Case Rep Neurol 2014;6:38?43
DOI: 10.1159/000358820
Published online: February 8, 2014
? 2014 S. Karger AG, Basel
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Salvatore Monaco, MD
Department of Neurological and Movement Sciences
University of Verona, Policlinico G.B. Rossi, P. le L.A. Scuro 10
IT?37134 Verona (Italy)
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[see article]
Anti-N-Methyl-D-Aspartate Receptor Encephalitis Causing a Prolonged Depressive Disorder Evolving to Inflammatory Brain Disease
Sara Mariottoa Stefano Tamburina Alessandro Salviatia Sergio Ferraria
Marco Zoccaratoc Bruno Giomettoc Laura Bertolasia Franco Alessandrinib Maria Donata Benedettia Salvatore Monacoa aDepartment of Neurological and Movement Sciences, University of Verona, and bDivision of Neuroradiology, AOUI, Verona, and cDepartment of Neurology, Regional Hospital ?Ca? Foncello?, Treviso, Italy
Key Words
Anti-N-methyl-D-aspartate receptor encephalitis ? Optic neuritis ? Depressive disorder
Abstract
Background: Anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis is a rapidly evolving condition that combines psychiatric and neurologic manifestations. Much remains unclear about its clinical onset and subsequent course. Although successful treatment depends on diagnosing the disorder early and therefore minimizing long-term complications, this is a difficult task owing to the atypical onset of this condition and the prolonged clinical course that has been observed in some patients. This report, illustrating a patient with slowly progressing psychiatric manifestations, unusual imaging and electrophysiological features, extends the information on varied clinical phenotypes.
Case Report: A 32-year-old woman suffered from an isolated depressive disorder for 4 months before undergoing psychiatric evaluation. During the following 5 months, she manifested hypersexuality, dysarthria, imbalance, dyskinesias and decreased word output. Brain magnetic resonance
imaging (MRI) showed multifocal hyperintense T2/FLAIR lesions, a few contrast-enhanced, involving the corona radiata, the periventricular white matter, the deep gray nuclei, the optic nerves and the brainstem. MRI spectroscopy disclosed confluent bilateral demyelination and focal optic nerve involvement suggesting widespread encephalitis. Visual evoked potential
studies indicated a demyelinating disorder. Serological screening and total body positronemission tomography yielded negative findings for malignancies. Cerebrospinal fluid examination disclosed IgG oligoclonal bands and nti-NMDAR antibodies. Corticosteroids and intravenous immunoglobulin provided only slight improvement, whereas switching to cyclophosphamide markedly improved her neurological status.
Conclusion: In patients with a prolonged clinical course, including psychiatric and neurological symptoms, the differential diagnosis should be anti-NMDAR encephalitis. This report expands the known disease phenotypes in this emerging condition. ? 2014 S. Karger AG, Basel
so they took her down to Oregon Health and Science University in Portland where she was finally diagnosed. (2 other girls had been recently treated there.)