Objective To explore the characteristics of hereditary spastic paraplegia with thin corpus callosum (HSP-TCC). Methods Clinical, electrophysiological, MRI features of 3 patients with HSP-TCC were reported. The genetic characteristics were reviewed. Results 3 patients revealed difficulty in walking, slowly progressive weakness, spasticity of the lower limbs and mental impairment. The electromyogram in 2 cases showed neurogenic damage in lower limbs muscle, and 1 case showed peripheral nerve damage. Cerebral MRI showed an extremely thin corpus callosum on sagittal image. The locus of 15q13-15 has been identified for HSP-TCC, but some HSP-TCC families have not been linked to this locus.Conclusion HSP-TCC is a common subtype of complicated HSP inherited by autosomal recessive mode. Brain MRI showed extremely thin corpus callosum. Electromyogram many reveal neurogenic damage.

Objective To study the characteristics of peripheral nerve conduction (PNC) in advanced-stage amyotrophic lateral sclerosis (ALS) patients undergoing mechanical ventilation.Methods The sensory conduction velocity (SCV),motor conduction velocity(MCV),distal motor latency(DML) and amplitude of compound muscle action potential(CMAP) were determined in three ALS patients undergoing mechanical ventilation.The clinical data and PNC characteristics of the patients were analyzed.Results The 12 motor nerves were abnormal in all the three patients,including absence of responsive wave (66.7%,8/12),decreased amplitude of CMAP(33.3%,4 /12),prolongation of DML(16.7%,2 /12) and reduction of MCV(16.7%,2 /12).The CMAP amplitude of patients gradually decreased with the progression of disease,and finally led to adverse reactions.The SCV and amplitude of sensory nerve action potential were normal in all the three patients.Conclusion ALS patients undergoing mechanical ventilation may have severely damaged motor nerve conduction,and their sensory nerve is generally not affected,with the specific reason remains to be further studied.

Objective To elucidate the clinical, electrophysiological, neuropathological features of two cases of hereditary neuropathy with liability to pressure palsies (HNPP) in one pedigree, and to review the literature of HNPP, so as to promote the understanding and diagnostic acuity of the disease. Methods Detailed electromyogram, motor and sensory conduction velocity, and distal motor latency were measured for clinically affected and unaffected nerves in the two patients. Sural nerve biopsy was performed for case one and the specimen was observed under light microscope and elcctronmicroscope. The cases reported in China up to the present were collected. Results Case one was an 18 year old male with a 9 year history of recurrent weakness and numbness of limbs precipitated by compression or stretch. Case two was his father. Although he had not experienced clinical episode of limb weakness and numbness, physiological examination revealed signs of peripheral neuropathy. Eletrophysiological study demonstrated diffuse peripheral nerve damage with decreased nerve conduction velocity, delayed distal motor latency, especially a decrease in motor conduction velocity at common entrapment sites, including clinical unaffected nerves. Sural nerve biopsy showed that myelin sheath of most myelinated fibers with normal axons was thickened. Some thickened myelin sheath was seen to invaginate into the axon. No onion bulb was found and unmyelinated fibers were relatively normal. Only 9 cases of HNPP were reported in China, but no DNA analysis was performed for any of them. Conclusions HNPP is a rare disease with autosomal dominant inheritance. Nerve conduction study is an important diagnostic method for screening. Its definite diagnosis relies on the typical pathological findings in nerve biopsy specimen. Sural nerve biopsy could be avoided for diagnosis if the family history were positive and nerve conduction study should show diffuse peripheral nerve damage

Objective To explore the risk factors of hospital infection for hypertensive intracerebral hemorrhage ( HIGH). Methods 408 patients from Department of Neurology whose length of stay equaled or exceeded 72 hours and who fitted in with the criterion for the diagnosis of hospital infection were chosen as the HICH infection group while HICH patients without hospital served as the control group. The risk factors for HICH were studied by means of both single factor analysis and multifactor analysis. Results Of more than 30 possible risk factors that were studied by means of single factors analysis, more than 20 manifested marked difference (P < 0.05). Uncondition Logistic regression analysis of the more than 20 variables indicated that rise of inhalation of food resulting from indwelling, stomach tube,kinds of antibiotics used,spray inhalation and fasting blood-glucose,accidental were the risk factors of HICH infection. Conclusion HICH infection results from the synergetic action of various factors and hospital infection could be controlled and reduced by strengthening supervision.

