Binder-Free LiMn2 O4 Nanosheets on Carbon Cloth for Selective Lithium Extraction from Brine via Capacitive Deionization.

In this study, a three-step strategy including electrochemical cathode deposition, self-oxidation, and hydrothermal reaction is applied to prepare the LiMn2 O4 nanosheets on carbon cloth (LMOns@CC) as a binder-free cathode in a hybrid capacitive deionization (CDI) cell for selectively extracting lithium from salt-lake brine. The binder-free LMOns@CC electrodes are constructed from dozens of 2D LiMn2 O4 nanosheets on carbon cloth substrates, resulting in a uniform 2D array of highly ordered nanosheets with hierarchical nanostructure. The charge/discharge process of the LMOns@CC electrode demonstrates that visible redox peaks and high pseudocapacitive contribution rates endow the LMOns@CC cathode with a maximum Li+ ion electrosorption capacity of 4.71 mmol g-1 at 1.2 V. Moreover, the LMOns@CC electrode performs outstanding cycling stability with a high-capacity retention rate of 97.4% and a manganese mass dissolution rate of 0.35% over ten absorption-desorption cycles. The density functional theory (DFT) theoretical calculations verify that the Li+ selectivity of the LMOns@CC electrode is attributed to the greater adsorption energy of Li+ ions than other ions. Finally, the selective extraction performance of Li+ ions in natural Tibet salt lake brine reveals that the LMOns@CC has selectivity ( α Mg 2 + Li + $\alpha _{{\mathrm{Mg}}^{2 + }}^{{\mathrm{Li}}^ + }$ = 7.48) and excellent cycling stability (100 cycles), which would make it a candidate electrode for lithium extraction from salt lakes.

Estrogen deficiency induces bone loss through the gut microbiota.

Postmenopausal osteoporosis is a common bone metabolic disease, and gut microbiota (GM) imbalance plays an important role in the development of metabolic bone disease. Here, we show that ovariectomized mice had high levels of lipopolysaccharide in serum and gut microbiota dysbiosis through increases in luminal Firmicutes:Bacteroidetes ratio. We depleted the GM through antibiotic treatment and observed improvements in bone mass, bone microstructure, and bone strength in ovariectomized mice. Conversely, transplantation of GM adapted to ovariectomy induced bone loss. However, GM depletion reversed ovariectomy-induced gene expression in the tibia and increased periosteal bone formation. Furthermore, bioinformatics analysis revealed that the G-protein-coupled bile acid receptor (TGR5) and systemic inflammatory factors play key roles in bone metabolism. Silencing TGR5 expression through small interfering RNA (siRNA) in the local tibia and knockout of TGR5 attenuated the effects of GM depletion in ovariectomized mice, confirming these findings. Thus, this study highlights the critical role of the GM in inducing bone loss in ovariectomized mice and suggests that targeting TGR5 within the GM may have therapeutic potential for postmenopausal osteoporosis.

Health related quality of life and its impacts by chronic diseases in urban community population, Shanghai, China in 2015.

This survey aimed to describe the health-related quality of life (HRQoL) and to explore the relationship between chronic diseases and HRQoL among urban residents in Shanghai, China. A cross-sectional study of 9 426 adults was conducted in Xuhui District of Shanghai, China in 2015. The EuroQol five-dimension three-level (EQ-5D-3 L) was used to measure HRQoL. The average age of subjects was 55.6 ± 17.4 years and 53% were female. Their mean values of utility and visual analogue scale (VAS) were 0.974 ± 0.099 and 80.00 ± 12.36, respectively, which were above the Chinese norm values. Women had lower scores compared with men. The utility value decreased with age, which accelerated after the age of 55 years. Chronic conditions including diabetes, tumor, cardiovascular disease, and respiratory disease, were significantly related to HRQoL, and the reported proportions of problems in the five dimensions increased with the number of chronic diseases (p < 0.05). Chronic diseases except for respiratory disease had a negative effect on HRQoL utility value and VAS score after the adjustment for covariates (p < 0.05). Chronic diseases had a negative impact on both EQ-5D-3 L utility and VAS scores, although the health-related quality of life for the study was above the national average.

[Upper motor neuron involvement in Kennedy disease evaluated by triple stimulation technique].

