Objective To investigate the incidence rate, onset time and electrophysiological characteristics of rapid eye movement sleep behavior disorder (RBD) and the relationship between RBD and synucleinopathies as well as the electrophysiological diagnostic criteria of RBD in Parkinson' s disease (PD) and multiple system atrophy (MSA). Methods Sleep survey and night video-polysomnography (NPSG)were used to study sleep disturbance of PD and MSA. (1) Subjective sleep assessments: All subjects,including 66 PD patients, 65 age and sex matched healthy controls and 30 MSA patients, completed the sleep questionnaires, and the RBD incidence rate and onset time were got. (2) Objective sleep assessments: 8 PD patients, 13 MSA patients, and 15 age and sex matched healthy controls underwent video-NPSG recording on two consecutive nights. Sleep architect were analyzed. The NPSG characteristics of RBD accompany with PD and MSA were analyzed, and the electrophysiological diagnostic varameters of it were determined. Results Patients with PD or MSA had a higher prevalence of RBD. RBD was found in 59. 1% (39/66) PD patients and 86. 6% (26/30) MSA patients, among those, 46. 2% ( 18/39 ) and 84.6% (22/26) had the waking symptoms of MSA and PD. The main NPSG characteristics of RBD of PD or MSA were chin REM without atonia (RWA) and increased movement. Conclusions The relatively higher RBD prevalence in MSA and PD patients indicates that RBD has close relationship with PD and MSA.Part of patients with RBD preceding neurology disease indicates that RBD may be the early marker of PD and MSA. The main NPSG characteristics of RBD accompany with PD and MSA are chin RWA and the motor manifestations. RWA and phasic EMG activity density are supposed to be the NPSG diagnostic parameters.

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 investigate the clinical effect of free superficial circumflex iliac artery flap combined with anterolateral thigh flap in repairing large skin defect of the hand.Methods:A retrospective case series study was conducted to analyze the clinical data of 9 patients with large skin defect of the hand admitted to Ningbo No.6 Hospital from January 2016 to December 2019，including 7 males and 2 females，aged 31-63 years［（45.3±5.6）years］. The area of skin defect reanged from 20 cm×15 cm to 25 cm×20 cm. One side of the superficial circumflex iliac artery flap and the contralateral anterolateral thigh flap were used for repair. Both recipient and donor sites were sutured at the first stage. The ilioinguinal region and thigh donor region were sutured directly. The area of superficial circumflex iliac artery flap was from 19 cm×6 cm to 23 cm ×10 cm and that of anterolateral thigh flap was 19 cm×9 cm to 23 cm×8 cm. The flap survival，healing of donor and recipient area and complications were observed. At 1，6 and 12 months after operation，the function of the flap was evaluated by the late functional evaluation criteria of the flap，hand function by the upper limb functional evaluation criteria of the Chinese Medical Association，and scar condition of the donor site by Vancouver Scar Scale（VVS）. At the last follow-up，the color，elasticity，appearance and scar of the donor site were observed.Results:All patients were followed up for 6-18 months［（12.5±2.3）months］. All flaps survived successfully. All patients achieved donor-site healing at the first stage，except that one patient experienced incision dehiscence in the thigh donor site after operation and healed after the second stage suture. The texture and appearance of the flaps were good. The flap in 5 patients was obviously bloated. Therefore，the secondary skin flap repair was selected，and the appearance was significantly improved after operation. Scores for function of the flap and the hand were improved over time（ P<0.05）. At 12 months after operation，the function of the flap was excellent in 3 patients，good in 4 and fair in 2，with the excellent and good rate of 78%；the result of hand function was excellent in 3 patients，good in 3 and fair in 3，with the excellent and good rate of 67%. The VVS score of the donor site decreased from（9.7±1.3）points at postoperative 1 month to（5.7±0.9）points at postoperative 6 months and（3.4±0.8）points at postoperative 12 months（ P<0.05）. At the latest follow-up，the color and elasticity of the flap was similar to that of the surrounding skin tissue，only with slight bloating；the scar of the donor site was small. Conclusions:The free superficial circumflex iliac artery flap combined with anterolateral thigh flap can cover large skin defect of the hand at one time，with good appearance and texture of the flap. The appearance and function of the wounded hand are good after operation，leaving only linear scar in the donor site.

