Objective To investigate the association between human leukocyte antigen DRB1gene (HLA-DRB1) polymorphisms and asthma among Mongolian and Han peoples in China. Methods Sequence-specific primer polymerase chain reaction (PCR-SSP) was used to detect frequencies of HLA DRB1 alleles in 45 Han and 45 Mongolian patients of asthma, as well as 46 healthy Han and 45 healthy Mongolian people as controls. Frequencies of each locus of HLA-DRB1 gene were calculated and odds ratio (OR) for each locus was estimated and tested by chi-square test. Results Frequency of HLA-DR9 allele was significantly higher in Han ( 13.33 %, OR of 7. 82) and Mongolian ( 16.67 %, OR of 4. 10) asthmatic patients, as compared to Han ( 2. 22 % ) and Mongolian ( 5.43 % ) controls, respectively ( P < 0. 05 ) ;frequency of HLA-DR11 allele in Mongolian asthmatic patients ( 10. 00 % ) was significantly higher than that in healthy Mongolians ( 1.11% ) ( P < 0. 05, OR of 11.00 ) ;and frequency of HLA-DR7 allele in Han asthmatic patients was significantly lower than that in healthy Han people ( P < 0. 05, OR of 0. 08 ). Frequency of HLA-DR7 allele in Han asthmatic patients (1.11% ) was significantly lower than that in Mongolian patients (10. 87 % ). Conclusions HLA-DR9 allele is a common in the people of Inner Mongolian Autonomous Region, and associated with susceptibility of asthma in Han and Mongolian peoples. HLA-DR11 allele is a unique susceptive gene for Mongolian asthmatic patients and HLA-DR7 allele is a protecting one for asthma in Han people.

Objective To investigate the usage of neostigmine test in diagnosis of myasthenia gravis (MG) initiated from extraocular muscles,including test methods and data analysis.Methods The clinical features of 100 consecutive patients with myasthenia gravis initiated from extraocular muscles were collected retrospectivelly,including initial symptom,number and department of visiting,evolution of symptoms and signs,methods and the judgment of therapeutic response for neostigmine test.Results (1) All patients with MG started from ptosis unilateral or bilateral or diplopia,among which 46％ (46/100)of patients with both ptosis and diplopia.Within 21％ (21/100) of patients,other muscles had been involved except for extrocular muscles before neostigmine test.Seventy-eight percent patients first consulted ophthalmology after (0.7 ± 0.3) month from onset.It took (1.6 ± 1.3) months for patients to visit neurologist for the first time.The average time for confirming diagnosis was (3.3 ± 2.1) months.(2) After (2.0 ± 1.5) times consulted at neurology department and (2.0 ± 1.8) months after symptoms onset,the patients undergone neostigmine test.Positive reaction rate showed lower in low dosage group (60.9％ (14/23) vs 98.3％ (58/59),x2 =18.30,P ＜0.01).Positive rate also showed lower in oral-take pyridostigmine bromide tablets group than neostigmine methylsulfate injection group(15/18 vs 98.3％ (58/59),x2 =4.428,P =0.035).(3) Sixtyfive percent patients had mild to moderate side effects after neostigmine injection,which disappeared in 60-120 minutes.Conclusions Non-standard operations of neostigmine test reduced the effectiveness of its diagnosis for MG initiated from extraocular muscles.Neurologist should attach importance totake standard neostigmine test to identify MG as soon as possible.

Objective To compare the predictive values of the classification of clinical symptoms(OCSP classification), brain images and artery abnormalities on prognosis of cerebral infarction. Methods Totally 116 acute ischemic stroke patients were divided into 4 subtypes based on the OCSP clinical classification. Lesion distributions were classified into cortical infarction, basal ganglion infarction, centrum ovale infarction and posterior infarction according to brain MRI image. Artery abnormalities were grouped into large artery disease and small artery disease according to the results of transcranial doppler, brain MRA, carotid duplex sonography or cerebral angiograph. The neurological function of patients 1 year after onset was evaluated by Barthel index and Rankin scale. Kaplan-Meier survival curves were graphed, and log-rank testing was performed to investigate the differences among stroke subtypes. Results OCSP clinical classification and brain image classification predicted the neurological function recovery 1 year after stroke onset, but it might be based on the size of the lesion whether it caused a dominant factor or not. Patients with small artery disease were found having a lower recurrence and mortality rate, as compared those with large artery diseases(0 vs 17.9%, Log-rank test, P=0.03). Conclusions There might be differences in predictive value among these 3 classification methods upon the prognosis of cerebral infarction.

