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Objective To compare the incidence rate of non-motor symptoms including constipation, depression,REM sleep disorder(RBD)in patients with Parkinson′s disease(PD)and essential tremor(ET). Methods Sixty patients with PD and 40 patients with ET treated in the department of neurology of Tianjin People′s Hospital from October 2015 to June 2016 were enrolled in the study.The clinical data were recorded.The incidence rates of constipation,depression,REM sleep disorder in PD patients and ET patients were compared in order to analyze the correlation among HY staging and the duration of constipation,depression and RBD in PD patients.Results The incidence rates of constipation,depression,REM sleep disorder in PD group were significantly higher than those in ET group(88.3%(53/60)vs.10.0%(4/40); 61.7%(37/60) vs.27.5%(11/40),51.7%(31/60)vs.7.5%(3/40)),the differences were statistically significant(χ2=60.08,11.22,20.86,P<0.05).Spearman rank correlation analysis showed that the HY staging of patients with PD was related to the duration of constipation,the duration of depression and the duration of RBD(r=0.570,0.369,0.439,P<0.01).Conclusion The degree of correlation between constipation and HY staging is relatively high in PD patients.PD patients with constipation,depression,REM sleep disorder can provide reference for early diagnosis and differential diagnosis of ET patients.
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Objective To investigate the relationship between polymorphisms of two iron-related genes Transferrin (Tf) 258G/S and Transferrin receptor (TfR) 82S/G and Parkinson's disease (PD) in north and northwest China.Methods Polymerase chain reaction and restrict on fragment length polymorphism (PCR-RFLP) was used to analyze the genotypes and allele frequency distributions of Tf258G/S and TfR 82S/G in 61 north china PD patient group and 70 randomly selected locally healthy control subjects,and in 56 northwest China PD patient group and 70 randomly selected locally healthy control subjects.Results (1) In allelic and genotypic distributions of Tf258 G/S,the allele G and genotype GS in two PD patient groups were both significantly higher than those in corresponding controls (P<0.05),but there was no significant difference between the two PD patient groups,or between the two controls (P>0.05).(2) There were no significant differences in allelic and genotypic distributions of TfR 82S/G between the two PD patient groups,or between the two controls (P>0.05).Conclusions There might be association of Tf 258 G/S gene polymorphism with north and northwest China PD patients;the allele G and genotype GS might increase susceptibility to PD.No association of TfR 82S/G gene polymorphism with north and northwest China PD patients is noted.
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Objective To investigate the brain glucose metabolism in different stage of mixed-type multiple system atrophy (MSA).Methods Forty-six MSA patients with cerebellar or Parkinsonian symptoms and 18 healthy controls with similar age as patients were included.According to the disease duration,the patients were divided into three groups: group 1 (≤ 12 months,n=14),group 2 (13-24 months,n=13),group 3 (≥ 25 months,n =19).All patients and controls underwent 18F-FDG PET/CT brain imaging.To compare metabolic distributions between different groups,SPM 8 software and two-sample t test were used for image data analysis.When P<0.005,the result was considered statistically significant.Results At the level of P<0.005,the hypometabolism in group 1 (all t>3.49) was identified in the frontal lobe,lateral temporal lobe,insula lobe,anterior cingulate cortex,caudate nucleus and anterior cerebellar hemisphere.The regions of hypometabolism extended to posterolateral putamen and part of posterior cerebellar hemisphere in group 2 (all t>3.21).In group 3,the whole parts of putamen and cerebellar hemisphere were involved as hypometabolism (all t>4.08).In addition to the hypometabolism regions,there were also stabled hypermetabolism regions mainly in the parietal lobe,medial temporal lobe and the thalamus in all patient groups (all t>3.27 in group 1,all t>3.02 in group 2,all t>3.30 in group 3).Conclusions Disease duration is closely related to the FDG metabolism in the MSA patients.Frontal lobe,lateral temporal lobe,anterior cingulate cortex and caudate nucleus can be involved at early stage of the disease.Putaminal hypometabolism begins in its posterolateral part.Cerebellar hypometabolism occurs early at its anterior part.Besides,thalamus shows hypermetabolism in the whole duration.18F-FDG metabolic changes of brain can reflect the development of mixed-type MSA.
