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Beh?et's disease (BD) is a chronic, relapsed, and inflammatory vascular disease, usually with multiple systems involvement. Recurrent oral and genital ulcers, uveitis and skin lesions are common clinical manifestations. When nervous system gets involved, it is called Neuro-Beh?et's disease (NBD). NBD can lead to parenchymal damage to the brain or spinal cord, cerebral venous thrombosis and arterial vasculitis. Our review focuses on the clinical manifestations, auxiliary examination, diagnosis and treatment of NBD.
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Histiocytic necrotic lymphadenitis is known as Kikuchi-Fujimoto disease (KFD).KFD is a systemic disease,which is characterized by fever,necrotizing lymphadenopathy and leukopenia.However,involvement of the central and peripheral nervous system is rare.It can present as aseptic meningitis,encephalomyelitis,peripheral neuropathy,cerebellar ataxia and tremor,which makes it difficult to be differentiated with other nervous system diseases.Our review summarizes the clinical manifestations and differential diagnoses of KFD involving nervous system in order to reduce misdiagnoses and mistreatments.
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Objective To summarize the clinical and the molecular genetic characteristics of type DYT11 dystonia by analyzing the clinical data and pathogenic gene mutation of type DYT11 dystonia of a myoclonus-dystonia syndrome (MDS) family.Methods A MDS family enrolled in the General Hospital of the People's Liberation Army Rocket Force in January 2018 was retrospectively analyzed.The clinical data of 11 affected family members were collected and genetic testing of four affected family members (including the proband) of the MDS family was conducted using a panel of dystonia-associated genes.Results The affected family members showed great differences in clinical characteristics and obvious clinical heterogeneity.Four affected family members had myoclonus and dystonia,two affected family members only had myoclonus and five affected family members only had dystonia.The results of genetic testing showed that the proband,his father and his grandfather had a mutation (c.835_839delACAAA) in SGCE gene,which is autosomal dominant and belongs to type DYT11 dystonia.Conclusions MDS shows clinical heterogeneity.Gene screening is of great importance for the diagnosis and treatment of dystonia with myoclonus.
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Objective To evaluate the validity and reliability of modified absolute and relative scale for myasthenia gravis (mARS-MG),and explore its clinical application value in evaluating the severity of MG.Methods One hundred and three MG patients,admitted to our hospital from March 2016 to February 2017,were enrolled in our study.A new measurement called mARS-MG was put forward,which had modified the items about bulbus medullae of ARS-MG.In mARS-MG,chewing and swallowing were split into 2 items.The swallow function was measured by water swallow test.An experienced neurologist performed 4 scales,ARS-MG,mARS-MG,Quantitative Myasthenia Gravis scale (QMG) and Myasthenic Muscle Scale (MMS),on these patients.Spearman correlation analysis was performed to assess the correlations of ARS-MG,mARS-MG,QMG,and MMS scores.Validity of ARS-MG and mARS-MG was constructed and evaluated by structural equation modeling.Besides,Cronbach's alpha coefficient was used to evaluate the internal consistency of the scales.Results Strong correlations were noted among ARS-MG,mARS-MG and QMG,MMS scores ([r[＞0.800,P＜0.05).The items in each scale were grouped according to different muscle groups;strong correlations among ARS-MG,mARS-MG,QMG and MMS scores were found in bulbus medullae,limbs and respiratory muscle (|r|＞0.900,P＜0.05).In the extraocular muscles' part,the correlations were also well (|r|＞0.650,P＜ 0.05);however,the correlations in facial part between these scales were not well.The chewing and swallowing items were highly correlated to QMG and MMS scores (|r|＞0.900,P＜0.05).Besides,the items of ARS-MG and mARS-MG were all reasonable;these two scales had good construct validity;the reliability analysis showed that the intemal consistency of ARS-MG and mARS-MG was good (Cronbach's α＞0.8).Conclusion Both ARS-MG and mARS-MG showed good validity and reliability in evaluating the severity of MG patients;they are all suitable for further promotion and clinical application,and the modified version is better than the original one in the bulbar part.
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Objective To investigate the correlation between opportunistic infections in patients with HIV /AIDS in Dali of Yunnan Province and CD +4 lymphocyte count,discover the incidence trend of opportunistic infections in patients with HIV /AIDS in Dali and instruct early diagnosis and treatment.Methods Choosing 454 cases of opportunistic infections in patients with HIV /AIDS in Infectious Disease Dali Prefectural Hospital were chosen,ana-lyze various opportunistic infections,examining CD +4 lymphocyte count and analyze the differences of opportunistic infections on CD +4 lymphocyte count.Results 454 cases opportunistic infections with HIV /AIDS,48.24% were HCV infections,38.72% of various tuberculosis consumption phthisis,28.41% of bacterial pneumonia and 25.77%of oral condida monilia infections,high rate of opportunistic infections when CD +4 lymphocyte count 350 /μL of 5.56%(χ2 =34.88,P <0.01);The high rate of opportunistic infections found on patients without the treatment of HAART(40.83%,U =9.05,P <0.01),comparing to 18.79% of those with the treatment.Conclusion The com-mon opportunistic infections in our area are HCV,various tuberculosis consumption phthisis,bacterial pneumonia and oral condida monilia infections;high rate of opportunistic infections happens when CD +4 lymphocyte count are low and lower rate on those with the treatment of HAART,offering an effective method on controlling opportunistic infections.
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Objective To explore the cerebral circulation characteristics of patients with persistent trigeminal artery (PTA) combined with cerebrovascular diseases and their clinical relevance.Methods Eight patients with PTA,admitted our hospital from March 2010 to March 2013 and conformed by DSA,MR radiography (MRA) or CT angiography (CTA),were chosen in our study; their cerebral circulation features and clinical manifestations were retrospectively analyzed.Results In these eight patients,two were combined with cerebral infarction,one with cerebral artery stenosis,two with cerebral infarction and cerebral artery stenosis,one with transient ischemic attack,and the left two with cerebral aneurysm.Four patients complained for paroxysmal dizziness (one of them with one-side weakness),two patients complained for numbness of a limb,One patient had headache and blepharoptosis and one patient had diplopia.Saltzman type Ⅰ was noted in six patients,type H in one and special type in one.The pathogenesis of cerebral infarction was related to PTA.Conclusion The clinical features of the patients with PTA often determine by merger cerebrovascular diseases; PTA can change the normal cerebral circulation; PTA detection can be helpful in the diagnosis of the pathogenesis of multiple cerebral infarction.
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This paper introduces the technical parameters,basic principle and investigation method of the multiparameter supervisor.The functions and implementation methods of its components are also presented.The supervisor can provide much physiological information of the patients to medical staffs.Thus,the level of medical supervision and analysis can be enhanced.The multiparameter supervision can be realized when the microcomputer system with PC-104construction is adopted to analyze the received signals and filter the interfering signals resulting from the random variations of frequency and amplitude.