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1.
Int J Mol Med ; 45(2): 333-342, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31894261

RESUMO

Ischemic stroke (IS) is a severe neurological disease and a major cause of death and disability throughout the world. A long non­coding (lnc)RNA, transcription factor (TF) and gene can form a lncRNA­mediated regulatory triplet (LncMRT), which is a functional network motif that regulates numerous aspects of human diseases. However, systematic identification and molecular characterization of LncMRTs and their roles in IS has not been carried out. In the present study, a global LncMRT network was constructed and the topological features were characterized based on experimentally verified interactions. An integrated approach was developed to identify significantly dysregulated LncMRTs in peripheral blood mononuclear cells of IS and these dysregulated LncMRT networks exhibited specific topological characteristics and a closer network structure than the global LncMRT network that was constructed. The variation of the risk score for LncMRTs indicated that there were multiple dysregulated patterns of LncMRTs in IS. Numerous core clusters were identified from dysregulated LncMRT networks and these core clusters could distinguish IS patient and matched control samples. Finally, functional analyses demonstrated that LncMRTs associated with IS participated in the regulation of the phosphatidylinositol 3­kinase/protein kinase B signaling pathway. In conclusion, the roles of the LncMRTs in IS were elucidated, which could be beneficial for understanding IS pathogenesis and treatment.

2.
Clin Neurophysiol ; 131(1): 243-258, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31761717

RESUMO

Standardization of Electromyography (EMG) instrumentation is of particular importance to ensure high quality recordings. This consensus report on "Standards of Instrumentation of EMG" is an update and extension of the earlier IFCN Guidelines published in 1999. First, a panel of experts in different fields from different geographical distributions was invited to submit a section on their particular interest and expertise. Then, the merged document was circulated for comments and edits until a consensus emerged. The first sections in this document cover technical aspects such as instrumentation, EMG hardware and software including amplifiers and filters, digital signal analysis and instrumentation settings. Other sections cover the topics such as temporary storage, trigger and delay line, averaging, electrode types, stimulation techniques for optimal and standardised EMG examinations, and the artefacts electromyographers may face and safety rules they should follow. Finally, storage of data and databases, report generators and external communication are summarized.

3.
J Med Genet ; 57(2): 109-120, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31506324

RESUMO

PURPOSE: Facioscapulohumeral muscular dystrophy (FSHD) is a common adult muscular dystrophy. Over 95% of FSHD cases are associated with contraction of the D4Z4 tandem repeat (~3.3 kb per unit) at 4q35 with a specific genomic configuration (haplotype) called 4qA. Molecular diagnosis of FSHD typically requires pulsed-field gel electrophoresis with Southern blotting. We aim to develop novel genomic and computational methods for characterising D4Z4 repeat numbers in FSHD. METHODS: We leveraged a single-molecule optical mapping platform that maps locations of restriction enzyme sites on high molecular weight (>150 kb) DNA molecules. We developed bioinformatics methods to address several challenges, including the differentiation of 4qA with 4qB alleles, the differentiation of 4q35 and 10q26 segmental duplications, the quantification of repeat numbers with different enzymes that may or may not have recognition sites within D4Z4 repeats. We evaluated the method on 25 human subjects (13 patients, 3 individual control subjects, 9 control subjects from 3 families) labelled by the Nb.BssSI and/or Nt.BspQI enzymes. RESULTS: We demonstrated that the method gave a direct quantitative measurement of repeat numbers on D4Z4 repeats with 4qA allelic configuration and the levels of postzygotic mosaicism. Our method had high concordance with Southern blots from several cohorts on two platforms (Bionano Saphyr and Bionano Irys), but with improved quantification of repeat numbers. CONCLUSION: While the study is limited by small sample size, our results demonstrated that single-molecule optical mapping is a viable approach for more refined analysis on genotype-phenotype relationships in FSHD, especially when postzygotic mosaicism is present.

