Synergistic Effect of Hydrogen and 5-Aza on Myogenic Differentiation through the p38 MAPK Signaling Pathway in Adipose-Derived Mesenchymal Stem Cells

Background and Objectives@#This study aims to clarify the systems underlying regulation and regulatory roles of hydrogen combined with 5-Aza in the myogenic differentiation of adipose mesenchymal stem cells (ADSCs). @*Methods@#and Results: In this study, ADSCs acted as an in vitro myogenic differentiating mode. First, the Alamar blue Staining and mitochondrial tracer technique were used to verify whether hydrogen combined with 5-Aza could promote cell proliferation. In addition, this study assessed myogenic differentiating markers (e.g., Myogenin, Mhc and Myod protein expressions) based on the Western blotting assay, analysis on cellular morphological characteristics (e.g., Myotube number, length, diameter and maturation index), RT-PCR (Myod, Myogenin and Mhc mRNA expression) and Immunofluorescence analysis (Desmin, Myosin and β-actin protein expression). Finally, to verify the mechanism of myogenic differentiation of hydrogen-bound 5-Aza, we performed bioinformatics analysis and Western blot to detect the expression of p-P38 protein. Hydrogen combined with 5-Aza significantly enhanced the proliferation and myogenic differentiation of ADSCs in vitro by increasing the number of single-cell mitochondria and upregulating the expression of myogenic biomarkers such as Myod, Mhc and myotube formation. The expressions of p-P38 was up-regulated by hydrogen combined with 5-Aza. The differentiating ability was suppressed when the cells were cultivated in combination with SB203580 (p38 MAPK signal pathway inhibitor). @*Conclusions@#Hydrogen alleviates the cytotoxicity of 5-Aza and synergistically promotes the myogenic differentiation capacity of adipose stem cells via the p38 MAPK pathway. Thus, the mentioned results present insights into myogenic differentiation and are likely to generate one potential alternative strategy for skeletal muscle related diseases.

Effect of recombinant human coagulation factor Ⅶa combined with Bakri balloon compression on oxidative stress and coagulation in patients with refractory postpartum hemorrhage / 中国输血杂志

【Objective】 To investigate the effects of recombinant human coagulation factor Ⅶa combined with Bakri balloon compression on oxidative stress and coagulation in patients with refractory postpartum hemorrhage. 【Methods】 Prospectively, 80 patients with refractory postpartum hemorrhage in Chengdu Fifth People′s Hospital from June 2019 to June 2022 were selected and grouped according to the random number table method. The control group (n=40) was treated with Bakri balloon compression, and the observation group (n=40) was treated with recombinant human coagulation factor Ⅶa combined with Bakri balloon compression. The bleeding-related indexes and adverse effects were observed in both groups, and the prenatal and 24 h postpartum oxidative stress, coagulation function and inflammatory factors were compared between the two groups. 【Results】 The blood loss in the observation group and the control group was (683.96±146.52) vs(796.63±152.41)mL during operation, (812.46±161.53) vs(965.39±166.22)mL in 2 h after delivery, (899.53±178.74) vs(1 084.31±203.67)mL in 24 h after delivery, and the transfusion volume was (512.31±104.76) vs(683.25±113.52)mL, and the onset time of hemostasis was (14.63±3.18) vs (21.72±5.29) min (P0.05). At 24 h postpartum, NE, Cor, SOD and MDA were higher than those before delivery in both groups, but the observation group was lower than the control group (P<0.05); TT, APTT and PT were longer and Fib was lower in both groups than before delivery, but TT, APTT and PT were shorter and Fib was higher in the observation group than in the control group (P<0.05); CRP, IL-8 and TNF-α were higher in both groups than before delivery, but the observation group was lower than in the control group (P<0.05). 【Conclusion】 Hemostasis in patients with refractory postpartum hemorrhage treated with recombinant human coagulation factor Ⅶa combined with Bakri balloon compression was effective, which can improve coagulation, reduce transfusion, decrease oxidative stress injury and inflammatory response without increasing adverse effects.

The key points in diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy / 中华神经科杂志

The diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy relies on clinical features, demyelinating changes on motor and sensory nerve conduction studies, elevated cerebrospinal fluid protein, peripheral nerve image, nerve pathology, and response to immune therapy. Each diagnostic proof should be interpreted in light of clinical background and other findings, to avoid misdiagnosis or overdiagnosis, treatment delay or unnecessary treatment with immunotherapy.

