# A Case of Syrian Child with Cerebral Infarction as an Extraintestinal Manifestation of Ulcerative Colitis

Authors: Riham Salloum; Nawras Alhalabi; Mohamad Anas Almidani
Journal: Case Reports in Gastrointestinal Medicine
 (2019)

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## Abstract

Thromboembolic complications are rare but well-recognized manifestation of ulcerative colitis, especially because of their associated high mortality. We report a case of a Syrian child admitted to Damascus Hospital with a one-day complaint of sudden onset of numbness followed by weakness in the left lower and upper limbs, right mouth angle deviation, and loss of sphincters’ control. Earlier, she was diagnosed with ulcerative colitis and treated with immunosuppressants. CT and MRI scans revealed focal infarction around the M2-M3 segments of the right middle cerebral artery; she was treated with Aspirin. On discharge, she had significant improved neurological examination and was able to walk. Subsequent proctocolectomy was performed. We highlight the importance of thromboembolism in ulcerative colitis as there is paucity in the literature regarding its management and its symptoms may be overlooked especially in high-load central hospitals. We conducted a brief literature search and summarized findings of similar reported cases.


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## Body

## 1. Introduction

Extraintestinal manifestations of idiopathic inflammatory bowel disease (IBD) have been reported in 25% to 36% of patients [1]. Common manifestations include sacroiliitis (14%) and peripheral arthritis (10.7%), while rare manifestations include ocular (8%), mucocutaneous (2.7%), and vascular (2%) [2].Neurologic manifestations in IBD appear to be more common than previously estimated with a reported incidence of cerebrovascular complications in 0.12% to 4% of all patients with IBD [3, 4]. Generally, it occurs as a postoperative complication and found more in Crohn's disease than ulcerative colitis (UC) [5]. Thromboembolic complications of UC are reported at an incidence of only 1.2%-7.5%, but are well recognized because of their associated high mortality [6–9] which occurs in 60% of cases [4].We report a rare case of a Syrian child who was suffered a cerebrovascular accident (CVA) as a complication of ulcerative colitis. To the best of our knowledge, this is the first documented case in Syria.


## 2. Case Presentation

A 15-year-old Syrian female was admitted to the hospital on November 2016 with a one-day complaint of sudden onset of numbness in the left lower and upper limbs, followed by weakness in the same areas, right mouth angle deviation, and loss of sphincters’ control. She did not experience headache, nausea, vomiting, convulsions, or coma.Eight months earlier, she developed massive rectal bleeding, colonoscopy was performed, and the patient was diagnosed with ulcerative colitis (UC). She was treated with mesalazine 1 gram three times daily, azathioprine 50 milligram daily, prednisolone 40 milligram daily, and cefuroxime 500 milligram tab twice daily for a week.She has no history of smoking, alcohol abuse, or illicit drug use. She did not report any suspected allergies and she has no other history of hypertension, diabetes mellitus, cardiac, rheumatological, or hematological disease.On examination, her vital signs are blood pressure 100/60 mmhg, Pulse 110/minute, respiratory rate 36/minute, and temperature 37.5°C. General examination revealed conjunctival pallor and pitting edema in the left lower limb and purple stretch marks extends on the whole lower limbs till the sacrum.On neurological examination, there was no impaired consciousness and the patient was awake and alert. Cranial nerves exam was only significant for left facial nerve palsy. Motor examination showed 5/5 strength in the right upper and lower limbs, 3/5 left upper limb, and 0/5 left lower limb; there was also hypotonia on the left limbs and normal tone on the right limbs without any atrophy. Reflexes examinations scored 2/4 for the right limbs (normal) and 1/4 for the left limbs (hyporeflexia). Right toes showed planter flexion and absence of the flexion for the left toes. No cerebellar abnormalities were noted in the right side; cerebellar exam was not performed on the left side due to limbs weakness. She scored 10 on National Institutes of Health Stroke Scale (NIHSS). Sensory examination revealed loss superficial and deep sensations on the left side and normal sensations on the right side. Other systematic examinations, including cardiac, respiratory, and gastrointestinal systems, were all normal.Investigations including blood tests showed evidence of pancytopenia (hemoglobin 4.4 g/dL, platelets: 66 x1000/mm3 dropped to 3 x1000/mm3 after in two days of admission, WBCs: 1.4 x1000/mm3 with 35% neutrocytes, 61% lymphocytes, 3% monocytes, and 1% eosinophils); urinalysis values were within normal ranges. Thrombophilic and immunological screening including homocysteine, factor V Leiden, protein C, protein S, antithrombin, lupus anticoagulant factor, and antiphospholipid antibodies were all insignificant.An emergency computerized tomography (CT) scan (Figure1) showed small hypodensity foci situated in the cortical and subcortical area in right partial lobe. Magnetic resonance imaging (MRI) (Figure 2) showed cortical and subcortical areas in the right temporoparietal fossa with high signal on T2 and FALIR studies. T1 study showed isointense foci in the cortical and subcortical area in the posterior part of the parietal lobe extending deeply through the posterior horn of the right lateral ventricle. Based on these findings, the accident is complicated with focal infarction around the M2-M3 segments of the right middle cerebral artery. Aspirin 162mg was given upon these findings and the prednisolone treatment was continued.Figure 1
Patient CT scan showing showed small hypodensity foci situated in the cortical and subcortical area in right partial lobe.Figure 2
Patient MRI showing cortical and subcortical areas in the right temporoparietal fossa with high signal on T2 and FALIR studies, in addition to isointense foci in the cortical and subcortical area in the posterior part of the parietal lobe extending deeply through the posterior horn of the right lateral ventricle on T1 study.Cardiac echocardiogram and carotid arteries Doppler ultrasound study were both normal. The patient did not complain of any symptoms related to her UC when she had the CVA, which indicates that the UC was not in active stage.On December 2016, the patient was able to walk and her neurological examination dramatically improved (NIHSS: 0); she was then discharged and referred to physical therapy. On January 2017, the patient suffered from overt rectal bleeding, she was admitted again to the hospital, and proctocolectomy was performed.A written informed consent was obtained from the patient before writing this report, Syrian Private University and Damascus Hospital Ethical Committee approved the report, and both are available upon request.


