| Objectives1. To analyze the data of the cross-sectional study in ALS/MND and investigate the incidence and current status of diagnosis and treatment of ALS/MND in domestic large general hospital.2. To evaluate the nerve conduction characteristics.3. To study cognitive affective impairment and its related factors in ALS/MND patients and their relatives.Material and Methods1. The characteristics of disease onset, current diagnosis and treatment status of ALS/MND patients in our outpatient department and inpatient department from January 2009 to April 2010 were retrospectively analyzed with epidemiological methods.2. Nerve conduction features were summarized, especially in advanced-stage patients.3. Patients with ALS/MND (n=100),their relatives (n=60) and healthy controls (n=100) were recruited in this study, their intelligence level and affection state were evaluated by the means of MMSE, SAS and SDS. All the patients and relatives were compared with those of healthy controls by t-test, the related impact factors were analyzed by multiple linear regression analysis.4. Questionaires were performed in 100 families using self-rating anxiety scale (SAS), self-rating depression scale (SDS).Results1. Cross-Sectional Study The ratio of male and female was 1.28:1. The onset age of patients mostly ranged from 50 to 60 years.25% patients were agricultral sectors.1.7% patients had positive family history.98.3% patients were sporadic ALS/MND. The mean time from onset to definite diagnosis was 16.2±13.4 months. The initial symptoms occurred mostly at cervical region (48.3%). A minority of patients with history of brain truma and poinson history including lead, mecurry.85.7% patients were diagnosed as ALS/MND within 3 times of visiting doctor. Some cases were misdiagnosed as lumbar spondylosis, cervical spondylosis, cerebral infarction and peripheral neuropathy. ALS,PBP and PMA accounted for 84.4%,8.9% and 6.7% respectively.59.4% patients had known what he or she suffered from.25.6% patients had taken riluzole.4.4% patients had accepted psychological therapy and 1.1% had taken anti-anxiety and depression drugs.2. Nerve Conduction 60.8% of patients had motor conduction abnormalities. In the 12 tested motor nerves of advanced-stage patients, CMAP were absent or not induced in 8(66.7%), amplitude of CMAP were decreased in 4(33.3%), DML were prolonged in 2(16.7%) and MCV were reduced in 2(16.7%). Sensory nerve conduction study was normal in majority of tested nerves.3. Cognitive and Affective Disorder Evaluation3.1. MMSE evaluation results of patients with ALS/MND MMSE was 27.84±1.23, significantly lower than that of control group (P<0.05). MMSE of male was slightly higher than that of female, but the difference was not statistically significant (P> 0.05). The MMSE of different aged patients was not statistically significant (P> 0.05). MMSE scores of patients with high education level, short course and higher ALSFRS were all greater than the opponents and with statistic significance (P<0.05). Multivariate analysis:the level of education, course and ALSFRS all have significant effects on the MMSE, and higher education, short course, higher ALSFRS are the protective factors for MMSE.3.2. Anxiety and Depression Evaluation SAS and SDS scores of patient group were 45.5±7.2,44.8±8.2, the ratio of anxiety, depression were 87.5% and 20.8%, and were all significantly higher than those of controls (P＜0.05). SAS and SDS scores of female were significantly higher than those of males (P<0.05). SAS and SDS scores of patiens with university and higher education were significantly higher than those of high school qualifications or below (P< 0.05). SAS and the SDS scores with ALSFRS<20 points were significantly higher than those of ALSFRS score>20 points (P< 0.05). Different ages, vocations, diagnostic types, course and informed or unknown patient were all not statistically significant for SAS and SDS (P> 0.05). Multivariate analysis revealed that patients with anxiety and depressive disorders were the results of many factors. Education and ALSFRS score on the anxiety and depression are significant, that is, lower education level, higher ALSFRS score were the protective factors for anxiety and depression disorders.Conclusions1. The occurrence was not different between male and female. The onset age ranged from 50 to 60 years old. The profession of patients was mostly agricultral sectors. Most patients were sporadic ALS/MND cases. The mean time from disease onset to definite diagnosis was 16 months. The initial site involved was mostly in cervical spinal region. Some patients with poinson history including lead and mecurry. A definite diagnosis of ALS/MND was made 2-60 months after the appearance of initial symptoms. The patients were misdiagnosed as lumbar spondylosis, cervical spondylosis, cerebral infarction and peripheral neuropathy respectively because of the varied clinical features of ALS/MND and doctor's poor understanding about the disease. Most patients were diagnosed as ALS, the rest were PBP and PMA. Only a minority of patients knew what he or she suffered from.2. Some patients present widespread motor nerve conduction damages especially in advanced stage.3. MMSE score of the patient group were lower than that of normal control group, and statistically significant (P<0.05) as well; higher education, short course, higher score of ALSFRS are the protective factors. Anxiety and depression are common in ALS/MND patients. Female, highly educated, lower score of ALSFRS are related to anxiety and depression.4. The SAS and SDS score, the incidence rate of anxiety and depression in patients'relatives are significant higher than those of the control group. The affected factors of anxiety mainly included that female gender and spouse. The affected factors of depression involved mental relative and lower score of ALSFRS.
|
PDF Full Text Request