First-line immunotherapy (steroids, IVIg), with escalation to rituximab in resistant cases, and anti-NMDAR antibody-positive AE with features of Kluver-Bucy syndrome are reported.
Abstract
ABSTRACT
Autoimmune encephalitis (AE) is a heterogeneous group of immune-mediated disorders of the central nervous system characterized by neuropsychiatric symptoms, seizures, cognitive decline, and movement abnormalities. Timely recognition and initiation of immunotherapy are critical to improving outcomes. We report three cases of AE presenting with varied clinical phenotypes from the Neurology department of Madras Medical College. The first, a 44-year-old male, presented with altered sensorium, action tremor, and myokymia, and was diagnosed with LGI1 and CASPR2 antibody-positive AE. The second, a 16-year-old girl, presented with seizures, progressive cognitive decline, behavioral disturbance, and focal weakness, confirmed as anti-NMDAR encephalitis. The third, a 14-year-old boy, developed seizures and behavioral changes following herpes simplex encephalitis and was later confirmed to have anti-NMDAR antibody-positive AE with features of Kluver-Bucy syndrome. All patients received first-line immunotherapy (steroids, IVIg), with escalation to rituximab in resistant cases.
Rationale: Autoimmune encephalitis in children often presents with neuropsychiatric symptoms and seizures and may progress to secondary epilepsy with prolonged functional impairment. Immunotherapy is the principal disease-modifying treatment, whereas comprehensive nursing care supports safety, complication prevention,...
During disease progression, innovative sequential therapy with efgartigimod, an FcRn antagonist, was followed by the anti-CD20 monoclonal antibody rituximab (RTX) to reduce the risk of profound B-cell depletion, thereby demonstrating synergistic efficacy.
Jun-Tong Liu, Wen-Ping Zhu, Lan Gao et al.· Frontiers in Immunology· 1 citation
Objective: Among autoimmune limbic encephalitis syndromes, glutamic acid decarboxylase 65 (GAD65) antibody-associated syndromes represent a relatively rare and clinically heterogeneous subtype characterized by prominent temporal lobe involvement and diverse neurological manifestations. GAD65 is an intracellular antigen...
Matthew S. Phillips, Malorie E. Watson, Brian Ramanauskas et al.· Clinical Neuropsychologist· 0 citations
Background Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is the commonest form of autoimmune encephalitis of childhood. However, little information is captured with formal neuropsychological testing. When the pre-illness history is incomplete, it is also hard to judge how much of the residual picture reflects...
Felipe Dalvi-Garcia, Iolanda de Salles Fonseca Carvalho, Julia Valeriano de Almeida et al.· Brain, Behavior, & Immunity...· 0 citations
Influenza-associated encephalitis (IAE) is a rare but serious complication of influenza infection, predominantly affecting children. While most reported cases present with moderate to severe neurological symptoms, mild and self-limiting forms remain underrecognized. We report the case of a previously healthy 9-year- ol...
Seyedeh Rojina Mortazavi, Dina Sadeghi, Solmaz Aziz-ahari· Case Reports in Clinical Pra...· 0 citations
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