Objective:To investigate the clinical manifestations, gene mutation characteristics, imaging and video electroencephalogram (VEEG) characteristics of patients with dentatorubral-pallidoluysian atrophy (DRPLA).Methods:The clinical data of 9 patients with genetically diagnosed DRPLA in the Neurology Center, Beijing Tiantan Hospital, Capital Medical University from January 2018 to January 2023 were collected, and the clinical data of DRPLA patients reported in China were retrieved and summarized.Results:A total of 45 cases were included. The clinical characteristics were summarized as follows: (1) The male to female ratio of 45 patients was 1.00∶1.25, and the age of onset was (28.11±14.58) years. (2) The main clinical symptoms of juvenile type, early-onset adult type and late-onset adult type were analyzed, and the results showed that the frequency of seizures in juvenile type (16/17) was higher than that in early-onset adult type (8/21) and late-onset adult type (2/7), with statistically significant difference (χ 2=15.971, P<0.001). In addition, the frequency of cognitive impairment in juvenile type (16/17) was also higher than that in early-onset adult type (15/21) and late-onset adult type (2/7), also with statistically significant difference (χ 2=10.177, P=0.005). Cognitive impairment, language disorder and involuntary movement were common in early-onset adult patients, and about half of the patients had ataxia. Ataxia and language disorder were more common in late-onset adult patients, while seizures and cognitive impairment were rare. (3) In imaging, cerebellum and brainstem atrophy was the most common, followed by cortical atrophy and white matter lesions. (4) The number of trinucleotide (CAG) repeats was 53-79, and there was a significant negative correlation between the number of CAG repeats and the age of onset ( r=-0.765, P<0.001), that means the younger the age of onset, the higher the number of CAG repeats. (5) In terms of electrophysiology, 21 patients provided complete VEEG data, of which slowed activity (52%, 11/21) and generalized discharge (71%, 15/21) were more common, and focal discharge (33%, 7/21) was uncommon. Conclusions:DRPLA patients can present with epilepsy, cerebellar ataxia, and other clinical manifestations. Brainstem and cerebellar atrophy and white matter lesions can be relatively characteristic in imaging. In terms of electrophysiology, slowed activity and generalized discharge are more common. DRPLA patients are easy to be misdiagnosed in clinical practice and genetic confirmation helps confirm the diagnosis.

Objective:To investigate the clinical manifestations, gene mutation characteristics, imaging and video electroencephalogram (VEEG) characteristics of patients with dentatorubral-pallidoluysian atrophy (DRPLA).Methods:The clinical data of 9 patients with genetically diagnosed DRPLA in the Neurology Center, Beijing Tiantan Hospital, Capital Medical University from January 2018 to January 2023 were collected, and the clinical data of DRPLA patients reported in China were retrieved and summarized.Results:A total of 45 cases were included. The clinical characteristics were summarized as follows: (1) The male to female ratio of 45 patients was 1.00∶1.25, and the age of onset was (28.11±14.58) years. (2) The main clinical symptoms of juvenile type, early-onset adult type and late-onset adult type were analyzed, and the results showed that the frequency of seizures in juvenile type (16/17) was higher than that in early-onset adult type (8/21) and late-onset adult type (2/7), with statistically significant difference (χ 2=15.971, P<0.001). In addition, the frequency of cognitive impairment in juvenile type (16/17) was also higher than that in early-onset adult type (15/21) and late-onset adult type (2/7), also with statistically significant difference (χ 2=10.177, P=0.005). Cognitive impairment, language disorder and involuntary movement were common in early-onset adult patients, and about half of the patients had ataxia. Ataxia and language disorder were more common in late-onset adult patients, while seizures and cognitive impairment were rare. (3) In imaging, cerebellum and brainstem atrophy was the most common, followed by cortical atrophy and white matter lesions. (4) The number of trinucleotide (CAG) repeats was 53-79, and there was a significant negative correlation between the number of CAG repeats and the age of onset ( r=-0.765, P<0.001), that means the younger the age of onset, the higher the number of CAG repeats. (5) In terms of electrophysiology, 21 patients provided complete VEEG data, of which slowed activity (52%, 11/21) and generalized discharge (71%, 15/21) were more common, and focal discharge (33%, 7/21) was uncommon. Conclusions:DRPLA patients can present with epilepsy, cerebellar ataxia, and other clinical manifestations. Brainstem and cerebellar atrophy and white matter lesions can be relatively characteristic in imaging. In terms of electrophysiology, slowed activity and generalized discharge are more common. DRPLA patients are easy to be misdiagnosed in clinical practice and genetic confirmation helps confirm the diagnosis.

Objective:To investigate the status of professional cognition of the vocational medical students and educate the professional values for the vocational medical students. Method:A questionnaire survey was conducted among 360 vocational medical students. SPSS 16. 0 was used to analyze the data in the present study. Result:Vo-cational medical students have career pursuit and understand profession basic requirements. The students have pragmatic attitude, however, they have unobvious continued willingness to invest and haven 't form clear integral professional cognition. Conclusion: Professional values of the vocational medical students can be cultivate by strengthening ideal and belief, humanities accomplishment, medical professionalism, professional ideological edu-cation, internship and social practice.

Doctors' taking part-time jobs has become prevalent phenomena.But there are blanks of the construction of law in it、the juristic relation between doctors and certified medical treatment institution and medical treatment institution of part-time job、the rights and obligations With the development of market economy,It is necessary to probe into those key juristic problems concerning the phenomena.

Education of humanistic spirit in higher medical education is not illusive,isolated or even contrary to major curricula,but close to professional knowledge and medical sciences.The joint between humanistic spirit and medical sciences lies in the scientific spirit and the spirit of virtue.We should foster humanistic spirit in medical students by taking various measures from the above mentioned two aspects.

OBJECTIVE:To prepare red sage gel for implantation and to establish a method for its quality control.METHODS:With carbamer934as vehicle,2%red sage gel was prepared;a HPLC method for the determination of tanshinone in gel was established.RESULTS:The calibration curve of tanshinoneⅡ A was linear in the concentration range of16.59～33.18ng/ml,Y=9723X—2569(n=5),r=0.9987.CONCLUSION:The preparation of red sage gel was simple,its quality was stable;the method of quality control was rapid and accurate.

OBJECTIVE:To establish a HPLC method to determine the content of compound tramadol tablets METHODS:Stationary phase:Kromasil C18 column,internal standard:phenacetin,mobile phase:methanol-water-triethlamine(55∶45∶0 2),adjusting pH to 4 2 with acetic acid,flow rate∶0 7ml/min,detecting wavelength∶267nm RESULTS:The linear range of tramadol (TR) was 50～500?g/ml(r=0 9 999,n=5) The linear range of nefopam(NFP) was 50～500?g/ml(r=0 9 999,n=5) The average recovery of TR was 99 69% The average content of TR was 100 71%,RSD=0 76%(n=5) The average recovery of NFP was 99 76% The average content of NFP was 99 64%,RSD=0 62%(n=5) CONCLUSION:The method is accurate,rapid and simple