

Please cite this article in press as: Pasi M et al. Rapidly progressive cognitive impairment in a patient with high flow dural arteriovenous fistulas, cerebral sinus thrombosis and protein S deficiency. J Clin Neurosci (2014), http://dx.doi.org/10.1016/j.jocn.2013.12.025

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Case Report / Journal of Clinical Neuroscience xxx (2014) xxx–xxx

Fig. 1. Pre-treatment digital subtraction angiography (DSA) showing (A) Cognard type IIa + IIb fistula on the left transverse sinus (black arrow) fed by branches of the right middle meningeal artery (arrow head) and right occipital artery (arrow) and (C) Cognard type IIa + IIb fistula on the torcular sinus (arrow head) fed by branches of right occipital artery (arrow) and by the neuromeningeal branch of the right ascending pharingeal artery (black arrow). Post-treatment DSA showing the obliteration of the left transverse sinus fistula (B) and the partial obliteration of the torcular fistula (D). Pre-treatment cerebral axial CT scan with contrast showing hypodensity in the left occipital lobe close to the recent hemorrhage and diffuse venous dilatation (E). Pre-treatment axial cerebral fluid attenuated inversion recovery (FLAIR) MRI showing swelling and white matter hyperintensity of the left hemisphere (G). MR venography showing extensive cerebral venous thrombosis (F). Axial post-treatment FLAIR MRI showing no residual hyperintensity and substantial regression of the left occipital lesion at 6 months after discharge (H).

Table 1 Neuropsychological evaluation of the patient at presentation and follow-up Cognitive domain

Test

Global functioning Memory

Mini Mental Status Evaluation+ [11] Rey Auditory Verbal Learning + [12]

Psychomotor speed Executive functions

Language

Trail Making Test A (time to complete)# [13] Trail Making Test B (time to complete)# [13] Stroop test (time to complete)# [14] Symbol digit test (correct answers)+ [16] Digit cancellation test+ [15] Verbal fluency (words in 3 minutes)+ [17]

Visuo-spatial

Clock Drawing test+ [18]

Attention

Short term Long term

Phonemic Semantic

Presentation 4/05/2012

3 month follow-up 14/09/2012

6 month follow-up 5/02/2013

18/30* 17/75* 0/15* 277* 300* 140* 6* 16/50* 8* 13* 4/10*

27/30 28/75 5/15 56 300* 43° 30 38/50 21 28 9.5/10

28/30 28/75 7/15 50 201 35 31 39/50 12° 26 8/10

In this table raw scores are shown. To determine cognitive performance, raw scores have been adjusted for age and education according to correction norms based on Italian adult samples. Abnormal performance: an adjusted score below the outer confidence limit for the 5th centile of the normal population. ° Borderline performance: an adjusted score between the outer and inner confidence limits for the 5th centile of the normal population. + Higher scores correspond to a better performance. # Lower scores correspond to a better performance. *

MRI showed a reduction of the extensive hyperintense area on T2-weighted, FLAIR and DWI sequences in the left hemisphere. Six months after discharge, the patient was asymptomatic and a third neuropsychological examination revealed normal cognitive performance in all the investigated domains except for a slight impairment in phonemic fluency (Table 1). In the same period, MRI showed a complete regression of the extensive hyperintensity on T2-weighted, FLAIR and DWI sequences; follow-up DSA revealed the reduction of venous engorgement with a progressive normalization of the angiographic phases (Fig. 1B, D, H).

3. Discussion High flow DAVF may be a cause of vascular cognitive impairment due to cerebral venous congestion and should be considered particularly in patients with rapidly progressive cognitive impairment because they are potentially treatable. The frequency of cognitive disorders in patients with DAVF is overall quite low but the reported rates are discordant. For example, in a series of 92 patients with transverse-sigmoid sinus DAVF, Obrador and coworkers found 11 patients with mental deterioration and the same percentage

Please cite this article in press as: Pasi M et al. Rapidly progressive cognitive impairment in a patient with high flow dural arteriovenous fistulas, cerebral sinus thrombosis and protein S deficiency. J Clin Neurosci (2014), http://dx.doi.org/10.1016/j.jocn.2013.12.025

