Abstract
BackgroundAnti-N-methyl-D-aspartate receptor encephalitis is a rare autoimmune disease characterized by severe neurological and psychiatric symptoms and a difficult diagnosis.The disease is often secondary to a neoplastic lesion, seldom diagnosed years later. Psychiatric symptoms are prevalent in adults; neurologic symptoms are more evident in children, who typically present primarily with neurological symptoms. To the best of our knowledge, the association with juvenile idiopathic arthritis has not been described.Case presentationWe report the cases of two caucasian girls with an atypical presentation.The first patient was an 8-year-old girl with normal psychomotor development. Over a 4-month period she developed behavioral problems, speech impairment, and deterioration in academic skills. Within 8 months from the onset of symptoms, choreic movements gradually appeared. Hematological, neuroradiological, and neurophysiological examinations were negative; however, her symptoms worsened and treatment with prednisone was started. Although her choreic movements improved within 1 month, her neuropsychological and behavioral symptoms continued. Anti-N-methyl-D-aspartate receptor antibodies in cerebrospinal fluid and in blood were detected. Therapy with intravenously administered immunoglobulins was administered, without improvement of symptoms. After 2 months of steroid treatment, she suddenly started to pronounce some words with a progressive improvement in language and behavior.The second patient was a 14-year-old girl with classic anti-N-methyl-D-aspartate receptor encephalitis, treated successfully with intravenously administered immunoglobulins and methylprednisolone, followed by orally administered prednisone, who developed chronic arthritis of the hip. The arthritis was confirmed by magnetic resonance imaging and associated to antinuclear antigen antibody positivity.One year after the encephalitis presentation, an ovarian cystic mass was identified as a teratoma. The surgical resection of the mass was followed by the resolution of the psychotic spectrum and arthritis.ConclusionsAnti-N-methyl-D-aspartate receptor encephalitis in pediatric patients can present initially with neuropsychological and behavioral symptoms.In the literature, the association of anti-N-methyl-D-aspartate receptor encephalitis with juvenile idiopathic arthritis is not yet described: to the best of our knowledge, this is the first case reported. The link to a neoplastic lesion can explain the favorable course of encephalitis and arthritis, after the surgical resection of the mass. Early diagnosis and treatment can improve the patient’s outcome.
Highlights
Anti-N-methyl-D-aspartate receptor encephalitis is a rare autoimmune disease characterized by severe neurological and psychiatric symptoms and a difficult diagnosis
The link to a neoplastic lesion can explain the favorable course of encephalitis and arthritis, after the surgical resection of the mass
Speech was restored and she was completely asymptomatic and no further relapses occurred. She started a 3-day course of high-dose pulse intravenous methylprednisolone (30 mg/kg per day), followed by orally administered prednisone (2 mg/kg per day weaned over 3 months) and intravenously administered immunoglobulin (IVIG) 400 mg/kg per day for 5 days
Summary
Many patients are first followed by psychiatrists: pediatricians and psychiatrists must consider anti-NMDAR encephalitis as a possible cause of acute psychosis in children and adolescents without a history of psychiatric events [5, 8]. In these patients, an accurate follow-up must exclude an occult neoplasm; the diagnostic pattern must include imaging with neoplastic biomarkers (such as CA125, CA19.9) for the identification of the most common neoplasm: ovarian teratoma [11].
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