Objective To identify the clinical and neurophysiological features of multiple system atrophy (MSA) and explore diagnostic values using various techniques in electro neurophysiology Methods Forty four subjects diagnosed as multiple system atrophy (MSA) during 1983 to 2001 in PUMC Hospital were studied retrospectively about their clinical features, routine electromyography (EMG), nerve conductive velocity (NCV), somatosensory evoked potential (SEP), motor evoked potential (MEP), brainstem auditory evoked potential (BAEP) and visual evoked potential (VEP) The clinical features and the electroneurophysiologic results were analyzed in different subgroups (probable MSA and possible MSA, OPCA and non OPCA, classified MSA and unclassified MSA) Results Most patients were found to be suffered from autonomic failure (88 6%) and cerebella dysfunction (88 6%) The pyramidal sign was seen in 72 7% of patients No remarkable statistical differences were found in all of the above three domains The incidence of Parkinsonism was 36 4% of all the patients, which showed significant differences between probable and possible MSA (54 5% vs 18 2%), non OPCA and OPCA (50% vs 7 1%), unclassified MSA and classified MSA (52% vs 15 8%) EMG was abnormal in 7 patients (38 1%), all of them were neurogenic impairments The rates of abnormal EMG were varied differently between probable and possible MSA (54 5% vs 22 2%), non OPCA and OPCA (53 8% vs 14 3%), unclassified MSA and classified MSA (58 3% vs 12 5%), but without statistical differences The abnormal rates in BAEP,SEP, VEP and MEP were 56 7%, 28%, 23 1% and 20 0%, respectively, and there were no significant differences between subgroups Conclusions It should be hard to divide MSA into groups only by the clinical manifestations All EPs were abnormal in some extent The most sensitive test is BAEP in current study, but there were no differences among the subgroups in the rates of abnormal EPs The difference of EMG and NCV between the groups showed that the technique might be helpful in the diagnosis of MSA

Amyotrophic lateral sclerosis is a progressive neurodegenerative disorder involving primarily motor neurons in the cerebral cortex,brainstem and spinal cord.The clinical diagnosis of ALS may be categorized into four levels of certainty according to clinical and electrophysiological examination,depending on the presence of upper and lower motor neuron signs together in the same topographical anatomic region in either the brainstem(bulbar cranial motor neurons),or cerival,or thoracic,or lumbosacral spinal cord(anterior horn motor neurons).Electromyography is of great importance in the diagnosis of ALS.Neruoimaging studies and clinical laboratory studies are helpful to exclude other disorders.

Amyotrophic lateral sclerosis(ALS)is a progressive neurodegenerative disorder.Mutations in the Cu,Zn-superoxide dismutase(SOD1)gene cause ALS by an unknown gain-of-function mechanism.Current researches show that diminishing SOD1 mutation enzymatic activity does not account for motor neuron lose in ALS,while direct toxicity of variant SOD1 protein or aggregation of SOD1 protein may impart cell function and cause motor neuron death.Though motor neuron death is selective,other cells also participate in this course.The author reviewed recent advances in the relationship between mutant SOD1 and Amyotrophic Lateral Sclerosis.

ALS is a progressive neurodegenerative disease affecting both lower and upper motor neurons,including the pyramidal tract.There have been no objective and effective method to evaluate the lesions of upper motor neurons.With non-invasive,sensitive and macroscopic advantages,the application of Regular MR,MRS,DWI,function MR and VBM on ALS will undoubtedly have great potentials in the diagnosis,disease monitor and therapy of ALS.

Objective To quantitatively estimate upper motor neuron (UMN) lesion with the triple stimulation technique (TST) in amyotrophic lateral sclerosis (ALS). Methods Fifty ALS patients and 22 normal controls were enrolled in the study. Patients were examined clinically with conventional transcranial magnetic stimulation (TMS) and TST at abductor digiti minimi. Central motor conduction time (CMCT),motor evoked potential ( MEP), resting motor threshold ( RMT), compound muscle action potential ( CMAP), modified ashworth scale ( MAS), Medical Research Council scale and modified ALS scale were assessed and their correlation to disease progress was analyzed. Results The TST amplitude ratio was significantly decreased in ALS patients with UMN signs(28 cases 62. 0% (40. 7%, 75.9% ) ), compared with controls ( 96. 9% ± 2. 6% ) and ALS patients without UMN signs ( 22 cases 95.6% ( 85.4%,100. 0% ) ;Z = -4. 827, -5.435, both P =0. 000). The abnormal rates of the TST amplitude in ALS with UMN signs, ALS without UMN signs and controls were 89. 3%, 27.3% ,9. 1% respectively. The abnormal rates of the TST amplitude, the latency of MEP, CMCT, RMT in detecting UMN lesions were 89. 3%,64. 3%, 53.6%, 64. 3% , 78. 6%, respectively. The TST amplitude was significantly correlated to tendon reflex in right arm ( r = 0. 690, P = 0. 000), with modified ashworth scale ( MAS, r = - 0. 772,P = 0. 000),with diagnostic degree ( r = 0. 483, P = 0. 000), with RMT ( r = - 0. 774,P = 0. 000), the latency of MEP (r = - 0. 444, P = 0. 005 ), motor evoked potential/compound muscle action potential of erb' s ( MEP/ CMAPerb, r = 0. 685, P = 0. 000 ), MEP/CMAPerb in facilitation ( r = 0. 770, P = 0. 000). Conclusions TST appears to be a more accurate and sensitive measure of detecting and quantifying UMN abnormality in ALS patients than the other parameters. TST may reveal the subclinical UMN impairment in ALS and provide an accurate diagnosis assessment for UMN loss in ALS and an objective scale for monitoring the progression of disease.

