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 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 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 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

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.

0.05).Conclusion these results suggest that ?_2-AR 16、27 locus genetic polymorphism is not correlated with asthma.

Objective We investigated the effect of Guhong on the capability of brain to resist ischemia.Methods MCAO model was established in SD rats.TTC staining was performed in Guhong group with intraperitoneal injection and routine MCAO model group.Infarction volume was compared.Immunochemistry was performed to observe Caspase 3 expression in peri-infarct cortice of both of the groups.Results Guhong intraperitoneal injection significantly reduced the infarction volume(P﹤0.05).Caspase 3 expression was lower in Guhong group as compared with the model group.Conclusion Guhong may strengthen the capability of brain to resist ischemia.Anti-apoptosisinduced by Guhong may be the one of the underlied mechanisms.