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Neuropsychiatric Autoimmune Encephalitis without VGKC-Complex, NMDAR, and GAD Autoantibodies: Case Report and Literature Review

Najjar, Souhel MD*,†; Pearlman, Daniel BA*,‡; Devinsky, Orrin MD*,†,§,∥; Najjar, Amanda; Nadkarni, Siddhartha MD*,†,∥; Butler, Tracy MD*,†; Zagzag, David MD, PhD

Cognitive & Behavioral Neurology: March 2013 - Volume 26 - Issue 1 - p 36–49
doi: 10.1097/WNN.0b013e31828b6531
Case Reports

We report a patient with a seronegative autoimmune panencephalitis, adding a subtype to the emerging spectrum of seronegative autoimmune encephalitis, and we review the sparse literature on isolated psychiatric presentations of autoimmune encephalitis. (A PubMed search for “seronegative autoimmune encephalitis,” “nonvasculitic autoimmune inflammatory meningoencephalitis,” and related terms revealed <25 cases.) A 15-year-old girl developed an acute-onset isolated psychosis with prominent negative symptoms and intermittent encephalopathy. Despite clinical worsening, her brain magnetic resonance imaging (MRI) scans remained normal for 7 years. Serology was negative for voltage-gated potassium channel (VGKC)-complex, N-methyl-D-aspartate receptor (NMDAR), and glutamic acid decarboxylase (GAD) autoantibodies. We excluded genetic, metabolic, paraneoplastic, degenerative, and infectious etiologies. The patient’s symptoms remitted fully with immune therapy, but recurred in association with widespread bihemispheric brain lesions. Brain biopsy revealed mild nonvasculitic inflammation and prominent vascular hyalinization. Immune therapy with plasma exchanges cleared the MRI abnormalities but, 10 years after onset, the patient still suffers neuropsychiatric sequelae. We conclude that autoimmune panencephalitis seronegative for VGKC-complex, NMDAR, and GAD autoantibodies is a subtype of autoimmune encephalitis that can present with pure neuropsychiatric features and a normal brain MRI. Immunologic mechanisms may account for psychiatric symptoms in a subset of patients now diagnosed with classical psychotic disorders. Delay in starting immune therapy can lead to permanent neuropsychiatric sequelae. We propose a standardized classification system for the autoimmune encephalitides, integrating earlier pathology-oriented terms with more recently defined serologic and clinical phenotypes.

*New York University Comprehensive Epilepsy Center

Department of Neurology

Division of Neuropathology, Department of Pathology

§Department of Neurosurgery

Department of Psychiatry, New York University School of Medicine, New York, NY

Geisel School of Medicine at Dartmouth College, The Dartmouth Institute for Health Policy and Clinical Practice, Lebanon, NH

The authors declare no conflicts of interest.

Reprints: Souhel Najjar, MD, New York University Comprehensive Epilepsy Center, 223 E 34th St, New York, NY 10016 (e-mail: mna1024231@aol.com).

Received October 23, 2011

Accepted October 9, 2012

© 2013 Lippincott Williams & Wilkins, Inc.