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 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

Objective To investigate the diagnostic value of the split-hand sign in amyotrophic lateral sclerosis ( ALS).Methods Ninety ALS patients, 41 patients with other neuromuscular disorders and 71 normal controls were recruited for conventional nerve conduction study.Compound muscle action potential ( CMAP) amplitude recorded from abductor pollicis brevis ( APB) , abductor digiti minimi ( ADM) and first dorsal interosseous (FDI), CMAP amplitude ratios, CMAP amplitude differences and split-hand index ( SI) were analyzed.Results The APB/ADM CMAP amplitude ratio was significantly lower in the ALS patients (0.44(0.44)) than that in the patients with other neuromuscular disorders (1.31(0.87);z=6.967, P<0.01) and the normal controls (0.99(0.42);z=7.687, P<0.01).The FDI/ADM CMAP amplitude ratio was significantly decreased in the ALS patients ( 0.79 ( 0.46 ) ) compared with that in the normal controls ( 1.23 ( 0.39 ); z =5.899, P <0.01 ).The FDI/ADM CMAP amplitude ratio was comparable between the ALS patients and the patients with other neuromuscular disorders ( 0.93 ( 0.62 );z=1.737,P=0.081).SI was significantly lower in the ALS patients (2.42 (3.14)) than that in the patients with other neuromuscular disorders (10.10(6.54);q=7.947, P<0.05) and the normal controls (17.93(8.32);q=10.827, P<0.05).SI <5.2 can help differentiate ALS from mimic disorders, with a sensitivity of 83.33% and specificity of 96.43%.Conclusions The split-hand sign appears to be a specific feature of ALS.SI robustly differentiates ALS from mimic disorders and potentially facilitates an earlier diagnosis of ALS.

Objective To analyze the symptoms and signs in forty-five Lambert-Eaton myasthenia syndrome (LEMS) patients retrospectively. Characteristics of electrophysiological examinations were investgated. Methods Forty-five LEMS patients were reviewed and information gathered regarding clinical complains neurological symptoms, and other concomitant diseases. The records showed that repetitive nerve stimulation (RNS) and nerve conduction velocity (NCV) were performed in all patients. Needle electromyography (EMG) and skin sympathetic response (SSR) were performed in some patients. Results (1) The mean age of neurological clinical onset age was (51.2 ±6. 8) years old. The two most common symptoms were slight weakness of lower extremities ( n = 35 ) and upper extremities (n= 5). Dysarthria was found in 3 patients and neck weakness in 2 patients. Tendon reflex decreased and disappeared in 38 patients. Autonomic nervous system manifestations were presented in 30 patients. (2) RNS increasing was observed in all patients from 156% to 636%. Low frequency RNS abnormalities were found in 29 patients.Sensory nerve conduction velocity abnormalities or sensory nerve conduction velocity combined motor nerve conduction velocity abnormalities were found in 19 patients ( 42% ). Of the 30 patients who underwent a needle EMG examination, 20 had myogenic or neurological damage. Thirteen abnormal findings were observed in 25 patients who underwent SSR examination. Conclusion The most common manifestations were weakness in lower extremities and autonomic nervous system dysfunction. Many abnormal electrophysiological results were found in LEMS patients, including NCV and EMG abnormalities. These findings indicated that clinical manifestations exceed the neuromuscular junction and perhaps included the peripheral nerve and muscle.

Objective To assess the correlations between muscle strength and amplitude of compound muscle action potential(CMAP)with blood potassium level in hypokalemic periodic paralysis after long exercise test(ET).Methods ET of abductor digiti minimi(ADM)was performed on 78 patients with hypokalemic periodic paralysis.Ulnar and median CMAP amplitude,blood potassium level,muscle strength of ADM,palmar interossei muscle and abductor pollicis brevis were measured before and 120 minutes after exercise.The correlations of muscle strength,CMAP amplitude and blood potassium level were analyzed.Results Ulnar CMAP amplitude was(4.6 ±2.7)mV after ET and(9.6 ±3.2)mV before ET(t =16.047,P =0.000)in 78 patients with hypokalemic periodic paralysis,respectively.Median CMAP amplitude was(10.9 ± 4.2)mV after ET and(11.2 ± 3.9)mV before ET(t =0.673,P =0.822),respectively.After ET,muscle strength of ADM decreased in 76 patients,score on MRC was less than Ⅲ in ADM but V in palmar interossei muscle and abductor pollicis brevis in 41 patients,the blood potassium level was tested in 10 of them,which was(3.8 ±0.3)mmol/L before ET and(3.9 ±0.4)mmol/L after ET(t =0.395,P =0.702).Conclusion In patients with hypokalemic periodic paralysis,blood potassium level is not the key factor affecting muscle strength and CMAP amplitude after ET.

