Objective To observe the possibility and efficacy tolerability of topiramate(TPM) in transferring monotherapy for the patients wi th partial seizures with or without secondary general seizure,as well as to find out the optimum way of the transferring.Methods After T PM add-on therapy 64 patients with seizures reduced ≥50%, it reduced about 1/ 4 of AEDs or one drug each time according to every two weeks until gradually to stop fully and transferring monotherapy.Results 18 patients we re successful with TPM monotherapy (28.13%), 35 patients were half success(54.69 %), 11 cases for failure (17.19%). The less AEDs combined, the higher possibilit y of transferring monotherapy would be. The rate of success for cabamazepine see med higher than valproate.Conclusion It was possible to transfe r monotherapy by TPM. The rate of success was 28.13%. It was easier to reduce ca bamazepine than valproate. In the transferring monotherapy it was better to redu ce one after another, in principle the one of the little dose was reduced first, and then the hepatic enzyme inducing agents was reduced. Even if the transferrin g was half success,the kinds of concomitant AEDs could be reduced at least, it w as in line with the policy of medicine application.

Objective To explore the dynamic alteration of intracellular free calcium concentration([ Ca2+]i),ATP level and membrane Ca2+-Mg2+ATPase acti vity of erythrocyte in the patients with acute cerebral infarction(CI).Methods we examined [Ca2+]i,ATP level and membrane Ca2+-Mg2+ATPase activity of erythrocyte in 30 patients with acute CI and 28 health controls by Fluorescence Activated Cell Sorter. Results [Ca2+]i in erythrocyte increased significantly in CI group(P<0.01),while the ATP level and membrane Ca2+-Mg 2+ATPase activity were lower than the controls(P<0.05,P<0.0 01).The above result was more remarkable in the elderly group than the young one .The dynamic alteration of [Ca2+]i in erythrocyte increased obv iously during 1～2 days after the attack,and reached the peak in 3～7 day s,it decreased slowly to the slightly low level at the beginning of th e attack in about two weeks,but it was still higher than the controls.The dynamic alteration of ATP level and Ca2+-Mg2+ATPase activit y after acute CI,it decreased significantly during 1～2 days after the at tack,and reached the lowest in 3～4 days.this status could last about one week.Then both of them increased slightly. There was remarkable negative correlation betwe en RBC [Ca2+]i and ATP level or membrance Ca2+-Mg2+ A TPase activity (r=-0.904,r=-0.978,P<0.05).There was positive correl ation between ATP lev el and membrane Ca2+-Mg2+ATPase activity(r=0.835,P<0.05 ).Conclusion There was calcium overload [Ca2+]i in th e intracellular of erythrocyte in acute CI,ATP level and membrane Ca2+ -Mg2+ATPase activity of erythrocyte CI was involved in the pathologi cal course of calcium overload,and related to the age.

Objective To explore the relationship between the protein expression of PCNA and biological behavior science in benignant pituita ry adenoma.Methods The protein expression of PCNA in 58 patien ts with pituitary adenoma were determined by ABC immunohistochemical method.Results The PCNA index was significantly higher in the patients with recurrent pituitary adenomas than in nonrecurrent ones(P<0.05).There was no significantly difference between bleeding and unbleeding group,cystic and noncystic group,large type and unlarge type group(P>0.05 respectively) .Conclusion The protein expressions of PCNA reflected the proli ferative activties of pituitary adenomas, and could be taken as one of the indic ators to evaluate recurrence and prognosis of the tumor.

Objective　To investigate the incidence， clinical characters and pathogenesis of hyponatremia following acute stroke in the elderly patients.Methods　The clinical,serum natrium and CT data of 41 cases with hyponatremia following acute stroke in the elderly patients were retrospectively analysed.Results The incidence of hyponatremia following acute stroke in the elderly patients was 16.02% (taking hemorrhagic stroke),the occurrence of the patients' coma induced by hyponatremia and the fatality rate were higher than the controls (P＜0.05);Hyponatremia correlated closely with the localization of lesions in CT, the focus in brain ganglion (induding thalamus) and hematocele in the ventricles of brain caused easily hyponatremia.Conclusion　Dysfunction of hypothalamus-pituitarium was one of the risk factors in causing hyponatremia following acute stoke in the elderly patients.

