When Neurology Meets Gastroenterology: An Unusual Case of Ulcerative Colitis

Question: A 47-year-old man was hospitalized in neurology department after right limb convulsions, drowsiness, and aphasia. The patient suffered from diarrhea for 1 month and had a 10-year history of ulcerative colitis (UC). He had no other disease like hypertension, diabetes, obesity, or atrial fibrillation, and he also denied smoking or drinking alcohol. Laboratory tests showed a hemoglobin concentration of 90 g/L, a platelet count of 389 × 109/L, and a d-dimer concentration of 4854 ng/mL. A fecal occult blood test was positive, and fecal calprotectin rose to 402 μg/L. Magnetic resonance imaging showed edema in the left frontoparietal (Figure A). Three days after admission, despite the treatment of mesalazine for UC and mannitol for cerebral edema, the volume of diarrhea was still 2500 mL/d, and the patient fell into a coma. Another magnetic resonance imaging and magnetic resonance venography were performed (Figure B, C). After further treatment, his consciousness improved, colonoscopy, and hematoxylin and eosin staining were also performed. What is the diagnosis, and what might cause the man’s rapid clinical progression? See the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. Combining clinical manifestation, laboratory tests and imaging data, cerebral herniation due to aseptic cerebral venous sinus thrombosis (CVST) was diagnosed. Magnetic resonance imaging showing edema in the left frontoparietal, and herniation beneath the falx cerebri (Figure B). Magnetic resonance venography revealing embolization of the superior sagittal sinus, with the lack of visualization of the left frontotemporal superficial vein and its branches (Figure C). A cranial decompressive craniectomy was urgently performed, and thrombosis of the posterior group of superior cerebral veins and veins adjacent to the central sulcus was confirmed. After consideration, anticoagulant therapy was commenced. Seven days later, the patient's consciousness improved. A colonoscopy showed that the mucosa was congestive, edematous and erosive, and multiple ulcers were covered with exudate (Figure D). Hematoxylin and eosin staining revealed crypt abscesses and cytomegalovirus inclusions (Figure E, F). Cytomegalovirus DNA was detected in the biopsies. The diarrhea soon improved after methylprednisolone and ganciclovir treatment. Another colonoscopy suggested mucosal healing after 7 weeks (Figure G). UC can promote hypercoagulability and thrombosis.1Novacek N. Weltermann A. Sobala A. et al.Inflammatory bowel disease is a risk factor for recurrent venous thromboembolism.Gastroenterology. 2010; 139: 779-787Abstract Full Text Full Text PDF PubMed Scopus (201) Google Scholar The progression of CVST in this patient was most likely to be associated with UC, because there were no other relevant risk factors.2Saposnik G. Barinagarrementeria F. Brown Jr., R.D. et al.Diagnosis and management of cerebral venous thrombosis: a statement for healthcare professionals from the American Heart Association/American Stroke Association.Stroke. 2011; 42: 1158-1192Crossref PubMed Scopus (1211) Google Scholar To our knowledge, this report presents the first case of cerebral herniation caused by CVST in a patient with UC. Entities including cytomegalovirus act synergistically to promote thrombosis,3Broucker C.D. Plessier A. Ollivier-Hourmand I. et al.Multicenter study on recent portal venous system thrombosis associated with cytomegalovirus disease.J Hepatol. 2022; 76: 115-122Abstract Full Text Full Text PDF PubMed Scopus (3) Google Scholar which indicate that coinfection with cytomegalovirus might exacerbate clinical progression with life-threatening cerebral herniation. Thus, increased suspicion and multidisciplinary treatment are warranted by physicians.