Objective To investigate the diffusion tensor imaging(DTI)and magnetic resonance spectroscopy(MRS)changes in amyotrophic lateral sclerosis(ALS)and to study on the physiopathology of ALS.Methods Thirty-eight AIS patients,8 patients with pure lower motor neuron involvement and 5 patients with mixed-type cervical spondylosis and 34 healthy controls were enrolled and examined with the conventional brain MRI,DTI and 1H-MRS.Results T2-weighted and T2 FLAIR images revealed abnormal high signals in bilateral pyramidal tract positions in 21%(8/38)of ALS patients,which was higher than that in the coaex When compared with the control group,the values of FA in the precentral gyms(0.492±0.059 vs 0.552±0.045,F=17.150,P<0.01),the posterior limb of the internal capsule(0.679±0.048 vs 0.727 4-0.031.F=19.481.P<0.01)and the cerebral peduncle(1eft:0.740 4-0.038 vs 0.761 4±0.024.F=4.290;right:0.720 ±0.044 vs 0.746 ±0.034,F=3.264,both P<0.01)of the ALS group were decreased significantly.so were the values of NAA/Cr.And the FA reduction rate was 10.9%,6.6% and 2.8%-3.5% respectively.There was also a significant decrease in FA in the anterior limb of the internal capsule and the occipital lobe in the ALS group.Conclusion Not only does the application of DTI and MRS help to demonstrate upper motor neuron involvement in the brain and facilitate the diagnosis and differential diagnosis of ALS,but also increases the understanding of the lesion distribution and physiopathology in ALS.

Objective To retrospectively analyze clinical manifestations of autonomic nervous system (ANS) and skin sympathetic response (SSR) in Lambert-Eaton myasthenic syndrome (LEMS).Methods Fifty-three LEMS patients' medical records were reviewed and information regarding clinical symptoms and signs of ANS and SSR testing results were collected.Results ( 1 ) The most common initial symptom of LEMS was weakness of lower extremities ( n =41 ) and the most common symptom of ANS dysfunction was constipation ( n =25 ) and dry-mouth ( n =23),which could be occurred before the onset of the legs (n =7).(2) In symptoms of ANS,cardiovascular system dysfunction was found in 4 patients include one of ingone of bradycardia,one of postural hypotension and 2 of tachycardia- Secretory glands dysfunction was found in 34 patients:23 dry-mouth,6 dry-eyes,and 8 patients sweating dysfunctions.Twenty-eight patients complained of alimentary dysfunction including constipation and diarrhea.Bladder dysfunction was found in 2 patients,who complained of urinary incontinence.Seven male patients complained of sexual dysfunction.Abnormal skin scratch test was found in 17 patients.(3) SSR was performed in 33 patients and 18 found abnormal.Conclusions ANS manifestations are common and prominent in LEMS patients.SSR abnormality is also common in LEMS.More electrophysiology tests are needed in LEMS patients.

Objective To explore the correlation between diffuse neurogenic changes on electromyography and diagnosis of amyotrophic lateral sclerosis (ALS).Methods Retrospective study was performed based on database of motor neuron disorders collected from January,2002 to December,2008.The category of disease with diffuse neurogenic changes at the first examination was summarized.The electromyography (EMG) manifestation in ALS patients at the first examination and the results after follow-up were reviewed.The factors affecting EMG manifestation in ALS were analyzed with binary Logistic regression.Results In 298 patients with diffuse neurogenic changes on EMG,192 cases (64.4％ ) were diagnosed of ALS,36 ( 12.1％ ) progressive muscular atrophy,13 (4.4％ ) Kennedy' s disease,10 (3.4％)Hirayama disease,9 ( 3.0％ ) cervical spondylosis combined with lumbar spondylosis,6 ( 1.3％ ) spinal muscular disease,5 ( 1.7％ ) multifocal motor neuropathy,5 ( 1.7％ ) ALS-plus disease,4 ( 1.3％ )myopathy,3( 1.0％ ) hereditary motor neuropathy,3 ( 1.0％ ) axonal motor neuropathy,2(0.7％ ) postpolio syndrome,and 10 (3.4％) with no definite diagnosis.In total 213 patients who were diagnosed with ALS after follow-up,at their first examinations,8 (3.8％) had neurogenic changes in two regions and 13(6.1％ ) had neurogenic changes in one region,and they all developed to diffuse neurogenic changes after follow-up for 3 to 24 months.Logistic regression analysis showed that the EMG change at first examination was not related to duration from onset,symptom location at onset,age at onset and gender.Conclusion Diffuse neurogenic changes on EMG can present in many disease including ALS.Neurogenie changes in one or two regions on EMG can be the manifestation of ALS at early stage.

