Opsoclonus-myoclonus syndrome in anti-N-methyl-D-aspartate receptor encephalitis.
BACKGROUND
Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis has been recently reported as autoimmune/paraneoplastic encephalitis, affecting mostly young females.


OBJECTIVE
To describe opsoclonus-myoclonus syndrome in association with anti-NMDAR antibodies.


METHODS
Case report.


METHODS
Geneva University Hospital. Patient A 23-year-old woman with opsoclonus-myoclonus syndrome.


RESULTS
Two weeks after an episode of gastroenteritis, the patient developed symptoms of depression associated with psychomotor slowing, progressive gait instability, and opsoclonus-myoclonus. Cerebrospinal fluid examination showed mild lymphocytic pleocytosis and intrathecal IgG synthesis with oligoclonal bands. The patient's condition worsened rapidly to an akinetic mutism, followed by a period of agitation, delirium, and hallucinations. These gradually subsided; however, a frontal behavior and executive dysfunction persisted 5 months after symptom presentation. No tumor was found. Anti-NMDAR antibodies were found in the cerebrospinal fluid.


CONCLUSIONS
Opsoclonus-myoclonus may occur in patients with anti-NMDAR encephalitis. Prompt diagnosis of this disorder is important because after tumor removal and immunomodulatory therapies it has a relatively good prognosis.
Kurian
Mary
M
Department of Neurosciences, Faculty of Medicine, University of Geneva, Geneva, Switzerland.
Lalive
Patrice H
PH
Dalmau
Josep O
JO
Horvath
Judit
J
D000405:Akinetic Mutism / Q000276:immunology; D001323:Autoantibodies / Q000032:analysis / Q000134:cerebrospinal fluid; D020274:Autoimmune Diseases of the Nervous System / Q000134:cerebrospinal fluid / Q000503:physiopathology*; D001526:Behavioral Symptoms / Q000276:immunology; D015415:Biomarkers / Q000032:analysis / Q000134:cerebrospinal fluid; D003072:Cognition Disorders / Q000276:immunology; D003693:Delirium / Q000276:immunology; D003866:Depressive Disorder / Q000276:immunology; D018450:Disease Progression; D004660:Encephalitis / Q000134:cerebrospinal fluid / Q000276:immunology* / Q000503:physiopathology*; D056344:Executive Function / Q000502:physiology; D005260:Female; D005625:Frontal Lobe / Q000503:physiopathology; D020233:Gait Disorders, Neurologic / Q000276:immunology; D005759:Gastroenteritis / Q000150:complications / Q000276:immunology; D006212:Hallucinations / Q000276:immunology; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D043025:Oligoclonal Bands; D053578:Opsoclonus-Myoclonus Syndrome / Q000134:cerebrospinal fluid / Q000276:immunology* / Q000503:physiopathology*; D016194:Receptors, N-Methyl-D-Aspartate / Q000276:immunology*; D016896:Treatment Outcome; D055815:Young Adult
D001323:Autoantibodies; D015415:Biomarkers; D016756:Immunoglobulins, Intravenous; D043025:Oligoclonal Bands; D016194:Receptors, N-Methyl-D-Aspartate