## 3. Discussion

Increased coagulability and thrombosis due to IBD were first described in 1936 [29]. Intestinal inflammation may lead to increased risk for thrombosis through several pathways: activation of coagulation cascade, decreasing anticoagulant activity, inducing hypofibrinolysis, malabsorption, and hypercatabolism with vitamin deficiencies [4]. Most patients with IBD do not have demonstrable specific coagulation defects [30]. Dehydration, immobility, sepsis, surgery, and corticosteroid therapy are also risk factor for thrombosis in IBD patients [31]. The precise mechanism of these factors remains unclear. Arterial thrombosis particularly strokes may be considered a rare condition [17] but with high morbidity and mortality [32–34].Males and females may be equally affected which correlates with previously reported cases. The cerebral vascular involvement seems more frequent among younger IBD patients [4]. Conventional CT scan or MRI is used to define the cerebral affected areas. At this moment, no guidelines are available for the treatment of cerebral thrombosis and strokes in IBD [17].By reviewing previous literature, Schneiderman et al. were the first to report similar case with thrombosis of the left internal carotid artery (ICA) and occlusion of the left distal basilar artery in two separate patients with UC respectively [26]. The youngest patient reported was a 1-year-old girl with UC, who was complicated with bacterial endocarditis and subsequent infarctions of both middle cerebral arteries (MCA) [27].We have conducted a brief literature search and summarized cases reported on Cerebral Arterial Thrombosis associated with UC using modified version of Katsanos et al. [35] table (Table 1).Table 1
Cases reported in the international of Cerebral Arterial Thrombosis associated with ulcerative colitis.
Author Date Sex Age UC Activity IBD Treatment Neurological symptoms Site of cerebral injury/neurological findings Risk Factor Thrombophilia screening Anticoagulation/antiplatelet treatment Follow-up/outcome Calabro et al. [10] 2011 F 49 No  Right hemiparesis, aphasia, altered conscious Left frontotemporal infarct, lesion in the left mesencephalic area, left ICA stenosis Hypercholesterolemia, elevated Lpa  LMWH (nadroparine 0.6 ml/d), aspirin (300 mg/d) / clopidogrel (75 mg/d) Improved Casella et al. [11] 2013 M 25 Yes  Left hemiparesis, confusion, sensitive impairment, bladder incontinence Right MCA infarct Anemia  Local thrombolysis/ LMWH and aspirin (100 mg/d) Complete recovery Chetri et al. [12] 2002 M 42 Yes Steroids, 5-ASA Left hemiplegia Right MCA infarct and right CCA occlusion Anemia   Improved Fukuhara et al. [13] 1993 M 18 No Steroids Left hemiparesis Right paramedian branch of the basilar artery infarct, Ventromedial pons Anemia   Complete recovery Hilton-Jones et al. [14] 1985 M 20 No   Basal ganglia infarct Anemia, abnormal platelet aggregation   Recurrent thrombotic episodes, death Houissa et al. [4] 2011 M 24 Yes Steroids Left hemiplegia Left MCA infarct  Neg LMWH Complete recovery Houissa et al. [4] 2011 F 25 Yes 5-ASA Left hemiparesis Tenticular & right thalamic infarcts Thrombocytosis Neg LMWH Partial recovery (Residual right hemiparesis) Jorens et al. [15] 1990 M 31 Yes 5-ASA Right hemiparesis, nonconfluent aphasia Recent ischemic lesion in left internal capsule and old ischemic lesion in left basal ganglia Anemia, thrombocytosis, pro-C, pro-S and prothrombin deficiency, history of TIA   Partial recovery Jorens et al. [16] 1991 M 32 Yes  Left hemiparesis, stupor, hemiataxia Temporoparietal cerebrovascular ischemic lesion    Complete recovery Joshi et al. [17] 2008 M 55 Yes Steroids, 5-ASA Left hemiparesis Right parietal lobe infarction Thrombocytosis Neg LMWH Partial recovery Joshi et al. [17] 2008 M 24 Yes Steroids, 5-ASA Headache, altered conscious, global aphasia Left MCA infarct Thrombocytosis Neg  Complete recovery, subsequent epilepsy Keene et al. [18] 2001 F 13 Yes Steroids, subtotal colectomy Seizure Multiple bilateral cerebellar and corona radiata infarcts Anemia   Complete recovery Kelly et al. [19] 2014 M 38 No Steroids, AZA, infliximab bilateral lower limb claudication, acute confusion with associated ataxia and diplopia right medial temporal lobe extending posterior to the occipital lobe  Neg Warfarin Controlled Lloyd-Still and To masi [20] 1989 M 5 Yes Steroids, 5-ASA Right hemiparesis, seizure Left MCA arteritis Anemia   Partial recovery, epilepsy developed 10 years later Mayeux and Fahn [21] 1978 M 17 Yes Steroids  Right posterior frontal area