Transplacental transfer of NMDA receptor antibodies in an infant with cortical dysplasia
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A confused and agitated 18-year-old woman presented to the emergency unit with orolingual movements, eye deviation, and a temperature of 38°C. The symptoms had begun 2 weeks prior to the admission when she developed a severe headache associated with pathologic laughing and intermittent episodes of upgaze deviation. A urine pregnancy test was positive and a transvaginal ultrasonography showed a 9-week-old fetus. An MRI of the brain was unremarkable and results of the CSF analysis were also unremarkable apart from a CSF pleocytosis (62 lymphocytes) and slightly elevated protein (55 mg/dL; normal range 0–45 mg/dL). Extensive microbiologic and serologic studies with CSF were all negative. She gradually lost consciousness, experienced respiratory failure, and was intubated. There were semirhythmic movements consisting of complex patterns of mouth opening, chewing, facial grimacing, synchronous flexion-extension, and supination-pronation limb movements, which persisted during the period of unresponsiveness. She also had generalized hyperreflexia, persistent hyperthermia, and a full bladder. Three EEGs showed diffuse slow waves with no epileptic discharges. A diagnosis of anti-NMDA receptor (NMDAR) encephalitis was made on clinical grounds and strongly positive serum NMDAR antibodies.
Acknowledgments
Acknowledgment: The authors thank Dr. Leslie Jacobson for testing the antibodies and their titrations.
Footnotes
Author contributions: Dr. Jagota: research project conception, organization, and execution, statistical analysis design and execution, writing of the first draft. A. Vincent: research project execution and manuscript review and critique. Dr. Bhidayasiri: research project organization and execution, statistical analysis review and critique, and manuscript review and critique.
Study funding: The Rachadaphiseksompot Endowment Fund of the “Strengthen CU's Researcher's Project” and Chulalongkorn University, Research Unit (RU) grant number GRU 56-012-30-002, to the Chulalongkorn Center of Excellence on Parkinson's Disease & Related Disorders, Bangkok, Thailand.
Disclosure: P. Jagota reports no disclosures relevant to the manuscript. A. Vincent and the University of Oxford hold patents and receive royalties and payments for antibody tests. R. Bhidayasiri serves as an associate editor of BMC Neurology and was an editor-in-chief of Thai Journal of Neurology, and receives funding from the Rachadaphiseksomphot Endowment Fund part of the “Strengthen CUs Researchers Project” and Research Unit (RU) grant of Chulalongkorn University, Bangkok, Thailand, royalties from International Neurology (Wiley-Blackwell) and Movement Disorders: A Video Atlas (Humana Press), and honoraria from Boehringer-Ingelheim, Glaxo-SmithKline, Abbott, and Novartis Pharmaceuticals. Go to Neurology.org for full disclosures.
- Received October 8, 2013.
- Accepted in final form January 24, 2014.
- © 2014 American Academy of Neurology
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