Objective To compare the concentration of 3-nitrotyrosine and tyrosine and the ratio of 3-nitrotyrosine/tyrosine in the cerebrospinal fluid (CSF) of patients with sporadic amyotrophic lateral sclerosis (SALS) and those with healthy nervous system for definiting the role of free radicals oxidative damage in pathogenesis of SALS. Methods Subjects consisted of 15 patients with SALS and 15 patients with norml nervous system who underwent lumbar anesthesia for surgery. Their CSF was analyzed by using high performance liquid chromatography (HPLC). Results The 3-nitrotyrosine concentration was higher in patients with SALS (228.52?124.30 nmol/L) than that in controls (112.86?47.10 nmol/L) (P

Objective To study the motor evoked potential (MEP) evoked by transcranial electric stimulation in the masseter muscles of patients with amyotrophic lateral sclerosis (ALS), and to compare the results obtained from a control population, so as to establishe a method to evaluating the impairment of corticobulbar projections in ALS.Method Transcranial electrical stimulation was used in 20 patients with ALS and 30 matched normal control.Central motor conduction time (CMCT) was calculated.Results Responses to direct activation of the trigeminal motor root (R-MEP) were able to be recorded in all ALS.The latency and amplitude of R-MEP was (3.44?0.53) ms and (2.79?2.19) mV respectively.They had nonsignificant differences as compared to the controls.Responses to activation of corticobulbar descending fibers (C-MEP) were absent or delayed in 12 ALS patients, and CMCT was significantly prolonged ( P

BACKGROUND: Cervical intumescence is the earliest and easy attacking part of motor neuron disease (MND). With the development of patients'condition, cervical part and medulla oblongata region were involved early,and the trigemino-cervical reflex (TCR) was involved earliest.OBJECTIVE: To establish electromyography of TCR and analyze the value in the diagnosis of MND of the TCR.DESIGN: Case-control study.SETTING: Room of Electrophysiologic Study, Department of Neurology,Third Hospital, Peking University.PARTICIPANTS: Thirty MND patients,mainly amyotrophic lateral sclerosis (ALS) patients and 70 healthy volunteers who were examined at Third Hospital, Peking University from 2002 to 2005. MND patients accorded with the diagnostic standard of Spain El Escorial conference.METHODS: The subjects held the heads slightly raised when lying supine to make the sternocleidomastoid muscles contracted slightly. Electrical stimulation was applied to the infraorbital nerves and latency and amplitude of wave were recorded from the bilateral sternocleidomastoid muscles. The detecting instrument was Keypoint electromyography (EMG).The surface EMG activity was recorded via Ag/AgCl.MAIN OUTCOME MEASURES: Latency of positive wave/ negative wave (P20/N30); square root of the ratio of the amplitudes, that is, value A.RESULTS: Stimulation of the infraorbital nerve on one side of controlled people produced bilateral positive and negative waves. In the MND group,7 patients showed normal (23.3%), 8 patients showed absence of wave (26.7%), 11 patients were delayed in latency (36.7%), and extreme asymmetry of reflection of the two sides was found in 4 patients (13.3%).In the MND group, the latency of positive and negative waves of TCR (P20/N30) was markedly longer than that in the normal control group,and the difference was significant. Square root of the ratio between the amplitudes after and before stimulation (square root of the ratio between peak to peak value and amplitude of wave before stimulation, that was,value A) was lower remarkably than that in the normal control group, and the difference was significant.CONCLUSION: The TCR can be reliably measured. It may help examine the cervical-bulbar lesion and diagnosing motor neuron disease in an early period.

Objective:to investigate the clinical feature and dynamic changes of the cervical dural sac and spinal cord during neck flexion in Hirayama disease(juvenile muscular atrophy of distal upper extremity).Methods:Clinical data were taken and MRI in neutral neck position and a fully flexed neck position were performed on 27 cases of Hirayama disease.Results:(1)All patients were consistent with the diagnostic criteria of Hirayama disease who had asymmetric muscular atrophy and weakness of the hand and forearm.All patients were young males and right handed of whom 77.8% had initial symptoms before they were 19 years old.More patients(20 cases,74%)had muscular atrophy in the right hand than in the left at onset.The duration after disease onset was from 2-72 months[(26.48?15.57)months].(2)In neutral neck position by MIR examination,16 patients showed abnormal cervical curvature,14 showed atrophy of the lower cervical cord and 2 patients had intramedullary abnormal high signal.(3)In a fully flexed position of the neck,all patients showed forward displacement and flattening of the lower cervical cord,and a crescent-shaped high signal area behind the cord.(4)The crescent-shaped area was enhanced on T1-weighed imaging and disappeared after the patient returned to a neural position in one case.Conclusion:Hirayama disease occurs mainly in young males.There are obviously dynamic changes of the cervical cord during neck flexion in Hirayama disease by MRI examination,which can help the doctor make diagnosis in the early stage.

Objective To assess the value of electromyography (EMG) of rectus abdominis muscle in the diagnosis of polyneuropathy. Methods 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.Results 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)μV was higher than that of the controls (373.78±56.46)μV (t=2. 01, P < 0. 04) and lower than that of patients with ALS (537.19±159. 04)μV (t=2. 32, P<0.03). Conclusion EMG of rectus abdominis muscle might be used to find the lesion of intercostal nerve in polyneuropathy.

