Objective To explore the effects of VEGF/bFGF sustained release microspheres combined with hyperbaric oxygen therapy on survival rates of uhralong random pattern flaps in rats.Methods Modified McFarlane flap rat models were randomly divided into 4 groups,VEGF/bFGF microspheres + hyperbaric oxygen group,VEGF/bFGF microspheres group,hyperbaric oxygen group and control group.At the end of 7 days,survival rates of flaps were calculated (Survival rate =Survival area/total area × 100％).The tissue samples were taken from middle portion of flaps to detect the neovascularization.Immunohistochemistry was used to detect the differential expression of VEGF.Results Seven days after the random pattern flap operation in rats,the survival rates of VEGF/bFGF microspheres combined with hyperbaric oxygen therapy experimental group,VEGF/bFGF microspheres experimental group,hyperbaric oxygen therapy experimental group,and control group were (89.54 ± 3.23) ％,(73.54 ± 4.57) ％,(71.89 ± 2.26) ％,(50.36 ± 2.37) ％ respectively.The neovascularization counts of each group in part Ⅱ were (35.14 ± 4.21)/mm2,(23.34 ± 2.53)/mm2,(25.22 ±2.73)/mm2 and (17.37 ± 5.73)/mm2 respectively.VEGF cumulative absorbance in VEGF/bFGF microspheres + hyperbaric oxygen group,VEGF/bFGF microspheres group,hyperbaric oxygen group and control group were 78.39 ± 9.12,52.42 ± 13.59,49.84 ± 12.93,29.24 ± 10.35 respectively.In each experimental result group,there were significant difference between the combination group and the other groups (F =189.956,P ＜ 0.05),but there was no significant difference between VEGF/bFGF microspheres experimental group and hyperbaric oxygen therapy experimental group.Conclusion VEGF/bFGF sustained release microspheres combined with hyperbaric oxygen therapy can promote flap neovascularization,increase the flap blood supply and then improve the survival of random pattern skin flaps.

Objective To assess the utility of changes of muscle strength and compound muscle action potential (CMAP) of abductor digiti minimi (ADM) muscle after the exercise test in diagnosis of hypokalaemic periodic paralysis during inter-attack period. Methods Exercise test was applied on 59 patients with hypokalaemic periodic paralysis and 38 control subjects during inter-attack period. The changes of CMAP amplitude, muscle strength and the range of abduction of ADM muscle at 120 minutes after exercise were calculated and compared between the two groups. Results At 120 minutes after exercise, the M50 (M25, M75 ) of decrease in amplitude of CMAP recorded in ADM muscles were 54. 1% ( 43.1%,66. 3% ) in patients with periodic paralysis and 11.1% (2. 0%, 21.3% ) in control subjects(Z =6. 731,P=0.000), M5o(M25,M75) of decrease in range of abduction of ADM muscles were 39.4% (26.3% ,48. 9% ) in patients with periodic paralysis and 7. 8% ( 1.3%, 13.7% ) in control subjects ( Z = 5. 519,P=0. 000). The muscle strength of ADM muscle was less than Ⅳ grade in 96. 3% (52/54) patients with periodic paralysis and 8.6% ( 3/35 ) in control subjects ( x2 = 68.2, P = 0. 000 ). The sensitivity and specificity for decrease in CMAP amplitude ( best cutoff = 30% ) in diagnosis of hypokalaemic periodic paralysis were 87.5% and 93.7%, respectively. The sensitivity and specificity for decrease in range of abduction of ADM muscle ( best cutoff = 20% ) were 87.5% and 90. 5%, respectively. Conclusion Exercise test is recommended to apply on patients with suspected hypokalaemic periodic paralysis during inter-attack period. The decrease in range of abduction of ADM muscle more than 20% at 120 minutes after exercise supports the diagnosis of hypokalaemic periodic paralysis.