4.
Artigo em Inglês | MEDLINE | ID: mdl-31619447

RESUMO

OBJECTIVE: To describe the detailed clinical characteristics, immunotherapy, and long-term outcomes of patients with anti-NMDA receptor (NMDAR) encephalitis in China. METHODS: A single-center, prospective study. Patients who met the diagnostic criteria were enrolled from 2011 to 2017 and followed up. The clinical features, treatment, and long-term outcomes were collected prospectively. Factors affecting the long-term prognosis were analyzed. RESULTS: The study included 220 patients. The most common clinical presentations were psychosis (82.7%) and seizures (80.9%). Of the patients, 19.5% had an underlying neoplasm; of which ovarian teratoma was 100% of tumors in females and only one male had lung cancer. Most patients (99.5%) received first-line therapy (glucocorticoids, IV immunoglobulin, or plasmapheresis alone or combined), and only 7.3% received second-line immunotherapy (rituximab, cyclophosphamide alone, or combined). Long-term immunotherapy (mycophenolate mofetil or azathioprine >1 year) was administered to 53.2% of patients. During the first 12 months, 207 (94.1%) patients experienced improvement, and 5 (2.3%) died, whereas 38 (17.3%) experienced relapses. At 12-month follow-up, 92.7% had favorable clinical outcomes (modified Rankin Scale score ≤2). CONCLUSIONS: Patients in China present with psychosis and seizure frequently but have a low percentage of underlying neoplasms. Re-enforced first-line immunotherapy is effective in managing anti-NMDAR encephalitis in the acute phase. Although relapse is relatively common, with combined first-line and long-term immunotherapy, most patients reached favorable outcomes.

5.
Muscle Nerve ; 61(1): 104-107, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31587332

RESUMO

BACKGROUND: The abduction range of the little finger in the long exercise test (ET) has rarely been reported in patients with hypokalemic periodic paralysis (HypoPP) during inter-attack periods, and the diagnostic value requires clarification. METHODS: The long ET was performed in 43 HypoPP patients during inter-attack periods and in 20 healthy controls (HCs). The compound muscle action potential (CMAP) and the abduction range of the little finger were recorded concurrently. RESULTS: There were significant differences in the percent changes of the CMAP amplitudes and the abduction ranges after exercise between HypoPP patients and the HCs. The curve of percent changes in abduction ranges overlapped substantially with that of the CMAP amplitudes, and the sensitivity, specificity, and cutoff values were 0.860, 0.900, and 22.6%, respectively. CONCLUSIONS: The abduction range of the little finger can serve as a novel parameter in the long ET for the diagnosis of HypoPP during inter-attack periods.

6.
Thorac Cancer ; 2019 Dec 10.
Artigo em Inglês | MEDLINE | ID: mdl-31823509

RESUMO

Immune checkpoint inhibitors (ICIs) can cause adverse reactions in the nervous system. The incidence rate is 0.1%-12% and 80% of nervous system adverse reactions occur within the first four months of application. ICIs can cause diseases of various parts of the nervous system including central nervous system diseases such as aseptic meningitis, meningeal encephalitis, necrotizing encephalitis, brainstem encephalitis, transverse myelitis, etc., and peripheral neuropathy such as cranial nerve peripheral neuropathy, multifocal nerve root neuropathy, Guillain-Barré syndrome, spinal nerve root neuropathy, myasthenia gravis, myopathy, etc. For these complications of the nervous system, diagnosis could be difficult. Physicians require a specific collection of nervous system symptoms and signs, combined with supplementary examinations including imaging, cerebrospinal fluid cytology, EEG or electromyography in order to exclude infection or malignant tumor before reaching a final diagnosis. With regard to treatment, ICIs should be discontinued in severe cases, and large doses of glucocorticoid or gamma globulin administered, and supportive treatment may be necessary. If severe adverse reactions of the nervous system occur, the prognosis could be poor.

