To explore the effectiveness of "near peer learning" (NPL) in the electromyography(EMG)teaching module for neurology residents.

Objective:To explore the surgical techniques and effects of transfer of the free chimeric functional thoracodorsal artery perforator flap (TDAPF) with latissimus dorsi in reconstruction of dynamic muscle and soft tissue defects in forearm.Methods:From January 2014 to December 2020, a total of 13 transfer surgery of free chimeric functional TDAPF with vascularised latissimus dorsi were performed in the Department of Hand Surgery, Plastic & Reconstructive Surgery, Ningbo Sixth Hospital, to reconstruct forearm composite defects. The patients were 12 males and 1 female with an average age of 33.2 years old. They all had open forearm injuries, with 5 in the left and 8 in the right. Removal of inactivated muscles, exploration and repair of blood vessels and nerves were performed in emergency surgery, and VSD were applied after the surgery. Phase II reconstructive surgery were completed within 4 to 12 days, with 7.5 days in average. The wounds and flaps sized were 9.0 cm×8.0 cm - 21.0 cm×11.0 cm and were 10.0 cm×9.0 cm - 22.0 cm×12.0 cm, respectively. The volume of transferred muscles ranged were 9.0 cm × 2.0 cm × 1.5 cm - 19.0 cm × 9.0 cm × 1.5 cm. Free chimeric functional muscular flaps were transferred to reconstruct the musculus flexor digitorum profundus in 4 patients, the musculus extensor digitorum communis in 8 patients, the musculus flexor carpi radialis in 3 patients, and the musculus flexor pollicis longus in 1 patient. Reconstruction of both of musculus flexor carpi radialis and musculus extensor digitorum communis with 2 functional sub-blocks of latissimus dorsi were performed in 3 patients. All donor sites were closed primarily. All patients were included in the postoperative follow-up to evaluate the appearance of flaps, range of motion of the digits, recovery of muscle strength and gripping power, at the outpatient clinics or through the telephone interview.Results:A total of 12 flaps survived uneventfully after reconstructive surgery. One flap developed a vascular crisis and it was rectified after surgical exploration. Postoperative follow-up ranged from 17 to 52 months, with a mean of 34.1 months. Appearances of limbs and flaps were good without obvious bulky, hyperpigmentation or scar contracture. Four patients with reconstructed musculus flexor digitorum profundus showed muscle strength recovery of M 4, with the fingertips measured lower than 2.0 cm from the centre of palm when clenching a fist, and the average gripping strength of the hand reached 27.5% (20%-35%) to the healthy side. Five patients with reconstructed musculus extensor digitorum communis showed muscle strength recovery of M 4, and there was no obvious limitation in fingers flexion and extension, with the average gripping strength of the hand reached 75.4% (65%-80%) to the healthy side. Of the 3 patients with reconstruction of both power muscles, the recovery of muscle strength of musculus flexor carpi radialis was at M 4 in all the 3 patients, and the musculus extensor digitorum communis was at M 4 in 1 and M 3 in 2 patients. However, the patient who received reconstruction of musculus flexor pollicis had no significant recovery in muscle strength. Conclusion:Transfer of free chimeric functional TDAPF combines the benefits of a perforator flap and a functional muscle transfer together. This surgical technique can effectively reconstruct damaged muscle groups in forearm and resulting in good hand movement. Additionally, it can also restore the aesthetic appearance of forearm, hence makes it an excellent option for complex wound coverage.

Objective:To investigate the clinical and electrophysiological characteristics of facial onset sensory motor neuronopathy (FOSMN) syndrome.Methods:Ten patients diagnosed with FOSMN syndrome in Peking Union Medical College Hospital from January 2012 to December 2022 were included. The clinical and electrophysiological characteristics of patients were analyzed and summarized, and the genetic testing was also performed in these patients.Results:The age of onset was (56.6±6.5) years, and the longest survival duration of disease was 10 years. All patients had numbness around the face and mouth as the first symptom and abnormal blink reflex. A total of 52 sensory nerve conduction nerves were detected, among which 2 median nerves and 2 μlnar nerves showed decreased amplitude of sensory nerve action potential. Needle electromyography showed neurogenic lesions, with both progressive and chronic denervation. Whole exome sequencing identified the heterozygous variant c.272A>C in the exon 4 of the SOD1 gene resulting in the amino acid change p.Asp90Ala in 1 patient. In all patients, the disease progressed relentlessly and eventually led to involvement of respiratory muscle. Conclusion:FOSMN syndrome is characterized by abnormal blink reflex and sometimes abnormal sensory nerve conduction may be shown on electrophysiologic testing.