The changes in European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy / 中华神经科杂志

European Academy of Neurology/Peripheral Nerve Society revised the guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Typical CIDP and CIDP variants were distinguished. The levels of diagnostic certainty of CIDP and possible CIDP can be defined according to the changes in motor and sensory nerve conduction studies. The utility of cerebrospinal fluid, peripheral nerve image, and nerve biopsy in diagnosis of CIDP was suggested. Serum auto-antibodies, including anti-nodal and paranodal antibodies, anti-myelin-associated glycoprotein antibodies were discussed. Monoclonal protein should be tested routinely in CIDP. Principles for treatment of CIDP were recommended. This guideline updated the development in CIDP and is more suitable for clinical practice.

The Progression and Problems in Diagnosis and Treatment of Rare Neurological Diseases / 罕见病研究

Rare neurological diseases contain of a great variety of different disease and difficult to diagnose and cure. In the past years, many drugs are approved for treatment of the rare diseases, bringing hope to patients. However, these drugs are usually very expensive, so clinicians and patients have to face the delimma of the accessibility and appropriate usage of these drugs. On the other hand, there are many rare diseases could be treated by cheaper drugs. However, diagnosis of those rare diseases is challenging and clinician's lacking knowledge of the rare diseases leads to delay in diagnosis. Improving the competence in early diagnosis of rare neurological diseases is essential for the patients to live a quality life.

Recent research focus in peripheral neuropathy / 中华神经科杂志

Peripheral neuropathy consists of a group of diseases due to different pathogenesis. This article discussed the recent research focus in four categories of peripheral neuropathies, including immune mediated neuropathy, diabetic neuropathy, hereditary neuropathy and chemotherapy-induced neuropathy.

Dog-Bone double button in shoulder arthroscopic treatment of acute acromioclavicular dislocation / 中华创伤骨科杂志

Objective:To investigate the clinical efficacy of Dog-Bone double button in the treatment of acute acromioclavicular dislocation under shoulder arthroscopy.Methods:A retrospective analysis was conducted of the 20 patients with acute acromioclavicular dislocation who had been treated at Department of Sports Medicine, Northern Jiangsu People's Hospital of Jiangsu Province from November 2018 to December 2020 by Dog-Bone double button under shoulder arthroscopy. They were 11 males and 9 females, aged from 31 to 63 years. Recorded were their visual analogue scale (VAS), Constant-Murley shoulder function score and range of shoulder anteflexion at preoperation and the last follow-up, as well as complications and the X-ray parameters at one month postoperation and the last follow-up [including coracoclavicular distance (CCD), distance between the upper and lower Dog-Bone titanium plates (DDD), angle between the coracoid process tunnel and the tangent line of the superior clavicle (CTCA), and widths of the clavicle tunnel and the coracoid process tunnel].Results:The 20 patients were followed up for 6 to 12 months (average, 10.5 months). Their preoperative VAS score, Constant score, and range of shoulder anteflexion were 3.0 (3.0, 4.0), 57.0 (54.3, 61.5) and 130° (110°, 140°), which were significantly improved to 0 (0, 0.8), 90.0 (86.5, 91.0) and 170° (170°, 180°) at the last follow-up ( P<0.05). Their CCD, DDD, CTCA, and widths of the clavicle tunnel and the coracoid process at one month postoperation were (5.0±1.0) mm, (32.4±3.5) mm, 91.7° (88.5°, 104.9°), 3.0 (3.0, 3.0) mm and 3.0 (3.0, 3.0) mm, which were significantly improved to (6.3±1.3) mm, (32.8±3.7) mm, 84.8° (81.8°, 92.0°), 3.5 (3.4, 3.6) mm and 3.2 (3.1, 3.3) mm with the exception of DDD at one month postoperation ( P<0.05). The last follow-up observed postoperative reduction loss in only one patient. Conclusion:For acute acromioclavicular joint dislocation, the Dog-Bone fixation technique under shoulder arthroscopy can lead to fine surgical efficacy and patient satisfaction, because it has advantages of limited trauma, good functional recovery of the shoulder, and effective prevention of reduction loss.

Advances in study of immune-related small fiber neuropathy / 中华神经科杂志

Small fiber neuropathy is a peripheral neuropathy that selectively involves unmyelinated C-type fibers and (or) thin myelinated Aδ-type fibers. It is mainly manifested as abnormal pain and temperature perception and autonomic symptoms. Changes in certain inflammatory markers and antibodies related to autoimmune diseases can occur in patients with immune-related small fiber neuropathy. This article aims to explore the immunological progress in small fiber neuropathy.

