Cerebral venous thrombosis in ulcerative colitis and review of the … · 2019. 8. 1. · (long...
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BRIEF COMMUNI CATION Cerebral venous thrombosis in ulcerative colitis and review of the literature Hurn I CJ IAUN, BM, FRCP, FRCPC, JEFF H BECKMAN, MD, FRCPC, TERENCF G SPARLIN<.,, MD, FRCPC H CHAUN, JH BECKMAN, TG SPARLING. Cerebral venous thrombosis in ulcerative colitis and review of the literature. Can J Gastroenterol 1991 ;5(4 ): 129- l32. A JO-year-o ld man with a n e ighl year his tory of u lcera ti ve colitis developed left occipital h ead ach e, mental confu sion, dysphasia and righr-sided weakness when his bowel di sease was asy mptomati c. Invest i gatio ns revealed thwmbos is of the ce rebral sag itta l s inus and left transverse s inu s. The literatu re relating to cereb rovascu lar co mplica tions associated with ulcerative co litis is reviewed, and th e possible pathogenetic mech anisms of venou s thro mbosis in ulcerative co lit is are di scussed. The importance of recogn izing that venous thrombosis may occur in association with ulcera tive co litis in remission is emphasized. Key Words: Cere bral venous thrombosis, R emiss i on phase, Ulcerative c olitis , Vas- cular complicati ons Thrombose veineuse cerebrale et colite ulcereuse - Observation et tour d'horizon de la litterature RESUME: Cep hal ee occip itale, confu sion mentalc, dysph asic er affaiblisseme nt du cote <lro it ont etc notes chez un patient age de 30 ans et atteint depuis huit ans d'une colite ulcereuse al ors asymptomatique. Les exa mcns o nt revc le unc thrombose du sinus sag ittal ct du s inu s lat era l ga uche. Le~ auteurs effectue nt un tour d'horizon de la litte rature traitan t des compli cat io n s ce rebrovasc ulaires associees a la co lite ulcere use. lls examinent les mecanismes pathogc niqu es possibles <le la thrombose veineuse dans ces ci rconstances et so ulignent qu 'il est important de reconnattre qu'elle pe ut surve nir ch cz la personn c attei nte d'.me colite ulcereuse en re mission. Di1Jision.s of Gastrocmernl oRJ , Neurology and I l ematol oRY, Departme ni of Medicrne, Uniuersity HosJ>ital; a nd Universiiy of British Colwnb1a, Va nco1.wer, Bmi sh Columbia Correspondence and reprims : Dr Hugh Chaun, Suite 60 1, 805 Wesi Broadway, Vancouver , B riiish Columbia V5Z 1 KI. Telephone ( 604) 872 - 07 17 R ecei 1Jed for Jmhlication Apri l 25, 1991. Acce/ >ted June 3, 199 / CAN J GAS TROENTEROL VOL 5 Nu 4 J UL Y/A Um JST 1991 T IIE ASS <.X IATION OF VASCULAR l e~ ions with 1J iopathic inflam- matory bowel disease (IBD) is we ll recognized (I). Vascul ar co mplica tions as~ociatcd wit h ulcc rau vc co li tis in- clude veno us th ro mbosis (2 -4), arteria l th rombosis (2,5), Takayasu's disease (6) , bchem ic skin les ions (7), a nd vas- culitis (8,9). Bargen a nd Barker in l 936 (2) first drew at ten ti on to thP serious imp li cnrions of th rombocmbo li sm co mpli cat ing ulcerat ive co li ti s. Si nce Ha rrison and Truelove (10) first report- ed cere bral veno us thro mbosis in two ulcerative co litb patie nts, th ere hav e been o ther we ll doc umented rep orts of the association of ce rebrovascular dis- ease with ulcerative colitis; the majo r- ity h ave occ urred wi th act ive bowel di sease. Thi s report desc ri bes a patient w ith ul cerative co litis in remissi on who dev e lo ped ce rebra l sagittal s inus a nd left transverse sinus thrombosis. CASE PRESENTATION A 30-year -o l<l accountant, a n on- smoker, was transferred from another hospital on Dece mber 6, 1990 for eva luation of ce rebra l h emorrhag ic in - farction. The patient had a history of ulcerative co litis sin ce 1982, wh en he 129
Transcript of Cerebral venous thrombosis in ulcerative colitis and review of the … · 2019. 8. 1. · (long...
