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

被引:28
作者
Najjar, Souhel [1 ,2 ]
Pearlman, Daniel [1 ,6 ]
Devinsky, Orrin [1 ,2 ,4 ,5 ]
Najjar, Amanda [3 ]
Nadkarni, Siddhartha [1 ,2 ,5 ]
Butler, Tracy [1 ,2 ]
Zagzag, David [3 ]
机构
[1] NYU, Comprehens Epilepsy Ctr, New York, NY 10016 USA
[2] NYU, Sch Med, Dept Neurol, New York, NY 10016 USA
[3] NYU, Sch Med, Div Neuropathol, Dept Pathol, New York, NY 10016 USA
[4] NYU, Sch Med, Dept Neurosurg, New York, NY 10016 USA
[5] NYU, Sch Med, Dept Psychiat, New York, NY 10016 USA
[6] Dartmouth Coll, Dartmouth Inst Hlth Policy & Clin Practice, Geisel Sch Med, Lebanon, NH 03756 USA
关键词
neuroinflammation; autoimmune; psychosis; encephalitis; seronegative; LIMBIC ENCEPHALITIS; BIPOLAR DISORDER; GLUTAMATE TRANSPORTER; RECEPTOR ANTIBODIES; PREFRONTAL CORTEX; MESSENGER-RNA; SCHIZOPHRENIA; BRAIN; DEPRESSION; EXPRESSION;
D O I
10.1097/WNN.0b013e31828b6531
中图分类号
B84 [心理学]; C [社会科学总论]; Q98 [人类学];
学科分类号
03 ; 0303 ; 030303 ; 04 ; 0402 ;
摘要
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.
引用
收藏
页码:36 / 49
页数:14
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