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Wavelet entropy,as a powerful quantitative parameter to measure the ordering/disordering level of multi-scale dynamical behavior for nonlinear signals,provides information of complex degree in nonlinear dynamical process.Recently,the wavelet entropy is attracting more and more attention in electroencephalogram (EEG) signal analysis,which is employed by domestic and overseas scholars to investigate the complex degree of EEG,evoked potential and event-related potential,and to profoundly reveal the dynamic mechanism of physiological electrical activity in the brain.It is mainly used in the research of perception,cognitive activity,dynamic observation of epileptic EEG signals,sleeping,internet addiction and rehabilitation of brain after injury.Not only can the wavelet entropy represent the dynamic evolution process of the frequency synchronization for stimulated EEG signals,but also distinguish the states before and after epileptic seizure,as well as to deepen the understanding of brain dynamics mechanism.The wavelet entropy is becoming a new tool for investigating cognition and exhibits a good application prospect in EEG signal analysis.
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Wavelet entropy is a quantitative index to describe the complexity of signals. Continuous wavelet transform method was employed to analyze the spontaneous electroencephalogram (EEG) signals of mild, moderate and severe Alzheimer's disease (AD) patients and normal elderly control people in this study. Wavelet power spectrums of EEG signals were calculated based on wavelet coefficients. Wavelet entropies of mild, moderate and severe AD patients were compared with those of normal controls. The correlation analysis between wavelet entropy and MMSE score was carried out. There existed significant difference on wavelet entropy among mild, moderate, severe AD patients and normal controls (P<0.01). Group comparisons showed that wavelet entropy for mild, moderate, severe AD patients was significantly lower than that for normal controls, which was related to the narrow distribution of their wavelet power spectrums. The statistical difference was significant (P<0.05). Further studies showed that the wavelet entropy of EEG and the MMSE score were significantly correlated (r= 0. 601-0. 799, P<0.01). Wavelet entropy is a quantitative indicator describing the complexity of EEG signals. Wavelet entropy is likely to be an electrophysiological index for AD diagnosis and severity assessment.
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Sujet âgé , Humains , Maladie d'Alzheimer , Diagnostic , Études cas-témoins , Électroencéphalographie , Entropie , Analyse en ondelettesRésumé
Objective To investigate the interactive mechanism of dopamine and α-synuclein in SH-SY5Y cells,and to explore their effects on Parkinson's disease (PD).Methods The concentration of exogenous dopamine was definited by MTT method.The effect of exogenous dopamine on the expressions of α-synuclein and caspase-3 active fragments and malondialdehyde (MDA) content were detected by Western blot and commercial kit.Recombinant plasmid of wild-type α-synuclein was transfected into SH-SY5Y cells.Level changes in dopamine concentration,MDA content and expression of caspase-3 active fragment were observed.Furthermore,the effect of GBR12935,an inhibitor of dopamine transporter,on these outcomes were detected.Results Intracellular dopamine concentration was increased by 16 folds in SH-SY5Y cells which were added 300 umol/L exogenous dopamine for 24 hours as compared with blank controls (t=7.32,P<0.01).Expressions of α-synuclein and caspase-3 active fragments and MDA content were increased in exogenous dopamine treated group as compared with blank controls (t=4.92,17.14,6.55,all P<0.01).Intracellular dopamine concentration,MDA content and expression of caspase-3 active fragment were increased in SH-SY5Y cells transfected with recombinant plasmid of wild-type α-synuclein as compared with SH-SY5Y cells transfected with empty plasmid (F =32.97.107.80,55.54,all P<0.01),whlie these increases could be partially inhibited by GBR12935.Conclusions Dopamine promotes the expression of α-synuclein,while over-expressed α-synuclein increases intracellular dopamine concentration,which forms a vicious cycle of cytotoxicity in SH-SY5Y cells.
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Objective To study the regional cerebral glucose utilization with 18 F-fluorodeoxyglucose (FDG) PET and to investigate the correlation between cerebral glucose metabolism and the clinical characteristic of progressive supranuclear palsy (PSP).Methods A total of 13 patients with PSP and 30 matched healthy controls were performed 18F-FDG PET imaging at rest state.Visual inspection and statistical parametric mapping (SPM) were used to investigate regional cerebral metabolic rate of glucose (rCMRglc).Results Based on the visual inspection,PET imaging in the PSP patients showed that the focal hypometabolic areas mainly included the bilateral frontal cortex,midbrain and subcortical structures.Compared to the controls,voxel-based analysis showed that the regional glucose metabolism decreased in bilateral superior,middle frontal gyrus,cingulate gyrus,midbrain and subcortical structures including basal ganglion and thalamus,which were consisted with the clinical characteristics,such as vertical gaze palsy,pseudobulbar palsy,postural instability,axial rigidity,dementia and so on.Conclusion 18 F-FDG PET imaging is helpful for the early diagnosis of PSP.