OBJECTIVE: To explore the presence of subclinical upper motor neuron (UMN) dysfunction in Kennedy disease (KD) patients with triple stimulation technique (TST). METHODS: At our hospital from July 2013 to July 2014, a total of 20 KD patients with clinical manifestations, genetic testing and routine electrophysiological tests were examined by triple stimulation technique (TST) combining transcranial magnetic stimulation (TMS) of motor cortex with peripheral collision studies. And the results were expressed by TST amplitude ratio (TST test/TST control). RESULTS: All patients had typical presentations and were genetically proved. The expansion size of polymorphic tandem cytosine-adenine-guanine (CAG) repeat in the first exon of androgen receptor gene was (44 ± 5). Electromyography showed acute and chronic neurogenic damage. Sensory nerve action potentials declined in amplitude. TST amplitude ratio was normal for 13 KD patients and 7 cases were significantly altered. Tendon hyperreflexia was found in 1 patient with normal TST. In patients with abnormal TST, central motor conduction time (CMCT) was normal in 4 patients and tendon hyperreflexia was found in 5 patients. The possible reasons for 7 patients with abnormal TST were concurrent lacunar infaction (n = 2), concurrent spondylotic myelopathy (n = 2) and KD alone (n = 3). CONCLUSION: Subclinical involvement of UMN may be present in KD patients while other potential causes must be excluded.

[Neurophysiologic studies of POEMS syndrome and its related diseases].

OBJECTIVE: To explore the electrophysiological characteristics of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS) syndrome and its related diseases. METHODS: The electrophysiological characteristics were analyzed retrospectively from June 2000 to June 2013 in patients with POEMS syndrome, monoclonal gammopathy of undetermined significance (MGUS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Electromyography and nerve conduction examinations of median, ulnar, tibial, peroneal and sural nerves were performed. RESULTS: In patients with POEMS syndrome, the amplitude of compound muscle action potential (CMAP) of tibial and peroneal nerves was lower than that of median and ulnar nerves ((1.02 ± 0.23) vs (2.12 ± 0.30) mV, (P < 0.05) ); compared with patients with CIDP, the amplitude of CMAP for POEMS patients was lower ((2.12 ± 0.30) vs (3.94 ± 0.52) mV, (1.02 ± 0.23) vs (3.65 ± 0.57) mV) (P < 0.05) while terminal latency indices (TLI) was higher ((0.41 ± 0.13) vs (0.22 ± 0.01), (0.45 ± 0.16) vs (0.24 ± 0.13) ) (P < 0.05) and the occurring rate of conduction block (CB) and temporal dispersion (TD) was lower (P < 0.05). Compared with MGUS patients, the amplitude of CMAP for POEMS patients was lower ((2.12 ± 0.30) vs (3.81 ± 0.83) mV, (1.02 ± 0.23) vs (3.82 ± 0.63) mV) (P < 0.05) ; sensory conduction velocity of POEMS patients was faster ((33 ± 5) vs (20 ± 3) m/s, (28 ± 4) vs (18 ± 3) m/s)(P < 0.05)). CONCLUSION: Nerve conduction studies of POEMS syndrome implicate both axonal loss and demyelination.Uniform demyelination is more predominant in nerve trunk rather than distal nerve terminals. CB and TD occur less frequently and axonal loss is length-dependent.

[Study on chemical constituents from crop pathogenic fungus active fraction of Wisteria sinensis tumor].

OBJECTIVE: To study the chemical constituents from the crop pathogenic fungus active fraction of Wisteria sinensis tumor. METHODS: The chemical constituents were extracted of different concentrations and isolated by silica gel and Sephadex LH-20 column. The chemical structures of components were further elucidated by the physicochemical characters and MS, NMR spectral data. RESULTS: Ten compounds were isolated and identified as dibutyl phthalate (1), diacetonealcohol (2), pinoresinol (3), stellasterol (4), oleanolic acid (5), olean-12-ene-3-oxo-22,24-diol (6), betulinic acid (7), 2',4',4-hydroxy-chalcone (8), avicularin (9) and quercetin-3-O-ß-D-glucopyranoside (10). CONCLUSION: All the compounds are isolated from the genus for the first time.