Objective:To investigate the effect of tourniquet-reperfusion augmented infrared thermography(TRAIRT) on locating the dominant perforator to assist design of free medial sural artery perforator flap.Methods:The data of patients with skin soft tissue defect of upper limb repaired by free medial sural artery perforator flap in Department of Hand Surgery, Ningbo Sixth Hospital from May 2019 to January 2022 were retrospectively analyzed. Color Doppler ultrasonography (CDU) and TRAIRT were used to locate the dominant perforator auxiliary flap design before surgery and after anesthesia. In the TRAIRT video, the hot spot with "early emergence, high brightness and fast expansion" was selected as the advantageous perforator. During the operation, the flap was elevated and transferred to the affected area to cover the defect wound according to the designed mark points, and fixed by the absorbable sutures with an interrupted suturing method, then end-to-end vascular anastomosis was performed successively. The donor area was sutured directly or sutured with full thickness skin grafts depending on the area of the wound. The donor and recipient areas of the flap were observed and the patients’ satisfaction was recorded. The gold standard was the actual location of the perforating vessel found during the operation. Compared with the gold standard, the location of the perforating vessel explored by TRAIRT and CDU was considered to be accurate (positive) if the distance was less than 10 mm. The sensitivity (accurate number of perforators/actual number of perforators during operation ×100%) and positive predictive value (accurate number of perforators/total number of perforators×100%) of the two methods were calculated, expressed as %, and the sensitivity of the two methods was compared by paired χ2 test. Kappa coefficient was used to analyze the consistency of the two methods to detect perforating vessels. The perforator detection time of TRAIRT and CDU were recorded, expressed as Mean±SD, and statistically analyzed by paired sample t-test. Results:A total of 23 patients were included, consisting of 14 males and 9 females, agd 21-70 years old, average age of 43 years old. The wounds were on forearm in 5 cases, wrist in 2 cases, and hand in 16 cases. The wound area was 4.5 cm × 5.5 cm-6.5 cm × 12.0 cm. Intraoperative flap incision area was 5.0 cm × 6.0cm-7.0 cm × 13.0 cm. After operation, 22 cases of flaps survived, 1 case had superficial necrosis at the distal end, which healed after repeated dressing change. The donor area of flaps healed well, with primary suture in 20 cases and full thickness skin grafting in 3 cases. Postoperative follow-up was 5-16 months (mean 8.4 months). The flaps had no bulge, swelling and abrasion. The texture and color were similar to the medial calf skin, and the scars in the donor and recipient areas were not obvious. The patients were satisfied with the recovery. Among the 23 patients, 49 perforators were found by TRAIRT, 50 perforators were found by CDU, and 53 perforators were found intraoperatively. The sensitivity of TRAIRT and CDU was 88.7% (47/53) and 90.6% (48/53), with no significant differences ( P>0.05), and the positive prediction value was 95.9% (47/49) and 96.0% (48/50) respectively. The Kappa coefficient was 0.89, indicating a good consistency between the two methods. The time required for TRAIRT to detect perforators was significantly shorter than that of CDU, with statistical significance [(6.52±2.02) min vs. (17.87±2.49) min, P<0.01]. Conclusion:TRAIRT has a good consistency with CDU in detection of medial sural artery perforator. The application of TRAIRT in designing medial sural artery perforator flap for wound repair is good, and it has advantages of short time taking, simple operation, economy and non-invasiveness.