Objective To investigate the characteristics of F-waves in patients with Kennedy's disease.Methods Thirty two patients with Kennedy's disease and 30 male healthy volunteers,who visited the Department of Neurology,Peking Union Medical College Hospital between August 2013 and July 2014,were recruited consecutively for this study.Motor nerve conduction study and F-wave examination were performed on the median,ulnar,tibial and peroneal nerves of all participants.A series of 100 electrical stimuli were employed to obtain F-waves.The F-wave parameters in all tested nerves were compared between patients with Kennedy's disease and normal controls including F-wave minimum latency,F-wave persistence,mean and maximum F-wave amplitude,the frequency of giant F-waves.Results The mean Fwave amplitude (median nerve:patients with Kennedy's disease 375.0 (298.3) μV,healthy volunteers 297.0(145.0) μV,Z =-3.378,P ＜0.01;ulnar nerve:patients with Kennedy's disease 397.5(295.0) μV,healthy volunteers 293.0 (101.8) μV,Z =-3.968,P ＜ 0.01;tibial nerve:patients with Kennedy's disease 374.5 (227.3) μV,healthy volunteers 294.0 (160.5) μV,Z =-3.144,P =0.002;peroneal nerve:patients with Kennedy's disease 346.5 (292.8) μV,healthy volunteers 146.5 (69.3) μV,Z =-6.864,P ＜ 0.01),maximum F-wave amplitudes (median nerve:patients with Kennedy's disease 1 291.0 (952.0) μV,healthy volunteers 846.5 (523.0) μV,Z =-4.823,P ＜ 0.01;ulnar nerve:patients with Kennedy's disease 1 663.5 (1 374.0) μV,healthy volunteers 760.5 (341.8) μV,Z =-6.813,P ＜ 0.01;tibial nerve:patients with Kennedy's disease (1 054.1 ± 451.6) μV,healthy volunteers (652.5-± 172.5) μV,t =5.380,P ＜ 0.01;peroneal nerve:patients with Kennedy's disease (840.4 ± 494.1) μV,healthy volunteers (370.2 ± 202.0) μV,t =6.475,P ＜ 0.01),frequency of giant F-waves (median nerve:patients with Kennedy's disease 0.0％ (7.2％),healthy volunteers 0.0％ (0.0％),Z =-5.149,P ＜ 0.01;ulnar nerve:patients with Kennedy's disease 3.1％ (10.5％),healthy volunteers 0.0％ (0.0％),Z =-7.026,P ＜0.01;tibial nerve:patients with Kennedy's disease 0.0％ (3.3％),healthy volunteers 0.0％ (0.0％),Z =-4.651,P ＜0.01;peroneal nerve:patients with Kennedy's disease 3.3％ (28.2％),healthy volunteers 0.0％ (0.0％),Z =-5.532,P ＜0.01),and frequencies of patients with giant F-waves (median nerve:patients with Kennedy's disease 78.1％ (25/32),healthy volunteers 10.0％ (3/30),x2 =29.016,P ＜ 0.01;ulnar nerve:patients with Kennedy's disease 87.5％ (28/32),healthy volunteers 10.0％ (3/30),x2 =37.200,P ＜ 0.01;tibial nerve:patients with Kennedy's disease 62.5％ (20/32),healthy volunteers 6.7％ (2/30),x2 =21.085,P ＜ 0.01;peroneal nerve:patients with Kennedy's disease 68.8 ％ (22/32),healthy volunteers 10.0％ (3/30),x2 =22.209,P ＜ 0.01) in all nerves examined were significantly higher in patients with Kennedy's disease than in the normal controls.The F-wave persistence in all nerves examined was significantly lower than in the normal controls (median nerve:patients with Kennedy's disease 52.5％ (36.3％),healthy volunteers 98.0％ (7.0％),Z =9.010,P ＜ 0.01;ulnar nerve:patients with Kennedy's disease 71.0％ (28.3％),healthy volunteers 100.0％ (1.0％),Z =9.455,P ＜ 0.01;tibial nerve:patients with Kennedy's disease 100.0％ (2.0％),healthy volunteers 100.0％ (0.0％),Z =4.255,P ＜ 0.01;peroneal nerve:patients with Kennedy's disease 33.1％ ± 23.9％,healthy volunteers 46.9％ ± 27.2％,t =-2.848,P =0.007).Giant F-waves were detected in multiple nerves and often appeared symmetrically on the same nerves between the left and right sides in patients with Kennedy's disease.No significant correlations were found between the pooled frequency of giant F-waves and disease duration in patients with Kennedy's disease(r =0.162,P =0.418).Conclusions The results showed increased F-wave amplitudes,increased number of giant F-waves,especially giant F-waves detected in multiple nerves or appearing symmetrically combined with low persistence,consistent with the pathologic features of chronic and unselected loss of anterior horn cells in patients with Kennedy's disease.

0.9999 ( n =5). The recovery was 101.4% , RSD = 3.26% ( n =6).Conclusion:: The method is proved to be rapid, accurate and reproducible, and suitable for components determination for Shexiangzhitong Plaster.