Objective To summarize the clinical, electrophysiological, pathological characteristics of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Methods From Jan 1990 to Dec 2003, 32 patients of CIDP were admitted by our hospital, 14 male and 18 female. The age range of onset of illness was 13 to 74 years old (averaging 42.56) with the prime period of 40 to 50. Before hospitalization, the course was from 2 months to 5 years. Among all patients, there were 8 cases of a relapsing course and 24 cases of a chronic progressive course. Twenty-four patients were treated with corticosteroids, while 7 received immuglobin (IG) and corticosteroids. The clinical data before and after the treatment was studied retrospectively. Results Most of the patients had subacute or chronic onsets. The common initial symptoms are numbness, paresthesia, and extremity weakness. Diplopia, decreased visual acuity, dysarthria, and dysphagia could also be found initially. It was usually a symmetric sensorimotor neuropathy with either a relapsing course or a chronic progressive course. It could be accompanied with autonomic dysfunction and cranial nerve involvement. Electromyogram demonstrated that the motive and sensory nerve conduction velocities were slow. The sural nerve biopsy showed demyelination and remyelination. `IG and corticosteroids were both effective. Conclusion CIDP might result in widespread peripheral nerve damages, in which autonomic dysfunction and cranial nerve involvement were common. The dominant electrophysiological changes showed peripheral nerve demyelination accompanied by axon degeneration. The sural nerve biopsy played an important role of diagnosis. The treatment with IG and corticosteroids was a most effective way for CIDP nowadays.

Objective To investigate the serum creatinine (SCr) level in patients with sporadic amyotrophic lateral sclerosis (sALS) and to explore the relationship between the SCr level and the clinical characteristics.Methods A total of 80 patients with sALS,80 patients with multiple system atrophy (MSA) and 80 patients with tension-type headache (TTH) were enrolled in the study.The SCr levels were compared among the three groups.The association between the SCr level and the revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R),the forced vital capacity (FVC) percentage of predicted (FVC％ pred),the site of symptom onset,the duration of disease and the rate of disease progression was evaluated in the sALS group.Results The SCr level was significantly decreased in the sALS group than the other two groups [(60.86 ± 16.80) μmol/L vs (70.05 ± 12.79) μmol/L and (66.97-± 14.14) μmol/L,P ＜ 0.01].In the sALS group,the SCr level was positively correlated with the ALSFRS-R (r =0.315,P =0.005),while no correlation was found between the SCr level and the FVC％ pred,the site of symptom onset,the duration of disease and the rate of disease progression (all P ＞ 0.05).Conclusion The SCr level is an important biochemical index in the patients with sALS and might play an important role in monitoring the disease progression.

AIM: To study the protective effects of the extract of Ginkgo biloba (EGB) on brain tissues in experimental diabetic rats and explore its possible mechanisms. METHODS: Thirty male Sprague-Dauley rats were divided into three groups: normal control group, diabetic group and EGB-treated group. Strepozotocin were injected intraperitoneally on the later two groups to induce diabetes, EGB-treated group was injected intraperitoneally with EGB, and the others were treated with normal saline at the same volume. After five weeks, the content of endothelin (ET), malondial dehyde (MDA), NO2~-/NO 3~-, and the activity of superoxide dismutase (SOD), nitric oxide synthase (NOS) were determined in brain homogenate, and the level of blood glucose, insulin and ET were measured respectively. In addition, the morphologic changes of the brain tissue were studied by light microscopy and electron microscopy, respectively. RESULTS: In diabetic group, there were degeneration of neuron, brain edema, softened focus and demyelination in the white matter of brain by light microcopy. There were expansion of mitochondria of neuron and gliocyte, the shortened of crista, demyelination of neurofiber and injury of blood-brain-barrier by the electron microscopy. After treated with EGB, the pathological changes decreased in brain under light microcopy and electron microcopy. Compared with diabetic rats, the activity of SOD and the level of serum insulin increased, while the level of blood ET, the activity of NOS, the content of ET, MDA, NO2~-/NO3~- decreased in EGB-treated rats (P