Objective To study the features of pain evoked potentials in patients with amyotrophic lateral sclemsis(ALS)and evaluate the pain pathway in these patients.Methods Sixty patients with ALS and 60 controls were set on supine position.The contact heat evoked potential stimulator with a diameter of 27 mm and an area of 573 mm2was used to elicit pain and contact heat evoked potentials(CHEP)in an accelerating speed of 70 ℃/s.Thermal stimuli were given at 54.5 ℃ to three body sites:the dorsum of hand,proximal volar for aml and C7. CHEP was recorded at spots of Cz and Pz.The features of CHEP was observed At the same time,somatosensory evoked potential(SEP)was assesed.Results The figure and latency of CHEP in ALS patients were normal.The latency was:the dorsum of hand:(561.2±28.6)ms; proximal volar forarm:(540.1±39.2)ms;C7:(512.7±31.4)ms.There were no significant differences of latency and SEP between tlle ALS patients and the controls((558.7±30.2),(536.6±23.5), (501.8±26.0)ms,t=4.23,4.51,3.74,P＞0.05).Conclusion Patients with ALS have a normal CHEP,suggesting that the pain pathway in patient with ALS is intact.

Objective To analyze the clinical features of 27 Chinese patients with Kennedy's disease(KD), a sex-linked inheritance disorder. Methods We collected the clinical data of 27 KD patients whose diagnosis were confirmed by gene examination to analyze their clinical features, as well as their serum levels of sex hormones and biochemical indicators. Results Patients with spinal and bulhar muscular atrophy tend to have an adult onset, exhibiting a slow progression of lower motor neuronal weakness and atrophy involving limbs and bulbar zones. Part of the invalids presented signs of androgen insensitivity such as gynecomastia or reduced sexual function, even though their degrees of sex hormones were normal. There was mild motor functional lesion which correlated with the course of the disease ( r = 0. 77, P = 0. 000). The degrees of creatine kinase ( ( 920. 10 ± 495.54 ) U/L ) and triglyceride ( ( 3.27 ± 2. 78) mmool/L) increased mildly and was significantly different from the levels of healthy ones (107.20 U/L,t =7.517,P =0.000;1.40 mmol/L, t =2.687,P =0.017). Conclusions Basically, Chinese KD patients present the same clinical features as the literature reports, however they have distinctive aspects such as elevated degree of triglyceride. The present research can help us to understand the features of Chinese KD more particularly.

Objective To compare two common techniques for motor unit number estimation (MUNE), multiple point stimulation(MPS) and incremental stimulation, and determine which is preferable in the follow-up study of patients with amyotrophic lateral sclerosis (ALS).Methods MPS or incremental stimulation MUNE was recorded respectively in 120 ALS patients at baseline and month 3, 6, 9 ,and 12 after study entry.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 the wrist, 6 cm above the wrist, elbow and 6 cm above the elbow.Individual motor unit responses were obtained by moving thestimulating electrode and isolating threshold responses.Then, with finely graded stimulus intensity at one point, 3 steps in a CMAP were investigated.For incremental stimulation, stimulus intensity was slowlyincreased 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.Single motor unit potential were obtained by subtracting amplitudes of each response from that of the prior response.The values obtained from two methods were compared.Results The value of MUNE declined in the follow-up period.MUNE obtained from MPS was the same as that gained from incremental stimulation at the baseline and the 3rd, 9th, 12th month after study entry, while MUNE obtained in MPS was more than that obtained from incremental stimulation at the 6th month after study entry ( 88 ± 6 and 47 ± 5;t = 1.72, P = 0.04).Conclusions Both MPS and incremental stimulation are certain in the follow-up study of patients with amyotrophic lateral sclerosis.The value of MUNE obtained from two methods might be different in some period.

Objective To explore the significance of proton magnetic resonance spectroscopy(1H- MRS)in patients with amyotrophic lateral sclerosis(ALS).Methods Single-voxel1 H-MRS was carried on the preeentral gyri in 110 patients with ALS and 24 patients with lower motor neuron syndrome (LMNS) compared with 89 healthy controls.The upper motor neuron involvement of the patients was assessed by upper motor neuron(UMN)signs and the reflex scale,and the disease severity was evaluated by the AIJS function rating scale(ALS-FRS)and APPEL ALS rate scale(AARS).Results Compared with the healthy controls(1.62±0.18),the NAA/Cr of patients with ALS(1.40±0.25)remarkably decreased (t= -5.007,P=0.000),however,it did not change in patients with LMNS(1.60±0.17)as compared with that of the controls.The NAA/Cr of patients with ALS was also lower than that of patients with LMNS(t= -2.660,P=0.009).Furthermore,the NAA/Cr of patients with definite ALS was lower than those of patients with probable and possible ALS(definite vs probable:t=-2.626,P=0.010;definite vs possible: t=-2.537,P=0.013).On the other hand,patients with pronounced UMN signs had a lower NAA/Cr ratio than those with probable UMN signs(t=-2.827,P=0.006).In patients with asymmetric clinical presentation,the NAA/Cr ratio was significantly lower in the precentral gyrus contralateral to the clinically predominantly affected side(t=-2.652,P=0.011).The NAA/Cr was correlated with the reflex scale scores,ALS-FRS score,and AARS and its sub-items(P＜0.05). Conclusions 1H-MRS is useful in studying ALS.The marker NAA/Cr of precentral gyrus may reflect the UMN degeneration in ALS,which is related to the disease severity and progression.However,it might not be helpful in the early diagnosis and differential diagnosis of the disease.