Cerebral autosomal recessive arteriopathy with subcortical infarcts and leukoencephalopathy (CARASIL) is an autosomal recessive hereditary cerebral artery disease in the adolescence,and its main clinical manifestations are dementia,stroke,low back pain and alopecia.At present,most cases are from Japan.3-6 exon point mutations in the HTRA1 gene are associated with the onset of CARASIL.Brain histopathological examination showed a small arterial intimal thickening medial smooth muscle cell loss and hyalinization.Brain MRI showed a diffuse white matter abnormal signal and multiple subcortical infarcts.The diagnosis mainly depends on the characteristic clinical symptoms,imaging characteristics and genetic testing.It should be differentiated from cerebral autosomal dominant arteriopathy with sulcortical infarcts and leucoencephalopathy.

Rho/Rho-kinase signaling pathway is a ubiquitous one in vivo.It plays a role of "molecular switch"by regulating the polymeric state of intracellular actin cytoskeleton,and participates in the regulation of various cellular functions.Rho/Rho-kinase signaling pathway plays an important role in the risk factors.pathogenesis and pathophysiological processes of ischemic cerebrovascular disease.The inhibition of Rho/Rho-kinase signaling pathway may achieve a significant neuroprotective effect.

Intercellular adhesion molecule-1 (ICAM-1) is one of the major molecules in mediating the adhesion between leucocytes and endothelial cells. The article mainly elaborates the expression of ICAM-1 and its effect during cerebral ischemia; it also discusses the interaction between ICAM-1 and other inflammatory cytokines. Anti-ICAM-1 treatment is expected to become one of effective methods in the management of cerebral ischemic injury.

Leukoaraiosis is caused by a variety of diseases. It presents diffuse low density areas in periventricular and subcortical areas (centrum semiovale) on CT or presents diffuse high signals on T2-weighted imaging. Demyelination of white matter fibers caused by arteriopathy is one of the major causes of leukoaraiosis. This article reviews the clinical manifestation, imaging and pathological features of leukoaraiosis, as well as its pathogenesis.

Objective To investigate the relationship between the plasma inflammatory cytokines and carotid atherosclerosis in patients with ischemic cerebrovascular disease(ICVD).Methods The levels of plasma interleukin (IL)-6,matrix metalloproteinase (MMP)-8 and soluble CD40 ligand (sCD40L) in 64 patients with ICVD were detected by enzyme-linked immunosorbent assay (ELISA),the severity of bilateral carotid atherosclerosis was measured by colour ultrasound.The results were compared with those of non ICVD controls. The association of the levels of plasma IL-6,MMP-8,sCD40L and the severity of carotid atherosclerosis were analysized. Results The levels of plasma IL-6,MMP-8,and sCD40L in patients with ICVD were significantly higher than those in control group(all P

Objective To explore the clinical and pathological features of leprous neuritis.Methods Sural nerve biopsied specimens from one patient with leprous neuritis were analyzed with histopathology and electron microscopy.Results The main clinical characteristics included injuries of sensory nerves with numbness and pain in the distal limbs, vegetative nerve functional disturbance, and the asymmetrical bulky ulnar nerve. The pathological changes were the presence of lymphocytic infiltrates in the endoneurial and epineurial spaces, a mild stenosis of small vessel with a large number of perivascular lymphocytic and mononuclear phagocytic infiltrates combined with epithelioid cells form. Anti-acid staining demonstrated the positive leprosy bacillus in nerve fibers. A nearly completely loss of myelinated fibers and obvious connective tissue proliferation were also seen in the sural nerve. The electron microscopy observed marked axonal degeneration of unmyelinated nerve fibers with many leprosy bacillus in Schwann' cells. This case was treated with Aminophenylsulfone and Dehydrocortisone for one year and his condition had improved.Conclusions Diagnosis of leprous neuritis depends on the nerve biopsy, and the early treatment may relieve disability.

Objective To explore the relationship between the apoptosis of cerebellar granule cell and the changes of caspases-3 and Bcl-2 in rats with methylmercury intoxication.Methods We chronologically observed the pathological changes of cerebellum in rats given methylmercury[4 mg/(kg?d)] at day 11, 15, 18 and 19. The apoptosis of cerebellar granule cell was detected by terminal deoxynucleotidyl transferase-mediated dUTP nick-end-labeling (TUNEL) method, and the relationship between the apoptosis and the expression of caspases-3 and Bcl-2 was evaluated.Results At day 18, some sparse TUNEL positive granular cells could be observed, mainly in deep lamina adjacent to the white matter. At day 19, the apoptotic cells markedly increased, while the number of granular cells decreased with a well preservation of Purkinje cells demonstrated by calvindine immunostaining. MRF-1 and glial fibrillary acidic protein(GFAP) dyeing demonstrated severe microglia reaction and astrocytosis. From day 11, caspasese-3 mRNA expression up-regulated, increased gradually and reached peak at day 18, then decreased markedly at day 19. In contrast, Bcl-2 mRNA expression down-regulated from day 11, and decreased obviously at day 19. Zic-1 as a marker of granule cell decreased gradually after methylmercury administration and indicated degeneration of granule cells.Conclusion Cerebellar granular cell degeneration induced by methylmercury is related to apoptosis mediated by caspases-3 and Bcl-2 down regulated.