7.
Muscle Nerve ; 2019 Dec 14.
Artigo em Inglês | MEDLINE | ID: mdl-31837161

RESUMO

INTRODUCTION: Detailed morphological features of the peripheral nerves could improve the diagnostic sensitivity in some polyneuropathies. In this study we aimed to establish multiple-site, cross-sectional area (CSA) reference values for peripheral nerves of upper extremities in a healthy Chinese population. METHODS: One hundred eleven healthy subjects, 15 to 70 years of age, were prospectively recruited. CSA at predetermined sites of the median, ulnar, radial nerves, and brachial plexus was measured bilaterally. Ten consecutive sites were studied along the median/ulnar nerves. RESULTS: The CSA at ten sites of the median nerve ranged from 5.59 ± 0.89 to 8.43 ± 1.30 mm2 , and for the ulnar nerve from 2.94 ± 0.57 to 5.63 ± 1.08 mm2 . Both age and gender correlated with nerve CSA at most sites. DISCUSSION: CSA was not uniform along the length of the median and ulnar nerves. The multiple-site reference values could be helpful for investigating polyneuropathies in the Asian population.

8.
Eur J Paediatr Neurol ; 23(5): 755-759, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31679561

RESUMO

3-Hydroxyisobutyryl-CoA hydrolase (HIBCH) deficiency is a recently described disease resulting from mutations in HIBCH with no effective treatment. Here, we report a female Chinese patient presenting with exercise-induced dystonia and bilateral symmetrical hyperintensities of the globus pallidus on brain MRI associated with novel HIBCH mutations (c.1027C>G;p. H343D and c.383T>A;p.V128D). After treatment for 1 year with a low-valine diet, both clinical symptoms and brain lesions improved substantially. We propose that HIBCH deficiency should be considered in the differential diagnosis for patients with exercise-induced dystonia, particularly if bilateral symmetrical lesions in the globus pallidus are present. A low-valine diet is a potentially promising treatment for HIBCH deficiency.


Assuntos
Anormalidades Múltiplas/dietoterapia , Erros Inatos do Metabolismo dos Aminoácidos/dietoterapia , Distúrbios Distônicos/dietoterapia , Exercício , Tioléster Hidrolases/deficiência , Anormalidades Múltiplas/diagnóstico por imagem , Erros Inatos do Metabolismo dos Aminoácidos/complicações , Erros Inatos do Metabolismo dos Aminoácidos/diagnóstico por imagem , Criança , Distúrbios Distônicos/complicações , Feminino , Globo Pálido/diagnóstico por imagem , Sequenciamento de Nucleotídeos em Larga Escala , Humanos , Imagem por Ressonância Magnética , Mutação de Sentido Incorreto , Tioléster Hidrolases/genética , Resultado do Tratamento
9.
Hum Mutat ; 2019 Nov 09.
Artigo em Inglês | MEDLINE | ID: mdl-31705731

RESUMO

Exonic deletions and duplications within DMD are the main pathogenic variants in Duchenne and Becker muscular dystrophies (DMD/BMD). However, few studies have profiled the flanking sequences of breakpoints and the potential mechanism underlying the breakpoints in different fragile regions of DMD. In this study, 896 Chinese male probands afflicted with DMD/BMD were selected from unrelated families and analyzed using multiplex ligation-dependent probe amplification of the DMD gene, in which we identified exon deletions in 784 subjects and duplications in 112 subjects. Deletions occurred most frequently in the genomic region encompassing exons 45-55, accounting for 73% of all deletion patterns. Furthermore, to unravel the potential mechanism that induced breaks, DMD gene capture and sequencing were performed to identify the breakpoints in 37 subjects with deletions encompassing exons 45-55 of DMD; we found that DMD instability did not arise from a single cause; instead, long-sequence motifs, nonconsensus microhomologies, low-copy repeats, and microindels were embedded around the breakpoints, which may predispose DMD to instability. In summary, this study highlights the heterogeneous characteristics of the flanking sequences around the breakpoints and helps us to understand the mechanism underlying DMD gene instability.

