Objective To explore the clinical and MRI features multiple system atrophy (MSA) and the significance of diagnosing its clinical subtypes.Methods Clinical data and MRI features of 28 patients with MSA were retrospectively analyzed and compared.Results Cerebellar signs (75.0%) were dominant clinic features in olivopontocerebellar atrophy (OPCA). Brain MRI showed atrophy of pontine (91.7%) and cerebellar vermis (91.7%), fourth ventricle dilatation (83.8%) and T2WI high intensity in pontocerebellar region (63.6%). Extrapyramidal signs (80.0%) were found in striatonigral degeneration (SND) with apparent basal ganglia changes such as putamen atrophy (60.0%) and lineal T2WI high intensity of the lateral margin of the putamen (putamen slit) (80.0%). Shy-Drager syndrom (SDS) presented mainly with autonomic nerve system (81.8%), and autonomic nerve system failures appeared earlier and more severe than the other two subtypes of MSA. Conclusions MRI examination may be useful in diagnosis of MSA and its subtypes. Pontine atrophy, T2WI high intensity, especially pontine cross sign may support diagnosis of OPCA. However, putamen atrophy and putamen slit are the specific imaging signs in SND.

Objective To observe the mitochondrion and nucleus ultrastructural damage and caspase-3 expression in hippocampal CA_3 neurons during kainic acid(KA) induced status epilepticus(SE) in rats.Methods SE was induced for 2 h with KA in adult male Wistar rats.3,12 and 24 h later the rats were killed and the hippocampal CA_3 subareas were taken out to make brain sections.The neuronal damage on the whole with light microscope and the ultrastructure of mitochondrion and nucleus with electron microscope.Caspase-3 expression of the same area was examined with immunohistochemical staining.Results 24 h after SE,by the light microscope examination,the KA group showed that the neurons put scattered disorder and nucleus shrink firmly.3 h after SE,electron microscope examination showed swelling cristae and ruptured membrane of mitochondria.The change of nucleus were significant margination of chromatin 24 h after SE.Compared to normal control group,the caspase-3 expression increased 12 h after SE,the average number of positive cell and the gray scale were obviously higher(all(P

Objective To investigate Guillain Barre syndrome (GBS) combined with demyelinating neuropathy in central nervous system(CNS), and explore the possible mechanism and the relationship between the two.Methods 3 cases GBS combined with demyelinating neuropathy in CNS were observed clinically and the datum of laboratory were analysed.Results Case 1, a 28 year old man had symptoms of general flaccid paralysis and coma.The result of blood gas analysis was normal. CSF showed an albuminocytological dissociation, delayed nerve conduction velocity and missed F waves. Brain MRI showed multifocal T 2 Wight Image high signs in white matter of bilateral brain and cervical spinal cord. The patient is getting recovery by treatment with plasma and immunoglobulin. Case 2 , a 5 year old girl with progressive weakness of her limbs and respiratory arrest, appeared confusion,dully light reflex and absent corneal reflex, at last she died because of rejecting treatment.Case 3,a 12 year old boy with progressive weakness of his limbs and the difficult of relieving the bowels.Brain MRI was normal.Spinal MRI showed multifocal T 2 weight imagine hight signs from T 5 to L 4.CSF showed an albuminocytological dissociation.EMG showed a delayed nerve conduction velocity.Conclusion GBS combined with disorders of consciousness are mostly severe, the pathological mechanism is unclear. It is suggested that auto immunoreaction caused by P 1 myelin basic protein can relate to around and CNS demyelination.