[Clinical and neuro electrophysiologic study of flail arm syndrome].

OBJECTIVE: To explore the clinical and neuroelectrophysiological characteristics of flail arm syndrome (FAS). METHODS: The clinical and neuroelectrophysiological characteristics were analyzed retrospectively from July 1, 2006 to July 1, 2012 in FAS patients (n = 56), those with upper limb onset amyotrophic lateral sclerosis (ALS) (n = 60) and those with upper brachial plexus neuropathy (n = 22) from our hospital. Nerve conduction study of musculocutaneous, axillary, median, ulnar, radial, tibial, peroneal and sural nerves and electromyogram (EMG) of bulbar, cervical, thoracic and lumbosacral regions were conducted. RESULTS: In FAS patients, the ratio of male-to-female was 5:1 and they were characterized by symmetric, proximal wasting and weakness of upper extremities, the involved muscles demonstrated neurogenic damage on EMG and the amplitude of compound muscle action potential (CMAP) of arms decreased. Compared with ALS patients, the amplitude of motor unit action potential (MUAP) of deltoid muscle and biceps muscle was higher in FAS patients ((1531 ± 76) vs (898 ± 57) µV; (1433 ± 57) vs (872 ± 75) µV) (F = 13.25, 6.33; P < 0.05). Compared with upper brachial plexus neuropathy patients, the amplitude of MUAP of first dorsal interosseous, deltoid and biceps muscles was higher in FAS patients ((1263 ± 24) vs (507 ± 42) µV; (1531 ± 76) vs (564 ± 27) µV; (1433 ± 57) vs (593 ± 36) µV) (F = 12.32, 16.71, 8.35; P < 0.05). CONCLUSION: The clinical manifestations of FAS are symmetric, proximal wasting and weakness of arms. And the involved muscles show denervation on EMG and the amplitudes of CMAPs decrease in motor nerves of upper extremities.

[Clinical and neurophysiologic profiles of sensory neuron disease].

OBJECTIVE: To explore the clinical and electrophysiological characteristics of sensory neuron disease (SND). METHODS: The clinical and electrophysiological characteristics were analyzed from November 2007 to November 2012 in 57 patients with sensory neuron disease and another 95 with sensory polyneuropathy. Nerve conduction studies of median nerve, ulnar nerve, tibial nerve, peroneal nerve and sural nerve and electromyogram (EMG) of bulbar, cervical, thoracic and lumbosacral region, somatosensory evoked potential (SEP) and contact heat evoked potential (CHEP) were performed. RESULTS: The amplitude of sensory nerve action potential (SNAP) decreased in 50 SND patients and disappeared in another 7. The parameters of SEP and CHEP were abnormal. The amplitude of SNAP was lower in median and ulnar nerve than in sural nerve ((0.6 ± 0.2) µV, (0.7 ± 0.2) µV vs (1.5 ± 0.5) µV; t = 2.42, 2.38; P < 0.05). The latencies of SEPs were longer in SND patients than in those with sensory polyneuropathy (t = 1.99, 1.99, 2.00, 2.07, 1.99; 1.98, 1.99, 2.02, 1.98, 1.99; P < 0.05).Comparing with those with sensory polyneuropathy, the latencies of CHEP were longer in SND patients when the stimuli was applied at hand dorsum, proximal volar forearm and anticus tibialis (t = 2.01, 2.00, 2.02; P < 0.05). No difference existed in latencies between 2 groups when the stimuli was at the levels of C7 and T12 (t = 0.97, 0.68; P > 0.05). CONCLUSION: Sensory neuron diseases usually present with sensory symptoms and ataxia. The amplitude of SNAP decreases or disappears especially in upper extremities. Both SEP and CHEP are abnormal.

[Phenolic derivatives from Wisteria sinensis sweet caulis].

OBJECTIVE: To study the phenolic derivatives from Wisteria sinensis. METHODS: From the petroleum ether extraction of 95% alcohol permeating extraction, 12 compounds were isolated and purified with silica gel and Sephadex LH-20 column chromatography. Their structures were determined on the basis of physicochemical properties and spectroscopic analysis. RESULTS: They were identified as formononetin (1), (3S)-vestitol (2), (6aS, 11aS)-medicarpin (3), (-+/-) isoduartin (4), (3S)-7,1'-dihydroxy-8,3'-dimethoxyisoflavan (5), isosakuranetin (6), p-hydroxy-benzaldehyde (7), 11b-hydroxy-11b, 1-dihydromedicarpin (8), 3-hydroxy-medicarpin (9), syringaresinol (10), 5-hydroxy-7-methoxychromone (11), vanillin (12). CONCLUSION: All the compounds are isolated from this plant for the first time.

