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Antibodies to voltage-gated potassium channels (VGKC) are associated with acquired neuromyotonia, limbic encephalitis, and Morvan's syndrome. The antibodies are often not associated with malignancy and have shown good clinical response to immunomodulatory therapies. A record review identified five patients with laboratory evidence of antibodies to VGKC who underwent plasma exchange (PE) as part of their immunosuppressive therapy for neurologic disease. Four of the patients presented with limbic encephalitis and one with neuromyotonia. Symptoms included memory impairment, seizures, and personality changes. All PE were 1.0 volume and were performed on an every-other-day schedule. Replacement fluid was 5% normal serum albumin except when a bleeding risk was identified and then fresh frozen plasma was added. Four of five patients were also receiving concurrent immunosuppressive therapy including corticosteroids. Of the five patients treated with PE, three had sustained improvement in symptoms for 6-17 months following PE. Two patients did not have signs of improvement at a limited follow-up. One patient had recurrence of her symptoms, which responded to additional PE. These cases, as well as the reports in the literature, suggest that PE could be a useful adjunctive therapy for patients with VGKC antibodies and neurologic symptoms.

Morvan's syndrome is a rare disorder characterized by neuromyotonia, hyperhidrosis, and central nervous system dysfunction. We report a patient with features of this syndrome, but who initially presented with breathing difficulties. Concentric needle electromyography showed an abundance of myokymic and neuromyotonic discharges. Exercise tests and repetitive nerve stimulation showed a decrement-increment response of compound muscle action potentials. Antibodies against voltage-gated potassium channels were not detected on repeated testing, but the presence of oligoclonal bands in the cerebrospinal fluid (CSF) suggested an autoimmune etiology. At follow-up over 3 years, no cancer was found. Electrophysiological in vitro studies of effects of patient serum and CSF on rat nerves provided no evidence of altered voltage-gated sodium or potassium conductances. We conclude that putative humoral factors do not block ion channels acutely but may cause channel dysfunction with chronic exposure.

Limbic encephalitis (LE) is often associated with lung, thymic, or testicular tumours and antibodies to Hu, CV2, or Ma2 (Ta) antigens. In these cases, it generally has a poor prognosis. Here we describe two patients with symptoms of LE, negative for typical paraneoplastic antibodies, in whom antibodies to voltage-gated potassium channels (VGKC) were detected retrospectively in serial serum samples. Patient 1 had a thymoma recurrence, but in patient 2 no tumour has been detected in the years following presentation. Plasma exchange was effective in reducing VGKC antibody levels, with substantial improvement in mental symptoms in patient 1. In patient 2, the VGKC antibodies fell spontaneously over two years, with almost complete recovery of mental function. Although neither patient had obvious neuromyotonia at presentation, both showed excessive secretions. We suggest that patients with limbic symptoms and excessive secretions should be tested for VGKC antibodies, and, if they are present, prompt and effective immunosuppressive treatment should be considered.