Objective:To investigate the effect of tourniquet-reperfusion augmented infrared thermography(TRAIRT) on locating the dominant perforator to assist design of free medial sural artery perforator flap.Methods:The data of patients with skin soft tissue defect of upper limb repaired by free medial sural artery perforator flap in Department of Hand Surgery, Ningbo Sixth Hospital from May 2019 to January 2022 were retrospectively analyzed. Color Doppler ultrasonography (CDU) and TRAIRT were used to locate the dominant perforator auxiliary flap design before surgery and after anesthesia. In the TRAIRT video, the hot spot with "early emergence, high brightness and fast expansion" was selected as the advantageous perforator. During the operation, the flap was elevated and transferred to the affected area to cover the defect wound according to the designed mark points, and fixed by the absorbable sutures with an interrupted suturing method, then end-to-end vascular anastomosis was performed successively. The donor area was sutured directly or sutured with full thickness skin grafts depending on the area of the wound. The donor and recipient areas of the flap were observed and the patients’ satisfaction was recorded. The gold standard was the actual location of the perforating vessel found during the operation. Compared with the gold standard, the location of the perforating vessel explored by TRAIRT and CDU was considered to be accurate (positive) if the distance was less than 10 mm. The sensitivity (accurate number of perforators/actual number of perforators during operation ×100%) and positive predictive value (accurate number of perforators/total number of perforators×100%) of the two methods were calculated, expressed as %, and the sensitivity of the two methods was compared by paired χ2 test. Kappa coefficient was used to analyze the consistency of the two methods to detect perforating vessels. The perforator detection time of TRAIRT and CDU were recorded, expressed as Mean±SD, and statistically analyzed by paired sample t-test. Results:A total of 23 patients were included, consisting of 14 males and 9 females, agd 21-70 years old, average age of 43 years old. The wounds were on forearm in 5 cases, wrist in 2 cases, and hand in 16 cases. The wound area was 4.5 cm × 5.5 cm-6.5 cm × 12.0 cm. Intraoperative flap incision area was 5.0 cm × 6.0cm-7.0 cm × 13.0 cm. After operation, 22 cases of flaps survived, 1 case had superficial necrosis at the distal end, which healed after repeated dressing change. The donor area of flaps healed well, with primary suture in 20 cases and full thickness skin grafting in 3 cases. Postoperative follow-up was 5-16 months (mean 8.4 months). The flaps had no bulge, swelling and abrasion. The texture and color were similar to the medial calf skin, and the scars in the donor and recipient areas were not obvious. The patients were satisfied with the recovery. Among the 23 patients, 49 perforators were found by TRAIRT, 50 perforators were found by CDU, and 53 perforators were found intraoperatively. The sensitivity of TRAIRT and CDU was 88.7% (47/53) and 90.6% (48/53), with no significant differences ( P>0.05), and the positive prediction value was 95.9% (47/49) and 96.0% (48/50) respectively. The Kappa coefficient was 0.89, indicating a good consistency between the two methods. The time required for TRAIRT to detect perforators was significantly shorter than that of CDU, with statistical significance [(6.52±2.02) min vs. (17.87±2.49) min, P<0.01]. Conclusion:TRAIRT has a good consistency with CDU in detection of medial sural artery perforator. The application of TRAIRT in designing medial sural artery perforator flap for wound repair is good, and it has advantages of short time taking, simple operation, economy and non-invasiveness.

Objective: To evaluate the value of cerebrospinal fluid markers expecially total-tau protein (T-tau), phosphorylated-tau protein (P-tau) in diagnosis and differentiation of sporadic Creutzfeldt-Jakob disease (sCJD). Methods: sCJD (according to 2009 Brain criteria, 2018 Neurology amended criteria), Alzheimer's disease (AD; the National Institute on Aging at National Institutes of Health and the Alzheimer's Association revised guidelines 2011 criteria) and other patients without cognitive impairment, matched for sex and age, in the Department of Neurology, Peking Union Medical College Hospital from 2018 to 2019 were enrolled. Twelve sCJD patients, 49 AD patients and 14 normal controls were enrolled. Cerebrospinal fluid (CSF) specimens were collected through gravity dropping directly, and further stored in -80 ℃ and disposed according to widely used standards. The levels of T-tau and P-tau were measured by ELISA. The data on electroencephalogram and neuroimaging findings of sCJD patients were recorded. Moreover, specimens of sCJD patients were sent to the Chinese Center for Disease Control and Prevention to test 14-3-3 protein and PRNP genotype. Results: Using Mann-Whitney U test, T-tau concentrations were found higher in patients with sCJD (1 211(448, 2 227) pg/ml) than in AD patients (549(314, 1 078) pg/ml; U=178, P=0.034 9), and both groups had higher T-tau than the control group (127(79, 192) pg/ml; U=20, 73, P<0.01). The level of P-tau was significantly increased in AD patients (72(58,109) pg/ml) compared to the control group (27(15, 42) pg/ml; U=82, P<0.01), but not in sCJD patients (32(24, 47) pg/ml). The T-tau/P-tau ratio was higher in sCJD patients (29.77(20.01, 54.53)) than in AD patients (7.45(4.79, 10.43); U=87, P<0.01). Twelve sCJD patients had cotical hyperintensity on diffusion weighted imaging and five had periodic three-phase waves on electroencephalogram. Nine sCJD patients, whose CSF samples were tested in the Chinese Center for Disease Control and Prevention, carried an M/M genotype at codon 129 and E/E at codon 219. Conclusion The CSF tau level and T-tau/P-tau ratio are significantly increased in sCJD, which may promote the diagnosis and differentiation of sCJD in routine clinical setting.