10.
BMC Neurol ; 19(1): 283, 2019 Nov 12.
Artigo em Inglês | MEDLINE | ID: mdl-31718577

RESUMO

BACKGROUND: As health behavior varies with increasing age, we aimed to examine the potential barriers in calling emergency medical services (EMS) after recognizing a stroke among 40-74- and 75-99-year-old adults. METHODS: Data were obtained from a cross-sectional community-based study (FAST-RIGHT) that was conducted from January 2017 to May 2017 and involved adults (age ≥ 40 years) across 69 administrative areas in China. A subgroup of residents (153675) who recognized stroke symptoms was analyzed. Multivariable logistic regression models were performed in the 40-74 and 75-99 age groups, separately, to determine the factors associated with wait-and-see behaviors at the onset of a stroke. RESULTS: In the 40-74 and 75-99 age groups, the rates of participants who chose "Self-observation at home" were 3.0% (3912) and 3.5% (738), respectively; the rates of "Wait for family, then go to hospital" were 31.7% (42071) and 33.1% (6957), respectively. Rural residence, living with one's spouse, low income (< 731 US $ per annum), having a single avenue to learn about stroke, and having friends with stroke were factors associated with waiting for one's family in both groups. However, unlike in the 40-74 age group, sex, number of children, family history, and stroke history did not influence the behaviors at stroke onset in the 75-99 age group. CONCLUSIONS: Different barriers from recognizing stroke and calling an ambulance exist in the 40-74 and 75-99 age groups in this specific population. Different strategies that mainly focus on changing the "Wait for family" behavior and emphasize on immediately calling EMS are recommended for both age groups.


Assuntos
Serviços Médicos de Emergência , Comportamentos Relacionados com a Saúde , Conhecimentos, Atitudes e Prática em Saúde , Acidente Vascular Cerebral , Adulto , Idoso , Idoso de 80 Anos ou mais , Ambulâncias , China/epidemiologia , Estudos Transversais , Feminino , Humanos , Modelos Logísticos , Masculino , Pessoa de Meia-Idade , Pobreza , População Rural , Acidente Vascular Cerebral/epidemiologia
11.
Front Neurol ; 10: 1055, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31649606

RESUMO

Introduction: Motor nerve conduction block (CB) is the main electrophysiological feature of multifocal motor neuropathy (MMN). Increased cross-sectional area (CSA) can be detected by nerve ultrasound in MMN. In this study, we aim to analyze the correlation between CB and CSA in MMN. Methods: Twelve patients with MMN were recruited. Ultrasonography tests and motor nerve conduction studies (NCSs) were performed on median and ulnar nerves simultaneously. CSA was measured at 10 consecutive sites on those nerves, meanwhile nerves were traced continuously and recorded thoroughly under ultrasound. Results: In motor NCSs, 12 definite CB and 12 probable CB areas were detected across standard segments of median and ulnar nerves. With ultrasound studies, increased CSA was detected at 36 sites. There were 9 standard segments with CB and increased CSA, 15 segments with CB but normal CSA, and 27 segments with increased CSA but no CB. Discussion: In MMN, motor nerve CB was not always consistent with increased CSA.

12.
Zhongguo Fei Ai Za Zhi ; 22(10): 633-638, 2019 Oct 20.
Artigo em Chinês | MEDLINE | ID: mdl-31650945

RESUMO

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.

13.
J Asthma ; : 1-8, 2019 Oct 14.
Artigo em Inglês | MEDLINE | ID: mdl-31514546

RESUMO

Purpose: The aim of this study was to investigate the efficacy and safety of tratinterol hydrochloride in bronchial asthma (BA) treatment. Methods: Patients enrolled in this study were distributed randomly into a treatment group (tratinterol hydrochloride) and an active control group (procaterol hydrochloride) and were treated for 2 weeks after running-in. The end points were changes in pulmonary function and clinical symptoms after administration. Safety indices were physical examinations, laboratory testing and spontaneous reporting. Findings: We enrolled 732 subjects, -365 in the treatment group and 367 in the active control group. Forced expiratory volume (FEV1), significantly increased in both group after treatment (P < 0.05). Least-squares (LS) means were -0.03/in the full-analysis set (FAS) and -0.02 in the per-protocol set (PPS) set, and 95% confidence intervals (CIs) for these sets were -0.09 to 0.03 and -0.08 to 0.04, respectively. Forced expiratory volume (FVC), morning peak expiratory flow (PEF) and asthma scores were significantly different with pretreatment (P < 0.05). There was no difference in asymptomatic days or frequency of relief medicine use (P > 0.05). No serious adverse events occurred. Implications: Tratinterol hydrochloride was effective, safe and not inferior to procaterol hydrochloride in treating BA.