BRIEF COMMUNICATION
Cerebral venous thrombosis in ulcerative colitis and review
of the literature
Hurn I CJ IAUN, BM, FRCP, FRCPC, JEFF H BECKMAN, MD, FRCPC, TERENCF G SPARLIN<.,, MD, FRCPC
H CHAUN, JH BECKMAN, TG SPARLING. Cerebral venous thrombosis in ulcerative colitis and review of the literature. Can J Gastroenterol 1991 ;5(4 ): 129- l32. A JO-year-old man with an e ighl year history of ulcerative colitis developed left occipital headach e, mental confusion , dysphasia and righr-sided weakness when his bowel disease was asymptomatic. Invest igations revealed thwmbosis of the cere bral sagitta l sinus and left transverse sinus. The literature relating to cerebrovascular complications associated with ulcerative colitis is reviewed, and the possible pathogenetic mech anisms of venous thrombosis in ulcerative colit is are discussed. The importance of recognizing that ven ous thrombosis may occur in association with u lcerative colitis in remission is emphasized.
Key Words: Cerebral venous thrombosis, Remission phase, Ulcerative colitis , Vascular complications
Thrombose veineuse cerebrale et colite ulcereuse -Observation et tour d'horizon de la litterature
RESUME: Cepha lee occipita le, confusion mentalc, dysphasic er affaiblissement du cote <lro it ont etc notes chez un patient age de 30 ans et atteint depuis huit ans d'une colite ulcereuse a lors asymptomatique. Les examcns ont revcle unc thrombose du sinus sagitta l ct du sinus lateral gauch e. Le~ auteurs effectue nt un tour d 'horizon de la litterature traitant des complications cerebrovasculaires associees a la colite ulcereuse. lls examinent les mecanismes pathogcniques possibles <le la thrombose veineuse dans ces circonstances et soulignent qu 'il est important de reconnattre qu'elle peut surve nir ch cz la personnc atteinte d'.me colite ulcereuse en remiss ion.
Di1Jision.s of GastrocmernloRJ , Neurology and I lematoloRY, Departmeni of Medicrne, Uniuersity HosJ>ital; and Universiiy of British Colwnb1a, Vanco1.wer, Bmish Columbia
Correspondence and reprims: Dr Hugh Chaun, Suite 601, 805 Wesi Broadway, Vancouver , Briiish Columbia V5Z 1 KI. Telephone ( 604) 872-0717
Recei1Jed for Jmhlication April 25, 1991. Acce/>ted June 3, 199 /
CAN J GASTROENTEROL VOL 5 Nu 4 JUL Y/A Um JST 1991
T IIE ASS<.X IATION OF VASCULAR
le~ ions with 1J iopathic inflammatory bowel disease (IBD) is well recognized (I). Vascular complications as~ociatcd with ulccrauvc coli tis include venous th rombosis (2-4), arterial thrombosis (2,5), Takayasu's d isease (6) , bchem ic skin lesions (7), and vasculi tis (8,9). Bargen a nd Barker in l 936 (2) first drew at tention to thP serious implicnrions of th rombocmbolism complicating ulcerative coli t is. S ince Harrison and Truelove (10) first reported cerebral venous thrombosis in two ulcerative colitb patients, there have been other well doc umented reports of the association of cerebrovascular disease with ulcerative colitis; the majority have occurred wi th active bowel disease. This report describes a patient with ulcerative colitis in remission who deve loped cerebral sagittal s inus and left transverse s inus th rombosis.
CASE PRESENTATION A 30-year-ol<l accountant, a non
smoke r, was tra nsferred from another hospital on Decembe r 6, 1990 for evaluation of cerebral hemorrhagic infarction. The patient had a history of ulcerative colitis since 1982, when he
129
CHAUNec al
Figure 1) Left Magnecic resonance imaging (MRI) scan showing increased signal of superior sagirtal sinus and sigmoid sinus indicacive of venoui chrombosis. Centre MRI scan showing cransverse sinus chrombosis. Right MR/ scan showing lefc parietal venoiL, infarccion
haJ presented with severe diarrhea with bleeJing, weight loss anJ anemia. He was treated in hospital with blood transfusion, sulphasalazine and intravenous hydrocortisone, fo lloweJ by prccJnisone for two months. The patient continued to take sulphasalazine until December 1988, and had occasional abJominal cramps and rectal hleeJing. In miJ-Ocrober 1990, he Jevcloped an acute exacerhation of the ulcerative colitis which responded rapidly 10 treatment with preJnisonc anJ sulpl,asalazine in hospital, and he became asymptomatic.