Objective: To summarize the clinical characteristics and nerve conduction damage in patients with early POEMS syndrome, and to explore the value of segment nerve conduction velocity in the diagnosis of POEMS syndrome. Methods: A total of 73 patients with POEMS syndrome and 27 healthy controls in Peking Union Medical College Hospital from September 2009 to June 2019 were recruited in this study. The motor and sensory nerve conduction characteristics of median, ulnar, tibial, and peroneal nerves and the clinical features of the participants were analyzed. The analysis parameters included: (1) distal motor latency (DML), compound muscle action potential (CMAP); (2) the median velocity from elbow to wrist, the median velocity from axillary to elbow, the ulnar velocity from the site below elbow to wrist, the ulnar velocity from the site above elbow to below elbow, the ulnar velocity from axillary to the site above elbow, the tibial velocity from ankle to knee, the peroneal velocity from ankle to fibulae capitulum; (3) sensory nerve conduction velocity and amplitude of these nerves; (4) terminal latency indices (TLI) of median; (5) motor nerve conduction blocks. Results: Peripheral nerve damages were the initial symptoms in thirty-two patients in this group, accounting for 43.8% (32/73), and 81.3% (26/32) of these patients only showed numbness in lower extremities. All POEMS syndrome patients with numbness had abnormal sensory nerve conduction, and 9.5% (7/73) of patients without sensory symptoms also had abnormal sensory nerve conduction. On the other hand, the decrease of CMAP amplitude corresponded to clinical muscle strength decline and motor dysfunction. In the patients with POEMS syndrome, motor nerve conduction in the lower limbs were more likely to be affected and the damages were more severe than in the upper limbs: the proportion of CMAP disappearance in the lower limbs and upper limbs was 47.6% (112/235) vs 2.8% (7/252; χ²=133.698, P<0.01). Sensory nerve conduction damage was more severe than motor nerve conduction: the proportion of amplitude disappearance in sensory and motor conduction was 43.0% (141/328) vs 24.4% (119/487; χ²=133.698, P<0.01). The slowing of motor nerve conduction velocity was more common than the decrease of CMAP amplitude: the rate of slowing down of motor nerve conduction was 88.7% (432/487), and the rate of decrease of amplitude was 52.8% (257/487; χ²=151.905, P<0.01). The DML of median and ulnar nerve in the POEMS syndrome group was longer than that in the control group (median nerve: 4.4 (3.7, 5.0) ms vs 3.2 (3.0, 3.5) ms, U=854.000, P<0.01; ulnar nerve: 3.1 (2.7, 3.8) ms vs 2.5 (2.2, 2.7) ms, U=1 077.500, P<0.01). The TLI of median nerve in patients with POEMS syndrome was significantly higher than that of healthy controls (0.40±0.11 vs 0.35±0.06, t=3.466, P=0.001). There was no statistically significant difference in nerve conduction velocity between the forearm segment and the upper arm segment in the POEMS group. Conclusions Neurological damages were common in patients with POEMS syndrome with sensory nerve damage often being the initial clinical manifestation. In patients with POEMS syndrome, the nerve damage in the lower limbs was more severe than in the upper limbs, and sensory nerve damage was more severe than motor nerve damage. Segmental motor nerve conduction results suggested that demyelination damage occurred earlier than axonal damage, and proximal lesions were slightly heavier than distal lesions. No conduction block was detected in these patients. These characteristics of segmental nerve conduction can provide more evidence for clinical diagnosis of POEMS syndrome.

