Cerebral venous sinus thrombosis in a patient with type 1 ... Hosseini.pdf · Cerebral venous sinus thrombosis (CVST) is an uncommon disorder with an estimated annual incidence of
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Case historyA 17-year-old British man of Indianorigin presented with a two-day history of severe generalised head -ache followed by blurred vision andvomiting on the day of admission. Hewas known to have type 1 diabetesmellitus since the age of 11 years. Hehad been treated with a basal bolusinsulin regimen using glargine andNovoRapid. Despite regular inputfrom medical and nursing teams, hisglycaemic control had been deterio-rating (HbA1c 12.7%, 11.8%, 9.8%,and 9.6% at 0, -3, -6 and -12 monthsfrom admission respectively). He had no known diabetic micro- ormacrovascular complications and wasnormotensive. He was a full-time student living with his parents, withno history of thrombophilia in thefamily. He denied any history of smok-ing, using any recreational drugs oralcohol consumption.
On admission, general physicaland systemic examinations werewithin normal limits and there wereno meningism, papilloedema, pyram-idal or cerebellar signs. His blood glucose was 11mmol/L. An initial urinary dipstick showed 2+ ketonesalthough subsequent repeats were allnegative and arterial blood gas analy-sis excluded acidosis – serum pH 7.38(7.35–7.45), calculated osmolality305mmol/kg, base excess -1.4mEq/L(±2mEq/L) and serum bicarbonate23mmol/L (21–28mmol/L). Therest of his routine haematologicaland biochemical profiles were withinnormal limits. An urgent CT scan of
his brain (Figure 1a) raised the suspicion of a dural venous sinusthrombosis. A subsequent magneticresonant venogram confirmed thediagnosis of sagittal sinus and lefttransverse sinus thrombosis (Figure1b). Anticoagulation was initiatedwith low molecular weight heparin,and maintained with warfarin. Whilein hospital he developed diplopia secondary to right 6th nerve palsythat was considered to be a false local-ising sign. The symptoms respondedwell to treatment and his neuro -logical signs improved prior to dis-charge from hospital. Investigationsfor underlying thrombophilic state,myeloproliferative disorders, auto -immune conditions and haemoglo-binopathy were all negative.
DiscussionCerebral venous sinus thrombosis(CVST) is an uncommon disorderwith an estimated annual incidenceof three to four cases per million.1Most patients are young adults orchildren and clinical presentation
is variable but often dramatic.Headache is the presenting symp-tom in 70–90% of cases, whileseizures, focal deficits, cranial nervesyndromes involving IX, X, and XI(jugular foramen syndrome) or III,IV and VI cranial nerve palsies,impairment of consciousness, visualdisturbances and papilloedemaoccur in 25–75% of cases.2,3Predisposing factors to CVST canonly be identified in 65–80% ofpatients, with more than one factoroften found in individual patients.4These include local and systemicconditions predisposing to venousthrombosis including pregnancy,puerperium, oral contraceptive pills,intracranial pathologies, malignan-cies, connective tissue and inflam-matory diseases, haematologicalconditions and drugs.2 As a generalrule, Virchow’s triad, as first formu-lated (venous stasis, vessel wallinjury, acquired or genetic changesin composition of blood), is still theprimary mechanism for the develop-ment of venous thrombosis.4
Cerebral venous sinus thrombosis in a patient with type 1 diabetes in theabsence of ketoacidosisAA Hosseini, RS Manjunatha, A Jacob, M El- Lahaw i, V Baskar*
Akram A Hosseini, MD, MRCPMohammed El-Lahawi, MBBS, MRCPNeurology Department, New CrossHospital, Wolverhampton, UKRashmi S Manjunatha, MBBS, MRCPVaradarajan Baskar, MD, MRCPWolverhampton Diabetes Centre, New
Cross Hospital, Wolverhampton, UKAbraham Jacob, FRCP, FRCPath,Haematology Department, New CrossHospital, Wolverhampton, UK
*Correspondence to: Dr VaradarajanBaskar, Wolverhampton Diabetes Centre,
New Cross Hospital, Wolverhampton WV100QP, UK; e-mail: [email protected]
Received: 28 April 2009Accepted in revised form: 2 July 2009
CASE REPORT
Cerebral venous thrombosis in type 1 diabetes without ketoacidosis
Diabetes remains a controversialrisk factor for venous thrombo -embolism. While diabetes itself is associated with several defects of platelet function, coagulation and fibrinolytic systems resulting in a hypercoagulable state,1,5 theincreased rate of thrombotic compli-cations observed is often in the pres-ence of additional risk factors.6 It isevident that the rate of thromboticcomplications in diabetic patientswith frequent episodes of hyper -ketonaemia and hyperosmolarity isincreased.1,6,7 Published literatureon CVST and diabetes is limited tocase reports predominantly amongpatients with type 1 diabetes and isin the context of diabetic ketoacido-sis (DKA), dehydration or hyper -osmolarity.1,7,8 In association withtype 2 diabetes, two cases of CVSTare reported: one presenting withDKA while the other patient hadtransverse sinus aplasia.9,10 To ourknowledge, this is the first report ofa CVST in association with type 1diabetes but in the absence of con-comitant ketoacidosis or any knownprothrombotic conditions.