Objective To investigate the characteristics of giant F-waves in patients with amyotrophic lateral sclerosis ( ALS ) and the relationship between giant F-waves and disease phenotype . Methods Motor nerve conduction study and F-waves were performed to the median , ulnar, tibial and peroneal nerves of 55 patients with ALS and 52 healthy volunteers.A series of 100 electrical stimuli were employed to obtain F-waves.The following F-wave variables were estimated: frequency of giant F-waves, frequency of patients with giant F-waves, the relationship between giant F-waves and lower motor neuron dysfunction , the relationship between giant F-waves and upper motor neuron dysfunction , the relationship between giant F-waves and disease duration , the relationship between giant F-waves and disease severity , and the relationship between giant F-waves and disease progression rate ( DPR).Results The frequencies of giant F-waves ( ALS: median nerve 0.00 ( 0.00 )%, ulnar nerve 0.00 ( 1.02 )%, tibial nerve 0.00 (0.00)%,peroneal nerve 0.00(0.00)%.Normal controls:median nerve 0.00(0.00)%,Z=-2.360, P=0.018;ulnar nerve 0.00(0.00)%,Z=-3.997,P<0.01;tibial nerve 0.00(0.00)%,Z=-3.006, P=0.003;peroneal nerve 0.00(0.00)%,Z=-3.006,P=0.003) and the frequencies of patients with giant F-waves (ALS:median nerve 13/55,23.6%,ulnar nerve 26/55,47.2%,tibial nerve 18/55,32.7%, peroneal nerve 16/55, 29.1%.Normal controls:median nerve 4/52,7.7%,χ2 =0.024,P=0.024;ulnar nerve 7/52,13.5%, χ2 =14.326,P<0.01; tibial nerve 6/52,11.5%, χ2 =6.897,P=0.009; peroneal nerve 6/52,11.5%,χ2 =5.042,P=0.025) in the median nerve, ulnar nerve, tibial nerve and peroneal nerve were significantly increased compared with those of the normal controls .No significant differences were found in the frequencies of upper motor neuron dysfunction between nerves with giant F -waves and nerves without giant F-waves in the median nerves , ulnar nerves , tibial nerves and peroneal nerves of ALS patients . The compound muscle action potential amplitude of nerves with giant F-waves was significantly higher than those of nerves without giant F-waves in the median nerves (11.20(5.80) mV vs 5.90(8.50) mV,t=2.883,P=0.004)and tibial nerves ((13.20 ±4.61) mV vs (10.69 ±4.76) mV,t=-2.222,P=0.028) of the ALS patients.No significant correlation was detected between the frequency of giant F-waves and disease duration or ALS functional rating scale in the ALS patients , while the frequency of giant F-waves correlated inversely with the DPR(r=-0.287,P=0.034).No significant differences were detected in disease duration between ALS patients with giant F-waves and those without giant F-waves.Compared with those in ALS patients without giant F-waves, the revised ALS Functional Rating Scale score (37.00(3.00) vs 42.00(4.75),Z=3.197,P=0.001) was more, the DPR (0.50(0.35)vs 0.90(0.43),Z=-3.033, P=0.002 ) was slower in ALS patients with giant F-waves. Conclusions The giant F-waves were significantly increased in the ALS patients than those in the healthy volunteers and were distributed asymmetrically between the left and right sides .These electrophysiological characteristics of ALS patients fitted well with progressive loss of anterior horn cells , and indicated differential involvement of different spinal motoneuron pools in the ALS patients .No correlations were found between the frequency of giant F-waves and disease duration .The appearance of giant F-waves may indicate loss of spinal motoneuron early in the disease course , and may suggest that the degree of reinnervation and functional compensation are relatively good after motoneuron loss .