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Objective To investigate clinical and imaging features of corticobasal degeneration syndrome (CBDS). MethodsA clinical, imaging and therapeutic analysis of 4 cases of clinically diagnosed with corticobasal degeneration was conducted. Results Asymmetric parkinsonism was the first symptom in all 4 cases who lacked of response to levodopa. Other symptoms including limb apraxia and anarthria occurred in all 4 cases,myoclonus occurred in three,dementia occurred in two,and involuntary movement occurred in one. All the patients had asymmetric frontoparietal cortical atrophy in the contralateral to the dominantly affected limbs on MRI.Asymmetric hypometabolism of the frontoparietal cortex and basal ganglia was observed on 18 F-FDG PET in all 4 cases.ConclusionsThe clinical features of CBDS are asymmetric parkinsonism,dementia,apraxia,anarthria,myoclonus,and involuntary movement.Brain MRI and 18 F-FDG PET are helpful to diagnosis of CBDS.There is no effective treatment for CBDS at this time.
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Objective To investigate the association between polymorphisms in monoamine oxidase B (MAO-B)and early-onset Parkinson's disease(EOPD).Methods Polymerase chsin reactionrestriction fragment length polymorphism was used to identify the genotypes of polymorphisms in MAO-B in 65 patients in EOPD group(early-onset age<50 years),60 in late-onset Parkinson's disease(LOPD) group(late-onset age≥160 years)and 66 healthy controls(<50 years).Results The frequency of AA genotype was higher in EOPD groups(49/65,75.4%)than in healthy controls(34/66,51.5%),and the difference between them was statistically significant(x2=8.075,P=0.018).The frequency of AA genotype between EOPD group and LOPD group,between LOPD group and healthy controls had no statistical significance.The frequency of AA genotype between male in EOPD group and male healthy controls,between female in EOPD group and female healthy controls had no statistical significance.The frequencies of AA genotype between male in EOPD group and LOPD group,between female in EOPD group and in LOPD group had no statistical significance.The frequency of AA genotype between male in LOPD group and in healthy controls,between female in LOPD group and female healthy controls had no statistical significance.The frequency of A alleles was higher in EOPD group(107/130,82.3%)than in healthy controls(87/132,65.9%)and the difierence between them was statistical significant(x2=9.165,P=0.002).The frequency of A allele between EOPD group and LOPD group,between LOPD group and healthy controls had no statistical significance. The frequency of A allele was higher in male EOPD group (60/70,85.7%) than in male healthy controls(51/72,70. 8% ), the difference between them was statistically significant (X2 =4. 606, P=0. 032) ;the frequency of A alleles was higher in female in EOPD group (47/60,78. 3% ) than in female healthy controls(36/60,60. 0% ), the difference between them was statistical significance( x2 =4. 728, P = 0. 030). The frequency of A alleles between male EOPD group and male LOPD group, between female EOPD group and female LOPD group had no statistical significance. The frequency of A allele between male LOPD group and male healthy controls, between female LOPD group and female healthy controls had no statistical significance. Conclusions The AA genotype of MAO-B is the risk factor of EOPD. The A allele of MAO-B is a risk factor of EOPD group for both male and female.
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Objective To study the clinical and imaging characteristics as well as cerebrospinal fluid chan-gea(CSF) of spontaneous intracranial hypotensian syndrome (SIHS).Methods The clinical characteristics, CSF and imaging data of 13 patients diagnosed as SIHS were retrospectively analyzed.Results All the 13 patients had orthostatic headache accompaning one or more numerous symptoms including nausea, vomiting, dizziness, diplopia and neck stiffness.All the patients had low CSF pressure,which was below 60 mm H2O and high CSF protein was in 5 patients, 8 had increased white cell counts and 9 had increased red cells counts;CT was performed in all patients.On CT scan the subdural effusion or small ventricles were compressed in 4 patients.MRI typically revealed diffused pachymeningeal enhancement in 2 patients;All the patients experienced relief of symptoms through conventional treatment.Conclusion Orthostatic headache is the most typical symptom in spontaneous intracranial hypotension syndrome and diffused pachymeuingeal enhancement is the most common imaging manifestation, and CSF hypovol-emia is the basis of pathophysiology of spontaneous intracranial hypotension syndrome.