Case Report / Journal of Clinical Neuroscience xxx (2014) xxx–xxx

(5/40 patients) was reported by Hurst and collaborators [1,2]. Ishii et al. found 10 patients with cognitive impairment in a series of 45 patients with transverse sigmoid-sinus DAVF [3]. Finally, in a more recent study reviewing 53 patients with DAVF in any intracranial location Kim et al. found dementia in just one patient with an arteriovenous shunt located in the transverse-sigmoid sinus [4]. The combination of high flow arteriovenous shunt and the obstruction of intracranial venous outflow has been reported to cause impairment of cerebral venous drainage [2]. In our patient, cerebral angiography revealed the presence of both high flow (through two large DAVF) and obstruction to intracranial venous outflow (by sinus thrombosis). The result was most likely venous hypertension causing widespread ischemia and progressive damage of brain parenchyma. Moreover, widespread venous hypertension has generated retrograde flow both in the cerebral sinuses and cortical veins, a major risk factor for intracranial bleeding. Our patient displayed a marked clinical improvement after the embolization procedure. This observation is in line with previous clinical series and case reports, but the reversal of cognitive impairment after DAVF treatment is not assured in all patients [5]. A Japanese study showed that in patients with cognitive impairment due to DAVF, the preservation of vasoreactivity on single photon emission computed tomography (SPECT) after acetazolamide challenge indicated that cognitive impairment may be reversible by DAVF treatment [5]. On the other hand, the loss of vasoreactivity indicated that cognitive impairment could not be improved by treatment with embolization or neurosurgical procedures. Unfortunately, our patient did not undergo a SPECT study to evaluate this hypothesis. Our patient showed left hemisphere diffuse white matter hyperintensity on T2-weighted, FLAIR and DWI cerebral MRI. This feature has been related to widespread venous congestion and consequently to chronic brain ischemia [6]. In line with previously reported cases, after the endovascular procedures our patient displayed progressive regression of the white matter hyperintensity that disappeared on his 6 month follow-up cerebral MRI [7,8]. In looking for possible causes of hypercoagulability, we found a protein S deficiency confirmed by a chromogenic assay. Indeed, heritable risk factors for venous thrombosis, such as antithrombin, protein C, and protein S deficiencies, have been associated with DAVF occurrence [9,10]. In conclusion, mental deterioration represents a rare but devastating consequence of intracranial high flow DAVF; successful treatment of the fistulas can result in significant improvement in the clinical status of these patients and in regression of white matter changes seen on cerebral MRI.

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Conflicts of Interest/Disclosures The authors declare that they have no financial or other conflicts of interest in relation to this research and its publication. References [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. [2] Hurst RW, Bagley LJ, Galetta S, et al. Dementia resulting from dural arteriovenous fistulas: the pathologic findings of venous hypertensive encephalopathy. AJNR Am J Neuroradiol 1998;19:1267–73. [3] Ishii K, Goto K, Ihara K, et al. High-risk dural arteriovenous fistulae of the transverse and sigmoid sinuses. AJNR Am J Neuroradiol 1987;8:1113–20. [4] Kim MS, Han DH, Kwon OK, et al. Clinical characteristics of dural arteriovenous fistula. J Clin Neurosci 2002;9:147–55. [5] Kai Y, Ito K, Kinjo T, et al. Reversibility of cognitive disorder after treatment of dural arteriovenous fistulae. Neuroradiology 2009;51:731–9. [6] Willinsky R, Terbrugge K, Montanera W, et al. Venous congestion: an MR finding in dural arteriovenous malformations with cortical venous drainage. AJNR Am J Neuroradiol 1994;15:1501–7. [7] Dabus G, Bernstein RA, Hurley MC, et al. Reversal of diffusion restriction after embolization of dural arteriovenous fistula: case report. Neurosurgery 2010;67:E1147–51. [8] Men S, Idiman F, Gulcu A, et al. Reversible cytotoxic edema associated with dural arteriovenous fistula: a case report. Eur J Radiol Extra 2004;52:1–5. [9] Gandhi D, Chen J, Pearl M, et al. Intracranial dural arteriovenous fistulas: classification, imaging findings, and treatment. AJNR Am J Neuroradiol 2012;33:1007–13. [10] Fujita A, Kuwamura K, Saitoh M, et al. Cerebral sinus thrombosis in a patient with protein S deficiency: a case report. No Shinkei Geka 1997;25: 467–72. [11] Folstein MF, Folstein SE, McHugh PR. ‘‘Mini-mental state’’. A practical method for grading the cognitive state of patients for the clinician. J Psychiatr Res 1975;12:189–98. [12] Carlesimo GA, Caltagirone C, Gainotti G. The mental deterioration battery: normative data, diagnostic reliability and qualitative analyses of cognitive impairment. The group for the standardization of the mental deterioration battery. Eur Neurol 1996;36:378–84. [13] Giovagnoli AR, Del Pesce M, Mascheroni S, et al. Trail making test: normative values from 287 normal adult controls. Ital J Neurol Sci 1996;17:305–9. [14] Caffarra P, Vezzadini G, Dieci F, et al. Una versione abbreviata del test di Stroop. Dati normativi nella popolazione italiana. Nuova Riv Neurol 2002;12:111–5. [15] Spinnler H, Tognoni G. Standardizzazione e taratura italiana di test neuropsicologici. Ital J Neurol Sci 1987;8:1–120. [16] Nocentini U, Giordano A, Di Vincenzo S, et al. The symbol digit modalities test – oral version: Italian normative data. Funct Neurol 2006;21:93–6. [17] Novelli G, Papagno C, Capitani E, et al. Tre test clinici di ricerca e produzione lessicale. Taratura su soggetti normali. Arch Psicol Neurol Psichiatr 1986;47: 477–506. [18] Manos PJ, Wu R. The ten point clock test: a quick screen and grading method for cognitive impairment in medical and surgical patients. Int J Psychiatry Med 1994;24:229–44.

Please cite this article in press as: Pasi M et al. Rapidly progressive cognitive impairment in a patient with high flow dural arteriovenous fistulas, cerebral sinus thrombosis and protein S deficiency. J Clin Neurosci (2014), http://dx.doi.org/10.1016/j.jocn.2013.12.025

Rapidly progressive cognitive impairment in a patient with high flow dural arteriovenous fistulas, cerebral sinus thrombosis and protein S deficiency.

Dural arteriovenous fistula (DAVF) may present with a variety of neurological symptoms, ranging from tinnitus to fatal hemorrhage. We report a case of...
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