TAILIEUCHUNG - Chapter 097. Paraneoplastic Neurologic Syndromes (Part 3)

Neuronal cell-surface antigens can be the target of antibodies in some patients with paraneoplastic encephalitis. A few of these antigens have been identified, including the NR1/NR2 subunits of NMDA receptors (Fig. 97-1) and voltage-gated potassium channels (VGKC). These disorders are more responsive to immunotherapy than those associated with immune responses to intracellular antigens. Figure 97-1 Antibodies to NR1/NR2 subunits of the NMDA receptor in a patient with paraneoplastic encephalitis and ovarian teratoma. Panel A is a section of dentate gyrus of rat hippocampus immunolabeled (brown staining) with the patient's antibodies. The reactivity predominates in the molecular layer, which is highly enriched. | Chapter 097. Paraneoplastic Neurologic Syndromes Part 3 Neuronal cell-surface antigens can be the target of antibodies in some patients with paraneoplastic encephalitis. A few of these antigens have been identified including the NR1 NR2 subunits of NMDA receptors Fig. 97-1 and voltage-gated potassium channels VGKC . These disorders are more responsive to immunotherapy than those associated with immune responses to intracellular antigens. Figure 97-1 Antibodies to NR1 NR2 subunits of the NMDA receptor in a patient with paraneoplastic encephalitis and ovarian teratoma. Panel A is a section of dentate gyrus of rat hippocampus immunolabeled brown staining with the patient s antibodies. The reactivity predominates in the molecular layer which is highly enriched in dendritic processes. Panel B shows the antibody reactivity with cultures of rat hippocampal neurons the intense green immunolabeling is due to the antibodies against the NR1 NR2 subunits of NMDA receptors. Only four of the antibodies listed in Table 97-2 have been shown to play a direct pathogenic role in PNDs all produce distinctive disorders of the peripheral nervous system. These are antibodies to P Q-type voltage-gated calcium channels VGCC in patients with the Lambert-Eaton myasthenic syndrome LEMS antibodies to acetylcholine receptors in patients with myasthenia gravis antibodies to VGKC in some patients with peripheral nerve hyperexcitability neuromyotonia and antibodies to ganglionic acetylcholine receptors in some patients with autonomic neuropathy. Common features of these four antibodies are that they target cell-surface molecules and that their passive transfer to animals reproduces the disorders. Plasma exchange or immunomodulation with intravenous immunoglobulin IVIg usually produces neurologic .

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