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ABSTRACT: 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).
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).
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).
TST may be used to detect proximal CB and facilitate the diagnosis of MMN.
Zhonghua yi xue za zhi 02/2012; 92(7):456-9.
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ABSTRACT: 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.
Amyotrophic Lateral Sclerosis 05/2011; 12(5):345-8. · 3.40 Impact Factor
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ABSTRACT: To explore the role of fasciculation potentials in the diagnosis of amyotrophic lateral sclerosis (ALS).
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.
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).
With different morphologies at different disease stages, fasciculation potentials may be useful in the earlier diagnosis of amyotrophic lateral sclerosis.
Zhonghua yi xue za zhi 02/2011; 91(7):482-4.
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ABSTRACT: To study the characteristic of chronic motor axonal neuropathy and the prognosis of it.
16 chronic motor axonal neuropathy (CMAN) patients were diagnosed by clinical manifestation and neurophysiologic manifestation. Follow-up study was done including living state, medicine they taken, change of physical sign and motor unit number estimation. 60 patients with amyotrophic lateral sclerosis were studies at the same time. The prognosis of these patients was compared.
After being treated with intravenous immunoglobulin therapy, the patients with CMAN showed a good clinical recovery except 6 patients who had the characteristic of ALS in the 18 months follow-up study, and the MUNE changed from 68 +/- 17 to 154 +/- 25. On the contrary, the patients with ALS had worse clinical manifestation, and the MUNE changed from 64 +/- 6 to 55 +/- 9.
Immunological factor may be involved in the mechanism of chronic motor axonal neuropathy which has a better prognosis and is difficult to differentiate from amyotrophic lateral sclerosis in the earlier stage.
Zhonghua yi xue za zhi 12/2009; 89(45):3193-5.
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ABSTRACT: To compare two common techniques for motor unit number estimation (MUNE), multiple point stimulation and incremental stimulation to determine which is preferable in patients with amyotrophic lateral sclerosis (ALS).
Surface recorded motor unit action potentials of median nerve or thenar muscle were measured in 60 ALS patients and 60 controls. The maximal baseline to negative peak compound muscle action potential (CMAP) amplitude was recorded. For multiple point stimulation, the stimuli sites included the skin of wrist, 6 cm above the wrist, elbow and 6 cm above the elbow. Individual motor unit responses were obtained by moving the stimulating electrode and isolating threshold responses with distinct morphologies. Then, with finely graded stimulus intensity at one point, 3 steps in a CMAP were investigated. 10 - 12 different single motor unit action potentials (SMUPs) were recorded. For incremental stimulation, stimulus intensity was slowly increased from subthreshold levels until a small all-or-none response was evoked. The intensity was slowly increased until the response increased in a quantal fashion. This process was repeated for a total of 10 increments. Individual motor unit amplitudes were obtained by subtracting amplitudes of each response from that of prior response. Both techniques were performed twice, electrodes changed and results averaged.
For controls, MUNE was 228 +/- 30 for multiple point stimulation and 198 +/- 26 for incremental stimulation. Test-retest correlation coefficients and coefficients of variation for mean of two MUNE were 0.88 - 0.91 and 13.20% - 15.24% for multiple point stimulation, 0.86 and 13.30% - 15.65% for incremental stimulation. For ALS patients, MUNE was 64 +/- 6 and 59 +/- 7 respectively.
Both MUNE methods are similarly reproducible and are equally effective at documenting progression of a lower motor neuron disorder in ALS patients.
Zhonghua yi xue za zhi 11/2009; 89(43):3051-4.
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ABSTRACT: To assess the value of electromyography ( EMG) of rectus abdominis muscle in the diagnosis of polyneuropathy.
109 patients with polyneuropathy were studied. The routine nerve conduction study was done and standard EMG was recorded from rectus abdominis muscle, first dorsal interosseous muscle and tibialis anterior muscle. The parameters studied included spontaneous activity fibrillation potentials (fib) and positive sharp waves (psw); duration, amplitude and percentage of polyphasic wave of motor unit action potential (MUAP) and pattern of recruitment. A group of controls and patients with amyotrophic lateral sclerosis (ALS) were studied at the same time. EMG parameters of rectus abdominis muscles were compared among patients with polyneuropathy, patients with ALS and the controls.
