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• W1998529061 abstract "There is increasing recognition of immune-mediated encephalitides resulting in seizures and status epilepticus. These disorders can be divided into limbic and cortical extralimbic encephalitides and may have a paraneoplastic or nonparaneoplastic etiology (Tuzun & Dalmau, 2007; Dalmau & Rosenfeld, 2008). Table 1 shows the most frequent antibodies identified in autoimmune encephalitides that may result in seizures and status epilepticus. In addition, there are other encephalitides that are likely immune mediated, but at the present time the associated syndrome or antibody specificity is unclear. These disorders are often defined with descriptive terms (e.g., steroid-responsive limbic encephalitis). Any paraneoplastic encephalitis involving the limbic system or cerebral cortex may result in seizures and status epilepticus. The associated antibodies include Hu, Ma2, CV2/CRMP5 (collapsin response mediator protein-5), and amphiphysin (Dalmau & Rosenfeld, 2008). Although there is strong evidence that the first three immune responses are mediated by cytotoxic T-cell responses, there are studies indicating that amphiphysin antibodies may be directly pathogenic (Sommer et al., 2005). Of these four immune responses, the anti-Hu antibodies are those most frequently described with seizures, epilepsia partialis continua, and status epilepticus (Shavit et al., 1999; Jacobs et al., 2003). The underlying tumors are small cell lung cancer (all antibodies), germ-cell tumors of the testis (Ma2), and thymoma (CRMP5). With the exception of encephalitis associated with Ma2 antibodies, in which approximately 30% of patients respond to tumor removal and immunotherapy, the other disorders are rarely treatment responsive (Dalmau & Rosenfeld, 2008). These include all the immune responses of Table 1 that are not in italics. A frequent feature of these immune responses [except for glutamic acid decarboxylase (GAD) and thyroid antibodies] is that the autoantigens are extracellular and, therefore, accessible to circulating antibodies. Although the presence of antibodies to GAD, voltage-gated potassium channels (VGKCs), or the NR1subunit of the N-methyl-d-aspartate receptor (NMDAR) associates with a limited set of syndromes and, therefore, provides useful diagnostic tests, the clinical significance of the other antibodies is unclear. For example, some antibodies of patients with lupus (e.g., directed to DWEYS consensus sequence of NR2B and NR2B) provide interesting animal models of brain autoimmunity, but their significance in human disease is unclear (Steup-Beekman et al., 2007; Fragoso-Loyo et al., 2008). GAD antibodies usually associate with nonparaneoplastic stiff-person syndrome and cerebellar dysfunction, but there are increasing numbers of reports showing that these antibodies also associate with subtypes of limbic encephalitis and refractory epilepsy (Saiz et al., 2008). VGKC antibodies associate with limbic encephalitis, Morvan’s syndrome, and neuromyotonia (Thieben et al., 2004; Vincent et al., 2004). Seizures and status epilepticus may occur in the first two disorders. The frequent development of hyponatremia may also favor seizures. About 20% of patients with VGKC antibodies have thymoma, small cell lung cancer, or, less frequently, other tumors. The disorder usually responds to corticosteroids, intravenous immunoglobulin, or plasma exchange. Antibodies to the NR1 subunit (or NR1/NR2 heteromers) of the NMDAR associate with a characteristic syndrome that presents with behavioral change or psychosis and usually progresses to a decline of the level of consciousness, catatonia, dyskinesias, autonomic instability, and frequent hypoventilation (Dalmau et al., 2008). Partial or generalized seizures occur frequently at early stages of the disease and in some patients may persist along the entire clinical course or at relapses (Niehusmann et al., 2009). The combination of true epileptic seizures and complex, elaborate orofacial and limb movements [without electroencephalography (EEG) correlates] complicates the clinical recognition of the seizures. EEG shows focal or diffuse slow activity with a poorly organized background, with or without epileptic activity. The disorder usually affects young women, 60% with ovarian teratoma, but it is being increasingly recognized in men and children. Less than 45% of girls have an underlying teratoma. Despite the severity and duration of the disorder, patients often respond to immunotherapy and, when appropriate, tumor removal; some patients improve spontaneously. The recovery is slow and may take many months. After recovery, most patients remain free of seizures. A recent study on limbic encephalitis showed that 64% of patients had antibodies to neuronal cell surface antigens, including VGKC and NMDAR, but in 24% the identity of the antigens was unknown (Graus et al., 2008). The fact that these types of antigens are localized in specific brain regions, such as hippocampus, and the epitopes are extracellular, suggest that the associated symptoms, including seizures, are potentially responsive to immunotherapy. An example is the recent characterization of one of these antigens as the GluR1/2 subunits of the alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor (AMPAR). Patients with these antibodies develop encephalitis that closely resembles a classical picture of limbic encephalopathy with frequent seizures. The disorder is responsive to immunotherapy, but has a tendency to relapse. In 7 of 10 patients the disorder was a paraneoplastic manifestation of tumors of the thymus, lung, or breast (Lai et al., 2009). In summary, recent studies in the field of paraneoplastic syndromes and autoimmune encephalitides provide several clues that suggest the autoimmune etiology of some seizure disorders that may result in status epilepticus, including the acute presentation of symptoms, the frequent detection of cerebrospinal fluid (CSF) pleocytosis and oligoclonal bands in the context of negative viral studies, and the detection of CSF antibodies reacting with the neuropil of hippocampus and the cell surface of neurons. I confirm that I have read the Journal’s position on issues involved in ethical publication and affirm that this report is consistent with those guidelines. Disclosure: The author declares no conflicts of interest." @default.
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• W1998529061 date "2009-11-18" @default.
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• W1998529061 title "Status epilepticus due to paraneoplastic and nonparaneoplastic encephalitides" @default.
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• W1998529061 doi "https://doi.org/10.1111/j.1528-1167.2009.02352.x" @default.
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