The field of autoimmune encephalitis has expanded tremendously over the recent years and now involves several types of diseases with different pathophysiology. Commonly, autoimmune encephalitis with pathogenic antibodies to cell surface proteins, those with antibodies to intracellular synaptic…
Background Neuroimaging findings in autoimmune encephalitis may include normal MRI as well as limbic and extra-limbic involvement. Prompt recognition allows early immunosuppression and improved outcomes.
Aim To gather data on mycophenolate mofetil (MMF) in paediatric autoimmune/immune‐mediated central nervous system (CNS) conditions, focusing on safety and factors that may affect MMF efficacy. Method Retrospective, multicentre study based on four paediatric neurology centres. Results Forty‐four children were included (30 females, 14 males): 19 had proven/suspected autoimmune encephalitis, 14 had inflammatory demyelinating CNS diseases, and 11 had other autoimmune/immune‐mediated CNS conditions. Before MMF, all received first‐line immune therapies, and 17 had second‐line rituximab and/or cyclophosphamide. MMF was started at a median of 9.5 months from disease onset (range 1–127mo) (median age 9y 4mo, range 1y 5mo–16y 5mo), and was used for median 18 months (range 0.3–73mo). On MMF, 31 patients were relapse‐free, whereas eight relapsed (excluding patients with chronic–progressive course). Relapses on MMF were associated with medication weaning/cessation, or with suboptimal MMF dosage/duration. Adverse events of MMF occurred in eight patients: six moderate (gastrointestinal, movement disorder, dermatological) and two severe (infectious). Interpretation MMF use in paediatric neuroimmunology is heterogeneous, although relatively safe. We have identified factors that may affect MMF efficacy and provide recommendations on MMF usage. What this paper adds Mycophenolate mofetil (MMF) use was heterogeneous with relatively common adverse events, although mostly not severe. MMF treatment reduced median annualized relapse rate, although 20% of patients relapsed on MMF. A high relapse rate pre‐MMF and late MMF start were associated with higher probability of relapsing on MMF. Most relapses were associated with suboptimal MMF dosage, short MMF duration, or concurrent medication weaning/discontinuation.
Brain on Fire author Susannah Cahalan joined University of Southern Mississippi alumna Jasmine Whiteside for a unique edition of the school’s University Forum Sept. 18, featuring both discussing their battle with a rare disease, anti-NMDA receptor encephalitis.
Objectives Anti-N-methyl-D-aspartate receptor encephalitis (anti-NMDARE) is a severe, but treatable disease. This study aims to give a detailed description of electroencephalogram (EEG) results in paediatric and adult patients to improve disease recognition, and analyses the predictive value of…
Objectives Anti-N-methyl-D-aspartate receptor encephalitis (anti-NMDARE) is a severe, but treatable disease. This study aims to give a detailed description of electroencephalogram (EEG) results in paediatric and adult patients to improve disease recognition, and analyses the predictive value of the first EEG for the final clinical outcome.
Methods This nationwide cohort study includes patients with N-methyl-D-aspartate receptor antibodies confirmed with cell-based assay and immunohistochemistry in serum and cerebrospinal fluid. EEG recordings were re-evaluated by two experienced neurophysiologists, mixed with control EEGs for blinding. Initial EEG as well as follow-up registrations were analysed.
Results 35 adults and 18 children were included. Only two patients (4%) had a normal EEG. During the first recording, the majority of the patients had normal posterior rhythm (71%), which was associated with better modified Rankin Scale at final outcome (OR 4.74; 95% CI 1.56 to 14.47; p=0.006). In addition, EEGs showed focal (73%) or diffuse (67%) slowing. The first EEG was severely abnormal in 26%. However, 8 of 14 patients with a severely abnormal first EEG still had a favourable outcome. During the course of the disease, extreme delta brushes (EDBs) were present in 6 of 53(11%)patients.
Conclusions The first EEG commonly shows normal posterior rhythm with focal or diffuse slowing. Although the sensitivity of an abnormal EEG is high (96%), normal EEG does not exclude anti-NMDARE. EDBs are only present in severely affected patients. The first EEG recording is predictive of the final clinical outcome.