The mRS scores after treatment (1.7??1.7, mean??SD) were significantly decreased compared with those before treatment (4.2??0.9) in the 13 treated patients (P?0.005). antithyroid antibodies. Nineteen of the 78 patients had anti-NAE antibodies; however, 5 were excluded because they were double positive for antibodies to the VGKC complex including LGI1. No antibodies against the test. All of the statistical analyses were performed using SPSS Statistics ver. 17.0 (SPSS Japan Inc, Tokyo, Japan), and P?0.05 was considered significant. 3.?Results 3.1. Antibodies against the NAE, NMDAR, VGKC complex, LGI1, Caspr2, GABABR, and AMPAR1/2 We detected anti-NAE antibodies in the serum of 19 of the 78 (24%) patients with LE and suspected HE. Physique ?Figure1B1B shows representative immunoblotting findings for a recombinant amino NAE with patient sera (Cases 4 and 13). The anti-NAE antibody titers of the patients ranged from 1/320 to 1/40,960 (Table ?(Table1,1, Fig. ?Fig.1A).1A). Anti-NMDAR antibodies were not detected in any of the 19 patients during either of the examinations using the conventional methods and the cell-based assay kit, suggesting the reliability of the assay kit. Antibodies to the Caspr2, GABABR, and AMPAR1/2 were also not detected in any of the 19 patients. In contrast, 5 patients (26%) were positive for anti-VGKC complex antibodies in addition to anti-NAE antibodies. Four of the 5 patients who had anti-VGKC complex antibodies were also positive for anti-LGI1 antibodies. Table 1 Demographic and clinical features of patients with limbic encephalitis associated with anti-NAE antibodies. Open in a separate window 3.2. Clinical features To investigate the clinical findings of LE associated with anti-NAE antibodies, we excluded the 5 patients who were double-positive for anti-VGKC complex antibodies including anti-LGI1 antibodies because these additional antibodies could affect the clinical findings in these patients. The clinical features and outcomes of the remaining 14 patients who were positive only for anti-NAE antibodies are displayed in Tables ?Tables11 and ?and2,2, while those for the 5 double-positive patients are shown in Supplemental Table 1. Table 2 Demographic and clinical features of patients with limbic encephalitis associated with anti-NAE antibodies, classified into onset type. Open in a separate window The patients consisted of 5 men and 9 women, with a median age 62.5 years (range: 20C83). The duration between onset and admission varied, with a range from 1 day to 4 months. Eight of the 14 (57%) patients showed an acute onset of less than 2 weeks (Cases 1C8), whereas the other 6 patients showed a subacute onset of within 2 weeks to 4 months (Cases 9C14). Three patients had a history of Hashimoto thyroiditis and had been adequately treated. Among the 14 patients, consciousness disturbance (71%) and memory disturbance (64%) were frequently observed, Xanthopterin (hydrate) followed by psychiatric symptoms (50%) and seizures (43%) (Table ?(Table2).2). Psychiatric Tmem1 symptoms included hallucinations, delusions, mood changes, agitation, and abnormal behavior. All seizures observed in the patients were generalized convulsions. Involuntary movements and respiratory impairment were rare (14% and 7%, respectively). Interestingly, the frequencies of consciousness disturbance and seizures were significantly higher in the acute-onset group (100% and 75%, respectively) than in the subacute-onset group (33% and 0%, respectively) (P?0.05 and?0.01, respectively). In contrast, psychiatric symptoms and memory disturbance were more frequently observed in the subacute-onset group (83% and 100%, respectively) than in the acute-onset group (25% and 38%, respectively) (P?=?0.10 and <0.05, respectively). These findings suggest that symptoms of LE associated with anti-NAE antibodies are not uniform and may differ depending on onset type. Regarding thyroid function, all 14 patients were clinically euthyroid except for 1 patient with low T4 who presented with no symptoms related to hypothyroidism. Hyponatremia, defined as a serum Xanthopterin (hydrate) sodium concentration of less than 135?mEq/L, which is frequently observed in LE associated with anti-LGI1 antibodies, was observed in Xanthopterin (hydrate) 6 (43%) patients at admission (Table ?(Table2).2). When the CSF was examined, some type of abnormality was observed in 12 of 13 patients (92%). The cell counts and protein concentrations in the CSF ranged from.