infarction    Slowly improved Nogami et al. [22] 2007 F 26 Yes Steroids Left hemiparesis Right MCA Infarct and right CCA occlusion Severe anemia  Heparin (15.000 U/d) Massive GI bleeding, no improvement Paradis et al. [23] 1985 F 12 Yes  Right hemiparesis, seizure Left major cerebral vessels occlusion Anemia, thrombocytosis   Complete recovery Patterson et al. [24] 1971 M 11 Yes   Cerebral emboli    Colectomy Richard et al. [25] 2014 F 42 Yes Steroids, AZA Sudden right hemiplegia Right MCA Slight hyperhomocysteinemia  LMWH, Enoxaparin sodium, Aspirin Salloum et al. (our case) 2016 F 15 No Steroids, 5-ASA, AZA Left hemiparesis, 7th CN palsy M2-M3 segments of the Right MCA Pancytopenia Neg Aspirin 162mg Colectomy, complete recovery Schneiderman et al. [26] 1979 M 34 Yes  Right hemiplegia, nonfluent aphasia Left ICA thrombosis Thrombocytosis  Thrombectomy, heparin/warfarin Schneiderman et al. [26] 1979 F 12 Yes Steroids, 5-ASA Hemianopia, headache, seizures Distal basilar artery defect extending to the left PCA Elevated fVIII   Death Tomomasa et al. [27] 1993 F 1 Yes Steroids, 5-ASA Right hemiplegia, altered conscious, seizures Left anterior & MCA infarct/ right MCA infarct Thrombocytosis, bacterial endocarditis   No improvement Yassinger et al. [28] 1976 F 15 Yes Steroids  Right frontal lobe infarction    Recovered M = male; F = female; 5-ASA = 5-aminosalicylic acid; AZA = azathioprine; CN = cranial nerve; ICA = internal carotid artery; MCA = middle cerebral artery; CCA = common carotid artery; PCA = posterior cerebral artery; LPa = lipoprotein; Pro-C = protein C; Pro-S = protein S; TIA = transient ischemic attack; fVIII = factor VIII; LMWH = low molecular weight heparin; GI = gastrointestinal.About half of the patients were on corticosteroid treatment and more than one-third of them were being treated with 5-aminosalicylic acid (5-ASA) at the time of the cerebrovascular event [35]. The predominant neurological symptom on admission in most of the case reports was left or right sided hemiparesis; our patient was also admitted with left hemiparesis as the main complication. In addition, the right or left MCA was the most frequent sites of cerebral arterial thromboembolism depicted on imaging studies similar to our patient that manifested with cerebral arterial thromboembolism around the M2-M3 segments of the right MCA as confirmed by imaging studies [35]. As previously reported, the risk of arterial thromboembolic events may be increased in patients with active disease [4], although our patient did not have an active disease by the time of CVA.Thrombocytosis and anemia were the most commonly observed potential risk factors for cerebral arterial infarction in the laboratory analysis [35], although there is no sufficient evidence supporting the theory of solitary thrombocytosis causing thromboembolic phenomena [26]. Our patient contrary presented with pancytopenia which may be due to immunosuppressants given [36].Our patient did not have a demonstrable specific coagulation defects, in comparison; hyperhomocysteinemia [4] and other acquired deficiencies of antithrombin III and protein S have previously been reported in similar cases [30, 35]. Finally, smoking and severe dehydration have been mentioned in only two cases contrary to our patient.Our patient received Aspirin as a treatment after she was diagnosed with the cerebrovascular accident. She had improved neurological examination on discharge and she was able to walk; she was then discharged and referred to physical therapy, although as reported in previous cases also most of the patients also recovered either without any or with minor neurological deficits; either they received anticoagulation or antiplatelet treatment or not [35] (Table 1).Clinical experience treatment of arterial ischemic cerebral lesions in patients with IBD is very limited due to lack of enough trials [37]. More studies are required to clarify the correlation between IBD and the thrombophilias and to evaluate the role of anticoagulant therapy and proctocolectomy in the management of these patients. Similarly, there is also lack of steady evidence and official guidelines for stroke management in both children and adults with IBD comorbidity. Both American Heart Association and European Stroke Organization guidelines for stroke management and prevention in the general population are currently presented as a reference point for the treatment of IBD patients who are complicated by an ischemic cerebral event [38–40].



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