Published online Oct 26, 2020. doi: 10.12998/wjcc.v8.i20.4866
Peer-review started: April 11, 2020
First decision: June 13, 2020
Revised: July 27, 2020
Accepted: September 25, 2020
Article in press: September 25, 2020
Published online: October 26, 2020
Asymptomatic cytomegalovirus (CMV) infection is common in children; in contrast, in children with a weakened immune system, invasive CMV can occur. This is the first case report of a severe manifestation of CMV esophago-enterocolitis in a girl diagnosed with anti-N-methyl-D-aspartate-receptor (anti-NMDAR) encephalitis who received only a moderate dose of corticosteroid therapy.
A 12-year-old-Thai girl presented with acute behavioural change and headache for 6 d. Electroencephalogram and positivity for NMDAR autoantibodies were compatible with anti-NMDAR encephalitis. Hence, she received pulse methylprednisolone 10 mg/kg per day for 4 d and continued with prednisolone 1.2 mg/kg per day. On day 42 of corticosteroid therapy, she developed unremitting vomiting and diarrhoea. Endoscopy showed multiple ulcers and erythaematous mucosa along the gastrointestinal tract. Tissue CMV viral load and viral-infected cells confirmed CMV esophago-enterocolitis. Therefore, the patient received ganciclovir 5 mg/kg per dose every 12 h for 3 wk and then 5 mg/kg per dose once daily for 3 wk. Unremitting diarrhoea slowly improved from stool output 1-4 L per day to 1-2 L per day after 3 wk of treatment. Pulse methylprednisolone 20 mg/kg for 5 d was initiated and continued with prednisolone 1 mg/kg per day. After this repeated pulse methylprednisolone treatment, surprisingly, diarrhoea subsided. Immunologic work-up was performed to rule out underlying immune deficiency with unremarkable results.
Unremitting diarrhoea from CMV esophago-enterocolitis subsided with antiviral and methylprednisolone therapy, implying the immune and NMDAR dysregulation in anti-NMDAR encephalitis.
Core Tip: We report a girl with behavioural change who was finally diagnosed with anti-N-methyl-D-aspartate-receptor (anti-NMDAR) encephalitis. She had unremitting vomiting and diarrhoea after 43 d of corticosteroid therapy and was later diagnosed with cytomegalovirus (CMV) esophago-enterocolitis. Surprisingly, these symptoms subsided after antiviral therapy followed by repeated pulse methylprednisolone targeting the anti-NMDAR encephalitis. CMV esophago-enterocolitis in a patient with anti-NMDAR encephalitis has not been reported before and is very rare in patients who receive only moderate-dose corticosteroids. We highlighted that immune dysregulation in autoimmune encephalitis and the linkage of anti-NMDAR receptors and the enteric nervous system might be the cause of severe gastrointestinal involvement.