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<p><b>OBJECTIVE</b>To study the association of two polymorphisms of ubiquitin carboxy-terminal hydrolase-L1 gene(UCH-L1), the 54C/A in exon 3 and the 277C/G in exon 4, with sporadic Parkinson's disease(PD) in Hans from North China.</p><p><b>METHODS</b>Polymerase chain reaction-restriction fragment length polymorphism was used to investigate the genotype and allele frequencies of the UCH-L1 C/A and C/G loci, in a case-control study including 75 sporadic PD and 100 randomly selected healthy control subjects.</p><p><b>RESULTS</b>(1)There was significant difference between PD patients and controls in the frequencies of UCH-L1 genotype and C/A allele(P<0.05). The frequencies of allele A and genotype AA were both significantly lower in PD patients than that in the controls(P<0.05).(2)There was no polymorphism in the UCH-L1 C/G locus in all cases and controls.</p><p><b>CONCLUSION</b>(1)There might be association between the polymorphisms of UCH-L1 C/A locus and sporadic PD in Han population from North China.(2)There is no polymorphism in the UCH-L1 C/G locus in Hans from this region.</p>
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Adulte , Sujet âgé , Sujet âgé de 80 ans ou plus , Femelle , Humains , Mâle , Adulte d'âge moyen , Asiatiques , Génétique , Ethnies , Génétique , Exons , Génétique , Fréquence d'allèle , Prédisposition génétique à une maladie , Génotype , Maladie de Parkinson , Génétique , Polymorphisme génétique , Ubiquitin thiolesterase , GénétiqueRésumé
Objective To investigate the clinical characteristics and mutations of guanosine triphosphate eyclohydrolase (GCH) Ⅰ gene in patients with dopa-responsive dystonia (DRD). Methods Five families with 18 affected family members and 17 patients with sporadic DRD were examined. Patients were allocated into 3 groups according to onset time, either in childhood, or in adolescence or adult. Interview, physical examination, psychologic testings and CT/MR scan were performed. Mutation screening was performed on 26 patients and 1 normal family nember. Thirty-five healthy control subjects were matched for age and sex. Statistical analysis were conducted with the use of SPSS 13.0 computer software. Results(1)Most of patients started with dystonia. The main clinical manifestation was dystonia too. There was no difference among 3 groups.(2) There were significant differences in diurnal fluctuation among 3 groups(15/15,6/6,7/14, χ2=13.125,P=0.001). Diurnal fluctuation negatively correlated with age (r=-0.720, P<0.01).(3)The differences in postural tremor were also found among 3 groups (7/15,5/6,1/14, χ2=8.073, P=0.018). Postural tremor positively correlated with age (r=0.399, P=0.018).(4)There were differences in exaggeration of tendon among three groups(11/15,1/6,4/14, χ2=8.309, P=0.016). Exaggeration of tendon reflexes negatively correlated with age (r=-0.429, P=0.010).(5)The scores of Hamilton Depression Scale and Hamilton Anxiety Scale in patients were higher than those in controls.(6)DNA sequencing revealed a heterozygous A224G missense mutation(Tyr75Cys)located within exon 1 in one autosomal dominant inheritance family. Conclusions The manifestations of DRD varies. The clinical course is closely correlated with age. A missense mutation(A224G)in coding region of the GCH 1 gene probablyleads to the occurrence of DRD.
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Objective To detect the relationship between the genotypes of the 2642 deletion polymorphism (delta 2642) in IT15 gene and the age of onset of Huntington disease (HD). Methods Peripheral blood samples were collected from 29 patients with HD and 38 gender- and age-matched controls. All patients with HD were diagnosed by gene diagnosis. The CAG trinucleotide repeats of the 29 patients with HD outnumbered 40. Polymerase chain reaction-restriction fragment length polymorphism and polyacrylamide gel electrophoresis technique were used to detect the genotypes of delta 2642. Results No B/B genotype was detected in both 2 groups. The genotype frequencies of A/A and A/B in the IT15 delta 2642 polymorphism was 65.5% and 34. 5% in HD patients,92. 1% and 7. 9% in the controls respectively (x2 = 7. 435, P =0. 006). The frequency of the B allele was 17.2% in the HD group and 3. 9% in the control group (P = 0. 010, OR = 5.07, 95% CI 1.47-15.12). Analysis showed no significant difference between A/A genotype patients and A/B genotype patients for CAG trinucleotide repeats(P =0. 188). HD patients with A/B genotype (37.33±6. 46) had an earlier onset than the patients with A/A genotype (47.10± 10. 86, t = 2. 491, P = 0. 019). Conclusions These data demonstrated that variations in IT15 delta 2642 polymorphisms may be a genetic factor that influences the variability in HD age of onset. HD patients with delta 2642 A/B genotype have an earlier onset than the patients with A/A genotype.
