NMDA receptor antibodies associated with distinct white matter syndromes
| Autor: | Angela Vincent, Sarosh R. Irani, Michael Absoud, Yael Hacohen, Leslie Jacobson, Ming K. Lim, Jean-Pierre Lin, Sunil P. Pullaperuma, Patrick Waters, Mike Pike, Camilla Buckley, Evangeline Wassmer, Ata Siddiqui, Cheryl Hemingway |
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| Jazyk: | angličtina |
| Rok vydání: | 2014 |
| Předmět: |
Pathology
medicine.medical_specialty biology business.industry medicine.medical_treatment Disease Immunotherapy medicine.disease Article Myelin oligodendrocyte glycoprotein White matter Leukoencephalopathy medicine.anatomical_structure Neurology Immunology medicine biology.protein Neurology (clinical) Antibody business Encephalitis Neuroinflammation |
| Zdroj: | Neurology® Neuroimmunology & Neuroinflammation |
| ISSN: | 2332-7812 |
| Popis: | Objective: To report the clinical and radiologic findings of children with NMDA receptor (NMDAR) antibodies and white matter disorders. Method: Ten children with significant white matter involvement, with or without anti-NMDAR encephalitis, were identified from 46 consecutive NMDAR antibody–positive pediatric patients. Clinical and neuroimaging features were reviewed and the treatment and outcomes of the neurologic syndromes evaluated. Results: Three distinct clinicoradiologic phenotypes were recognized: brainstem encephalitis (n 5 3), leukoencephalopathy following herpes simplex virus encephalitis (HSVE) (n 5 2), and acquired demyelination syndromes (ADS) (n5 5); 3 of the 5 with ADS had myelin oligodendrocyte glycoprotein as well as NMDAR antibodies. Typical NMDAR antibody encephalitis was seen in 3 patients remote from the first neurologic syndrome (2 brainstem, 1 post-HSVE). Six of the 7 patients (85%) who were treated acutely, during the original presentation with white matter involvement, improved following immunotherapy with steroids, IV immunoglobulin, and plasma exchange, either individually or in combination. Two patients had escalation of immunotherapy at relapse resulting in clinical improvement. The time course of clinical features, treatments, and recoveries correlated broadly with available serum antibody titers. Conclusion: Clinicoradiologic evidence of white matter involvement, often distinct, was identified in 22% of children with NMDAR antibodies and appears immunotherapy responsive, particularly when treated in the acute phase of neurologic presentation. When observed, this clinical improvement is often mirrored by reduction in NMDAR antibody levels, suggesting that these antibodies may mediate the white matter disease. Neurol Neuroimmunol Neuroinflammation 2014;1:e2; doi |
| Databáze: | OpenAIRE |
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