Objective To describe the clinical spectrum,especially sleep disorder in three patients diagnosed with Morvan syndrome.Methods Three consecutive patients were identified with Morvan syndrome in the Department of Neurology, Peking Union Medical College Hospital between December 2014 and March 2016.The character in three cases has been studied from several aspects such as clinical presentation, imaging, polysomnography (PSG), cerebrospinal fluid and serum.Results Serum test showed serum contactin-associated protein 2 (CASPR2)antibodies strongly positive (+++) and leucine-rich glioma inactivated protein 1 antibodies positive (+) in three patients.Neuropsychiatric features, neuromyotonia, neuropathic pain, dysautonomia, agrypnia excitata presented in all three patients.The agrypnia excitata was characterized by severe insomnia, excessive motor activity during the night.Agrypnia excitata was diagnosed in three patients according to their history.PSG was finished in case 2 and case 3.PSG in one patient (case 2) documented severe insomnia (sleep efficiency was 59%), lack of cyclic sleep organization with a predominance of stage 1 non-rapid eye movement sleep episodes intermixed with brief rapid eye movement, and a marked reduction of spindles and delta sleep;PSG in another patient (case 3) revealed complete absence of recognizable sleep.Sleep disorders and other symptoms resolved completely or almost completely in two patients (case 1,case 2) who received immunotherapy.Case 3 died from sudden cardiac death before immunotherapy.Conclusions Morvan syndrome usually is associated with high-titer CASPR2 antibodies in serum.Agrypnia excitata is cardinal manifestation of Morvan syndrome in association with a spectrum of neurologic presentations.Early immunotherapy could provide a favorable outcome.

Objective To identify the spectrum of sleep disorders in the patients diagnosed with limbic encephalitis associated with anti leucine rich glioma inactivated protein 1 (LGI1) antibody.Methods Thirteen patients were identified with limbic encephalitis associated with LGI1 antibody in the Department of Neurology,Peking Union Medical College Hospital between December 2012 and August 2017.The characters of the 13 cases were studied from several aspects such as clinical presentation,imaging,polysomnography,cerebrospinal fluid and serum.Results Serum test and/or cerebrospinal fluid test showed LGI1 antibody positive in all the 13 patients.Clinical presentation included:cognitive impairment,seizures,neuropsychiatric features.Sleep disorders presented in all patients,including insomnia,dream enactment behaviors,hypersomnia,involuntary movement.Polysomnogram in the 13 patients revealed that sleep efficiency decreased in 10 patients (＜ 80％),N3 sleep decreased in 10 patients,rapid eye movement sleep decreased in 13 patients;period limb movement disorder was identified in seven patients,period limb movement index was 15.6-224.4/h,four of the seven patients also had frequent irregular motor activity;six patients had frequent faciobrachial dystonic seizures (20-83 times throughout the night);rapid eye movement sleep behavior disorder (RBD) was diagnosed in three patients.Sleep disorder resolved almost completely in 10 of 13 patients who received immunotherapy.Conclusions Sleep disorders are cardinal manifestations of limbic encephalitis associated with LGI1 antibody,including insomnia,RBD,hypersomnia,involuntary movement.They may respond favorably to immunotherapy.

Objective: To compare the efficacy of Bracka method and Duckett method in the treatment of proximal hypospadias. Methods: Forty patients with hypospadias were treated by 2 stages(Bracka), 42 patients treated by transverse preputial island flap (Duckett) from January 2014 to January 2016. Mean age at first stage surgery were (19.70±6.62) months and (20.33±5.03) months in Bracka group and Duckett group, respectively. There were 10 cases of proximal penile type, 25 cases of penoscrotal type, 5 cases of perineal type in group 1. There were 11 cases of proximal penile type, 27 cases of penoscrotal type, 4 cases of perineal type in group 2. There was no significant difference in age and hypospadias classification between the two groups(P>0.05). All operations were performed by the same doctor. Urethral plate reconstruction with preputial graft was performed in group 1; stage Ⅱ Duplay urethroplasty repair was carried out 6-8 months after stage Ⅰ. Results: Urine tube was placed for 2 weeks after operation and followed up for 36-63 months(mean 47.6 months). After stage I repair, penile straightening and wide, smooth appearance of graft were confirmed. There was no fistula, split, urethral diverticulum or other complications, one case with urethral opening stenosis who was restored after urethral dilatation .After stage II repair, urethral fistula was noted in 3 cases(7.5%), stricture in 1 cases(2.5%). No other complications occurred . The total rate of complications was 10%(10/40). Urethral fistula was noted in 7 cases(16.7%), stricture in 3 cases(7.1%), penile head dehiscence in 3 cases(7.1%) and diverticulum in 1 case(2.4%) in group 2. The total rate of complications was 33.3%(14/42). The incidence of total complications between the two groups was statistically significant (P=0.011). Conclusions Bracka method can be used to treat proximal hypospadias. It has high safety and low incidence of complications.