Immune checkpoint inhibitors (ICIs) can cause adverse reactions in the nervous system, with the incidence rate ranging from 0.1% to 12%, and 80% occurring within the first 4 months of ICI application. It can cause lesions in various parts of the nervous system, including aseptic meningitis, meningoencephalitis, necrotizing encephalitis, brainstem encephalitis, transverse myelitis and other central nervous system diseases. It can also cause cranial peripheral neuropathy, multifocal radicular neuropathy, Guillain-Barre syndrome, spinal radicular neuropathy and myasthenia gravis, myopathy, etc. For these complications of the nervous system, diagnosis could be made by sufficient collection of disease manifestations combined with imaging, cerebrospinal fluid examinations, electro-encephalogram or electro myography to exclude infection or tumor progression. In the treatment of severe cases, ICIs should be discontinued and treated with high doses of glucocorticoid or gamma globulin with systemic support. After neurological adverse reactions, the prognosis of severe cases is poor.

Objective To characterize the brain microstructure changes of amyotrophic lateral sclerosis (ALS) patients with various levels of cognitive impairment as measured by diffusion tensor imaging (DTI).Methods A total of 55 ALS patients and 20 healthy controls (HC) were enrolled in the Department of Neurology of Peking Union Medical College Hospital From September 2013 to March 2017,and all participants underwent neuropsychological assessments and DTI scans.According to their cognitive performance,ALS patients were further subclassified into ALS with normal cognition (ALS-Cn,n =27),ALS with cognitive impairment (ALS-Ci,n =17) and ALS-frontotemporal dementia (ALS-FTD,n =11)subgroups.Comparisons of voxel-based and atlas-based fractional anisotropy (FA) and mean diffusivity (MD) data were conducted among the four subgroups.Results In the voxel-based analyses,the FA showed significant differences in cingulate gyms,corpus callosum,brain stem and cerebellum,and MD showed significant differences in bilateral frontal lobe,temporal lobe,cingulate gyms,corpus callosum,and cerebellum among the four subgroups.Besides,when compared to ALS-Ci,ALS-Cn and HC groups in the order,the areas of involvement were larger and differences were more significant in ALS-FTD group.In the atlas-based analyses,the FA and MD of the corticospinal tracts revealed no difference within the patients groups,but decreased FA and increased MD were found compared to HC group.The ALS-IFD patients manifested widespread white matter fiber integrity damage and microstructure impairment in the extramotor areas compared to other three groups.Conclusion The brain white matter structural patterns of ALS patients correlate with their cognitive function,and there is a gradient of alterations across the ALS-Cn,ALS-Ci and ALS-FTD continuum.

Objective To explore diagnostic value of amplitude of low-frequency fluctuation ( ALFF) on cognitive impairment in amyotrophic lateral sclerosis ( ALS ) using resting-state functional magnetic resonance imaging ( MRI ) .Methods Sixteen ALS patients from neurological clinic in Peking Union Medical College Hospital were enrolled between November 2013 and April 2015.The patients were divided into two groups by the presence (ALSi, n=7) or absence (ALSu, n=9) of cognitive impairment. Routine MRI structural images and resting-state functional MRI were collected for comparison between groups through voxel-based morphometry ( VBM ) and ALFF.Results ( 1 ) Neuropsychological analysis showed significant differences in Montreal Cognitive Assessment score (22.9 ±2.0 vs 25.8 ±2.3, t=2.622, P=0.020), Frontal Assessment Battery score (12.4 ±1.6 vs 15.1 ±1.4, t=3.600, P=0.003), animal listing test (13.6 ±1.8 vs 16.7 ±2.9, t=2.482, P=0.026), naming test (2(1) vs 0(1), Z=-2.746, P=0.006), similarity test (7.9 ±3.7 vs 17.3 ±2.8, t=5.846, P=0.000) and clock drawing test (2(2) vs 3(0), Z=2.516, P=0.012).(2) VBM analysis showed no significant differences in both gray matter and white matter density between the two groups .(3) ALFF analysis showed significantly increased signals in widespread areas of bilateral cerebrum and cerebellum in ALSi group compared to ALSu group . Conclusion ALFF value has the potential to provide more valuable imaging basis for early diagnosis on cognitive impairment in ALS.