BACKGROUND: It is believed that repetitive transcranial magnetic stimulation (rTMS) may produce such neurophysiological effects as regulating regional cerebral blood flow, neurotransmitters, local metabolism, and neuronal remodeling after nerve tissue injuries. The prognosis ofischemic stroke is related with the cortical function reconstruction in the ipsilateral and contralateral hemisphere of the lesion. Currently studies have not defined whether rTMS can affect the cortical function, protect ischemic neurons and promote motor functional recovery after cerebral ischemia.OBJECTIVE: To investigate the effects of rTMS on rat motor cortical excitability and neural function in acute stage of cerebral ischemia-reperfusion injury.DESIGN: Completely randomized experiment.SETTING: Electroneurophysiological Laboratory of Peking Union Medical College Hospital.MATERIALS: The experiment was completed in the Zoological Research Center of Peking Union Hospital from January to June 2004. Totally 22adult male healthy Wistar rats were randomly divided into treatment group and the control group with 11 in each.METHODS: After determination of the average motor threshold of the right hind limbs, which was 22% of the maximum output, the rats were subjected to middle cerebral artery occlusion for 1 hour followed by reperfusion for 72 hours. At each time point of immediately and at 12, 36 and 60 hours after the initiation of reperfusion, the rats in the treatment group received rTMS treatment (20 Hz, 40% maximum output, 5 seconds for each session with an between-session interval of 2 minutes for a total of 10 sessions), and the site for motor threshold evaluation was used for rTMS stimulation; the rats in the control group recevied no treatment after model establishment. Motor threshold testing was performed in both groups 4 hours after the last session of treatment to avoid immediate-early effects of rTMS on the motor threshold. At 24 and 72 hours of reperfusion, the scores of neural function were recorded according to evaluation systems. All the rats with scores between 1 and 3 were enrolled in statistical analysis were evaluated between.MAIN OUTCOME MEASURES: ① Motor threshold of the rats in both groups before and after injury; ② Neural function scores at 24 and 72 hour reperfusion; ③ Infarct volume at 72 hour of reperfusion.RESULTS: Totally 13 rats entered the final result analysis. Before injury,motor threshold in the treatment and control group was similar (P=0.71),and after the injury, the motor threshold of the control group was 1.49times that of the treatment group but such difference was not statistically significant [(41.62±24.73)% vs (28.00±9.35)%, t=-1.17, P=0.27]. At 24hours of reperfusion, the functional scores of the treatment group and control group were not significantly different (P=0.46), but at 72 hours, the scores of the treatment group were significantly lower than that of the control group (1.60±1.52 vs 7.75±3.62, t=-3.57, P=0.004). The average infarct volume of two groups was (62.00±60.88) mm3 and (20.00±12.41) mm3 at 72 hours of reperfusion, respectively, which, after logarithm transformation,was not significantly different between the two group (t=-1.31, P=0.22),but when the infarct volume was transformed into Log10 values, a significant difference occurred between them (P=0.045).CONCLUSION: rTMS may stabilize and prevent the increment of the motor threshold, time-dependently relieve the neural function disability and reduce the infarct volume after cerebral ischemia-reperfusion injury.

Objective To analyze the associations between alleles of TNF?-308 and myasthenia gravis(MG) and its subgroups. Methods A biallelic polymorphism at position -308 in the promoter region of TNF? was screened by polymerase chain reaction (PCR) amplification and NcoⅠ restricted enzyme. One hundred MG patients and one hundred healthy controls of Chinese were analysed. Results The allele TNF?2(A) was found significantly increased in all patients (0.32 in MG vs 0.21 in healthy controls, P0.05), showing a trend of increase in patients with an late onset of disease (0.34) compared to age matched healthy controls (0.10, P