Because of the relationship between the quality and the processing technology of Chinese herbal pieces was very close, we proposed concerning the problems of Chinese herbal pieces characters and processing experi-ence. Then, we made use of intelligent sensory technique to objectively express the characters of Chinese Herbal pieces, and explored to set the methods and models about evaluating the quality of Chinese herbal pieces of appear-ance characters and internal quality. To realize the expression of experience terms such as huoli and huohou in the process of Chinese herbal pieces objectively, we adopted on-line infrared temperature measurement and biologi-cal evaluation technologies, and established new technologies and methods of Chinese herbal pieces process quality control in the process.We established a workable monitoring technology, which could not only achieve the controlla-bility and stability of processing quality, also ensured the safety and effectiveness of Chinese herbal pieces.

OBJECTIVE: Apolipoprotein (Apo) E- ε 4 is the first identified important susceptible gene of late-onset family and sporadic Alzheimer disease. Apo E E4 or ε 4 chained other genetic products probably play a certain actions in occurrence of Alzheimer disease, but their biological basis is unknown.To explain the relevant biological mechanism between ApoE and Alzheimer disease is still the key in next study.DATA SOURCE: Computer Medline database is used to search the relevant papers from January 1978 to January 1999, with the words of "develop", "ApoE" and "Alzheimer", limited in English version. Simultaneously, the relevant papers are looked up from China Journal Databasefrom January 1978 to December 2003, with the words of "ApoE, Alzheimer disease and development", limited in Chinese version.STUDY SELECTION: Preliminary examination is done to the data. Inclusive criteria: relevant papers on ApoE gene and Alzheimer disease, including the study of both animal experiment and clinical basic research.Exclusive criteria: repeated study, summarized and Meta analysis papers.DATA EXTRACTION: Totally 55 relevant papers are collected, of which, 20 papers are included and 33 papers are excluded due to repeated experiment and summarized papers.DATA SYNTHESIS: Of 20 experiments, 16 experiments explain that ApoE E4 or ε 4 chained other genetic products probably play a certain actions in occurrence of Alzheimer disease and 4 researches propose that ApoE- ε 4 is the susceptible gene of Alzheimer disease.CONCLUSION: It is viewed in majority at present that ApoE genotype determination cannot substitute any clinical and imaging diagnostic method, which is probably onlya kind of assistant diagnosis to improve the specialty in clinical diagnosis. Beingthe susceptible gene of Alzheimer disease, ApoE- ε 4 is the important discovery in its research no doubt, but,many relevant questions are still at experimental and hypothesis stages. In the future, the great consideration should be still drawn on ApoE genotype determination of Alzheimer disease.

Objective To investigate the clinical,neuroimaging, pathological features and prognosis of brainstem encephalitis. Methods The clinical and neuroimaging features and prognosis of 15 cases with brainstem encephalitis were analyzed retrospectively.Among them,1 case of brain autopsy was studied by HE,K-B and immunohistochemical staining. Results In 7 cases,the history of viral infections before the onset of disease was obtained.The initial neurological manifestation was dizzy in 6 cases,diplopia,headache and vomiting in 2 cases respectively,and unconciousness,ptosis and facial numbness in 1 case respectively.The main clinical features were shown that 15 cases demonstrated the involvement of cranial nerves,9 cases pyramidal tract and 5 cases spinothalamic tract,8 cases ataxia,4 cases Horner sign and 2 cases unconsciousness.MRI found single or multiple lesions in the corresponding parts of brainstem in 10 cases.In case of brain autopsy,neuropathological examination revealed local hemorrhagic necrotizing softening lesions in the pontine tegmentum and lateral medulla.A marked infiltration of lymphocytic inflammatory cells and macrophages were observed in the necrotic area.Myelin loss,axonal swelling and mild increase of reactive astrocytes were also observed in the cranial nerve nuclei.There was no positive finding of immunohistochemical staining by using the primary antibodies against HSV-I,Ⅱ,EB,CMV and toxoplasma.The prognosis for other surviving 14 cases were benign,and no relapsing case was found after 39.9 months of follow-up. Conclusions The course of brainstem encephalitis seems to be monophasic and benign,which indicates that it might be not the first episode of multiple sclerosis.The underlying causes of the disease are usually difficult to be identified.

