The effect of delayed anti-NMDAR encephalitis recognition on disease outcome

Abstract

Anti-NMDA receptor encephalitis is an acute form of brain inflammation that is potentially lethal but has a high probability for recovery with treatment. Although the clinical picture of anti-NMDAR encephalitis is usually recognizable due to its relatively well-known symptoms, the disorder can sometimes present itself in an unpredictable and atypical way. In this case report, we wish to present the influence of different delay times prior to the establishment of diagnosis. Thus, our first patient was diagnosed with anti-NMDAR encephalitis 4 years after the initial symptoms, the second one after 8 years, and the third one after 13 months. The outcomes of the three presented patients indicate the importance of being aware of many clinical presentations of this disorder, as its early diagnosis greatly affects the outcome and may reduce permanent damage, especially in cognitive functions.

Clinical Features, Treatment, and Outcomes Among Chinese Children With Anti-methyl-D-aspartate Receptor (Anti-NMDAR) Encephalitis

“…However, in our study, EDB patterns were detected in only 2/18 (11.1%) patients, likely due to differences in the time of EEG recording and individual differences in patients within the various study groups. Since prompt diagnosis is crucial (29), we recommend use of video-EEG monitoring for all patients with suspected anti-NMDAR encephalitis (30). Nonetheless, a recent study suggested that EDB is also not unique to anti-NMDAR encephalitis and can occur (albeit rarely) in patients with mesial temporal lobe epilepsy.…”

Never Too Late to Treat NMDAR Encephalitis: A Paediatric Case Report and Review of Literature

“…9,10 Numerous retrospective studies indicate a good prognosis, including fewer relapses, with early diagnosis and treatment of anti-NMDAR encephalitis. 11,12 A large cohort study indicated that treatment delay greater than 4 weeks was associated with worse 1-year functional status. 13 However, timely treatment is not always possible, given the complex presentation that may lead to psychiatric or non-autoimmune epileptic misdiagnoses, and lack of proven treatment options in refractory cases.…”

“…Lengthier delays to efficacious therapy have been documented in several case reports. Sulentic et al 11 discussed that, despite delayed diagnosis and therefore immunotherapy ranging from 13 months to 8 years after initial presentation, three patients only had minimal residual cognitive deficits compared with their pre-morbid baselines. Another case report reviewed three patients with treatment refractory disease receiving tocilizumab 2-4 years postsymptom onset, with substantial recovery months after treatment initiation.…”