Bone mass loss in chronic heart failure is associated with sympathetic nerve activation.

PURPOSE: Chronic heart failure causes osteoporosis, but the mechanism remains unclear. The sympathetic nerve plays an important role in both bone metabolism and cardiovascular function. METHODS: Thirty-six adult male SD rats were randomly divided into the following four groups: sham surgery (Sham) group, guanethidine (GD) group, abdominal transverse aorta coarctation-induced heart failure + normal saline (TAC) group, and TAC + guanethidine (TAC + GD) group. Normal saline (0.9 % NaCl) or guanethidine (40 mg/kg/ml) was intraperitoneally injected daily for 5 weeks. Then, DXA, micro-CT, ELISA and RT-PCR analyses were performed 12 weeks after treatment. RESULTS: The bone loss in rats subjected to TAC-induced chronic heart failure and chemical sympathectomy with guanethidine was increased. Serum norepinephrine levels were increased in rats with TAC-induced heart failure but were decreased in TAC-induced heart failure rats treated with guanethidine. The expression of α2A adrenergic receptor, α2C adrenergic receptor, osteoprotegerin (OPG), and osteocalcin in the tibia decreased in the TAC-induced heart failure group, and the expression of ß1 adrenergic receptor, ß2 adrenergic receptor, receptor activator of nuclear factor-κ B ligand (RANKL), and RANKL/OPG in the tibia increased in the heart failure group. In addition, these changes in gene expression levels were rescued by chemical sympathectomy with guanethidine. CONCLUSIONS: TAC-induced chronic heart failure is associated with bone mass loss, and the sympathetic nerve plays a significant role in heart failure-related bone mass loss. MINI ABSTRACT: The present study supports the hypothesis that heart failure is related to bone loss, and the excessive activation of sympathetic nerves participates in this pathophysiological process. The present study suggests a potential pathological mechanism of osteoporosis associated with heart failure and new perspectives for developing strategies for heart failure-related bone loss.

[A study of motor unit number estimation by multiple point stimulation in patients with Hirayama disease].

OBJECTIVE: To explore the significance of motor unit number estimation (MUNE) by using multiple point stimulating technique to evaluate patients with Hirayama disease (HD). METHODS: Multiple point stimulating technique was used to estimate the motor unit number of abductor pollicis brevis and abductor digiti minimi in 35 normal subjects [14 - 33 years old, mean (20.9 ± 4.0) years old, 33 men and 2 women] without nerve and muscle disease and 69 patients definitely diagnosed as HD [16 - 35 years old, mean (21.46 ± 6.61) years old, 67 men and 2 women]. The differences between the two groups were examined by Fisher's exact test and t test. RESULTS: There were 42 patients with atrophy and 27 patients with normal clinical manifestation of left hand. For right hand there were 54 patients with atrophy and 15 normal. For controls, the MUNE value of left abductor pollicis brevis was 226.97 ± 30.59, while that of right side was 228.31 ± 25.35. The MUNE value of left abductor digiti minimi was 237.43 ± 30.78, while that of right side was 240.20 ± 37.73. For HD patients, the MUNE of left abductor pollicis brevis and abductor digiti minimi was 145.66 ± 126.10 (t = 5.07, P < 0.01) and 102.20 ± 112.67 (t = 9.31, P < 0.01) respectively, while those of right hand was 149.72 ± 117.80 (t = 5.31, P < 0.01) and 64.23 ± 69.27 (t = 16.76, P < 0.01) respectively. MUNE of left abductor digiti minimi in 17 patients that was below 200 among 27 patients with normal clinical manifestation (χ(2) = 9.57, P = 0.002). MUNE of right abductor digiti minimi in 12 patients that was below 200 among 15 patients with normal clinical manifestation (χ(2) = 4.64, P = 0.03). CONCLUSIONS: The differences of MUNE values by multiple point stimulating technique between the normal subjects and the HD patients is significant, which suggests this method is very useful to evaluate HD in the early state.

