Bi-insular cortical involvement in anti-NMDA-receptor encephalitis – a case report. Issue 1 (December 2016)

• Record Type: Journal Article
• Authors: Hegen, Harald; Uprimny, Christian; Grams, Astrid; Virgolini, Irene; Ramberger, Melanie; Beer, Ronny; Helbok, Raimund; Pfausler, Bettina; Schmutzhard, Erich
• Is Part Of: BMC neurology. Volume 16:Issue 1(2016)
• Page Start: 1
• Page End: 4
• Publication Date: 2016-12
• Subjects: NMDA receptor -- Autoimmune encephalitis -- Insular cortex -- Hippocampus -- Medial temporal lobe -- Frontal lobe -- MRI -- PET -- Case report; Neurology -- Periodicals; 616.8005
• DOI: 10.1186/s12883-016-0653-9
• Languages: English
• ISSNs: 1471-2377

Abstract

Abstract Background Anti-NMDA-receptor encephalitis is an immune-mediated inflammatory disorder of the central nervous system. Brain MRI is unremarkable in at least 50 % of patients and highly variable in the remaining patients with signal abnormalities in different brain regions. Only scarce reports exist on other imaging modalities. Case presentation A 31-year-old woman sub-acutely developed psychosis, behavioural changes, amnesia, alternating states of agitation and mutism, fever and epileptic seizures. Clinically suspected diagnosis of anti-NMDA-receptor encephalitis was confirmed by the detection of anti-NMDA receptor antibodies in CSF and serum. During the acute phase, brain MRI abnormalities were found in both insular cortices and hippocampi, whereas F18 -FDG-PET showed hypermetabolism bilaterally in insular and prefrontal cortex. After resection of the underlying ovarian teratoma and with multimodal immunotherapy the patient substantially improved reaching a modified Rankin Scale score of 2 after 3 months. At follow-up, both hippocampi were still affected on MRI, whereas insular cortex appeared normal; however, both regions showed prominent glucose hypometabolism. Conclusions Here, we report bi-insular cortical abnormalities on MRI and F18 -FDG-PET in a patient with anti-NMDA-receptor encephalitis during the acute phase and after clinical improvement.