**4. Conclusions**

154 Venous Thrombosis – Principles and Practice

imaging and the cerebral angiography has made possible the early diagnoses and has changed the knowledge about this condition (Bousser et al., 2007; Breteau et al.,2003; Preter et al., 1996). The increase of the data about it in the scientific literature enlarges the aspects studied and poses new questions. One of these questions is related to the functional outcome after CVST and the impact of cognitive impairments and behavioural changes on it. Due to the heterogeneity of study methodology and design, it is very difficult to compare the obtained results. Another important obstacle is due to the differences in the diagnostic process, in the extent of control for the underlying causes for CVST, for the clinical

Most of the authors reported good functional outcome after CVST (Cakmak et al.,2003; de Bruijn et al., 2001; Ferro et al., 2001; Ferro et al., 2002; Hameed et al.,2006; Kirmani et al., 2005). In a 77 months follow-up 86% of the patients recovered without neurological sequelae (Preter et al., 1996). The conclusions from the largest, for the time being, international study - ISCVST (Ferro et al., 2004) and from a large systematic review of 19 papers on CVST (Dentali et al., 2006) were for a better prognosis than reported previously. Most of the patients examined by Stolz et al. (2004) showed significant improvement on hospital discharge and 89% of them had significant functional improvement 12 months after the discharge. A review of long-term follow-up studies found that from 1943 patients examined, only 180 had poor recovery with permanent neurological deficit (Dentali et al.,2006). At the same time Bender et al. (2006) found that the severe CVST cases had not been studied in details and that cognitive impairments could influence negatively the quality of life of survived patients. De Bruijn et al. (2000) reported less favorable CVST outcome than

CVST is a condition that requires a prompt diagnose and treatment. The mode of onset of CVST is acute in 37,2% and sub-acute in 55,5% of the participants in ISCVST. Probably this is the reason for the lack of data about the cognitive functioning of the patients. But it is difficult to explain the absence of neuropsychological techniques from the set of methods, assessing the long-term recovery of surviving patients. Most of the outcome studies of CVST do not include testing of cognition and assessment of behavioural changes (Breteau et al., 2003; Cakmak et

The assessment of the extent of functional recovery after CVST is limited. The outcome after CVST is traditionally described by modified Rankin Scale score, that gives information only about the patient motor activity and the capacity for carrying out previous activities. Some

Abulia, executive deficits, and amnesia may result from thrombosis of the deep venous system, with bilateral panthalamic infarcts. Memory deficits, behavioral problems, or executive deficits may persist. The importance of more detailed examination and neuropsychological testing could be confirmed by a paper, reporting the results of functionally independent patients, having long-term symptoms with negative impact on their everyday functioning: 75% of patients, included in this 63 months follow-up, reported

Aphasia was a clinical feature of CVST, found in 19% of the participants from 21 countries in ISCVST study (Ferro et al., 2004). Aphasia, in general of the fluent type, results from left lateral sinus thrombosis with temporal infarct or hemorrhage. Recovery is usually favorable, but minor troubles in spontaneous speech and naming might persist. Cognitive assessment for aphasia, apraxia and working memory found aphasia in 3 patients and working memory deficit in 6 out of 34 patients in a follow-up study (Buccino et al., 2003). Preter et al. (1996)

al.,2003; de Bruijn et al., 2001; Ferro et al., 2001; Ferro et al., 2002; Stolz et al., 2004).

of the studies report data received by mail or by phone.

concentration impairments (Koopman et al.,2009).

presentations and the treatment strategy.

reported previously.

The main findings of this review were that all age groups can be affected by CVST and the large sinuses such as the superior sagittal sinus are most frequently involved. Extensive collateral circulation within the cerebral venous system allows for a significant degree of compensation in the early stages of thrombus formation. Systemic inflammatory diseases and inherited as well as acquired coagulation disorders are frequent causes, although in up to 30% of cases no underlying cause can be identified. The diagnosis is usually made by venographic studies with computer tomography (CT or CTV) or magnetic resonance imaging (MRI or MRIV) to demonstrate obstruction of the venous sinuses or cerebral veins by a thrombus. The MRI with venography (MRIV) is the investigation of choice; computed tomography alone will miss a significant number of cases. Additionally, the use of such precise neuro-imaging techniques gives the possibility for early diagnosis and treatment of emerging neuropsychological impairments in the set of the clinical symptoms in CVST; for instance, a cognitive deficit as part of the long-term outcome; a range of psychiatric complications and, last but not least, to collect data and explore the various aspects of the health-related quality of life or HRQOL) in such patients. It should be noted that the neuropsychological impairments that could be present during CVST are not well known, including various aspects of the assessment of the eventually impaired mental status. For instance, various data exist about disorientation, lack of coordination, incapacity to follow commands, neglect, apraxia, recent memory impairments and language impairments, among others. In the same time, very little is known about the long term outcome of CVST – for instance, it has been observed that many patients experience some persistent neurologic and cognitive deficits, but data are scarce and difficult to collect and summarise. The management of CVST includes treatment of the underlying condition; symptomatic treatment; the prevention or treatment of complications of increased intracranial pressure, ICH, or venous infarction; and typically, anticoagulation therapy. It has now been conclusively shown that intravenous heparin is the first-line treatment for cerebral venous sinus thrombosis because of its efficacy, safety and feasibility. Local thrombolysis may be indicated in cases of deterioration, despite adequate heparinisation. This should be followed by oral anticoagulation for 3-6 months. The prognosis of cerebral venous sinus thrombosis is