Objective To investigate the clinicopathological features and the pathogenesis of rhabdomyolysis caused by exertional heat stroke.Methods Eight patients including 6 military soldiers and 2 physical exercisers trained under high temperature environment were enrolled into this study.Their clinical manifestations were compatible with the diagnostic standard of exertional heat stroke.Consecutive blood biochemistry tests were performed in all patients,and electromyography and muscle biopsy in 3 cases.Results Eight patients were all male,with average age of 27.4 years old.The main clinical characteristics included myalgia in 4 patients,muscle weakness in 2 patients,fever in 5 patients,and tea-colored urine in 3 patients.One patient had acute renal failure,and 2 patients developed multiple organ failure syndromes and disseminated intravascular coagulation.One muscle biopsy done in the third day after the onset showed obvious muscle necrosis without inflammatory infiltrates,while the other 2 muscle biopsy done 2 weeks after the onset showed muscle necrosis companied by inflammatory phagocytic response.Six patients full recovered,1 patient partially recovered and one patient died.Conclusions Rhabdomyolysis caused by exertional heat stroke predominantly occurred in males.The main clinical features include muscle pain,weakness,significantly elevated serum creatine kinase and myoglobin level and myoglobinuria.Muscle pathology indicated muscle necrosis in the early stage and accompanied inflammatory infiltrates in the late stage.Most patients will get recovered with prompt diagnosis and treatment while the severe cases can be life-threatening.

Objective To explore clinical and pathological characteristics of neurological lesions in primary Sj(o)gren's syndrome (pSS).Methods Ten patients diagnosed as pSS with nervous system involvement were analyzed retrospectively with autoimmune antibody test,cerebrespinal fluid examination and Schivmer's test,six pSS patients with peripheral nervous system lesions (PNS-pSS) were examined with electromyography and nerve conduction velocity and four pSS patients with central nervous system lesions (CNS-pSS) were examined with cranial MRI to analyze their clinical and pathological characteristics.Salivary gland biopsy was performed for all the ten patients,sural nerve biopsy for those with PNS-pSS and brain biopsy or spinal autopsy for those with CNS-pSS.Results Clinically,six patients with PNS-pSS presented symmetrical sensory-motor peripheral neuropathy or pure sensory peripheral neuropathy and four patients with CNS-pSS presented multiple sclerosis,subacute transverse myelitis,encephalitis,pituitary stroke and acute meningitis with varied lesions in the myelin sheath and axon by electromyography and motor nerve conduction velocity.Cranial MRI showed lesions in the white matter of the brain,spinal cord and abnormal signals in the pituitary.Pathologically,sural nerve biopsy showed typical vasculitis and nonvasculitis characterized as degeneration of the axon and myelin sheath,salivary acinar gland biopsy showed its atrophy and infiltration with lymphocytes,and brain biopsy showed demyelination of the brain and spinal white matter,as well as infiltration of lymphocytes surrounding the veins,typical vasculitis and bleeding and necrosis of the pituitary.Conclusions Both central and peripheral nervous systems can be involved in pSS,with complicated clinical manifestations.Electrophysiology,cerebrospinal fluid tests and histopathological examinations by biopsy are essential in diagnosis for neurological lesions in patients with pSS.Inflammatory reaction of the blood vessels and tissues mediated by cell immunity may be involved in mechanism of its neurological lesions.

AIM: To study factors affecting the stability of nylon monofilament for intraluminal middle cerebral artery occlusion in rats.METHODS:Successful rates and infarct volume of ischemic model were compared by polyvinyl alcohol (PVA) coated and silicon coated nylon monofilament intraluminal occlusion of middle cerebral artery under condition of ligation and without ligation of peterygopalatine artery.RESULTS:The successful rates were 60% and 53% in two groups under condition of ligation of peterygopalatine artery, and 20% and 27% in other two groups under condition of without ligation of peterygopalatine artery respectively. The intracranial length of nylon monofilament were about 7 mm in successful model and 4 mm in failure model. Animals in the PVA coated nylon monofilament group showed neurological dificit signs earlier, and had a significantly larger infarct volume at 12 hours of ischemia than those in the silicon coated group.CONCLUSIONS:The ligation of peterygopalatine artery is critical in the success of making this kind of ischemic model. The appropriate position of nylon monofilament entering crania and the good expansibility of PVA play an important role in occlusion of blood flow. Because of the relatively lower successful rate, a new substitute with unique quality, proper hardness and better elasticity needs to be explored in the future.