Whtie at work on November 13, the patient complained of left occipital headache ant.I vom ited. He was confused, hat.I d ifficulty finJing words, and haJ weakness of the right arm anJ leg. He had no fever or prot.lromal il lness (including oti t i~ anJ mastoiditis), no history of rheurrnnic heart disease, recent dental or surgical procedure, intravenous Jrug abuse or homosexual contact, anJ no family history of neurological or collagen vascular Jisease. He hat.I no heart murmur or carotid bruit.
Cerebrospinal fluid analysis showed protein 0.5 1 g/L (normal 0.15 to 0.4), glucose 4.0 mmol/L, white blood cells 1/mm 3, red blood cells 560/mm 3. Computed tomography (CT) scan showed three hemorrhagic infarcts in the lefr hemisphere , one in the tempornl area and two in the deep white matter. Blood cultures were negative but the patient was treateJ empirically with broad spectrum antibiotics. He was started on phenytoin for seizure pro-
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phylaxis, and dexamethasone. The heaJache and mental confusion resolved, and speech and strength in the right limbs improved, showing only mild impai rment of fine finger movements of the right hand.
Magnetic resonance imaging (MRI) scan revealed evidence of sagittal sinus and left transverse sinus thrombosis (Figure l ). Cerebral angiogram confirmed almost total thrombosis of the sagittal sinus and partial thrombosis of the left transverse sinus (Figure 2). Complete bloot.l count ( inclut.ling platelets), erythrocyte sedimentation rate, prothrombin t ime, activated partial thromboplastin time, the C3 and C4 components of complement, protein C, plasminogen, a lpha-2 antiplasmin, and relative serum viscosity at 20°C anJ 37')C were normal. Antithrombin Ill was 2.10 (normal 0.9 1 ro l. 38) and protein S was 1.40 (normal 0.57 ro 1.20). A lupus anticoagulant was excludeJ by a normal dilute Russell viper venom time and a normal tissue thromboplastin inhibition test. Sugar water test and rheumato id factor were negative. The antinuclear factor was slightly positive with a titre of 1:80. Serum protein was 62 g/L (normal 63 to
82). Protein electrophoresis showed a lbumin 36.3 g/L (normal 37 to 50), a lpha-I-globul in 3.4 g/L (normal l.S co 3.2), a nt.I normal a lpha-2-globulin, beta-globulin anJ gamma-globulin. Other biochemical tests inc luding blood glucose, cholesterol and triglycerit.le were normal. Serology test for hum;;in immunodeficiency virus was negative. Plasma reagm test and
hepatitis B surface antigen were nonreactive. Electrocardiogram, cchocardiography and colour flow duplex ultrasound of both lower I imbs and pelvic veins were normal. CT scan and x-rays of the 1m1stoid and paranasal sinuses showed no significant abnormality. Sigmoidoscopy showed normal mucosa.
Treatment with Jexamethasone was changeJ to prednisone on tapering doses, finally JiscontinueJ on December 31. The patient was Jischarged on December 19, on warfarin and 5-am inosalicylic acid, anJ has remained well.
DISCUSSION Thromhoembolic complication~
have been known to he a significant cause of morbidity and mortality in pllt ients with IBD since their recognition in J 936 (2). The reported incidence of thrombosis has ranged hetwccn 1.3 ant.I 6.4% during life (3,ll), with a 25% mortality rate (l l) to 39% at post mortem ( 4 ). In the Mayo C linic series, 6 l of92 patients had deep venous thrombosis or pulmonary emholi, anJ only nine patients had cerehrovascular episodes ( 11 ).
Review of the Engl ish language literature discloscJ references to 22 patients with adequately documented descriptions of cerebrovascular complications which developed in association with ulcerative colitb ( 10-23). Th1: male to female rat in was 11: IO; the sex of one patient was not identifieJ. The patients rangeJ in age from five co 54 years (mean 28.3). The presenti ng fca-
CAN J GASTROENTl:ROL VOL 5 Nt)4 jL1LY/At.X,UST 1991
t '
0 0
II
Figure 2) Anrernposrenor (top) and lateral (bottom) t•iews of ihe venous phase of the left internal rnrorid angiowam show failure to opac1fy the superior sagictal smus ( n). There is only partial opac1ficacion of che left transverse smus (short arrows) and no opacification of the left sigmoid sinus or mremal jugular vein. Faine filling of che right transverse and sigmoid sinuses is seen (arrowheads). The inferior sagictal smus ( paired arrows) is slight/)' enlarged and nmluple small wrwous conical veins (long arrows) prot1ide collateral drainage to the left cavernous sinus ( curved arrows)
CAN) GASTROENTEROL VOL 5 No4 JULY/AUGUST 1991
Cerebral venous thrombosis in UC
rurcs included headache, loss of vision, aphasin, hem1paresis, :.eizurcs, ment,11 confusion and stupor. The cercbrovas, cular lesions were diagnosed c linically in l2 patients (with only limited data in four). The diagnosis wa, confirmed by angiography in six patients (twl) also al autopsy), nt operation in one, at autopsy in one, and by CT ~can in two, one of whom also underwenc MRI. The cerebrovascular events occurred during the acute phase of ulcerative colitis in all bur seven patients. T he disease was in rem ission in one patient, controlled in two and unknown in one. Three patients had had proctocolectomy -one developed superior sagiccal venous sinus th rombosb l O years after the operation. Nine of the 22 patients also had a history of exrracerebra l rhromhoembolic disease.