[Diagnostic role of triple stimulation technique in patients with multifocal motor neuropathy].

OBJECTIVE: To detect the conduction block (CB) between nerve root and Erb's point by triple stimulation technique (TST) in patients with multifocal motor neuropathy (MMN). METHODS: The subjects were recruited from Peking University Third Hospital during the period of April 2010 to April 2011. Twelve MMN patients, 30 healthy subjects, 30 patients with cubital tunnel syndrome and 30 patients with amyotrophic lateral sclerosis (ALS) underwent TST along with clinical assessments and nerve conduction studies. TST combined transcranial magnetic stimulation (TMS) of motor cortex with peripheral collision studies. The results were expressed by the TST amplitude ratio. And the conduction block was judged by TST amplitude ratio or the amplitude ratio of compound muscle action potential (CMAP). RESULTS: The TST amplitude ratio of healthy volunteers was 93.0% ± 2.7%. And it was 42.3% ± 7.1% in patients with MMN indicating a proximal CB. There were 47 CBs in distal segments by routine nerve conduction study. Compared with the baseline levels, the patients with definite MMN increased (χ(2) = 6.31, P < 0.05). The TST amplitude ratio (30.5% ± 4.8%) of those with ALS indicated the lesion of pyramid tract (t = 2.43, P < 0.05). And the TST amplitude ratio (92.2% ± 2.6%) of those with cubital tunnel syndrome was normal (t = 0.68, P > 0.05) while the nerve conduction velocity of cubital tunnel syndrome patients (below elbow-above elbow) was slower ((23.6 ± 3.5) m/s) (t = 2.00, P < 0.05). CONCLUSION: TST may be used to detect proximal CB and facilitate the diagnosis of MMN.

[Study on triterpenoids from Wisteria sinensis].

OBJECTIVE: To study the triterpenoids constituents from Wisteria sinensis Sweet Caulis. METHODS: The compounds were beta-solated and purified with silica gel and Sephadex LH-20 column chromatography from the petroleum ether extract. Their structures were determined on the basis of physicochemical properties and spectroscopic analysis. RESULTS: They were identified as beta-sitosterol palmitate (1), alpha-spinasterol (2), (22E, 24R)-5alpha, 8alpha-epidioxy-ergosta-6, 22-dien-3beta-ol (3), (22E, 24R)-ergosta-5, 7, 22-trien-3beta-ol (4), (22E, 24R) -ergosta-7, 22-dien-3beta-ol (5), 11alpha, 12alpha-oxidotaraxerol (6), lupeol (7), betulinic acid (8), 22-oxo-3beta, 24-dihydroxyolean-12-ene (9), 2alpha, 3beta, 23-trihydroxyolean-12-ene (10), soyasapogenol E (11), 3alpha, 21beta-dihydroxy-olean-12-ene (12). CONCLUSION: Compounds 1 - 12 are isolated from this plant for the first time.

[Target points: a discussion on acupuncture treatment of primary trigeminal neuralgia].

In this paper, issues of acupuncture using target points and depth of needling were discussed based on the theory of traditional Chinese medicine acupoints and combined with anatomical and neurological research of Western medicine. The theoretical evidence of acupuncture on nerve foramen and ganglion and the reasons for being nonstandard operating on acupoints were analyzed. This study summarized the method of using acupuncture at Xiaguan (ST7), Cuanzhu (BL2), Sibai (ST2) and Jiachengjiang acupoints to align with the spheno-palatine ganglion and additional nerve foramen (supraorbital, infraorbital and mental foramina) to treat primary trigeminal neuralgia. This study adhered to the Standards for Reporting Interventions in Clinical Trials of Acupuncture and helped in development of study standardization for acupuncture processes. The authors hoped that should help in significantly diminishing risk and improving therapeutic efficacy in clinic.

Selective removal of Sr(II) from saliferous radioactive wastewater by capacitive deionization.

