Late relapse of anti-N-methyl-d-aspartate receptor encephalitis with amusia and transiently reduced uptake in 123I-iomazenil single-photon emission computed tomography

Yamada, Naoki; Kuki, Ichiro; Hattori, Taeka; Yamamoto, Naohiro; Nagase, Shizuka; Nukui, Megumi; Inoue, Takeshi; Okazaki, Shin; Kawawaki, Hisashi; Horino, Asako; Sakuma, Hiroshi

doi:10.1016/j.braindev.2022.05.003

Cited by 2 publications

(1 citation statement)

References 12 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Several antibodies have been detected in a subset of pediatric patients with AE ( 3 , 10–13 ). Among these, the most common extracellular antibodies in pediatric patients target N-methyl-D-aspartate receptor (NMDAR) ( 1 , 13–15 ) and myelin oligodendrocyte glycoprotein (MOG) ( 16–18 ). However, it is essential to note that not all pediatric patients with AE have known autoantibodies, and the diagnosis of antibody-negative AE in pediatric patients can be challenging because of their diverse clinical features, often leading to delays in therapeutic interventions ( 3 ).…”

Section: Introductionmentioning

confidence: 99%

Higher incidence of acute symptomatic seizures in probable antibody-negative pediatric autoimmune encephalitis than in major antibody-positive autoimmune encephalitis

Yamada,

Inoue,

Kuki

et al. 2024

Front. Neurol.

Self Cite

View full text Add to dashboard Cite

PurposeTo delineate the characteristics of probable antibody-negative pediatric autoimmune encephalitis (probable Ab-negative AE), we compared the clinical features of probable Ab-negative AE to those of major antibody-positive AE.MethodsWe retrospectively reviewed the clinical features of 18 patients with probable Ab-negative AE, 13 with anti-N-methyl-D-aspartate receptor encephalitis (NMDARE), and 13 with myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). Clinical characteristics, neuroimaging findings, treatments, and outcomes were analyzed.ResultsThe age of onset and length of hospital stay were significantly higher in the NMDARE group than in the other groups (p = 0.02 and p < 0.01). Regarding initial neurological symptoms, acute symptomatic seizures in the probable Ab-negative AE group (67%) were significantly more frequent than in the NMDARE (15%) and MOGAD (23%) groups (p < 0.01). Paraclinical evidence of neuroinflammation within 1 month of disease onset revealed that single-photon emission computed tomography (SPECT) detected abnormal alterations in 14/14 (100%), cerebrospinal fluid (CSF) analysis in 15/18 (83%), and magnetic resonance imaging (MRI) in 11/18 (61%) in patients with probable Ab-negative AE. In the probable Ab-negative AE group, seven patients (39%) developed autoimmune-associated epilepsy, whereas one patient (8%) had both NMDARE and MOGAD (not statistically significant, p = 0.07).ConclusionPatients with probable Ab-negative AE exhibited acute symptomatic seizures as initial neurological symptoms significantly more frequently. They developed autoimmune-associated epilepsy more frequently than those with NMDARE and MOGAD, which was not statistically significant. SPECT within 1 month of disease onset might be a valuable surrogate marker of ongoing neuroinflammation and neuronal dysfunction, even in patients with negative MRI findings.

show abstract