EMG of rectus abdominis muscle in the patients with polyneuropathy showed neurogenic change. The amplitude of motor unit potential in patients with polyneuropathy (451.67 +/- 75.01) microV was higher than that of the controls (373.78 +/- 56.46)microV(t = 2.01, P < 0.04) and lower than that of patients with ALS (537.19 +/- 159.04) microV (t = 2.32, P < 0.03).
EMG of rectus abdominis muscle might be used to find the lesion of intercostal nerve in polyneuropathy.
Zhonghua nei ke za zhi [Chinese journal of internal medicine] 10/2009; 48(10):850-2.
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ABSTRACT: To identify the correlation between the revised amyotrophic lateral sclerosis (ALS) functional rating scale (ALSFRS-R) and the progression rate of ALSFRS-R (DeltaFS) at time of diagnosis and survival time in a prospective survey.
A total of 129 consecutive patents with definite or probable ALS were enrolled at our hospital from January 2002 to December 2005. The patients were monitored with a standard evaluation form recording clinical features, ALSFRS-R and forced vital capacity (FVC) every 3 months from visit to death or tracheostomy.
The mean age at onset was (52 +/- 11) years old. The median survival time from symptom onset was 45.71 months (95% CI = 35 to 51). In univariate analysis of Kaplan-Meier method, the outcome was significantly related to total ALSFRS-R and DeltaFS (P < 0.05). In Cox multivariate model, DeltaFS and FVC% were both statistically significant predictors of survival after adjusting for age and onset site (P < 0.05). Al-unit decrease in DeltaFS was associated with a 73.3% decreased risk of death.
ALSFRS-R is sensitive to the change over time and can predict survival at diagnosis. It can be used as a primary outcome measure in ALS clinical trial. DeltaFS at diagnosis can provide us the information of disease progression at one particular point. It is a more significant predictor of survival in patients with ALS as compared to total ALSFRS-R and time from onset to diagnosis.
Zhonghua yi xue za zhi 09/2009; 89(35):2472-5.
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ABSTRACT: Amyotrophic lateral sclerosis (ALS) is typically considered to be a disease of motor, not sensory, neurons. However, reports exist of sensory system involvement in ALS. In this study we aimed to study the characteristic of contact heat-evoked potentials (CHEPs) in patients with ALS and to evaluate the nociceptive pathway in these patients. Sixty patients with ALS and 60 controls had pain elicited by a CHEP stimulator with an accelerated velocity of 70 degrees C/s. Thermal stimuli were sent at 54.5 degrees C to three body sites: the dorsum of the hand, the proximal volar forearm, and the skin near C7. CHEPs were recorded from Cz and Pz. The onset negative peak latencies were 561.2 +/- 28.6 ms, 540.1 +/- 39.2 ms, and 502.4 +/- 26.2 ms when the dorsum of the hand, the proximal volar forearm, and skin near C7 were stimulated, respectively. There were no significant differences between the ALS patients and the controls with CHEP (P > 0.05). Our results suggest that the nociceptive pathway is intact and support the idea that small fibers and their sensory pathway are spared in ALS.
Muscle & Nerve 03/2009; 39(6):735-8. · 2.37 Impact Factor
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ABSTRACT: To study the feasibility of the bi-level positive airway pressure (BiPAP) non-invasive ventilator used in home mechanical ventilation for long-term tracheostomy-mechanical ventilation (TMV) in patients with amyotrophic lateral sclerosis (ALS).
Sixteen patients (12 men and 4 women, mean age 59 years) with ALS were selected for this study at Respiratory Department of the Shougang Hospital, Peking University from January 2002 to March 2008. After the disease had been controlled by anti-infective therapy and comprehensive treatment, the patients received TMV, through the improved ("Xiang's" connection) non-invasive BiPAP ventilator connected with tracheotomy tube, and on-going home mechanical ventilation (HMV). The blood gas was evaluated during invasive ventilation and non-invasive ventilation before discharge. Family members of the patients were trained for the use of non-invasive ventilators. The use of ventilators and the patients' condition were regularly followed and the survival rate calculated. Statistical analysis was carried out by using one-way ANOVA.