AIM: To investigate the protective effect of Ginkgo biloba extract (GBE) on diabetic testis and explore its possible mechanism. METHODS: Testicular structure of streptozotocin-induced diabetic rats was observed under light microscopy (LM) and transmission electron microscopy (TEM). Content of malondialdehyde (MDA), NO_2~-/NO_3~- and activity of superoxide dismutase (SOD), nitric oxide synthase (NOS) were determined in testicular homogenate. RESULTS: In diabetic rats, it was manifestated as deformation of seminiferous tubule, atrophy and shedding of germinal epithelium under LM, while expansion of smooth endoplasmic reticulum, formation of fatty vacuoles and decrease of lysosome obviously in the cytoplasm of sertoli cell under TEM, the injury of testicular tissue was improved by GBE. Compared with diabetic rats, activity of SOD increased while activity of tNOS and iNOS, content of MDA and NO_2~-/NO_3~- decreased in GBE-treated rats. CONCLUSION: GBE could effectively prevent the development of diabetic testis and the effect may be partly achieved by resisting lipid peroxidation,restraining the activity of testicular tissue iNOS and reducing the pathological alterations of NO. [

Objective To investigate the clinical features,prognosis and its influencing factors of cerebral venous sinus thrombosis (CVST).Methods The clinical data of 163 patients with CVST were analyzed retrospectively.The outcome was assessed with the modified Rankin Scale (mRS).The influencing factors on prognosis were analyzed by logistic regression model.Results Among the 163 CVST cases,headache was found in 140 cases,motor and (or) sensory deficits in 16 cases,coma in 6 cases,serious intracranial hypertension (＞ 350 mm H2O,1 mm H2O＝0.0098 kPa) in 68 cases,lateral sinus thrombosis in 129 cases,straight sinus thrombosis in 11 cases and intracranial hemorrhage in 21 cases.Follow-up data was obtained by telephone in 150 cases (92％).The mRS scores were 0-1 in 89 cases,2 in 13 cases,3-5 in 44 cases,and 6 in 4 cases respectively.Poor outcome (mRS score ＞ 2) was found in 29.4％(48/163) patients.The total mortality rate was 2.7％ (4/150).Univariate analysis identified factors associated with poor outcome were motor and (or) sensory deficits,straight sinus thrombosis,serious intracranial hypertension (＞ 350 mm H2O) and intracranial hemorrhage.In logistic regression analysis,serious intracranial hypertension(＞ 350 mm H2O; OR =0.169,95％ CI 0.053-0.541,P =0.003) and intracranial hemorrhage (OR =0.075,95％ CI 0.018-0.311,P =0.000) were identified as independent predictors of poor prognosis.Conclusions The clinical manifestations of CVST are complicated and nonspecific.It is still a disease that may lead to death or disability.Serious intracranial hypertension(＞ 350mm H2O) and intracranial hemorrhage were independent predictors of poor prognosis.

AIM: To study effect of extract of ginkgo biloba(EGb) on the lipid metabolism and the function of macrophages from diabetic rats.METHODS: Sprague-Dauley rats were divided into four groups: normal control group,high-fat group,diabetic group and EGb treatment group.At the end of experiment,the rats were sacrificed,the blood glucose,blood insulin and serum lipid were measured.The activity of superoxide dismutase(SOD),content of malondialdehyde(MDA),nitric oxide(NO) in alveolar macrophages(AM) and peritoneal macrophages(PM) were assayed.In addition,peroxisome proliferator activated receptor ?(PPAR?),CD36 mRNA expression in AM was measured by RT-PCR.RESULTS: The concentration of the blood glucose,blood insulin and total cholesterol(TC),total triglycerides(TG),low-density lipoprotein-cholesterol(LDL-C) in blood increased significantly in type 2 diabetic group.The supplement of EGb decreased blood glucose,blood insulin and TC,TG,LDL-C levels.The activity of SOD decreased,while the content of NO,MDA increased in the diabetic macrophages,the activity of SOD became increased,but the content of NO and MDA decreased in EGb-treated group.The mRNA expression level of CD36 and PPAR? in alveolar macrophages from diabetic group increased,while expression level of CD36 and PPAR? mRNA in EGb treated rats continued to rise.CONCLUSIONS: EGb corrected insulin resistance and ameliorated disturbance of lipid metabolism caused by type2 diabetes in rats.Adjustment of PPAR? and CD36 mRNA expression of as well as reduction of lipid peroxidation and NO level may be involved in this process.