The optimum treatment forCVST and its duration haveremained uncertain, especiallywhere there is no identifiable aetiol-ogy.11 Based upon limited available
evidence, anticoagulation appears tobe safe and probably reduces therisk of death or dependency.12Treatment with systemic or localthrombolysis has been reported incases with a deteriorating clinicalcourse despite anticoagulation therapy. There are, however, no randomised controlled trial data tosupport efficacy or safety of throm-bolysis in CVST.12 In the presence ofa transient risk factor, anticoagula-tion is generally recommended forthree to six months. Patients withidiopathic CVST or those withhereditary thrombophilia are anti-coagulated for longer periods giventheir higher risk of recurrence.13 Inan international study that was performed in 624 patients withCVST, the cumulative risk for recur-rent CVST or other thromboticevents was 6.5%, with over half notundergoing anticoagulation at thetime of their recurrence.14
While our case might be consid-ered among the 20–35% of CVSTcases with no identifiable cause,4 wespeculate whether long-standinghyperglycaemia may have played arole in increasing our patient’sthrombotic risk, and highlight apotential direct association betweenCVST and hyperglycaemic state evenin the absence of dehydration or
ketoacidosis. If such a relationshipbetween diabetes and CVST were tobe found, new prognostic measuresmight become necessary and thismay also have an implication on therisk of recurrence and duration ofanti coagulation in this group ofpatients.
Our case highlights the need forclinicians to consider CVST amongpatients with diabetes even in theabsence of ketoacidosis or hyper -osmolarity.
Conflict of interest statementThere are no conflicts of interest.
ReferencesReferences are available at www.practicaldiabetesinternational.com.
Figure 1. (A) CT scan of the brain suggested the possibility of a dural sinus thrombosis. (B) Magnetic resonancevenogram confirmed the diagnosis of sagittal sinus and left transverse sinus thrombosis
Key points• The risk of thrombosis in thevenous tree in diabetes is lesswell studied
• Cerebral venous sinusthrombosis (CVST) is arecognised but rare complicationof diabetic ketoacidosis
• Our case illustrates the need toconsider CVST in patients withpoorly controlled diabetes evenin the absence of ketoacidosis orhyperosmolarity
a b
CASE REPORT
Cerebral venous thrombosis in type 1 diabetes without ketoacidosis
4. de Freitas GR, Bogousslavsky J. Riskfactors of cerebral vein and sinusthrombosis. Front Neurol Neurosci2008; 23: 23–54.
5. Jones EW, Mitchell JRA. Venousthrombosis in diabetes mellitus.Diabetologia 1983; 25: 502–505.
6. Keenan CR, Murin S, White RH.High risk for venous thromboem-bolism in diabetics with hyperosmo-lar state: comparison with other acute
medical illnesses. J Thromb Haemost2007; 5: 1185–1190.
7. Kalis NN, van der Merwe PL,Schoeman JF, et al. Cerebral oedemawith coning in diabetic ketoacidosis.Report of 2 survivors. S Afr Med J1991; 79: 727–731.