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Isolated Psychiatric Symptoms in Children With Anti-N-Methyl-d Aspartate Receptor Encephalitis
Gombolay, Grace; Brenton, J Nicholas; Yang, Jennifer H; Stredny, Coral M; Kammeyer, Ryan; Fisher, Kristen S; Sandweiss, Alexander J; Erickson, Timothy A; Kannan, Varun; Otten, Catherine; Steriade, Claude; Vu, NgocHanh; Santoro, Jonathan D; Robles-Lopez, Karla; Goodrich, Robert; Otallah, Scott; Arellano, Janetta; Christiana, Andrew; Morris, Morgan; Gorman, Mark P; Kornbluh, Alexandra B; Kahn, Ilana; Sepeta, Leigh; Jiang, Yike; Muscal, Eyal; Murray, Kristy O; Moodley, Manikum; Hardy, Duriel
BACKGROUND:Isolated psychiatric symptoms can be the initial symptom of pediatric anti-N-methyl-d-aspartate (NMDA) receptor autoimmune encephalitis (pNMDARE). Here we report on the prevalence of isolated psychiatric symptoms in pNMDARE. We also assess whether initial neurodiagnostic tests (brain magnetic resonance imaging [MRI], electroencephalography [EEG], and/or cerebrospinal fluid [CSF] white blood cell count) are abnormal in children with isolated psychiatric symptoms and pNMDARE. METHODS:This multicenter retrospective cohort study from CONNECT (Conquering Neuroinflammation and Epilepsies Consortium) from 14 institutions included children under age 18 years who were diagnosed with pNMDARE. Descriptive statistics using means, medians, and comparisons for continuous versus discrete data was performed. RESULTS:Of 249 children included, 12 (5%) had only psychiatric symptoms without other typical clinical features of autoimmune encephalitis at presentation. All but one (11 of 12 = 92%) had at least one abnormal finding on initial ancillary testing: eight of 12 (67%) had an abnormal EEG, six of 12 (50%) had an abnormal MRI, and five of 12 (42%) demonstrated CSF pleocytosis. The single patient with a normal MRI, EEG, and CSF profile had low positive CSF NMDA antibody (titer of 1:1), and symptoms improved without immunotherapy. CONCLUSIONS:Isolated first-episode psychiatric symptoms in pNMDARE are uncommon, and the majority of children will exhibit additional neurodiagnostic abnormalities. Delaying immunotherapy in a child with isolated psychiatric symptoms and normal neurodiagnostic testing may be warranted while awaiting confirmatory antibody testing.
PMCID:11381152
PMID: 39094249
ISSN: 1873-5150
CID: 5757662
Risk of Seizure Recurrence Due to Autoimmune Encephalitis With NMDAR, LGI1, CASPR2, and GABABR Antibodies: Implications for Return to Driving
Rada, Anna; Hagemann, Anne; Aaberg Poulsen, Charlotte; Baumgartner, Tobias; Berki, Timea; Blaabjerg, Morten; Brenner, Juliette; Britton, Jeffrey W; Christiana, Andrew; Ciano-Petersen, Nicolás L; Crijnen, Yvette; Elišák, Martin; Farina, Antonio; Friedman, Alec R; Hayden, Zsófia; Hébert, Julien; Holtkamp, Martin; Hong, Zhen; Honnorat, Jerome; Ilyas-Feldmann, Maria; Irani, Sarosh R; Kovac, Stjepana; Marusic, Petr; Muñiz-Castrillo, Sergio; Ramanathan, Sudarshini; Smith, Kelsey M; Steriade, Claude; Strippel, Christine; Surges, Rainer; Titulaer, Maarten J; Uy, Christopher E; de Vries, Juna M; Bien, Christian G; Specht, Ulrich
BACKGROUND AND OBJECTIVES/OBJECTIVE:R). We hypothesized that after a seizure-free period of 3 months, patients with AIE have a seizure recurrence risk of <20% during the subsequent 12 months. This would render them eligible for noncommercial driving according to driving regulations in several countries. METHODS:R-AIE, who had been seizure-free for ≥3 months. We used Kaplan-Meier (KM) estimates for the seizure recurrence risk at 12 months for each antibody group and tested for the effects of potential covariates with regression models. RESULTS:R. DISCUSSION/CONCLUSIONS:Taking a <20% recurrence risk within 12 months as sufficient, patients with NMDAR-AIE and LGI1-AIE could be considered eligible for noncommercial driving after having been seizure-free for 3 months.