Objective To explore the effects of small incision in situ release on surgical indications,nerve conduction velocity and upper limb function in patients with carpal tunnel syndrome(CTS).Methods A total of 100 CTS patients admitted to our hospital from January 2018 to January 2022 were selected,they were randomly divided into the control group(50 cases,treated with traditional carpal tunnel release)and the observation group(50 cases,treated with small incision in situ release),the clinical data of CTS patients were collected and surgical indications,nerve conduction velocity,upper limb function and the incidence of complications were compared between the two groups.Results The total effective rate was 98.00％in the observation group and 84.00％in the control group(P＜0.05).In the observation group,the length of incision was(1.65±0.29)cm,the time of opening and closing incision was(4.85±1.02)min,the hospitalization time was(3.24±0.62)d,the intraoperative blood loss was(17.88±3.53)mL,and the VAS score was(3.03±0.56)points one day after operation.The control group were(4.02±0.81)cm,(10.06±2.28)min,(7.11±1.34)d,(24.37±5.27)ml,(4.04± 0.89)points,the differences were statistically significant(P＜0.05).After treatment,The thumle-wrist sensory conduction velocity of CTS patients in the study group was(46.05±8.39)m/s,the middle finger-wrist sensory conduction velocity was(45.05±8.95)m/s,the thenar muscles-wrist motor conduction velocity was(53.94±11.47)m/s,the FIM ADL score was(34.38±7.22)points,and FMA The upper limb score was(34.23±7.25)points,and the control group was(41.86±8.22)m/s,(40.88±8.28)m/s,(49.05±10.01)m/s,(27.81±6.01)points,(41.05±9.19)points.The difference between the two groups was statistically significant(P＜0.05).The total incidence of complications was 4.00％in the observation group and 20.00％in the control group(P＜0.05).Conclusion Small incision in situ release is effective in the treatment of CTS patients,which can improve the surgical indications and nerve conduction velocity,help patients recover upper limb function,and reduce the incidence of postoperative complications.

Objective:To report the clinical characteristics of a case of childhood amyotrophic lateral sclerosis (ALS) caused by SPTLC2 c.778G>A (p.Glu260Lys) mutation. Methods:Whole exon sequencing or whole genome sequencing data from 1 936 patients in the ALS cohort of Peking Union Medical College Hospital were screened for SPTLC2 gene mutations. Clinical data, laboratory examination, neurophysiological examination and genetic test results of the proband were collected. Results:Only one 9-year-old male child with SPLTC2 gene mutation was found. He was admitted to the Department of Neurology, Peking Union Medical College Hospital in December 2022 due to"progressive limb weakness for more than 4 years". Physical examination revealed atrophy and fasciculations of the tongue. Weakness of 4 limbs, muscle atrophy, as well as bilateral hyperreflexia, clonus, and Babinski sign were present. Whole genome sequencing indicated that SPTLC2 gene had c.778G>A (p.Glu260Lys) missense mutation, and no other pathogenic mutations of ALS related genes were detected. Sanger sequencing and family verification showed that neither father nor mother carried the mutation, suggesting that it was a de novo mutation. Nerve conduction velocity test showed no abnormalities, and electromyography suggested neurogenic lesions. Neurofilament light chain in cerebrospinal fluid and serum were increased significantly. The patient′s symptoms continued worsening even after oral administration of L-serine. Conclusion:SPTLC2 gene mutation can cause childhood ALS, and further study of its potential pathogenesis is helpful to uncover another potential pathway of ALS and a novel therapeutic target.

BACKGROUNDThe long-time exercise test (ET) is used to diagnose the primary periodic paralyses (PPs). However the reference values of ET are many and various. This study aimed to investigate the reference value of long-time ET in the diagnosis of PPs.

METHODSWe recruited 108 healthy subjects, 68 patients with PPs, and 72 patients with other diseases for the study. The procedure of ET was made on the basis of the McManis' method. Electrical responses were recorded from right abductor digiti minimi (ADM) muscle when stimulation of the ulnar nerve at the wrist. After the compound muscle action potential (CMAP) was monitored, subjects were then asked to contract the muscle as strongly as possible for 5 minutes. CMAPs were recorded for 2 seconds immediately after cessation of exercise, then every 5 minutes for 10 minutes, and finally every 10 minutes for 50 minutes. In general, the CMAP amplitudes will fall below the pre-exercise levels in an hour. The largest decrease was calculated and used as results of ET.

RESULTSThe CMAP amplitude decreases had no significant differences between groups when the healthy adults were grouped according to age, gender, height, weight and test time. Decreases in PPs patients (57.76%) were significantly more than in healthy subjects (15.21%) and other disease patients (18.10%, P < 0.001). Receiver operating characteristic (ROC) curve analysis showed that the best threshold is 35.50%.

CONCLUSIONSIn the long-time exercise test, threshold of 35.50% for the CMAP amplitude decrease was identified for abnormal. The result is not influenced by age, gender, height, weight, and test time. About 7.4% of healthy subjects were abnormal in ET.

Adult ; Aged ; Aged, 80 and over ; Exercise Test ; methods ; Female ; Humans ; Male ; Middle Aged ; Paralyses, Familial Periodic ; diagnosis ; Prospective Studies ; Reference Values

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 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.