Objective To study the risk of recurrence after a first unprovoked seizure and analyze the potential predictors of recurrence. Methods 150 patients with one or more recently unprovoked seizures who attended our hospital from October, 1998 to June, 2000, which included 66 patients having a first unprovoked seizure, were followed up for 2 years. Recurrence rate was estimated by Kaplan-Meier curves. Univariate and multivariate analyses of the potential predictors of recurrence were performed for the first unprovoked seizure patients using the Cox proportional hazards model.Results All the 150 patients had 109 relapses in 2 years, Kaplan-Meier estimate of recurrence rate was 73%(?3.6%), while 66 first unprovoked seizure patients had 36 relapses, with the recurrence rate 54%(?6.1%). Cox Univariate and multivariate analyses showed that symptomatic etiology increased the risk of recurrence, and other predictors of recurrence included abnormal electroencephalogram, the occurrence of seizures during sleep and first seizure lasting longer than 10 minutes, whereas an age of 3 to 12 years decreased this risk.Conclusion The recurrence risk after the first unprovoked seizure is lower than those who have two or more recent seizures. Several factors enable us to predict the recurrence risk after a first unprovoked seizure.

Objective To study the change of glutamate transporter (GluTs) function of hippocampus in seizures rats induced by KA, and explore the role of GluTs in the mechanism of epilepsy.Methods 60 adult male Wistar rats were randomly divided into two groups: KA group(Ⅰ group) and control group(Ⅱ group). Each group was randomly divided into five subgroups according to different time after kindling, including 4 h, 24 h, 48 h, 5 d,and 7 d after injection. GluTs function was studied by means of assay of 3H-L-glutamate uptake in synaptosomes and tissue sections of hippocampus.Results The function of GluTs in synaptosomes was singnificently decreased at any time (all P

Objective To investigate the clinical and electromyographic features of peripheral nerve involvement in MS. Methods The clinical and electromyographic (EMG) data of 29 MS patients were evaluated retrospectively.Results 18 (62.1%) patients showed clinical and/or electromyographic abnormalities of peripheral nerves. The clinical symptoms included extremity numbness in 16 cases (88.9%), limb inertia in 11 cases (61.1%), radicular pain in 5 cases (27.8%), autonomic nerve disorder and dysphagia in few patients. Signs included decrease of tendon reflex in 11 cases (61.1%), periphery or root form hypoesthesia in 9 cases (50%), muscle weakness in 7 cases (38.9%), myatrophy in 4 cases (22.2%), and reduced pharyngeal reflex in 1 case (5.6%). There was no difference in age of onset, course of disease, neurologic impairment and prognosis between the patients accompanied with peripheral neuropathy and the patients without peripheral neuropathy. The electromyographic abnormalities included spontaneous potential in 4 cases (13.8%), increased motor unit potential in 8 cases (27.6%), slow MCV in 15 cases (51.7%), slow SCV in 13 cases (44.8%), low amplitude in 9 cases (31.1%), and prolonged distal latency in 5 cases (17.2%). The neuropathies improved by treatment with corticosteroid in the all patients except one.Conclusions Some MS patients may accompany with peripheral nerve abnormalities, which may improve with the recovery of MS. EMG is useful to evaluale the site and degree of peripheral nerve damage.