The utility of nerve ultrasound in diagnosis of peripheral neuropathy / 中华神经科杂志

Clinical and electrodiagnostic studies are the mainstay in the diagnosis of polyneuropathy. The nerve ultrasound can detect morphological features of peripheral neuropathy, which is a powerful supplement for neuroelectrophysiological test. The features of nerve ultrasound in different demyelinating and axonal neuropathies and its utility for differentiating diagnosis are detailed in this article.

Focus on the International Conference on Peripheral Neurology 2019 / 中华神经科杂志

The International Conference of Peripheral Neurology 2019 was held in Genoa, Italy from June 22 to 25, and the conference included four topics: immune-related peripheral neuropathy, hereditary peripheral neuropathy, diabetic peripheral neuropathy and toxic peripheral neuropathy. The conference consists of special lectures, speeches and wall reports of the general assembly, plenary sessions in the morning and sub-sessions on several topics in the afternoon. We introduce four topical issues in this conference.

Motor nerve conduction and clinical characteristics of POEMS syndrome / 中华神经科杂志

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.

Interpretation of Chinese guidelines for diagnosis and treatment of Guillain-Barré syndrome 2019 / 中华神经科杂志

The neuroelectrophysiological test and immunotherapy for Guillain-Barré syndrome were further discussed according to Chinese guidelines for diagnosis and treatment of Guillain-Barré syndrome 2019. Neuroelectrophysiological test should be performed and interpreted according to clinical background in the diagnosis of Guillain-Barré syndrome. Intravenous immunoglobulin therapy should be adopted individually according to the severity and course of the disease and other factors.

Summary of the Twenty-ninth International Symposium on Amyotrophic Lateral Sclerosis-Motor Neuron Disease / 中华神经科杂志

The 29th International Symposium on Amyotrophic Lateral Sclerosis (ALS)-Motor Neuron Disease was held in Glasgow from December 7 to 9, 2018. The symposium was divided into 23 topics, with 109 special reports and paper′s exchange and 515 posters exchange. This article briefly introduces some topics of the symposium, involving basic researches, clinical researches and clinical trials. Among these, basic researches include genetics and genomics, axonal degeneration, disease models, and preclinical therapeutic strategies; Clinical researches include epidemiology, clinical progression, cognitive and psychological change, neuropathology, neurophysiology, neuroimaging and biomarkers.

Summary of the Twenty?ninth International Symposium on Amyotrophic Lateral Sclerosis?Motor Neuron Disease / 中华神经科杂志

The 29th International Symposium on Amyotrophic Lateral Sclerosis (ALS)?Motor Neuron Disease was held in Glasgow from December 7 to 9, 2018. The symposium was divided into 23 topics, with 109 special reports and paper′s exchange and 515 posters exchange. This article briefly introduces some topics of the symposium, involving basic researches, clinical researches and clinical trials. Among these, basic researches include genetics and genomics, axonal degeneration, disease models, and preclinical therapeutic strategies; Clinical researches include epidemiology, clinical progression, cognitive and psychological change, neuropathology, neurophysiology, neuroimaging and biomarkers.

Clinical Diagnosis and Treatment Recommendations for Adverse Reaction in the Nervous System Related to Immunocheckpoint Inhibitor / 中国肺癌杂志

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.

Summary of the Twenty-eighth International Conference on Amyotrophic Lateral Sclerosis-Motor Neuron Disease / 中华神经科杂志

The 28th International Conference on Amyotrophic Lateral Sclerosis (ALS)-Motor Neuron Disease was held in Boston from December 8 to 10, 2017. The conference covered 23 topics, 102 special topics and 446 papers. This article briefly introduces some topics of the conference, involving basic research, clinical research and clinical trials. Among these, basic studies include genetics, cell biology and pathology, and superoxide dismutase1 gene ALS related pathology; clinical studies include the progression of ALS disease, cognitive behavioral disorders, and biological markers.

Diffusion tensor imaging studies of amyotrophic lateral sclerosis patients with various levels of cognitive impairment / 中华神经科杂志

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.

Characteristics of giant F-waves in amyotrophic lateral sclerosis / 中华神经科杂志

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 .

Characteristics of giant F-waves in amyotrophic lateral sclerosis / 中华神经科杂志

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 .

The characteristics of F-waves in patients with Kennedy's disease / 中华神经科杂志

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.