文摘

Acquired diseases classically associated with VGKC-complex antibodies include peripheral nerve hyperexcitability (PNH), Morvan’s syndrome, limbic encephalitis (LE), and epilepsy. However, not all such patients have VGKC-complex antibodies and antibodies have been reported in patients without a defined immune-mediated syndrome. To analyse the clinical relevance of positive VGKC-complex antibodies requested on the basis of initial clinical suspicion. We retrospectively analysed patients with positive VGKC-complex antibodies (>100?pM) referred to our institution between 2001 and 2011.?1,614 VGKC-complex assays were performed in 1,298 patients. Titres >100?pM were detected in 57/1,298 (4?%) patients. A classic VGKC-complex channelopathy (60?%) was associated with VGKC-complex antibody titres >400?pM (p?=?0.0004). LGI1 or CASPR2 antibodies were only detected in classic VGKC-complex channelopathies (LE; n?=?3/4 and PNH; n?=?1/5). VGKC-complex antibody titres n?=?15/22; 68?%) but also a heterogeneous range of central and/or peripheral nervous system disorders. Electromyography was supportive of PNH in 65?% of cases and symptomatic treatment was beneficial in 46?% of patients. Irrespective of titre, the rate of malignancy in patients with VGKC-complex antibodies was higher than the age-matched national incidence of malignancy (OR 19.9, 95?% CI 8.97-4.0 p400?pM can help determine VGKC-complex antibody relevance. Antibody titres