Objective　To study the relationship between the changes of cytosine，thymine，guanine（CTG）repeat numbers and brain stem BAEP,SEP and VEP in patients with myotonic dystrophy（DM） and their family members.Methods　The repeat numbers of CTG，BAEP， SEP and VEP of DM gene were determined by PCR amplification and DNA hybridization in 5 patients with DM and 16 members from 3 families.Results　The repeat numbers of CTG in 10 normal persons were 30，BAEP，SEP and VEP were normal.The repeat numbers of CTG in 5 patients with DM were over 85，two cases of them were over 1605，they were significantly higher than the normal persons.4 cases in 16 family members were normal,CTG repeat numbers of 12 cases were over normal genes,CTG repeat numbers were related to clinical symptom, BAEP，SEP and VEP.Conclusion　The gene diagnosis of DM was found to be consistent with its clinical symptom，the changes of BAEP，SEP and VEP.

Objective　To study the plasticity of hippocampal formation in epilepsy.Methods　The optical density (OD)of synaptophysin positive immunoreactive product was examined by image pattern analysis instrument in hippocampus of pentylenetetrazol induced kindling epileptic rats. The examined areas included CA1,CA3 and the dentate gyrus.Results　The OD of synaptophysin positive immunoreactive product in hippocampal formation of kindling group was higher than the controls,especially in the mossy fiber layer of the area CA3 and the inner molecular layer in the dentate gyrus. Conclusion　The change of synaptophysin resulted from kindling, it also could result in the molecular elements of kindling maintenance.

Objective　To study the clinic, neuro-electrophysiology and molecular biology of Machado-Joseph disease (MJD).Methods　Family visiting, physical examination and the blood samples were analysed on molecular biology in 44 members of a family with MJD.The cases of inpatients were examined on cerebrospinal fluid and neuro-electrophysiology.Results　10 patients of the family attacked,which were consisted with autosomal dominant inheritance type. Age of the onset was 8～38 years old. The clinical characteristic was progressive severe spinocerebellar of ataxia,faciolingual myokymia,bulging eyes.Change of denervated muscle was revealed by neuro-etectrophysiological examination. Light atrophy was observed in cerebellar,brain stem, spinal cord.The genetic defect of MJD was located the long arm of chromosome 14 between D14S280 and D14S81, their distance was 3.0 cm.All tested patients had their CAG repeated expansion from 72 to 84 in the MJD gene.Conclusion　MJD is a neuro-degenerative disorder of autosomal dominant inheritance. The disease was clinically characterized by progressive severe spinocerebellar ataxia, no obvious changes of cerebrospinal fluid,neuro-electrophysiology, CT and MRI.The genetic defect of MJD was located the long arm of chromosome 14.The number of CAG repeated expansion mutation was associated with the age of the onset.

Objective To explore the clinical characteristics and molecular diagnosis of 4H syndrome.Methods The clinical data of 1 patient with 4H syndrome diagnosed by gene was retrospectively analyzed , and the clinical characteristics were analyzed combined with the literature .Results This child patient was male , 6 years and 8 months old, with hands shake for 1 years, mental and movement development backwardness , walking instability, teething delay .Ophthalmic examination showed myopia and optic atrophy .Brain MRI suggested a wide range of cerebral white matter lesions on both sides of the cerebral hemisphere .Gene examination showed POLR3A compound heterozygous mutation (c.1781T>G,c.2693delT).He was diagnosed as 4H syndrome.Conclusions The early manifestations of 4H syndrome are mental and movement development backwardness and teething delay .The main clinical features of 4H syndrome are leukodystrophy, myopia and ataxia.The genetic characteristics are POLR3A or POLR3B mutation.