Leukoencephalopathy with ataxia (LKPAT) is a rare autosomal recessive disorder caused by mutations of CLCN2 gene. LKPAT is clinically characterized by cerebellar ataxia, headache and cognition impairment. Brain magnetic resonance imaging showed characteristic hyperintensities along the pyramidal fiber tracts. Few cases have been reported so far. This article reported the clinical data of a 48 years old female patient with LKPAT for clinical reference.

Myasthenia gravis (MG) is an immune-mediated disorder of the neuromuscular junction that is characterized by fluctuating muscle weakness.Acetylcholine receptor antibody (AchR-Ab) is the first MG specific antibody being elucidated,however,the discovery of many other MG specific or related antibodies enriches its immunopathogenesis greatly.The detection of antibodies is very important for the diagnosis and classification of MG.It can be used to guide the clinical management and evaluate the response to treatment as well.With the development of laboratory medicine,the methods of antibody detection are also being optimized.In this paper,we will review the serum antibodies closely associated with MG and their mechanisms,detection methods and clinical significance.

Objective To investigate muscle MRI characteristics of lower limbs in Chinese patients with dysferlinopathy. Methods Detailed clinical information of 42 patients with dysferlinopathy confirmed by Western blot or DYSF genetic test were studied retrospectively, including age, course, serum creatinine kinase (CK) and modified Gardner?Medwin and Walto score, and T1WI, STIR image. Each muscle was scored according to its fatty degeneration evaluated on T1WI (fat replacement score). The patients were divided into 3 groups:Miyoshi myopathy (MM), limb girdle muscle dystrophy 2B (LGMD 2B) and preclinical stage (asymptomatic hyperCKemia or exercise intolerance). The data including the scores of each muscle between MM and LGMD 2B were compared by ANOVA analysis and Chi square test. The relationship of fatty replacement score with course and GM?W score was analyzed by Spearman rank correlation analysis. Results Thirty nine patients underwent thigh MR scanning and 36 patients underwent leg MR scanning. At the thigh level, there is no specificity that the fatty replacement was found in both the anterior and posterior parts while the rectus femoris, sartorius and gracilis were rarely involved. At the leg level, the most severely involved muscle was the soleus, followed by gastrocnemius. It formed a sandwich?like pattern that the anterior part (anterior and posterior tibial muscle and peroneus longus muscle) and the posterior part (medial and lateral gastrocnemius) were less involved than the middle part (soleus). Of 42 patients, 14 cases were MM, and 24 were LGMD 2B. The fat replacement score of each muscle between two groups showed no significant differences (F=0.066 to 3.907,P all>0.05) except for the adductor muscle (F=5.239, P=0.028), semimembranosus (F=6.703, P=0.014) and semitendinosus (F=7.689, P=0.009). Of 4 pre?symptomatic cases, 3 showed edema of posterior part of leg on STIR, especially soleus. In all patients, the fat replacement score correlated positively with course (rs=0.732, P=0.000) and GM-W score (rs=0.485, P=0.001). Conclusions The MRI of Chinese patient with dysferlinopathy was characterized by the milder involvement of rectus femoris, sartorius and gracilis muscle in the thigh and a sandwich?like pattern in the leg, which is helpful for differential diagnosis of inflammatory Myopathy versus other types of muscular dystrophy.

Objective To investigate the clinical and immune pathological features of Ullrich congenital muscular dystrophy (UCMD) with sarcolemma-specific collagen Ⅵ deficiency (SSCD). Methods The clinical aspects of 2 patients with SSCD were analyzed and the muscle specimens from them were studied by immunofluorescence. Results SSCD patients were clinically characterized by neonatal hypotonia with proximal contractures and distal hyperlaxity at birth or early infancy. Immunofluorescence staining revealed partial deficiency of collagen Ⅵ. Double immunofluorescence staining revealed sarcolemma-specific deficiency of collagen Ⅵ, while collagen Ⅳ intact in thesarcolemma. Conclusions The clinical picture and severity of UCMD with SSCD are similar to the cases with collagen Ⅵ complete deficiency. The proximal contractures and distal hyperlaxity are the clinical hallmarks of both types. Sarcolemma-specific collagen Ⅵ deficiency can be better demonstrated by double immunofluorescence staining.

