Anti-N-Methyl-D-Aspartate Receptor (NMDAR) Encephalitis in Children and Adolescents

Anti-N-Methyl-D-Aspartate Receptor (NMDAR) Encephalitis in Children and Adolescents

July 2009 | Nicole R. Florance, MD, Rebecca L. Davis, BA, Christopher Lam, MD, Christina Szperka, MD, Lei Zhou, BS, Saba Ahmad, MD, Cynthia J. Campen, MD, Heather Moss, MD, PhD, Nadja Peter, MD, Amy J. Gleichman, BS, Carol A. Glaser, MD, David R. Lynch, MD, PhD, Myrna R. Rosenfeld, MD, PhD, Josep Dalmau, MD, PhD
Anti-NMDAR encephalitis is a rare autoimmune disorder characterized by antibodies against the NMDA receptor, primarily affecting children and adolescents. A study identified 81 patients over an 8-month period, with 32 (40%) under 18 years old. Among these, 56% of women over 18 years old had ovarian teratomas, while only 9% of girls under 14 years old did. None of the male patients had tumors. Common symptoms included behavioral changes, speech deficits, seizures, and abnormal movements. Most patients showed improvement with immunotherapy or tumor removal, with 74% achieving full or substantial recovery. Neurological relapses occurred in 25% of cases. The disorder is increasingly recognized in children, with clinical features similar to adults but with less frequent or severe autonomic dysfunction. The diagnosis involves immunohistochemical and ELISA testing for NMDAR antibodies. Treatment includes immunotherapy and tumor removal, with recovery often taking months. The study highlights the importance of early diagnosis and multidisciplinary management. Despite progress, many questions remain about the underlying mechanisms and optimal treatment strategies.Anti-NMDAR encephalitis is a rare autoimmune disorder characterized by antibodies against the NMDA receptor, primarily affecting children and adolescents. A study identified 81 patients over an 8-month period, with 32 (40%) under 18 years old. Among these, 56% of women over 18 years old had ovarian teratomas, while only 9% of girls under 14 years old did. None of the male patients had tumors. Common symptoms included behavioral changes, speech deficits, seizures, and abnormal movements. Most patients showed improvement with immunotherapy or tumor removal, with 74% achieving full or substantial recovery. Neurological relapses occurred in 25% of cases. The disorder is increasingly recognized in children, with clinical features similar to adults but with less frequent or severe autonomic dysfunction. The diagnosis involves immunohistochemical and ELISA testing for NMDAR antibodies. Treatment includes immunotherapy and tumor removal, with recovery often taking months. The study highlights the importance of early diagnosis and multidisciplinary management. Despite progress, many questions remain about the underlying mechanisms and optimal treatment strategies.
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