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Objective To study the therapeutic effects of low frequency repetitive transcranial magnetic stimulation(rTMS)in cranial dystonia.Methods Twenty cranial dystoina patients were treated with low frequency rTMS.Their motor threshold,cortical silent period(CSP)were evaluated before and after the rTMS and after 1,2,6 months as well as the spares and Toronto Western Spasmodic Torticollis Rating Scale(TWSTRS)to evaluate the effects of rTMS in the treatment of cranial dvstonia.Results The patients scored(23.5±14.0)significantly lower after l and 2 months(17.6 ±14.3,18.5±14.2,t=2.632,2.149.both P<0.05).But there was an increasing tendeney of the score after 2 months.The 2-month efficient rate of low-frequency rTMS Was 60%(12/20),yet the long-term effect of rTMS was still to be studied.There was a very significant improvment of relaxed(46.5%±7.3%vs49.9%±9.2%,t=-3.235.P<0.05)and active threshold(40.2%±5.9%/)5 43.9%±8.8%,t=-2.339,P<0.05),prolongation of CSP((96.1±24.5)ms vs(121.6±27.7)ms,t=-7.223,P=0.000).Conclusion The low frequency rTMS is efficient to relieve the clinical symptoms of cranial dystonia.
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Objective To clarify the possible role of cyclooxygenase-2(COX-2), intercellular adhesion molecule-1(ICAM-1) and E-selectin in the inflammatory injury induced by cerebral ischemia and reperfusion. Methods The focal cerebral ischemia and reperfusion model was induced by a suture occlusion of the right middle cerebral artery. The rats were randomly assigned to four groups: sham operated group; 4 h,22 h and 46 h reperfusion groups. The expression of ICAM-1, E-selectin and COX-2 were detected by immuno-Western blot and immunohistochemical method. Myeloperoxidase (MPO) activity was detected by a commercial MPO kit. Results In the right cortex and striatum of the sham operated group and the 4 h, 22 h and 46 h reperfusion groups, the relative values of COX-2 were 0.7642?0.0763, 1.5382?0.1047, 1.6491?0.3265, 1.8020?0.3719 and 0.7104?0.0891, 2.2061?0.2143, 1.7897?0.3537, 1.8018?0.5703 respectively; the relative values of ICAM-1 were 0.6845?0.0531, 0.9115?0.0422, 0.9426?0.0407, 1.0756?0.0467 and 0.6583?0.0361, 0.9439?0.0746, 0.9975?0.1532, 0.8808?0.0497 respectively. Significant increase of MPO activity and expression levels of COX-2, ICAM-1 and E-selectin were shown from 4 h to 46 h in the striatum and cortex of rats following reperfusion. Immunohistochemical staining showed that the number of ICAM-1 and E-selectin positive vessels was increased in the border of ischemia. The positive cells of COX-2 were almost neurons in the cortex, but in the striatum, the cells were neurons, glias and vessel endothelium. Positive correlation between COX-2 and ICAM-1 expression in the cortex(n=6,r=0.973,P
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@# Objective To assess the clinical efficacy and safety of amantadine monotherapy and concomitant amantadine with salvia miltiorrhiza compound or selegiline of the treatment of Parkinson's disease.Methods The clinical trial was performed in the multicenter, open label study. Amantadine group: 35 cases, amantadine plus salvia miltiorrhiza compound group: 34 cases and amantadine plus selegiline group: 29 cases. The clinical efficacy had been assessed with modified Webster scale (WR) and motor dysfunction rating scale for Parkinson's disease (MDRSPD) with interval of two months for one year. The safety data included blood glucose, hepatic and renal function tests, blood and urine routine tests.Results The clinical improved rates were 42.9% (WR) and 37.1% (MDRSPD) in amantadine group, respectively. The clinical score was improved in 34.2% (WR) and 26.5% (MDRSPD) in amantadine plus salvia miltiorrhiza compound group, respectively. The clinical improvement was 51.1% (WR)and 48.3% (MDRSPD) in amantadine plus selegiline group, respectively. There were no significant differences among these three groups (t-test,P>0.05). The clinical marked efficacy rates in assessment of MDRSPD were 2.8% in amantadine group, 11.8% in amantadine plus salvia miltiorrhiza compound group and 27.6% in amantadine plus selegiline group, respectively. There was significant difference between amantadine group and amantadine plus selegiline group, but no significant difference between amantadine group and amantadine plus salvia miltiorrhiza compound group. The adverse event rates were 27.8% in amantadine group, 8.8% in amantadine plus salvia miltiorrhiza compound group and 31.0% in amantadine plus selegiline group, respectively. All these events were mild, of short duration and resolved without treatment. Conclusion There was some efficacy rate in all three groups. Comparing with amantadine group, there was higher marked efficacy rate in amantadine plus selegiline group.