Objective To evaluate Western blot analysis in diagnosing limb-girdle muscular dystrophy type 2A (LGMD2A). Methods The clinical records including their pathological and biochemical results of 4 patients with LGMD type 2 were reviewed. Histochemical and immunohistochemical staining were performed on muscle biopsy specimens from the four patients. The expressions of dysferlin and calpain-3 in muscles were analyzed by Western biol. Results All 4 LGMD patients shared some common clinical features, such as dorsal muscular atrophy of lower limbs and remarkably elevated CK. The immunohistochemical results showed partial or complete deficiency of dysferlin staining in all 4 LGMD patients. However, Western blot revealed that the calpain-3 protein in the muscle of patient 1 was completely absent, who was later diagnosed with LGMD2A. The other 3 patients had complete dysferlin deficiency with reduced calpain-3 expression and they were confirmed to be LGMD2B. Conclusions Western blot analysis of calpain-3 and dysfcrlin can be used to differentiate LGMD2A which shows absence of calpain-3 from other LGMD types which show dysferlin deficiency. Western blot is an invaluable method in clinical diagnosis of LGMD2A.

Objective To investigate the removal effect of immunoadsorption (IA) on associated antibodies and the efficacy in late-onset myasthenia gravis (MG). Methods A total of 25 late-onset MG patients were randomly selected to enroll in this study. IA therapy was given to 10 patients (IA group), while immunoglobin (0.4 g·kg-1·d-1) was administrated to the other 15 patients for 5 days(Ig group). The titers of Titin antibody (Titin-ab), acetylcholine receptor antibody (AchR-ab) and presynaptic membrane antibody (PrsmR-ab) were detected before and after the treatment. Quantitive MG (QMG) score was assessed before and immediately after the entire course of treatment. The clinical efficacy, the duration of respiratory support and in-hospital were compared between two groups. The correlation between three antibodies and QMG score was also analyzed. Results Compared with that before treatment, the Titin-ab PIN values, the AchR-ab PIN values, and the PrsmR-ab P/N values of IA group were all decreased significantly after treatment (P<0.05, respectively). The P/N value of Titin-ab in IA group was decreased by 54.7%～3.5%, which was significantly higher than that in Ig group(19.9%±3.1%) (P<0.01). QMG score reduced by 42.4%± 4.2% and 23.8%±3.7% in IA group and Ig group respectively (P<0.01, respectively). Symptoms were effectively ameliorated by both treatments, but the effective power of IA group was higher than that of Ig group (70% vs 40%, P<0.05). Remission time of IA group was significantly shorter than that of Ig group [(5.38±0.42) d vs (8.4±1.54) d, P=0.008), so was the duration of in-hospital [(13.50±0.50) d vs (16.50±0.50) d, P<0.05). The number of respiratory support in IA group was less than that in Ig group (1/10 vs 6/15, P<0.05). By the Pearson correlation analysis, the decrease of Titin-ab showed a better longitudinal correlation with the decrease of QMG score than the other two antibodies (r=0.6315, P<0.01). Conclusion IA can rapidly and effectively clear the pathogenic antibodies of late-onset MG patients and its short-term clinical efficacy is better than immunoglobin.

Rhizoma Pinelliae Fermentata(RPF)wasoneofthecommonlyusedChinesemateriamedica(CMM). According to the ancient and modern literatures on RPF, the historical evolution, fermentation methods, chemical compositions, efficacy and microbes of RPF were systematically summarized in this paper. Through the analysis on existing problems of fermentation strains, effective components, quality standard and fermentation process, the corresponding solutions were proposed. This work may provide an idea and reference for the further study of RPF.