Although most patients had active bowel disease at the time of thrombosis ( 11 ), the pat ient in the present report developed the complicat ion when his ulcerative colitis ha<l returned co a remiss ion phase. He had no other apparent risk fac tor for thromboembol ic disease or hypercoagulable state. The role of anticoagulant therapy in cerehral venous thromhosis is controversial ( LO, l4.2 l ), hut the dec ision co use it in this patient was made easier as his colon ic disease was asymptomatic.
T he pathogenesis of venous thrombosis in ulcerative colitis remains ohscurc. Hypercoagulahi lity associated with rhromhocytosis (18,24), elevated fibrinogen ( l l , 18,2 5), elevated factors V and VIII (l l,18,25 ), decreased antithromhin Il l (18,24) and accelerated thromboplastin generation (26) have been postulated as possible causative mechanisms. However, most patients were evaluated when their bowel dis, case was active an<l these abnormalities in coagularion, which occur in active IBO, have not been proven to be causative faccors in venous or arterial th romboembolic disease (27). When patients were studied during a quiescent phase of their d isease, no significant prcthrombotic abnormalities were found (28,29). W ith two exceptions (24,27) , prcviou stud ies did not assess the newly recognized t h rombotic d isorders involving defic iency of protein C
131
Cl !AUNet al
or protein S , the vitamin K-dependenc natural anticoagulants. No defic iency was demonstrated in the present patient. In a study of IBO patients with little or no disease activity (27), all standard coagulation tests as well as protein C and protein S were normal. However, a high inc ide nce of abnorma lities in fibrinolysis and ele vated levels of c irc ulating immune complexes
ACKNOWLEDGEMENTS: The authors thank Dr Douglas A Graeh, Head , Divis ion of N euroradio lngy, Vancouver Genera l Hospital, for his inva luable he lp with the photographs of the ce rebral ;m giograrn, and Dr Rohen R Love for his referral and update of the patient.
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manifestations of inflammatory bowe l disease. In: Freeman HJ , ed . Inflammatory Bowel Disease, vol IL Boca Raton : C RC Press Inc , 1989:51 -74.
2. Bargen JA, Barker NW. Extensive arterial anJ venous thrombosis complicating chronic ulcerative co li tis. Arch Intern Med 1936;58: 17-3 1.
3. Edward, FC , Truelove SC. The course and prognosis of ulcerative colitis. Ill. Complicat ions. G ut 1964;5: l -22.
4. G raefV, Baggenstoss Al I, Sauer WG, Spittell JA Jr. Venous th rombosis occurring in nonspecific ulce rative colitis. A necropsy study. A rch Intern Med 1966; 117:3 77-82.
5. Braverman D, &)goch A. Arterial thrombos is in ulcerative coliri~. Arn J Dig Db 1978;23:1148-52.
6. C hapman R, Dawe C , Whorwell PJ , Wright R. U lcerative colitis in assoc iation with Tabyasu's d isease. Arn J Dig Dis l 978;23:660-2.
7. C haun H, Day J , Dodd WA, Dunn WL. lschcmic skin lesions in ulcerative colitis. Can Med A'"oc J 1985;132:937-9.
8. McDermott V, McCarthy C F. A case of ulcerative colitis presenting as vasculitic purpura. Dig Dis Sci l 985;30:495-6.
9. Newton JA , McGiblxm DH, Marsden RA. Leucocytoclast1c vasculitis and angiooedema associated with inflammatory bo'wel Jiseasc. C lin Exp Dermarol 1984;9:61 8-23.
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were noted, and it was suggested that the presence of these abnonna lities may increase the risk of thrombosis in IBO.
The role of corticosteroids in thrombotic disease has been refuted (30), and hype rcoagulabil ity has not been associa ted with the use of sulphasalazine (15 ).
Cerebra l thrombosis is an uncommon but po tentia lly serious complication of ulcerative colitis. It is important
10. Harrison MJG , T ruelove SC. Cerebral venous thrombosis as a complication of ulcerative colit is. A m J Dig Dis 1967; 12: 1025-8.