There was no statistical difference in the blood gas before the use of non-invasive ventilator, 2 h and 1 d after the use of non-invasive ventilator, and before discharge, PaCO2 [(36+/-10), (42+/-11), (41+/-10), (42+/-11) mm Hg (1 mm Hg=0.133 kPa)], PaO2 [(84+/-11), (81+/-12), (87+/-14), (86+/-12) mm Hg], SaO2 [(96.7+/-1.3)%, (96.5+/-0.8)%, (96.8+/-1.2)%, (96.5+/-1.0)%] respectively, (F=1.21, 0.59, 0.97, 0.41, respectively, all P>0.05). All patients had no complaint of uncomfortable use, no intolerance to ventilators, and no ventilator breakdown. Fifteen patients were alive at the end of the follow-up (July 31, 2008). The mean time of using non-invasive ventilator was 39 months (range 4 to 66 months).
For ALS patients who need long-term ventilation support, the use of BiPAP non-invasive ventilators is a safe and effective alternative for invasive ventilators.
Zhonghua jie he he hu xi za zhi = Zhonghua jiehe he huxi zazhi = Chinese journal of tuberculosis and respiratory diseases 03/2009; 32(2):107-10.
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ABSTRACT: By determining the usefulness of motor unit number estimate (MUNE) and compound muscle action potential (CMAP) amplitude in patients with amyotrophic lateral sclerosis (ALS), we tried to find an effective way to stratify the disease stages. In all, 112 consecutive ALS patients were enrolled, among whom 73 were elicited in a longitudinal study. MUNE by the standard incremental technique, the average CMAP amplitude, total Medical Research Council (MRC) score, ALS-functional rating score (ALS-FRS), Appel ALS rating scale (AARS), and forced vital capacity (FVC) were performed at baseline and months 3, 6, and 12 after study entry. We found MUNE correlated with CMAP amplitude (P < 0.01) as well as MRC score (P < 0.01) in regionally concordant distal muscles. Both MUNE and CMAP amplitude correlated significantly with ALS-FRS (P < 0.05) and AARS (P < 0.01). A MUNE decrease was observed at months 3, 6, and 12 compared with baseline, and the rate of change at month 3 was 50.47%. The decrease in MUNE over the first 3 months was significantly greater than other measurements. We arbitrarily divided the patients into three stages: (1) rapid progression: the rate of change of MUNE and CMAP amplitude during the first 3 months exceeded 50%; (2) moderate progression: the rate of change of MUNE was greater than 50% but CMAP amplitude was less than 50%; (3) slow progression: the rate of change of both MUNE and CMAP amplitude were less than 50%. Comparing the rate of ALS-FRS descent per year using one-way ANOVA showed a significant difference among the three groups (P < 0.01).
Muscle & Nerve 02/2009; 39(3):304-9. · 2.37 Impact Factor
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ABSTRACT: To identify the correlation between the progression rate of motor unit number estimate (MUNE) at diagnosis and survival.
We included 129 patents with amyotrophic lateral sclerosis (ALS) enrolled in our hospital from January 2002 to December 2005. We recorded clinical features, ALS functional rating scale (ALSFRS), forced vital capacity (FVC) and electrophysiological data at diagnosis. The patients were monitored every 3 months from visit to death or tracheotomy.
Mean age at onset was (52.19+/-11.00) years. The median survival time from symptom onset was 45.71 months (95% CI=35 to 51). In univariate analysis of Kaplan-Meier method, outcome was significantly related to progression rate of MUNE (P<0.05). In the Cox multivariate model, progression rate of MUNE was a significant prognostic factor of survival (P<0.01). Using 2.9/month as cutoff point, the value beyond this point at diagnosis tended to have a shorter survival (P<0.05). A 1-unit decrease in the progression rate of MUNE was associated with a 34.2% decrease risk of death.
Progression rate of MUNE at diagnosis can provide the information of disease progression at one particular point, which may help to stratify patients and affect survival. It is the strongest prognostic factor of survival in patients with ALS compared with age, onset site and time from onset to diagnosis.
Beijing da xue xue bao. Yi xue ban = Journal of Peking University. Health sciences 12/2008; 40(6):629-32.
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ABSTRACT: To study the electrophysiological and pathological features of Kennedy disease (KD) and to make sure the functional and anatomical change of the sensory nerve of KD patients.
