{
  "abstract": "Childhood stroke is a rare occurrence, with around 400 children in the UK experiencing a stroke each year. Approximately one-third of childhood strokes are cryptogenic, meaning that the underlying cause remains unknown. 1 2 An association between coeliac disease (CD) and acute ischaemic stroke has been suggested due to the influence of chronic inflammation, abnormal immune response, endothelial damage and prothrombotic states.2–9 Here, we present a case of stroke in a 4-year-old girl with no known medical history who was later discovered to have CD.A previously healthy 4-year-old girl presented to the emergency department (ED) following a syncopal episode along with new left sided weakness and a one-day history of right sided headache. Her neurological assessment revealed unilateral weakness on the left side with reduced ability to hold her limbs against gravity and fatigue when walking without support. Her initial bloods revealed severe anaemia with Haemoglobin 4.8 g/dL, other blood counts were normal, including normal clotting profile. CT head revealed a thrombus in the entire internal cerebral vein. Extensive further bloodwork, including iron studies, haemoglobinopathy screen, thrombophilia screen and coeliac screen, confirmed iron deficiency anaemia and CD with positive anti-tissue transglutaminase IgA (titres >128) and IgG (titre 69.0), and positive IgA endomysial antibodies. This resulted in a non-biopsy diagnosis of CD.She received fluid boluses, one blood transfusion, and was commenced on treatment dose low-molecular-weight-heparin (LMWH) 100 units/kg twice daily (BD) following discussion with the tertiary paediatric haematology team. Conservative treatment with 72-hours of hourly neuro-observation and monitoring for signs of raised intracranial pressure was carried out following discussion with tertiary paediatric neurology and neurosurgical teams.Following 72-hours of observation, she was discharged with 5-days LMWH 100 units/kg BD then switched to oral rivaroxaban 5 mg BD for 3-months. Her repeat MRI showed that the deep cerebral veins, Vein of Galen and Straight sinus had recannalised. She is currently doing well, is asymptomatic and on a lifelong gluten free diet with no further acute ischaemic stroke events reported.Some studies have reported that CD is associated with an increased risk of cardiovascular disease, due to its association with other autoimmune diseases including autoimmune vasculitis, which can directly affect the cerebral vessels and increase the risk of stroke.4 7 9 This combination of chronic inflammation, cross-reacting antibodies and endothelial dysfunction may influence the higher incidence of ischaemic stroke, myocardial infarction and thromboembolism seen in those with CD and suggests that there is an autoimmune component for CD associated vasculopathy.7 9 We suggest that CD is a potentially treatable cause of cerebral vasculopathy, and CD serology should be considered when investigating cryptogenic stroke in childhood, even in the absence of typical gastrointestinal symptoms. It highlights the importance of considering CD as a differential diagnosis for ischaemic stroke, allowing for earlier diagnosis and management of CD.References Schulz UG. ‘Cryptogenic stroke – how to make sense of a non-diagnostic entity’. Maturitas 2019;122:44–50. doi:10.1016/j.maturitas.2019.01.004.Vazquez S, Thaker A, Nolan B, et al. ‘Incidence, characteristics, and outcomes of stroke in pediatric patients with celiac disease’. Life 2023;13(7):1445. doi:10.3390/life13071445.Conroy M, Allen N, Lacey B, et al. ‘Association between coeliac disease and cardiovascular disease: prospective analysis of UK biobank data’. BMJ Medicine. 2023;2(1). doi:10.1136/bmjmed-2022-000371.Fousekis FS, Beka ET, Milionis H, et al. ‘Thromboembolic complications and cardiovascular events associated with celiac disease’. Irish Journal of Medical Science (1971 -) 2020;190(1):133–141. doi:10.1007/s11845-020-02315-2.Laurikka P, Nurminen S, Kivela L, et al. ‘Extraintestinal manifestations of celiac disease: early detection for better long-term outcomes’. Nutrients 2018;10(8):1015. doi:10.3390/nu10081015.Doğan M. ‘Stroke and dilated cardiomyopathy associated with celiac disease’. World Journal of Gastroenterology 2010;16(18):2302. doi:10.3748/wjg.v16.i18.2302.Prastesi R, Gandolfi L, Friedman H, et al. ‘Serum IGA antibodies from patients with coeliac disease react strongly with human brain blood-vessel structures’. Scandinavian Journal of Gastroenterology 1998;33(8):817–821. doi:10.1080/00365529850171468.Ludvigsson JF, West J, Card T, et al. ‘Risk of stroke in 28,000 patients with celiac disease: a nationwide cohort study in Sweden’. Journal of Stroke and Cerebrovascular Diseases 2012;21(8):860–867. doi:10.1016/j.jstrokecerebrovasdis.2011.05.008.Goodwin FC, Beattie RM, Millar J, et al. ‘Celiac disease and childhood stroke’. Pediatric Neurology 2004;31(2):139–142. doi:10.1016/j.pediatrneurol.2004.02.014.",
  "authors": [
    {
      "affiliations": [
        "Maidstone and Tunbridge Wells NHS Trust"
      ],
      "name": "Marie-Sophie Gomm"
    },
    {
      "affiliations": [
        "Maidstone and Tunbridge Wells NHS Trust"
      ],
      "name": "Rohit Gowda"
    }
  ],
  "title": "OC96 Childhood stroke and coeliac disease – a case study",
  "uid": "e8c6794e-a8dd-50f2-952b-620e8dbb0cc2"
}