14.
Brain Behav ; 9(10): e01405, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31515973

RESUMO

OBJECTIVES: Patients with a history of cardiovascular surgery are at risk of stroke, and immediately calling emergency medical services (EMS) after stroke onset is crucial to receiving effective reperfusion therapy. We aimed to determine the effect of a history of cardiovascular surgery on patients' ability to recognize stroke and intent to call EMS. METHODS: We performed a cross-sectional community-based study from January 2017 to May 2017. A total population of 186,167 individuals, recruited from 69 administrative areas across China, was analyzed. Different multivariable logistic regression models were performed to identify the associations between cardiovascular surgical history and stroke recognition or intent to call EMS, respectively. RESULTS: 0.1% of the total population had a history of cardiovascular surgery. In the surgery group, the estimated stroke recognition rate (SRR) and correct action rate (CAR) were 84.9% and 74.7%, respectively. The prevalence of cardiovascular risk factors was significantly higher in the surgery group. Cardiovascular surgical history was not associated with recognition of stroke across different models. The surgery group was more likely to call EMS, but the difference was not significant after full adjustment (OR: 1.40, 95% CI: 0.99-1.98, p = .0572). CONCLUSIONS: Cardiovascular surgical history does not influence patients' likelihood of calling EMS more often at stroke onset. Patients receiving cardiovascular surgeries should be counseled regarding stroke recognition, proper response to stroke, and the importance of controlling risk factors.

15.
Mult Scler Relat Disord ; 36: 101375, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31491597

RESUMO

BACKGROUND: To investigate the efficacy and safety of non-invasive brain stimulation for fatigue in multiple sclerosis patients. METHODS: We searched MEDLINE, Embase, Web of Science, Cochrane Library, Chinese National Knowledge Infrastructure, and Wanfang databases up to October 25, 2018 (PROSPERO registration number: CRD42018112823). Randomized or pseudo-randomized, sham-controlled clinical trials evaluating the effect of non-invasive brain stimulation (NIBS) such as transcranial direct current stimulation (tDCS), transcranial magnetic stimulation (TMS), transcranial random noise stimulation (tRNS), transcranial alternating current stimulation (tACS), cranial electrotherapy stimulation, and reduced impedance non-invasive cortical electrostimulation were included. Two authors independently performed data extraction and risk of bias assessment according to Cochrane Handbook for Systematic Reviews of Interventions Version 5.0.1. The primary outcome was fatigue scores before and after stimulation and the secondary outcome was adverse events. RESULTS: Data from cross-over and parallel group studies were pooled using a generic inverse-variance approach. A total of 14 studies (11 for tDCS, 2 for TMS, and 1 for tRNS) recruiting 207 patients were included in the systematic review and meta-analysis. No eligible tACS, cranial electrotherapy stimulation or reduced impedance non-invasive cortical electrostimulation studies were found. Short-term and long-term treatment effects were significant for tDCS, whereas TMS and tRNS were not superior to sham stimulation. The available evidence supported the effectiveness of the 1.5 mA subgroup and bilateral S1 subgroup of tDCS. Adverse events were minor and transient but comparable between real and sham stimulation. CONCLUSIONS: tDCS is a safe and effective treatment for fatigue in MS patients. However, further studies are required to confirm our results in a large-scale population and to investigate the effectiveness of other NIBS subtypes.

16.
J Clin Neurosci ; 69: 74-80, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31447367

RESUMO

OBJECTIVES: To explore the cause of long survival but early onset and other prognostic factors among Chinese sporadic amyotrophic lateral sclerosis (ALS) patients. METHODS: Patients with ALS were recruited and followed up from Jan 2013 to Jan 2017. Phenotype and survival were compared among different age-at-onset groups. Candidate prognostic factors were analyzed by Kaplan-Meier method, Cox regression and Royston Parmar (RP) model dealing with breaches of proportional hazard assumption. RESULTS: In the cohort of 531 patients, mean age-at-onset was 53.68 years (SD:10.85) and overall estimated median survival time was 59 months (95% CI: 48.29-69.71). Pairwise comparison showed that patients above 65 years at onset were more frequently bulbar onset (adjusted residual: 3.0), less frequently lumbosacral onset (adjusted residual: -3.0), and had shorter survival compared with other age groups (p = 0.002). Cox and RP model demonstrated independent prognostic variables including age at onset, bulbar onset, diagnostic delay, MRC-score at first diagnosis and region of residence. CONCLUSIONS: This clinic-based study suggested that Chinese sporadic ALS patients had relatively long survival probably due to young age and less bulbar onset cases. Short diagnostic delay, low MRC-score and northern residence were also predicative of short survival. Reallocation of resources is needed to optimize quality care and prolong survival time.


