A Follow-up Investigation On Motor Neuron Disease(SP1)

BackgroundThe motor neuron disease are orphan disease with short mean survival time. At present, there are not Golden standard in clinic and specific treatment. Therefore, the motor neuron disease patients often find the treatment everywhere, causing the clinical data lose easily. It's inconvincible to clinician follow up the progression of disease.ObjectivesLong-term and timed follow up the motor neuron disease patients using systemic index of traditional chinese medicine and western medicine to initial investigate the regular of motor neuron disease and to find fators which affect life span. At the same time.establish motor neuron disease patients follow-up system and database.MethodsCollet general information of motor neuron disease patients and use ALSFRS, index of pulmonary function, index of swallowing function, DIT, electromyogram, treatment of traditional Chinese medicine and western medicine, and symptom rating scale of traditional Chinese medicine as observe content to follow up motor neuron disease patients timed.1. Basic data:We observed 43 cases with motor neuron disease in all, 5 patients loss of follow up, loss rate of follow up was 11.62%. Finally, we observed 38 patients(31 patients with ALS,6 patients with SMA,1 patient with PBP) include 25 male and 13 female, the male and female proportion is 1.92:1.The oldest was 77 years,the Youngest was 30 years, the mean age was 53.34±10.37 years old.The patients' course of disease is between 5 months and 109 months.The distribution of motor neuron disease patients'age section is normal distribution.Compared the age distribution between male and female patients, there is no notable statistics difference (P>0.05). The oldest male is 73 years, the Youngest male was 30 years, the mean age was 53.61±9.61 years old. The oldest male is 77 years, the Youngest male was 41 years, the mean age was 52.93±11.78 years old.Family history:All of 38 patients are sporadic cases with no inheritance.Occupation:38 patients include 22 manualworkers, accounting 57.89% and 16 nonmanualworkers, accounting 42.11%。There is no notable difference in MND patient's occupation.Comparison of the distribution between difference start parts:9 cases start from medulla,20 cases start from cervical cord,9 cases start from pars lumbalis medullae spinalis.Using the chi square test to compare the difference start parts between male and female, the chi square test value is 0.876 (P>0.05)Comparison of TCM syndrome distribution:38 patients, lung and spleen asthenia type accounted for 21 cases, steeped hot and humid type in 7 cases,1 case of liver and kidney asthenia type, Kidney and Spleen yang asthenia type in 5 cases,4 cases of Spleen asthenia type, distribution of male and female patients with TCM syndrome compared by x2 test, x2 value of 1.651 (P> 0.05).2. Auxiliary examination:23 patients underwent DTI examination,. Among them,13 patients'DTI result had abnormal (56.5%).10 patients'FA values decreased, Among them,3 patients'ADC values increased.8 patients'pyramidal tract became loose.Lung function and Respiratory muscle strength test:33 patients underwent Lung function tests and respiratory muscle strength check.10 patients had varying degrees of restrictive ventilatory disorders,18 patients'MMV decreased.6 cases of pulmonary diffusing capacity decreased.28 patients'PImax and 25 patients' PEmax decreased. These data above suggest that Some of MND patients have restrictive ventilatory disorders, with Pulmonary diffusion capacity decreased at the same time. PImax and PEmax have high abnormal rate, which is more sensitive to monitor and assess lung function of MND patients.3. ALSFRS-R Rating and⊿FS ALSFRS-R Rating:During follow-up period between September 2010 to March 2011, there are 37 patients accounting definite ALS, possible ALS and SMA patients,to complete ALSFRS-R Rating.The ALSFRS-R score of 27 survival patients (31.93±16.01) Included in the study were significantly higher than ALSFRS-R score of the death group(13.30±10.82).It is significant difference comparing the survival group and death group(P<0.01), and the survival group with ALSFRS-R score decreased during follow-up to 22.41±18.38Rate of disease progression⊿FS= (Initial follow-up ALSFRS-R score minus Last Initial follow-up ALSFRS-R score)/Initial follow-up to the last follow-up timeMaximum⊿FS is 3.20, minimum⊿FS is 0.71, average⊿FSisl.66. The average group,⊿FS greater than 1.66 group survival time was 25±1.38 months,⊿FS less than 1.66 group survival time was 32±2.06 months. There was no significant difference compared between the two group survival time. (log-rank p> 0.05).4. Treatment conditions:MND patients with early compliance of treatment is high.But because there is no specific treatment for advanced and the disease become more serious, coupled with high cost of treatment, patients were followed up 30 patients have not been any post-treatment.5. Survival outcome:to March 2011,10 died (6 males and 4 females), 28 patients survived, the mortality rate of 26.3%. Death of patients, 1 case of SMA patients, the other 9 cases of ALS patients.5 cases of patients start from cervical cord, patients start from medulla in 3 cases, 2 cases patients start from medulla; lung and spleen asthenia type 6 patients,4 cases of spleen asthenia The longest survival of 109 months, the shortest 16 months.MND patients with a median survival time was 30±3.02 months. Log-Rank for the use of male and female survival in patients with MND compared with men, the median survival in patients with MND was 32±4.70 months and the female MND patients with a median survival time was 28±3.16 months, x2 value of 6.391 (P<0.05).The difference is significant.Survival of patients with different starting positions as compared to patients start from medulla median survival of 25±5.84 months. The median survival in patients start from cervical cord of 30±3.29 months. patients start from pars lumbalis medullae spinalis.median survival period of 31±17.53 months. MND patients with different starting parts of the survival of the pairwise comparison, P values were greater than 0.05.MND patients with different TCM syndromes survival compared to patients with lung and spleen asthenia median survival time was 30±4.49 months. steeped hot and humid type patients median survival time was 25±19.25 months. Liver and kidney asthenia type patients, median survival was 37 months. Spleen and kidney yang asthenia type patients median survival time was 45±25.76 months. spleen asthenia type patients in the median survival time was 24±1.96 months. The different contrast between two TCM syndromes, spleen and kidney yang asthenia type patients'survival time than in spleen asthenia type patients'is significant difference. The remaining pairwise comparison TCM syndromes has no significant difference. There was not statistically significant.Conclusion1.MND patients in the age distribution of men and women, starting position distribution, the distribution of syndromes no difference.2. MND disease may be associated with manual labor, Agriculture, Forestry, animal husbandry and fishery.3. MND patients with high drug compliance in an early stage, most of the patients stop taking drugs because of little effect.4. DTI can find the abnormal pyramidal tract, it is useful to assist in the diagnosis of ALS.5. Some of MND patients have restrictive ventilatory disorders, with Pulmonary diffusion capacity decreased at the same time. PImax and PEmax have high abnormal rate, which is more sensitive to monitor and assess lung function of MND patients. But a single measured PIMAX, PEMAX value of the size and length of survival time of patients with MND was no significant correlation.6. With MND became worse, the ALSFRS-R scores were decreasing. It's sensitive scale to access the functional status of MND patients7.⊿FS value of the size was no significant relationship with the survival time of MND patients. So⊿FS is not sentitive indicator to assess MND patients survival time, but the results need further follow-up observation.8. MND patients with survival longer than men, female survival in patients with MND.9. Starting positions had no obvious effect on survival.10. Spleen and kidney yang significantly longer survival in patients with MND temper Deficiency MND patients, But the results still need further follow-up study. The remaining pairwise comparison TCM was no significant difference.