3.Three cases failed to respond to treatment.Two cases had recurrence 7 and 9 months after treatment.The effective rate was 82%. The ICSI score was decreased to 6.1?3.4 at 1 month,6.3?3.5 at 2 months (P

Objective Autosomal dominant pigmentary type of orthochromatic leucodystrophy (POLD) is a rare disease characterized pathologically by demyelination and an appearance of pigmentary glial and scavenger cells in cerebral white matter. We reported a northern Chinese family with autosomal dominant POLD. Methods Brain,skin and muscle biopsies as well as brain postmortem examination were performed in proband patient. Results Proband patient,a 39-year-old woman suffered a progressive motor disturbance,dysarthria and dysphagia,accompanied with respiratory difficulty and incontinence. She died after a disease interval of 2 years. Her father,uncle and two sisters also died of similar symptoms. The onset of disease occurred in between 34～56 years old and had an interval of 1～4 years. CT showed periventrically multifocal hypodensity lesion in all three women. MRI showed multifocal lesions in parietal and frontal white matter and there appeared hypodensity on T 1 weighted scan and hyperdensity on T 2 weighted scan. Diffuse demyelination,disappearance of axons,appearance of macrophages,proliferation of astrocytes and decrease of oligodendrocytes were found in the parietal and frontal white matter. Macrophages and glial cells contained lipopigments,which ultrastructurally consisted of membrane bounded intracytoplasmatic inclusions with fingerprint pattern,curved or straight parallel arrangement. The same lipopigments were also observed in brain biopsy specimens,but not in muscle and skin tissues. Conclusion Clinical and neuropathological findings confirmed that this family should be a case of having autosomal dominant POLD. Because the pathological changes found predominantly in glial cells in white matter and the eosinophilic lipopigments in glial cells presented with morphological features of lysosomes,the POLD should be a glial lysosomal disorder.

Objective To report on an autosomal recessive pyramidal tract, corpus callosum and peripheral nerve degeneration in a family and to study its relationship with other complicated hereditary spastic paraparesis. Methods Neurological examination revealed the following findings. Proband was a 20 year old man who spoke slowly and developed mental retardation in his childhood. Gait disturbance with pyramidal signs and mild cerebellar ataxia were found when the patient was 16. Slight sensory disturbance was present in the lower extremities. His 23 year old sister had similar symptoms at beginning of disease when she was 17. Their clinical courses were bad progressively. Electromyogram showed nerve conduction velocity decrease in the nerve medianus and neurogenic process in the muscle tibialis anterior. Cranial MRI, muscle and nerve suralis biopsies were examined in proband patients. Results MRI showed thin corpus callosum with cerebral and cerebellar atrophy as well as enlargement of ventricle system. Myopathological findings were characterized by angular atrophy fibers in small groups with appearance of hypertrophy fibers. The nerve suralis biopsy showed degeneration and regeneration of myelinated axons. Conclusion Our study confirms that this family is hereditary spastic paraparesis with mental retardation, thin corpus callosum and polyneuropathy reported mostly in Japan. Axonal polyneuropathy is a common pathological feature of this disease.

0 05) The overall incidence of adverse effects was between 12 9% and 28 8% There were no significant differences between these two groups Rivastigmine had no influence on vital signs and laboratory indexes Conclusion Rivastigmine may significantly improve the symptoms of the patient with AD and have a good safety and tolerability,being an ideal choice in treating AD

Objective To study the clinical features and pathological changes of muscles and peripheral nerves in Sjogren's syndrome cases accompanied with neuromuscular complications. Methods The muscle and peripheral nerve biopsies of 7 cases were performed and the specimens were tested by using histochemical and electron microscopic methods. Results The results revealed that myelinated fiber number in peripheral nerves was severely decreased in 2 cases, and the manifestations of atrophy, necrosis, vasculitis and mononuclear cell inflitration could be found in the muscle tissues in 5 cases. Conclusions The neurologic complications of Sjogren's syndrome appear commonly and early. The muscle and peripheral nerve biopsies indicate that complications and its degree,in patients with Sjogren's syndrome should be early diagnosed and treated.