Assuntos
Esclerose Amiotrófica Lateral/mortalidade , Idade de Início , Idoso , Esclerose Amiotrófica Lateral/diagnóstico , Esclerose Amiotrófica Lateral/patologia , Grupo com Ancestrais do Continente Asiático , Estudos de Coortes , Progressão da Doença , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Fenótipo , Prognóstico
17.
J Alzheimers Dis ; 71(2): 559-567, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31424402

RESUMO

BACKGROUND: Few studies have investigated the correlation between cerebral microbleeds (CMBs), a hemorrhagic imaging marker of cerebral small vessel disease (CSVD), and brain volume. OBJECTIVE: We investigated the association between the burden and locations of CMBs and brain volume in community-dwelling populations. METHODS: Data were obtained from 1,029 participants who underwent brain magnetic resonance imaging (MRI) and APOE genotyping. Volumes of the whole brain, subcortical white matter (WM), cortical gray matter (GM), and hippocampus were extracted. Linear regression models were used to investigate the relationship between the CMB burden and their location with structural changes. RESULTS: Regarding burden, participants with≥3 CMBs had significantly lower whole brain (ß= -1.124, p = 0.0133), subcortical WM (ß= -1.020, p = 0.0043), and hippocampus (ß= -0.015, p = 0.0088) volumes than those without CMBs. Regarding location and burden, the presence of≥3 strictly lobar CMBs was negatively associated with whole brain volume (ß= -2.838, p = 0.0088). Additionally, higher CMB burdens in strictly lobar locations or deep/mixed locations were associated with lower subcortical WM volume (ß= -1.689, p = 0.0482; ß= -0.872, p = 0.0464, respectively). Finally, the presence of≥3 deep/mixed CMBs was associated with lower hippocampus volume (ß= -0.018, p = 0.0088), and these associations were independent of other ischemic markers of CSVD. However, the CMB burden and distributional pattern did not correlate with cortical GM volumes. CONCLUSION: A higher CMB burden, in specific locations, is associated with decreased brain volumes in community-dwelling populations.

18.
Artigo em Inglês | MEDLINE | ID: mdl-31364874

RESUMO

Objective: Split-hand sign is a useful clinical and electrophysiological feature in the diagnosis of amyotrophic lateral sclerosis (ALS). We proposed a novel split-hand index (SI) using F-wave persistence (FP) and assessed its diagnostic utility in ALS. Methods: Eighty-three consecutive ALS patients were recruited, and 50 healthy, age-, and height-matched volunteers were used as a control group. Compound muscle action potentials (CMAP) and FP were recorded from the abductor pollicis brevis (APB), first dorsal interosseous (FDI), and the abductor digiti minimi (ADM) muscles. The SI derived from FP was calculated using the following formula: SIFP = (FPAPB ×FPFDI)/FPADM. The sensitivity and specificity of SIFP and SICMAP in differentiating ALS from healthy controls (HCs) were derived from receiver-operating characteristic (ROC) curve analysis. Results: Both SIFP and SICMAP were significantly reduced in ALS patients. The ROC curve analysis indicated that both SIFP and SICMAP reliably differentiated ALS from HCs [0.92 (95% CI: 0.88-0.95); 0.86 (95% CI: 0.82-0.91)], but SIFP showed better diagnostic accuracy than SICMAP (p = 0.04), with a high sensitivity (81.2%) and specificity (97%). In subgroup analyses, SIFP appeared to be a better variable than SICMAP for differentiation of ALS patients with normal CMAP from HCs, with an area under the curve of 0.87 (95% CI: 0.80-0.93), sensitivity of 69.4%, and specificity of 94%. Conclusion: The SIFP reliably distinguished ALS patients from HCs and may be more sensitive for determining the split-hand pattern of ALS than SICMAP, particularly in the early stage of the disease.