Objective To explore an effective method for establishing femoral head necrosis models in young dog and to evaluate osteogenesis of vascular pedicled periosteum transplanted in the repairment offemoral head necrosis of young dogs.Methods Animal models of femoral head necrosis were established by liguting and destroying femoral neck artery circles as well as freezing femoral heads with liquid nitrogen in 29 dogs.At 4 weeks, the animal models of femoral head necrosis were evaluated through X-ray, MRI, ECT,and 2 dogs were randomly executed and detectd by histology examination, then 29 dogs were divided randomly into experimental group(n = 12), control group(n = 9 )and blank group(n = 8).Dogs in the experimental group were treated with the vascular pedicled periosteum from isolateral great trochanter, dogs in the control group were treated with the vascular pedicled bone from isolateral great trochanter, and the blank group were without any treatment.4,8, 12 weeks postoperation,these femoral heads were examined morphologically, radiologieally(X-ray, MRI, ECT) and histologically.All animals were executed and detectd by histology at 12 weeks to observe the repairs of necrotic femoral heads at different periods.Results Twelve weeks postoperation, dogs femoral head figurations in the experimental group were normal, MRI signals were odds in femoral head, ECT studies shows decreased radionuclide compared with contralateral femoral head;capillary vessel and osteoblast multiplication and new traboeulacs were matured in histology examination.Dogs femoral head figurations in the contral group were irregular, MRI revealed high-low signals intermix in femoral head, ECT revealed obviously decreased radionuclide, osteoblast actived, new trabeculac and lipocyte were observed in histology examination.In blank group, femoral head figurations were distorted and sunk, while MRI signals were low, ECT showed no radionuclide uptaking.Collapsed trabeculas and many vacant bone lacuna were observed in HE staining.Dogs femoral head figurations in the blank group taken on distortion andsunk, MRI signals were low, without radionuclide revealed uptake, trabeculac collapse, many bone lacuna were vacant.At the same time, radionuclide counts of vascular pedicled periosteums/vascular pedicled bones in ECT were significantly different between experimental group and control group(P < 0.01 ).Conclusion The vascular pedicled periosteum has higher osteogenesis capability compared with the vascular pedicled bone and can effectively repair the femoral head necrosis of young dogs by this means.

Objective To evaluate the diagnostic accuracy of diffusion tensor imaging (DTI) of corticospinal tract in amyotrophic lateral sclerosis (ALS) and find optimal testing strategies and optimal cutoff values of DTI indices for individual patient discrimination.Methods Thirty-three ALS patients and 34 healthy controls,collected at Peking Union Medical College Hospital from June 2004 through July 2005,undergoing brain DTI studies and fractional anisotropy (FA) examinations along the corticospinal tract,were analyzed by receiver operating characteristic (ROC) curves.Results Compared with the controls,ALS group had significantly decreased FA values in subcortical white matter of the precentral gyrus,the posterior limb of the internal capsule and the cerebral peduncle.In ROC analysis,the average FA value of the former two positions showed the best performance with an area under the curve of 0.917,an optimal cut-off value of 0.604,a sensitivity of 0.759 and a specificity of 0.912.The corresponding data for the average FA of all the three positions and each single position were listed as follows:average of three 0.914,0.648,0.759,0.912; precentral gyrus 0.875,0.509,0.733,0.824; internal capsule 0.845,0.692,0.656,0.941 ; and cerebral peduncle 0.752,0.742,0.656,0.735.Conclusions FA values of the corticospinal tract have a good accuracy in detecting upper motor neuron involvement in ALS.Precentral gyrus and posterior limb of the internal capsule and the average FA values of these two positions were suggested as the preferred testing places and DTI indices for clinical use.