Cerebral Venous Sinus Thrombosis - Diagnostic Strategies and Prognostic Models: A Review 157

Ferro JM, Lopes MG, Rosas MJ *et al.* (2002). Long-term prognosis of cerebral vein and dural

Ferro, J. M., Canhao, P. (2011). Etiology, clinical features, and diagnosis of cerebral venous

Ferro, J. M., Canhao, P., Stam, J., Bousser, M.G., Barinagarrementeria, F.; ISCVST

Hameed B, Syed NA. (2006). Prognostic indicators in cerebral venous sinus thrombosis. *J Pak* 

Kirmani J , Janhua N, Kawi A et al. (2005). Therapeutic advances in interventional

Knopp, E. A. (1995). Venous disease and tumors. *Magn Reson Imaging Clin N Am* 3(3): 509-

Koopman K, Uyttenboogaart M, Vroomen PC et al. (2009). Long-term sequelae after cerebral venous thrombosis in functionally independent patients. *J Stroke* 18(3): 198-202. Koopman, K., Uyttenboogaart, M., Vroomen, P. C., van der Meer, J., De Keyser, J., Luijckx,

Kwan J, Günther A. (2006). Antiepileptic drugs for the primary and secondary prevention of

Leach, J. L., K. Meyer, et al. (2008). Large arachnoid granulations involving the dorsal

Manolidis, S., Kutz, J.W. Jr. (2005). Diagnosis and management of lateral sinus thrombosis.

Masuhr, F., Mehraein, S., Einhäupl, K. (2004). Cerebral venous and sinus thrombosis. *J* 

McGinn TG, Guyatt GH,Wyer PC, et al. (2000). Users' guides to the medical literature: XXII:

McNally M, et al. (2010). Validity of British Thoracic Society guidance (the CRB-65 rule) for

Plata R, Cornejo A, Arratia C, Perna A, Dimitrov BD, Remuzzi G, Ruggenenti P. (2002). Effects of ACE inhibition therapy in altitude polycytemia. *Lancet* 359: 663-666. Preter M, Tzourio C, Ameri A et al. (1996). Long-term prognosis in cerebral venous

Saposnik G, Barinagarrementeria F, Brown RD Jr, et al.; and the American Heart Association

G. J. (2009) Development and validation of a predictive outcome score of cerebral

seizures after intracranial venous thrombosis. *Cochrane Database of Systematic* 

superior sagittal sinus: findings on MR imaging and MR venography. *AJNR Am J* 

how to use articles about clinical decision rules. Evidence-Based MedicineWorking

predicting the severity of pneumonia in general practice: systematic review and

Stroke Council and the Council on Epidemiology and Prevention. (2011). Diagnosis and management of cerebral venous thrombosis: a statement for healthcare

diagnosis-of-cerebral-venous-thrombosis#H1)

278.

528.

*Stroke* 35: 664–670.

*Med Assoc* 56(11): 551-553.

neurology. *NeuroRx* 2(2): 304–323.

venous thrombosis. *J Neurol Sci* 276: 66–68.

*Reviews* Issue 3. Art.No.: CD005501.

*Neuroradiol* 29(7): 1335-1339.

*Otol Neurotol* 26:1045–1051.

Group. *JAMA* 284(1): 79–84.

meta-analysis. *Br J Gen Pract* 60(579): e423-433.

thrombosis. Follow-up of 77 patients. *Stroke* 27: 243–246.

*Neurol* 251 : 11–23

sinus thrombosis. Results of the VENOPORT Study. *Cerebrovasc Dis*; *13* (4) : 272-

thrombosis. UpToDate. Ver.19.2 (last updated on June 6, 2011, last accessed on July 12, 2011 at http://www.uptodate.com/contents/etiology-clinical-features-and-

Investigators. (2004). Prognosis of cerebral vein and dural sinus thrombosis: results of the International Study on Cerebral Vein and Dural Sinus Thrombosis (ISCVST).

generally favourable. A high index of clinical suspicion is needed to diagnose this uncommon condition so that appropriate treatment can be initiated. Complications, in a short- and a long-term, include but are not limited to cognition and behavioural changes.