Objective To study the genetic origin of mitochondrial dysfunction in patients with mitochondrial myopathy.Methods The esphagus carcinoma cells were cultured by ethidium bromide, and established stabile,cell line of long term survival mitochondrial DNA(mtDNA).The platelets of patients with mitochondrial myopathy and the normal controls were carried out cell fusion.the mitochondrial function of fusion cell was determined. Results The esphagus carcinoma cells were cultured by ethidium bromide for 12 days, the cells were completely depleted of mtDNA,which can be passed stably. The respiratory capacity of transformants derived from patients with mitochondrial myopathy was lower than those from the control.Conclusion The mtDNA mutation can play a role in the pathogenesis of mitochondrial myopathy.

Objective To investigate the early and long-term effects of enhanced ischemia/reperfusion injury on the transplanted abdominal aorta. Methods Abdominal aorta grafts from Sprague-Dawley (SD) rats were cold stored for 1 or 24 hours, and they were orthotopically transplanted to SD or Wistar recipients. The pathohistological changes and the expression of TGF-? 1 in the grafts were observed. The levels of serum lipid peroxides before and after transplantation were also measured. Results The intima was significantly thicker in aorta transplants which was cold stored, both in SD→SD and SD→Wistar groups 10 weeks and 6 weeks after transplantation, whereas grafts which were cold stored for 24 hours showed pronounced thickening as early as 2 weeks after transplation. Serum lipid peroxides levels were elevated significantly 2 hours post-transplantation in all groups, but they were lowered 4 and 24 hours post-transplantation. The expression of TGF-? 1 in 24 hours of ischemia became stronger 1 week after transplantation regardless the difference in strains of the animal. Conclusions The enhanced ischemia/reperfusion injury can aggravate the infiltration of inflammatory cells, intensify the expression of TGF-? 1, accelerate the thickening of intimal layer.

目的分析影响急性脑卒中就诊时间的相关因素。方法对326例急性脑卒中患者或家属进行问卷调查,用多变量对数回归分析模型统计不同变量和就诊时间的独立关系。结果发病6小时内就诊的患者占51.5%。就诊延迟主要与病情严重,患者对脑卒中症状的认识缺乏有关。年龄、首诊医院亦与就诊时间有关。结论对公众进行卒中症状及早期就诊重要性的教育是必要的。

AIM and METHOD:To determine the production of nitric oxide(NO) and change of NO synthase(NOS) activity in mitochondria isolated from the rat brains of the ischemia/reperfusion rat model produced by transient occlusion of middle cerebral artery on the following the points: 2 h after occlusion of artery and 30 min, 2h, 4h after reperfusion. RESULTS: After the occlusion of middle cerebral artery, the respiratory control rate(RCR) of mitochondria significantly decreased and slightly increased at 4h after reperfusion. Meantime, the production of NO in mitochondria increased significantly. But with the increase of perfusion, production of NO gradually decreased and reached normal level as in the control group. It also shows that cerebral ischemia increased NOS's activity significantly in the mitochondria and still kept a higher level than the control group although it decreased gradually after reperfusion. But the iNOS's activity did not show obvious change. The change of total NOS's activity depends on the change of cNOS's activity. CONCLUSION: The activation of NO/NOS system in the mitochondria might play an important role in the reperfusion injury during reperfusion of ischemic brain.

The etiological factor, diagnosis, as well as therapeutic results of 23 cases with urinary fistula following renal transplantation, at the Chengdu Military General Hospital, from December 1998 to December 2008, were analyzed retrospectively, including 21 cases with a renal transplantation, 2 cases with retransplantation; 9 cases adopt renal artery, renal veins to external lilac artery, external lilac vein anastomosis, 14 cases with renal artery to internal lilac artery, renal veins to external lilac vein anastomosis. 23 cases were followed-up for 6-12 months, 17 cases suffered urinary fistula at days 3-7 after transplantation, 6 cases occurred at days 7-10; there were 17 stoma fistulae, 4 distal end necrosis of ureter, 2 ureteral fistulae. 11 cases were received conservative treatment, and 12 cases with operation. Among the surgery patients, 9 cases received conventional operation and 1 of them returned with urinary fistula and then was cured by second operations; 3 patients received pedicled omentum transplantation and no recurrence or hydronephrosis happened with normal renal function. The one-time success rate was 92% (11/12), of which the repair success rate using pedicled omental was 100%. The results demonstrated that prevention plays an important role in urinary fistula, and ureter should be protected during the surgery. Meanwhile, stoma fistula should be avoided. Promptly treatment following urinary fistula is also necessary to reduce the damage of urinary fistula to the renal function.