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 investigate the clinical application and effect of end-to-side anastomosis in personalised free ilioinguinal flap transfer.Methods:From March, 2015 to July, 2020, 88 patients with soft tissue (bone) defect of limbs were treated. Different ilioinguinal flaps were designed according to the wound condition of patients, which were 48 cases of free superficial circumflex iliacartery perforator flap, 7 cases of free superficial epigastric artery perforator flap, 19 cases of composite tissue flap with iliac bone, 8 cases of combined flap of superficial circumflex iliac artery and superficial abdominal wall artery, and 6 cases of superficial circumflex iliac artery and superficial abdominal wall artery lobulated flap. The area of the flap was 4.0 cm×6.0 cm-10.0 cm×30.0 cm. The artery and recipient artery were anastomosed end-to-side: 36 cases to radial artery; 12 cases to ulnar artery; 18 cases to dorsalis pedis artery; 15 cases to anterior tibial artery; 7 cases to posterior tibial artery. Venous anastomosis of skin flap: 42 cases were anastomosed with 2 veins, which were superficial vein of the same name and accompanying vein; 46 cases were anastomosed with 1 superficial vein of the same name. The accompanying vein of the flap was anastomosed end-to-side with the accompanying vein of the main artery of the recipient area, and the superficial vein of the same name was anastomosed end-to-end with the accompanying vein or subcutaneous superficial vein of the recipient artery. Follow-up includes flap blood supply, blood supply to the distal limbs, appearance of both the donor site and the recipient area, and patient satisfaction.Results:There were 83 cases of flaps survived successfully, and 5 cases of crisis. Among them, 2 cases had artery crisis at 48 h after surgery. After exploration, it was found that 1 case caused by arterial thrombosis, and 1 case compressed by the stapler that anastomoses the vein. The other 3 cases had venous crisis at 72 h after surgery: after exploration, it was found that caused by thrombosis at the venous anastomotic site. The average follow-up period was 10 (range, 3-24) months. All flaps survived after re anastomosis or vascular transposition. The donor site and recipient site of the flap healed well. The blood supply of the flap was good and the texture was soft. There was no blood supply disorder in the distal limb.Conclusion:The end-to-side anastomosis technique is suitable for all kinds of free flap transplantation in ilioinguinal region, with high vascular patency rate. It can not only solve the problem of thin vascular pedicle of donor site flap, but also retain the main artery of recipient limb without affecting the distal blood supply.

The clinical manifestations and examination results of a case of Creutzfeldt-Jakob disease (CJD) admitted to the Department of Neurology of Peking Union Medical College Hospital,Chinese Academy of Medical Sciences in August 2020 were analyzed, and a comprehensive neuropsychological assessment and assessment of apraxia were conducted. The neuropsychological characteristics of apraxia in CJD patient and the progress in the research and evaluation of apraxia were reviewed. The patient was a 65-year-old male with insidious onset and progressive symptoms, whose clinical manifestations were apraxia, rapidly progressing dementia, and extrapyramidal symptoms. The magnetic resonance imaging showed hyper-intense signal in diffusion weighted imaging in bilateral cerebral hemispheres, and 14-3-3 protein in cerebrospinal fluid was positive, which were consistent with the probable CJD diagnostic criteria. The patient exhibited prominent signs and symptoms of ideomotor apraxia. It has been reported in the literature that apraxia can also be the main neuropsychological manifestation of CJD. It is necessary to pay attention to the standard evaluation and timely identification of apraxia in clinical diagnosis.

Propriospinal myoclonus （PSM） is a rare movement disorder. Three types of PSM have been reported：symptomatic，idiopathic and functional PSM. PSM at sleep onset is a subtype of idiopathic PSM. The current study summarized the clinical and neurophysiological characteristics of different types PSM. Methods The current study included 2 cases of PSM at sleep onset，2 cases of functional PSM，1 case of symptomatic PSM. Results Two patients with PSM at sleep onset had chronic course. Abdominal jerk was the main complaint，occurring at sleepwake transition. Mental disorders were happened in two cases with functional PSM，one of whom had various patterns of jerk and propagation. Neurophysiological examination showed that functional PSM had longer duration of muscle burst and faster velocity of recruitment. Moreover，myoclonic status was observed in PSM at sleep onset and symptomatic PSM. Conclusion PSM showed specific clinical neurophysiological characteristics according to different types. Differential diagnosis is required in clinics.