PMCID:11160480
PMID: 38838283
ISSN: 2332-7812
CID: 5757652
MRI Features and Their Association With Outcomes in Children With Anti-NMDA Receptor Encephalitis
Gombolay, Grace; Brenton, J Nicholas; Yang, Jennifer H; Stredny, Coral M; Kammeyer, Ryan; Otten, Catherine E; Vu, NgocHanh; Santoro, Jonathan D; Robles-Lopez, Karla; Christiana, Andrew; Steriade, Claude; Morris, Morgan; Gorman, Mark; Moodley, Manikum; Hardy, Duriel; Kornbluh, Alexandra B; Kahn, Ilana; Sepeta, Leigh N; Yeshokumar, Anusha; ,
OBJECTIVES:How brain MRI lesions associate with outcomes in pediatric anti-NMDA receptor encephalitis (pNMDARE) is unknown. In this study, we correlate T2-hyperintense MRI brain lesions with clinical outcomes in pNMDARE. METHODS:This was a multicenter retrospective cohort study from 11 institutions. Children younger than 18 years with pNMDARE were included. One-year outcomes were assessed by the modified Rankin Score (mRS) with good (mRS ≤2) and poor (mRS ≥3) outcomes. RESULTS:A total of 175 pNMDARE subjects were included, with 1-year mRS available in 142/175 (81%) and 60/175 (34%) had abnormal brain MRIs. The most common T2-hyperintense lesion locations were frontal, temporal, and parietal. MRI features that predicted poor 1-year outcomes included abnormal MRI, particularly T2 lesions in the frontal and occipital lobes. After adjusting for treatment within 4 weeks of onset, improvement within 4 weeks, and intensive care unit admission, MRI features were no longer associated with poor outcomes, but after multiple imputation for missing data, T2 frontal and occipital lesions associated with poor outcomes. DISCUSSION:Abnormal frontal and occipital lesions on MRI may associate with 1-year mRS in pNMDARE. MRI of the brain may be a helpful prognostication tool that should be examined in future studies.
PMCID:10219134
PMID: 37236807
ISSN: 2332-7812
CID: 5757642
Two cases of focal status epilepticus in pregnancy [Case Report]
Christiana, Andrew; Della Torre, Micaela; Serafini, Anna
The management of women with epilepsy (WWE) presents many challenges for physicians. The primary goal during pregnancy is to achieve the best possible control of seizures with the least adverse effects associated with exposure to antiseizure medications (ASMs). Even though the guidelines for managing pregnant WWE are expanding, no definitive guidelines exist for the treatment of status epilepticus (SE). Additionally, much of our data comes from the effect of generalized tonic clonic seizures on the fetus. There is very little data on the effect of focal seizures and even less on focal SE. Here we present two cases of pregnant WWE who presented in focal SE, who underwent simultaneous video-EEG monitoring and fetal heart tracing (FHT). During each focal seizure the FHT demonstrated a normal baseline heart rate with moderate variability. In the second case due to continuous seizures more aggressive treatment had to be started. This led to maternal relative autonomic instability as well as absent variability on FHT. These findings raise many questions on the management of focal SE during pregnancy including if the effect of treatment is worse than the seizures, and how do we balance our goals for both mother and fetus?
PMCID:8515390
PMID: 34693246
ISSN: 2589-9864
CID: 5344312