11. T albot RW, Heppell J, Dozois RR, Beare RW Jr. Vasc ula r complicat ions of inflammatory bowel disease. Mayo C lin Proc 1986;6 I: 140-5.
12. Borda lT, Southern R F, Brown W F. Cerebral venous th rombosis in
ulcerati ve colitb. Gastroenterology 1973;64: 11 6-9.
I 3. Mayeux R, Fahn S. S trokes and ulcerative colitis. Neuro logy l 978;28:571 -4.
14. Schneiderman JH, Sharpe JA, S utton DMC. Cerebral and retinal vascular cornplicarions of inflammatory bowel disease. Ann Neuro l 1979;5:331 -7.
15 . Markowitz RL, Ment LR, G ryboski JD. Cerebral thrornhoern bolic disease in pediatric and adul t inflammatory bowe l Jisease: Case report and rev iew of the li terature. J Pe<liacr Gastroentero l Nutr l 989;8:4 13-20.
16. Lloyd-Srill JD, Tomasi L. Neurovascular and th rombocmhol ic complications of mflarnrnarory bowel Jisease in ch ildhood. ] Pediatr Gasrroenrerol N utr 1989;9: l 10-5.
17. Kehoe EL, Newwrne r KL. Thromboernbo lic phe nomena in ulce rative colitis. Arc h Intern Med 1964;113:71 l-5.
18. Lam A , Borda IT, Inwood MJ, Thomson S. Coagula tion studies in ulcerative colit is and Crohn's d isease. Gastroenterology 197 5;68:245-5 1.
19. Ryan FP, Timperley WR, Preston FE, Holdsworth C D. Cerebral involvement with disseminated inrravascular coagulat ion in intest inal J isease. J C lin Pathol l 977;30:55 l -5.
20. Edwards KR. Hemorrhagic complications ,if cerebral arteritis. Arch N eurol I 977; 34:549-52.
21. Averback P. Primary cerehrnl vcnnus thrombosis 111 young adults: The
to recognize that its occurrence docs not correlate with the duration, activity or extent of the intestinal disease (14) . A s the present patie nt demonstrated, cerebral venous thrombosis should be considered in a patient presenting with an abrupt onset of headache, mental confusion and focal neuro logical sympmms, even when the ulcerative colitis is in apparent remission.
Ji verse marnfe:,ta t ions of ,i n underrecognizeJ Ji:,ease . A nn Neurol 1978; 3:81-6.
22. Yerby MS, Bailey G M. Superior sagin al sinus th rombosis l O year, after surgery for ulcerative col iris. S troke 1980; 11 :294-6.
2 3. Paradis K, Bernste in ML, Adelson JW. T hrombosis as a complication of inflammatory bowel d isease m children: A report of four cases. J Pediatr Gastroenterol Nutr I 985;4:659-62.
24. Morowitz DA, A llen LW, Kirsner JB. Thrombocytos is in chronic inflammatory bowel disease. Ann Intern Med 1968;68: 10 13-2 1.
25. Lee LC , Spirrell JA Jr, Sauer WG . Hypercoagulability associated with chron ic ulcerative colitis: C hanges in
blood coagulation factors. G amocnterology 1968;54:76-85.
26. Spittell JA Jr, Owen C A Jr, Thompson JH Jr, Sauer WG. I lypercoagulahiliry and th rombosis in chronic ulcerati ve coli t is. In: 1963-1964 Collected Papers in Medicine from the Mayo C linic anJ the Mayo Foundat ion, vol 53. PhilaJ elphia and London: WB Saunders Co, 1963:53-7.
27. Conlan MG, Haire W O, Burnett DA. Prm hro rnbo tic abnormalitie; in inflammatory bowel disease. Dig Dis Sci 1989;34: l 089-93.
28. Knot EAR, Ten Cate JW, Lecksrna OCl !, Tytgat G N , Vreekenj . No ev idence for a preth rombotic state in stable chronic inflarnrnarory howe l Jiscase. J C lin Pathol 1983;36: 1187-90.
29. Like AM, Stauffer JQ, Stuart MJ . Hemosratic alternrion~ in inflammatory bowe l d isease. Response to the rapy. A m J Dig Dis l 978;23:897 -902.
30. T niclove SC, Wim LJ. Cortisone in u lcerative colitis; final repo rt <ln therapeutic trial. Br Med J l 955;2: l 041 -8.
CAN J G ASTROENTEROL VOL 5 NO 4 J Lil. Y / A UGUST 1991
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