90Sr-containing radioactive wastewater during Fukushima nuclear accident (FNA) aroused extensive consideration for its disposal. Massive coexisted Na+ ions seriously inhibited Sr2+ removal, aggravating the expenditure of radioactive wastewater treatment. Herein, a chestnut shell derived porous carbon material modified with aryl diazonium salt (ADS) of sodium 4-aminoazobenzene-4'-sulfonate (SPAC) was developed as capacitive deionization electrode for selective removal of Sr2+ from saliferous radioactive wastewater. Based on ADS modification, the Sr2+ electrosorption capacity of SPAC electrode was improved to 33.11 mg g-1 with fast ion removal rate of 2.89 mg g-1 min-1, comparing with only 16.10 mg g-1 before modification. The isothermal adsorption and kinetics by SPAC electrode fitted well with Langmuir and pseudo-second-order model, achieving a maximum Sr2+ electrosorption capacity of 58.21 mg g-1, superior cycling stability, and excellent charge efficiency (77.63%). Fascinatingly, the SPAC electrode exhibited superhigh Sr2+ selectivity of 70.65 against Na+ in Na+-Sr2+ mixed solution with molar ratio of Na+:Sr2+ as 20:1. Density functional theory (DFT) simulation, combining with electrochemical and spectral analyses, revealed that the high overlap of electron cloud between Sr2+ ion and anionic sulfonic group (-SO3-) provided SPAC with remarkable selectivity of Sr2+ ion, and illustrated the ion-swapping mechanism of Sr2+ selectivity.

High-performance pseudocapacitive removal of cadmium via synergistic valence conversion in perovskite-type FeMnO3.

Cadmium pollution is a serious threat for the global drink water and natural environment. Herein, a poly-pyrrole coated dual-metal perovskite-type oxide FeMnO3 (PFMO@PPy) was developed firstly as pseudocapacitive cathode for the reversible capture and release of cadmium ions by asymmetry pseudocapacitive deionization (APCDI) technology, extending the library of CDI electrodes. Our work highlighted several points: (i) PFMO@PPy achieved a maximum Cd-removal capacity of 144.6 mg g-1, and maintained the retention rate of 93.4% after 15-cycle CDI process for up to 150 h, far beyond other previous work. (ii) PFMO@PPy showed the superior removal ratio (~90%) under different real water environments such as tap water, lake water and the groundwater. (iii) The superior Cd(II) electrosorption and desorption behavior is ascribed to the reversible synergistic valence conversion (Fe3+/Fe0 and Mn3+/Mn2+), which is confirmed by ex-situ XPS measurement and electrochemical tests. (iv) DFT calculations confirmed the synergistic effect from Mn and Fe elements in perovskite-type bimetallic oxide FeMnO3. This study paves a new way for promising future applications of perovskite-type oxides containing dual Faradic redox-activity for wastewater treatment and environmental remediation.

Upper trapezius electromyography aids in the early diagnosis of bulbar involvement in amyotrophic lateral sclerosis.

Electromyography (EMG), particularly measurements of the tongue or sternocleidomastoid, aids in the diagnosis of amyotrophic lateral sclerosis (ALS) and may be used to identify lower motor neuron lesions in the bulbar region. Abnormal trapezius EMG recordings were recently shown to be useful in diagnosing ALS. Here, we investigated the role of upper trapezius EMG in assessing bulbar involvement in ALS. Standard EMG measurements were recorded from the upper trapezius in ALS, cervical spondylotic myelopathy (CSM), and normal controls (NC). Forty-three CSM patients were examined pre-operatively and three months post-operatively. Greater spontaneous activity levels were observed in upper trapezius EMG measurements of ALS patients with a disease duration of ≤8 months (70%), compared with patients with a disease duration of >8 months (40%). Significant differences in motor unit action potential parameters were also noted between ALS and CSM or NC. Fewer spontaneous EMG recordings were detected in CSM post-operatively. Furthermore, no differences in neurogenic EMG measurements were observed in ALS between the trapezius and sternocleidomastoid muscles (p = 0.22). In conclusion, upper trapezius EMG recordings may provide valuable information for assessing the clinical and subclinical involvement of bulbar lower motor neurons in ALS patients, particularly at early disease stages.

[Role of fasciculation potentials in the diagnosis of amyotrophic lateral sclerosis].

