The levels of evidence (class I-IV) supporting the recommendations and ratings of recommendations (A-C, Good practice point [GPP]) are defined at the end of the "Major Recommendations" field.
Heparin Therapy
Current evidence shows that patients with cerebral venous and sinus thrombosis (CVST) without contraindications for anticoagulation (AC) should be treated either with body weight-adjusted subcutaneous low-molecular-weight heparin (LMWH) (180 anti-factor Xa U/kg/24 hour administered by two subcutaneous injections daily) or dose-adjusted intravenous heparin with an at least doubled activated partial thromboplastin time. Concomitant intracranial haemorrhage (ICH) related to CVST is not a contraindication for heparin therapy. For the reasons mentioned above, LMWH should be preferred in uncomplicated CVST cases. (Good practice point [GPP])
Thrombolysis
There is insufficient evidence to support the use of either systemic or local thrombolysis in patients with CVST. If patients deteriorate despite adequate AC and other causes of deterioration have been ruled out, thrombolysis may be a therapeutic option in selected cases, possibly in those without intracranial haemorrhage (ICH). The optimal substance (urokinase or recombinant tissue plasminogen activator [rtPA]), dosage, route (systemic or local), or method of administration (repeated bolus or bolus plus infusion) are not known (GPP).
Oral Anticoagulation
There are insufficient data about the optimal duration of oral AC in patients with CVST. Analogous to patients with a first episode of extracerebral venous thrombosis, oral AC may be given for 3 months if CVST was secondary to a transient risk factor, for 6 to 12 months in patients with idiopathic CVST and in those with "mild" hereditary thrombophilia. Indefinite AC should be considered in patients with two or more episodes of CVST and in those with one episode of CVST and "severe" hereditary thrombophilia (GPP).
Symptomatic Treatment
Control of Seizures
Prophylactic antiepileptic therapy may be a therapeutic option in patients with focal neurological deficits and focal parenchymal lesions on admission computed tomography/magnetic resonance imaging (CT/MRI). The optimal duration of treatment for patients with seizures is unclear (GPP).
Treatment of Elevated Intracranial Pressure
In patients with isolated intracranial hypertension (IIH) and threatened vision, possible therapeutic measures may include one or more lumbar punctures, acetazolamide and incidentally CSF-shunting procedures. There are no controlled data about the risks and benefits of certain therapeutic measures (e.g. steroids and decompressive surgery) to reduce an elevated intracranial pressure (with brain displacement) in patients with CVST. Antioedema treatment should be carried out according to general principles of therapy of raised intracranial pressure. In a very small subgroup of patients who deteriorate especially in the presence of large intracerebral haemorrhages, decompressive craniectomy might be an alternative treatment option in the future. Now, this therapy needs further investigation and should be regarded as experimental (GPP).
Definitions
Evidence Classification Scheme for a Therapeutic Intervention
Class I: An adequately powered prospective, randomized, controlled clinical trial with masked outcome assessment in a representative population or an adequately powered systematic review of prospective randomized controlled clinical trials with masked outcome assessment in representative populations. The following are required:
- Randomization concealment
- Primary outcome(s) is/are clearly defined
- Exclusion/inclusion criteria are clearly defined
- Adequate accounting for dropouts and crossovers with numbers sufficiently low to have minimal potential for bias
- Relevant baseline characteristics are presented and substantially equivalent among treatment groups or there is appropriate statistical adjustment for differences
Class II: Prospective matched-group cohort study in a representative population with masked outcome assessment that meets a–e above or a randomized, controlled trial in a representative population that lacks one criteria a–e
Class III: All other controlled trials (including well-defined natural history controls or patients serving as own controls) in a representative population, where outcome assessment is independent of patient treatment
Class IV: Evidence from uncontrolled studies, case series, case reports, or expert opinion
Rating of Recommendations
Level A rating (established as effective, ineffective, or harmful) requires at least one convincing class I study or at least two consistent, convincing class II studies.
Level B rating (probably effective, ineffective, or harmful) requires at least one convincing class II study or overwhelming class III evidence.
Level C rating (possibly effective, ineffective, or harmful) requires at least two convincing class III studies.
Good practice point (GPP) Where there was lack of evidence but consensus was clear the Task Force members have stated their opinion as good practice points.