Objective To investigate the relationship among pulsatility index ( PI) , sustained attention function and collateral circulation in patients with severe stenosis or occlusion of internal carotid artery .Methods One hundred and thirty-five patients with severe stenosis or occlusion of internal carotid artery were examined by DSA and were divided into different groups according to the type of collateral circulation .Meanwhile, 30 healthy aged people were selected as a control group .The peak systolic velocity ( Vs) , and diastolic velocity ( Vd) , mean velocity ( Vm) and PI of bilateral middle cerebral artery were obtained by TCD .Sustained attention was evaluated by missed and mistaken scores as well as average reaction time of continuous performance test .The results were compared and analyzed.Results (1)According to the type of collateral circulation , 135 patients with severe stenosis or occlusion of internal carotid artery were divided into groups with patency of anterior communicating artery (AcoA)(31 cases),posterior communicating artery (PcoA)(20 cases), AcoA+PcoA (19 cases), primary collateral circulation (with AcoA, PcoA and AcoA +PcoA) (70 cases), secondary collateral circulation (including ophthalmic artery, leptomeningeal collateral vessel and new blood vessels ) ( 15 cases ) , primary+secondary collateral circulation ( 13 cases) and group without collateral circulation (37 cases).(2)Compared with control group, Vs,Vd,Vm and PI all decreased significantly in every other group ( all P<0.05 ) .Compared with group without collateral circulation , Vd of group with AcoA or primary collateral circulation , Vd and Vm of group with AcoA +PcoA all increased significantly;PI of groups with AcoA , AcoA+PcoA, primary collateral circulation and primary +secondary collateral circulation all decreased (all P<0.05).Compared with group with PcoA, PI of groups with AcoA and AcoA +PcoA both decresed significantly (all P<0.05).Compared with group with secondary collateral circulation , Vs,Vd and Vm of group with primary collateral circulation , Vd and Vm of group with primary +secondary collateral circulation all increased significantly; PI of groups with primary collateral circulation and primary +secondary collateral circulation both decreased (all P<0.05).(3) Compared with control group, the missed, mistaken scores and average reaction time of CPT in every other group increased significantly ( all P<0.05 ) .Compared with group without collateral circulation , the missed, mistaken scores and average reaction time in groups with AcoA , AcoA+PcoA and primary collateral circulation all decreased significantly; the missed and mistaken scores of group with primary+secondary collateral circulation decreased significanly ( all P<0.05) .Compared with group with PcoA , the missed and mistaken scores of group with AcoA decreased significantly while the missed , mistaken scores and average reaction time of group with AcoA +PcoA all decreased significantly ( all P<0.05 ) .Compared with group with AcoA+PcoA, the missed, mistaken scores and average reaction time in group with AcoA increased sinificantly ( all P<0.05).Compared with group with secondary collateral circulation , the missed, mistaken scores and average reaction time in group with primary collateral circulation all decreased significantly ;the missed and mistaken scores in primary+secondary collateral circulation both decreased significantly ( all P<0.05 ) .( 4 ) PI positively related to average reaction time in groups with AcoA , primary and secondary collateral circulation ( r=0.441, r=0.364, r=0.552; all P<0.05 ) .PI positively related to missed scores in group with PcoA and group without collateral circulation (r=0.668, r=0.397;all P<0.05).PI also positively related to mistaken scores in the above groups (r=0.509,r=0.480;all P<0.05).Conclusion High PI on the affected side of patients with severe stenosis or occlusion of internal carotid artery may reflect impairment of sustained attention function .

Objective To explore the clinical characteristics and molecular diagnosis of 4H syndrome.Methods The clinical data of 1 patient with 4H syndrome diagnosed by gene was retrospectively analyzed , and the clinical characteristics were analyzed combined with the literature .Results This child patient was male , 6 years and 8 months old, with hands shake for 1 years, mental and movement development backwardness , walking instability, teething delay .Ophthalmic examination showed myopia and optic atrophy .Brain MRI suggested a wide range of cerebral white matter lesions on both sides of the cerebral hemisphere .Gene examination showed POLR3A compound heterozygous mutation (c.1781T>G,c.2693delT).He was diagnosed as 4H syndrome.Conclusions The early manifestations of 4H syndrome are mental and movement development backwardness and teething delay .The main clinical features of 4H syndrome are leukodystrophy, myopia and ataxia.The genetic characteristics are POLR3A or POLR3B mutation.