OBJECTIVE: To explore the role of fasciculation potentials in the diagnosis of amyotrophic lateral sclerosis (ALS). METHODS: A total of 60 ALS patients were recruited from August 2009 to August 2010 at our hospital. Standard examinations of electromyography and nerve conduction were performed. And fasciculation potentials were measured in the resting muscles with a band pass of 20 Hz-10 kHz, a sensitivity of 0.05 - 0.5 mV/D and a sweep speed of 5 ms/D. Each muscle was observed for 1 - 5 minutes. The amplitude, duration and phase of fasciculation potentials were recorded. RESULTS: The occurrence rate of fasciculation potential in sternocleidomastoid was more than that of fibrillation and positive sharp wave in ALS patients (36.7% vs 13.3%, χ² = 8.71, P < 0.05). The amplitude and duration of fasciculation potentials in ALS patients with a duration of over 18 months were higher than that of those with a duration of under 8 months [(1.8 ± 0.9) mV, (1.0 ± 0.6) mV; (15.2 ± 4.1) ms, (11.2 ± 3.0) ms; q = 3.43, 3.51, P < 0.05)]. The patients with definite ALS might increase if fasciculation potentials were considered as spontaneous potential (80% vs 60%; χ² = 5.71, P = 0.02). CONCLUSION: With different morphologies at different disease stages, fasciculation potentials may be useful in the earlier diagnosis of amyotrophic lateral sclerosis.

Upper Motor Neuron Signs in the Cervical Region of Patients With Flail Arm Syndrome.

Objective: We investigated upper motor neuron (UMN) signs in the cervical region in a Chinese clinic-based cohort of patients with flail arm syndrome (FAS) by clinical examination and neurophysiological tests such as triple stimulation technique (TST) and pectoralis tendon reflex testing. Methods: A total of 130 consecutive FAS patients from Peking University Third Hospital underwent physical examination and neurophysiological tests at baseline and 3 months, 6 months, 9 months, and 12 months later. Pyramidal signs, pectoralis tendon reflex and TST results were evaluated to estimate the function of cervical spinal UMNs. Results: At the first visit, weakness of the bilateral proximal upper limbs was found in 99 patients, while weakness of a single proximal upper limb was found in 31 patients. There were 49 patients with tendon hyperreflexia, 42 patients with tendon hyporeflexia and 39 patients with tendon areflexia. All except 4 of the patients had brisk pectoralis tendon reflex. The UMN score of the cervical region was 1.7 ± 0.4, and the lower motor neuron score of that region was 3.5 ± 0.3. The TSTtest/TSTcontrol amplitude ratio was 65.7 ± 7.5%. The latency of quantitative detection of the pectoralis tendon reflex was 7.7 ± 1.2 ms. In the follow-up study, the UMN score and the TSTtest/TSTcontrol amplitude ratio decreased, while the lower motor neuron score increased, and the latency of quantitative detection of the pectoralis tendon reflex remained steady. Conclusion: Although the signs of cervical spinal UMN dysfunction in patients with FAS were often concealed by muscle atrophy in the progression of the disease, TST and pectoralis tendon reflex could reveal it.

[Rare variants of HSPB1 are probably associated with amyotrophic lateral sclerosis].

OBJECTIVE: To explore the association between rare HSPB1 variants and amyotrophic lateral sclerosis (ALS). METHODS: We performed next-generation sequencing for 166 Chinese ALS patients to screen for possible pathogenic rare variants of HSPB1. The control individuals were obtained from 1000 Genome Project and an in-house whole-exome sequencing database. The Sequence Kernel Association Test (SKAT) and the SKAT-optimal test (SKAT-O) were used to identify the association between rare HSPB1 variants and ALS. RESULTS: We identified 3 possible pathogenic rare variants of HSPB1 (all were missenses), including c.379C>T (p.R127W), c.446A>C (p.D149A) and c.451A>C (p.T151P). Compared with 1000 Genome Project, SKAT p=3.61×10-7 and SKAT-O p=1.62×10-6; while compared with the in-house database, SKAT p=9.99×10-4, SKAT-O p= 1.80×10-3. We analyzed the phenotypes of rare HSPB1 variant carriers and found no specific clinical characteristics associated with these variants. CONCLUSIONS: Rare variants of HSPB1 are probably associated with the pathogenesis of ALS.