Electrophysiological and pathological tests were performed in 14 KD patients, including electromyogram, conduction velocities of median, ulnar, peroneal, tibial, and sural nerves, trigemino-cervical reflex (TCR), contact heat evoked potential (CHEP), and biopsy of the sural nerves. During TCR, the patients lay on the back, holding the heads slightly raised to make the sternocleidomastoid muscles contract slightly. Electrical stimuli were applied to the infraorbital nerves and recording electrodes were placed in the sternocleidomastoid muscles. For CHEP, contact heat was delivered via a circular thermode to excite selectively nociceptors with a rapid rising time at 70 degrees C/s to elicit pain and CHEP. Thermal stimuli were sent at 54.5 degrees C to three body sites: skin of back of hand, proximal volar forearm, and C7. The CHEPs were recorded from Cz and Pz.
The sensory conduction velocity and latency were almost normal, while the amplitude of the sensory nerve action potential (SNAP) declined to 0.65 - 2.85 microV. The latency of trigemino-cervical reflex was longer than normal with the onset peak latency of (38.9 +/- 7.0) ms. The configuration was asymmetric. The onset peak latencies when the skin of the back of hand, volar surface of forearm, and C7 were stimulated were (613 +/- 57), (595 +/- 32), and (489 +/- 37) ms respectively. Biopsy of the sural nerves showed that the large myelinated nerve fiber was decreased.
Sensory nerve is involved in the patients with Kennedy disease, including the large and small fibers.
Zhonghua yi xue za zhi 11/2008; 88(39):2771-4.
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ABSTRACT: To evaluate the long term efficacy and safety of long term home noninvasive positive pressure ventilation (HNPPV) in stable hypercapnic patients with chronic obstructive pulmonary disease (COPD).
Forty patients with stable severe COPD [arterial PaCO2>or=55 mm Hg (1 mm Hg=0.133 kPa)] after hospital discharge were divided into 2 groups: the HNPPV group (n=20) with conventional therapy and HNPPV, and the control group (n=20) with conventional therapy only. Parameters before and after 2 year follow-up observation were compared, which included dyspnea grade, scale for accessory muscle use, scoring for emotional disorders, lung function test, arterial blood gases, 6-min walking distance (6MWD), mean pulmonary arterial pressure (mPAP) by electrocardiography, mortality and hospitalization rates.
The age, gender, height, weight, dyspnea grade, scale for accessory muscle use, anxiety scores, depression scores, 6MWD, mPAP, arterial PaCO2 and PaO2, FVC, FEV1, and hospitalization rates of the 2 groups on admission were similar (t values were 1.08, 1.15, 1.20, 1.09, 0.86, 0.54, 0.00, 0.00, 0.43, 0.96, 0.76, 0.38, 0.26, 0.24 and 0.87 respectively, all P>0.05). The mortality was reduced slightly by HNPPV but with no statistically significant difference (3/20 and 8/20, respectively, chi2=3.27, P>0.05). After 2 years, the difference of the dyspnea grade, scale for accessory muscle use, anxiety scores, depression scores, arterial PaCO2 and PaO2, 6MWD, mean pulmonary artery pressure, and hospitalization rates in the HNPPV group [2.4+/-0.5, 2.6+/-0.6, 6.9+/-2.1, 6.1+/-1.6, (49.5+/-2.2) mm Hg, (60.8+/-4.7) mm Hg, (213+/-45) m, (30.3+/-2.2) mm Hg, (1.4+/-0.4) times/year] was statistically significant compared to the control group [3.9+/-0.3, 4.8+/-0.4, 11.2+/-2.6, 11.6+/-2.1, (61.5+/-2.3) mm Hg, (52.8+/-2.4) mm Hg, (127+/-23) m, (36.4+/-2.3) mm Hg, (3.9+/-0.3) times/year] (t values were 9.53, 10.83, 4.92, 7.83, 14.07, 5.41, 6.07, 4.81 and 10.22 respectively, all P<0.01).
Long-term use of home noninvasive positive ventilation in patients with stable severe COPD is effective and safe.
Zhonghua jie he he hu xi za zhi = Zhonghua jiehe he huxi zazhi = Chinese journal of tuberculosis and respiratory diseases 11/2007; 30(10):746-50.
