Introduction: Infliximab is a monoclonal antibody against TNF-α used in the treatment of various autoimmune diseases, including rheumatoid arthritis and inflammatory bowel disease. Previously reported neurologic complications in children include central and peripheral demyelinating disorders and neuropathies that usually occur months after initiation of therapy.

Case Description: A 7 year old male with a diagnosis of ulcerative colitis and primary sclerosing cholangitis received an infusion of infliximab after failing outpatient oral medications. He tolerated the infusion well but six hours later awoke with headache and emesis, rapidly became obtunded, and required intubation. His exam was notable for minimally reactive pupils and loss of all other brainstem reflexes. Cranial CT found hypodense lesions in the bilateral cerebral hemispheres, cerebellum and pons accompanied by hemorrhage. MRI showed diffusion restriction concerning for ischemic infarcts. On careful review, areas of ring-enhancement suggestive of either inflammation or blood-brain barrier degradation were found. MRA and MRV were unremarkable. Labs were notable for an extremely elevated d-dimer. CSF was grossly bloody but otherwise bland. Infectious studies were negative. Echocardiogram showed depressed ventricular dysfunction but neither intracardiac shunt nor thrombus were seen. Intracranial pressure was monitored and hyperosmotic therapy was administered. Malignant intracranial hypertension did not develop. Within three days, he lost all brainstrem reflexes and was terminally extubated. Autopsy was refused.

Discussion: This case is relevant to the neurologic community as it is the first to suggest that infliximab can cause fulminant, fatal CNS consequences even after the initial dose.