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Induction and Clinical Scoring of Chronic-Relapsing Experimental Autoimmune Encephalomyelitis
Published on: July 4, 2007
Autoimmune limbic encephalitis in 39 patients: immunophenotypes and outcomes
L Bataller1, K A Kleopa, G F Wu
1Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Journal of Neurology, Neurosurgery, and Psychiatry
|September 19, 2006
Summary
Many limbic encephalitis patients have CNS antibodies, including novel cell-membrane antigens. Antibodies to cell-membrane antigens, not just VGKC antibodies, are linked to favorable treatment responses in limbic encephalitis.
Area of Science:
- Neurology
- Immunology
- Neuroscience
Background:
- Approximately 40% of limbic encephalitis patients lack detectable central nervous system (CNS) antibodies.
- The frequency of immune-mediated limbic encephalitis in antibody-negative cases remains unknown.
Purpose of the Study:
- To characterize limbic encephalitis spectrum in a single institution and compare it with referred cases.
- To correlate clinical outcomes with autoantigen cellular location.
Main Methods:
- Prospective clinical case studies.
- Immunohistochemistry using rat brain, live hippocampal neurons, and HeLa cells expressing Kv potassium channels.
- Immunoblot analysis.
Main Results:
- 53% of institutionally identified patients had antibodies to known neuronal antigens (paraneoplastic or voltage-gated potassium channels [VGKCs]) or novel cell-membrane antigens (nCMAg).
- Across the entire series, 49% had antibodies to known antigens and 44% to nCMAg.
- Antibodies to cell-membrane antigens (VGKC or nCMAg) were significantly associated with treatment response (p = 0.003).
Conclusions:
- 82% of clinically identified limbic encephalitis patients possessed CNS antibodies.
- Treatment responsiveness extends beyond VGKC antibodies to include novel cell-membrane antigens.
- A significant proportion of patients (29% from one institution) had unknown autoantigens enriched in hippocampal neuronal cell membranes, associated with favorable outcomes.
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