19.
J Clin Neurosci ; 67: 10-13, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31272830

RESUMO

Motor conduction blocks (CBs) and decreased motor nerve conduction velocity (MCV) are both demyelination electrophysiological characteristics. Though CBs are both common in Lewis-Sumner syndrome (LSS) and multifocal motor neuropathy (MMN), they are two distinct disease groups, so their MCV and CBs electrophysiological characteristics may be different. In this paper, we aimed to discuss the relationship between CBs and MCV in Lewis-Sumner syndrome (LSS) and multifocal motor neuropathy (MMN). Sixteen patients with LSS and 11 with MMN were retrospectively collected. Motor nerve conduction studies were performed on bilateral median and ulnar nerves. 16/23 segments with CBs in MMN, and 50/53 segments with CBs in LSS had decreased conduction velocities respectively. There was significant difference in MCV between these two groups (MMN 53.1 ±â€¯12.5 m/s; LSS 34.3 ±â€¯17.1 m/s, mean ±â€¯SD, P < 0.05) as well as significant difference in MCV for segment with or without CBs between MMN and LSS patients (P < 0.05). Patients with LSS were prone to be found MCV decrease in segments of motor CB than patients with MMN (P = 0.017). Forty one segments in LSS patients and 6 segments in MMN had MCV ≤ 38 m/s (P < 0.05). There exist two relationships between CB and MCV no matter in LSS or MMN. The characteristics of MCV and CB can be used to distinguish these two disease groups.


Assuntos
Condução Nervosa/fisiologia , Polineuropatias/fisiopatologia , Adulto , Fenômenos Eletrofisiológicos/fisiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Síndrome
20.
Front Neurol ; 10: 565, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31191446

RESUMO

Background: The muscle patterns involved in the "split-leg" syndrome of amyotrophic lateral sclerosis (ALS) remains controversial. We sought to evaluate and reassess the pattern of the extensor digitorum brevis (EDB) and the abductor hallucis (AH) muscles' involvement in split-leg syndrome in ALS. Methods: We recruited 60 consecutive patients with ALS and 25 healthy controls (HCs). Compound muscle action potentials (CMAPs) and F-waves were recorded over the EDB and AH muscles in all subjects. For comparison, we classified patients into two categories based on the presence or absence of lower limbs symptoms. Results: The EDB/AH CMAP amplitude ratio was significantly reduced in patients with affected legs (0.33 ± 0.21, P = 0.007), whereas patients with unaffected legs had a ratio similar to that of the HCs. The EDB/AH ratios for the F-wave latencies, mean F-wave amplitude, mean F/M amplitude ratio, and the persistence of the total repeater F-wave shapes (index Freps) of the EDB-AH, were significantly increased in the affected leg group, whereas the EDB/AH ratio for F-wave persistence was significantly reduced. These findings indicated a greater loss of lower motor neurons (LMNs) innervating the EDB and dysfunction of spinal motoneurons innervating the EDB. In the unaffected leg group, the EDB, but not the AH, F-wave latencies, mean and maximal F/M amplitude ratios, and index Freps were significantly altered. Receiver operating characteristic curve analysis suggested that the EDB F-wave latencies, mean F/M amplitude ratios, and index Freqs (area under the curve [AUC] > 0.8) more strongly differentiated patients with ALS from the HCs compared to the EDB/AH CMAP amplitude ratio (AUC = 0.61). Notably, the EDB maximal F-wave latency and index Freqs reliably differentiated patients with unaffected legs (HCs), with AUCs of 0.83 (95% CI 0.76-0.91) and 0.81 (95% CI 0.72-0.89), sensitivities of 76 and 78%, and specificities of 76 and 78%, respectively. Conclusions: These results suggest preferential EDB compared to AH involvement in the split-leg syndrome of ALS. The EDB maximal F-wave latency and index Freqs robustly differentiated patients with ALS from HCs, which might facilitate an earlier identification of ALS.

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