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ABSTRACT: We examined the role of needle electromyography (EMG) of the rectus abdominis (RA) in assessing thoracic involvement in amyotrophic lateral sclerosis (ALS). Needle EMG of the RA was performed in 67 patients with sporadic ALS and 110 healthy controls. The presence of abnormal spontaneous activity, configuration of motor unit action potentials (MUAPs), and recruitment pattern of motor unit potentials were examined. In ALS patients, MUAPs in the RA were of prolonged duration, large amplitude, and showed increased prevalence of polyphasic waveforms compared to controls. Significant differences in MUAP parameters, presence of abnormal spontaneous potentials, and interference patterns were noted between ALS patients and controls. Additionally, we found that active denervation was more frequent in the RA of ALS patients with dyspnea than those without dyspnea. Thus, conventional needle EMG of the RA is a valuable electrophysiological method to assess clinical and subclinical involvement of thoracic lower motor neurons in patients with suspected ALS.
Muscle & Nerve 04/2007; 35(3):383-5. · 2.37 Impact Factor
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ABSTRACT: We examined the role of needle electromyography (EMG) of the rectus abdominis (RA) in assessing thoracic involvement in amyotrophic lateral sclerosis (ALS). Needle EMG of the RA was performed in 67 patients with sporadic ALS and 110 healthy controls. The presence of abnormal spontaneous activity, configuration of motor unit action potentials (MUAPs), and recruitment pattern of motor unit potentials were examined. In ALS patients, MUAPs in the RA were of prolonged duration, large amplitude, and showed increased prevalence of polyphasic waveforms compared to controls. Significant differences in MUAP parameters, presence of abnormal spontaneous potentials, and interference patterns were noted between ALS patients and controls. Additionally, we found that active denervation was more frequent in the RA of ALS patients with dyspnea than those without dyspnea. Thus, conventional needle EMG of the RA is a valuable electrophysiological method to assess clinical and subclinical involvement of thoracic lower motor neurons in patients with suspected ALS. Muscle Nerve, 2006
Muscle & Nerve 02/2007; 35(3):383 - 385. · 2.37 Impact Factor
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ABSTRACT: To establish a method to evoke cerebral potentials by stimulating nociceptive fibers, estimate the conduction velocities of nerve fibers mediating these responses, and assess the value of the potentials in carpal tunnel syndrome.
One hundred patients with carpal tunnel syndrome and 20 sex-, and aged-matched healthy persons underwent stimulation of contact heat delivered via a circular thermode to excite selectively nociceptors with a rapid rising time at 70 degrees C/s to elicit pain and contact heat evoked potential (CHEP). Thermal stimuli were sent at two intensity levels (49.5 degrees C and 54.5 degrees C) to three body sites: thenar eminence, dorsum of hand, and proximal volar forearm The CHEPs were recorded from Cz and Pz. The relation between the stimulus intensity and pain rating was observed, and the main components of the evoked potential were recorded. The nerve conduction velocity was calculated from the latency difference of the CHEP and center to center distance of distal and proximal stimulus arrays.
The pain intensity ratings were 3.2 +/- 0.3 and 4.4 +/- 0.5 when the thenar eminence was stimulated at the temperature of 49.5 degrees C and 54.5 degrees C respectively; and the ratings were 6.3 +/- 0.8 and 7.2 +/- 0.5 on the dorsum of hand and proximal volar forearm at the temperature of 54.5 degrees C respectively. Three components, Cz/N550, Cz/P750, and Pz/P1000, were found in the evoked potentials. The latency of the CHEP was significantly correlated with the arm length, and not significantly correlated with sex, age, and side difference of the arm. The conduction velocities of the Adelta and C fibers of the carpal tunnel syndrome patients were 12.0 m/s +/- 5.6 m/s and 0.6 m/s +/- 0.1 m/s respectively, both lowered, being 93.8% and 99.5% of those of the healthy persons (12.9 m/s +/- 7.5 m/s and 1.7 m/s +/- 0.4m/s respectively).
Elicited reliably, CHEP is mediated by Adelta and C fibers. The conduction velocity of C fibers in the patients with carpal tunnel syndrome is decreased.
Zhonghua yi xue za zhi 02/2007; 87(5):321-4.