BACKGROUND: Status epilepticus can result in neuronal injury.OBJECTIVE: To observe the mitochondrial ultrastructural damage and the changes of Fas, Bax and Caspase-3 expressions in hippocampal CA3 neurons of rats of different kindling, so as to provide theoretical evidence for the neuronal injury after epilepsy.DESIGN: A randomized c ntrol animal experiment.SETTINGS: Department of Neurology and Department of Anesthesiology,Qilu Hospital of Shandong University.MATERIALS: The experiments were carried out in the pathological laboratory of Shandong Academy of Medical Sciences between March and July2005. Totally 150 adult male SD rats of 260-300 g were provided by the experimental animal center of Shandong University (SCXK20030004), they were raised at room temperature and were free to the access of food and water.METHODS: The adult male Sprague Dawley (SD) rats were divided into intraperitoneal injection of kainic acid group and caudal venous injection of kainic acid group respectively ac cording to the method of random number table, and the rats were administrated by kainic acid injected intraperitoneally (12 mg/kg) and via caudal vein (10 mg/kg) respectively. Each group was divided into 5 subgroups, which were 3, 6, 24, 48 and 72 hours after status epilepticus groups respectively. Twelve successfully induced rats were selected from each subgroup, hippocampi were removed at different time points after the termination of status epilepticus, 2 were used for examination under electron microscope, 5 for the reverse transcription-polymerase chain reaction (RT-PCR) detection of Fas and Bax, and 5 for the immunohistochemical assay of Caspase-3. Another 12 rats were used as normal controls without any treatment. The materials were taken at24 hours after corresponding status epilepticus in the control group, and the specific distributions were the same as those in the subgroups. The mitochondrial structure was observed under electron microscope, the levels of Fas and Bax mRNA were detected with semi-quantitative RT-PCR, and the expression of Caspase-3 protein was determined with the immunohistochemical assay.MAIN OUTCOME MEASURES: ① Results of ultrathin section under transmission electron mcroscope; ② RT-PCR results; ③ Immunohistochemical results.RESULTS: Totally 132 rats were involved in the analysis of results. ①Mitochondrial structure under electron microscope: In the intraperitoneal injection group, the mitochondria swelled, and the neurons showed characters of apoptosis. In the caudal venous injection group, the mitochondria swelled, and accompanied by the membranous collapse, and the neurons manifested the necrosis. ② No expression of Fas and Bax was detected in the control group and caudal venous injection. In the intraperitoneal injection group, Fas expression appeared at 6 hours after status epilepticus, increased at 24 hours, reached the peak value at 48 hours, and lasted till 72 hours. ③ The Caspase-3 expressions began to increase 6 hours after status epilepticus in both the intraperitoneal injection group and caudal venous injection group(10.27±0.34, 15.21±0.34; P ＜ 0.001), and reached the peak values at 24 hours (25.36±0.47, 28.23±0.47; P ＜ 0.001); The higher expression of Caspase-3 lasted till 72 hours in the intraperitoneal injection group, but sharply decreased in the caudal venous injection group.CONCLUSION: Two different methods of administration result in different severity of mitochondrial damage and different expressions of Fas, Bax and Caspase-3, which further determines the molecular mechanisms of neuronal death.

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Objective To study the role of calcium homeostatic and kinetics in the epileptogenesis activity. Methods Hippocampal neurons were acutely isolated from controls and status epilepticus (SE) models induced by lithium-pilocarpine at different time point. The [Ca2+]i levels were detected by laser scanning confocal microscope. And the ability to restore resting [Ca2+]i levels after a brief exposure to 5 μmol/L glutamate in control and epileptic neurons were evaluated. Results The [Ca2+]i level of acute separated hippocampal neurons in the control rats was (95.4±22. 1) nmol/L After injection of lithium pilocarpine, the [Ca2+]i level in hippecampal neurons increased dramatically to (867.6±35.2) nmol/L, and decreased to (292.8 ± 18.3) nmol/L on the 7th day, lasting for about 30 days ((220. 8± 17.6) nmol/L), it is higher than that in the control group (t = 12. 55, P < 0.01). The distribution of neuronal [Ca22+]i showed that 92% of control neurons were in the normal range of [Ca2+]i level (25-150 nmol/L) ; After 6 hours, however [Ca2+]i levels of all SE neurons increased, and 85% of which were higher than 500 nmol/L; After 7, 14 and 30 days, there were 75%, 60% and 52% of SE neurons still manifested an elevated [Ca22+]i level, but less than 500 nmol/L. After the exposure to 5 μmol/L glutamate treatment for 2 minutes, [Ca2+]i of the control neurons restored to baseline values in (9. 5±3.4) minutes, whereas the SE rats of acute, latent and chronic phases did not (t = 5.08, 4. 56, 4. 21, all P < 0. 01). Conclusion Lithium-pilocarpine induced epilepsy causes a long-term alteration of calcium homeostatic mechanisms of hippocampus neurons, which may play an important role in the development and maintenance of spontaneous recurrent seizures.