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AIM: To study the efficacy and safety of dispersible formulation of levodopa-benserazide on the parkinson disease. METHODS: The multicenter, open-label, self-controlled trial was conducted at 23 hospitals in 15 cities. Two hundred and four patients with idiopathic parkinson who had received standard levodopa-benserazide previously participated in this study. Dispersible levodopa-benserazide instead of standard levodopa-benserazide for 8 wk as a course. The Webster rating scale and patient diary were applied to assess the efficacy and safety of dispersible levodopa-benserazide. RESULTS: The medication with dispersible levodopa-benserazide increased “on” time by 47 min, decreased “off” time by 11 min, and speeded the onset of “on” time by 37 min. The Webster score was improved by 25 %. Statistical significant difference was calculated (P<0.01). Slight and few adverse reactions were found. CONCLUSION: Dispersible formulation of levodopa-benserazide is a powerful anti-parkinsonian drug characterized by oral easy use and rapid reach to therapeutic action after ingestion. This drug is particularly used in the parkinsonian patients with morning akinesia, delayed onset of “on” time, afternoon “off” status and dysphagia.
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Objective To detect the mutations in coding region of the guanosine triphosphate cyclohydrolase Ⅰ (GCH1) gene in Chinese patients with dopa-responsive dystonia (DRD).Methods Two families with five affected family members and six patients with sporadic DRD were examined, as well as their eighteen relatives and twenty normal members. Mutation screening was performed using single-strand conformation polymorphism analysis followed by direct sequencing of the presumably mutated exons; in patients whose results showed a normal pattern on single-strand conformation polymorphism analysis, the entire coding region of the GCH1 gene was sequenced. To confirm the mutation, a pair of primers was designed, which produced a restrictive site for SphI.Results DNA sequencing revealed a new heterozygous A224G missense mutation (Tyr75Cys) located within exon 1 in one family with autosomal-dominant inheritance. The mutation was confirmed with restriction enzyme analysis; it was not present in 20 control alleles. Restriction enzyme analysis also detects two systematic gene mutation carriers. In patients from the other family and patients with sporadic DRD, no alterations in the translated portion of the GCH1 gene were observed. Direct sequencing also showed that there existed gene polymorphisms in intron 1 and intron 3 which neared the exon2 and exon3 respectively in Chinese.Conclusions We describe a new missense mutation (Tyr75Cys) in the GCH1 gene. Mutation in the coding region of the gene might be accounted for a part of patients with DRD.
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Objective To investigate the clinical features,diagnosis and treatment of dementia with Lewy bodies(DLB).Methods A clinical, neuropsychological ,neuroimaging and therapeutic analysis on 8 cases of clinically diagnosed dementia with Lewy bodies was conducted.Results Fluctuating cognition(FC) was the first symptom in all 8 patients,followed by persistent visual hallucinations and Parkinsonism. Patients showed moderate dementia and severe constructional disabilities .The ratings of FC were mild to moderate with scores 4-10(mean 7.4?2.1). The UPDRS scores ranged from 31 to 71 (mean 47.8?14.3). The polysomnography of the patient with REM sleep behavior disorder showed augmented submental muscle activity during REM and no EEG epileptiform activity. MRI or CT scans showed global brain atrophy in all 8 patients. SPECT of Sleep patient showed impaired striatal dopaminergic function . L-dopa and donepezil therapy were effective.Conclusions The main clinical features of DLB are fluctuating cognition, persistent visual hallucinations and extrapyramidal motor symptoms. The diagnosis relies on history and current symptoms, neuropsychological testing and findings of neuroimaging. L-dopa and cholinesterase-inhibitor drugs can improve mobility and cognitive symptoms in DLB.
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Objective To investigate the manifestations of myodystonia for recognizing it.Methods Symptomatic myodystonia,relationship between the age of onset and the affected sites,the main clinical types and sex in 292 consecutive cases of myodystonia by retrospective reviewed.Results Symptomatic myodystonia was found accounting for 21.23% in all cases and its major cause was perinatal stage anoxia.Patients who had had early onset (