Objective To investigate the changes in regional cerebral blood flow and its relationship with atrophy of the entorhinal cortex in patients with amnestic mild cognitive impairment (aMCI) and Alzheimer's disease (AD).Methods Twenty eight patients including ten cases with aMCI,nine case with mild AD and nine cases with moderate to severe AD were enrolled.The cognitive function was evaluated by revised version of Hasegawa's Dementia Scale (HDS-R) and Alzheimer's Disease Assessment Scale-cognitive part (ADAS-cog).Regional cerebral blood flow was evaluated by 99mTc-ECD SPECT and easy Z score imaging system (eZIS).Z scores of voxel-based specific regional analysis system for Alzheimer's disease (VSRAD) were used to assess the degree of atrophy of the entorhinal cortex.Results The Z-score in eZIS was lower in mild to severe AD group than in aMCI group [(1.57±0.46),(1.50±0.54) vs.(1.00±0.50),F=4.58,P=0.022],which showed that the regional cerebral blood flow of cingulate was lower in AD group than in aMCI group.The Z-score in eZIS in bilateral frontal lobes and left hippocampus were higher in moderate to severe AD group than those in aMCI group than in aMCI and mild AD group respectively [(1.43± 0.79) vs.(0.67±0.50),(0.88±0.64); (1.43±0.79) vs.(0.67±0.50),(0.75±0.46); (1.86± 0.50) vs.(1.33±0.50),(1.75±0.46),F=2.90,3.81 and 3.06,respectively,P=0.077,0.039 and 0.068],which showed that the regional cerebral blood flow of bilateral frontal lobes and left hippocampus were lower in moderate to severe AD group than in aMCI group.The score of HDS-R had a negative correlation (r =-0.568,-0.481,P=0.004,0.017) and the score of ADAS-cog had a positive correlation (r=0.462,0.459; P =0.030,0.032) with regional cerebral blood flow in hippocampus.Conclusions The regional cerebral blood flow of cingulate gyrus is decreased in early stage of AD,and involves in frontal lobe and hippocampus along with the deterioration of diseases.The changes of cognitive function are correlated with cerebral blood flow in hippocampus.The Z-score in eZIS can effectively evaluate the changes of regional cerebral blood flow in aMCI and AD patients.

Objective To determine whether sleep structures,the waveform components of NREM and rapid eye movement (REM) sleep, and the relationship between sleep variables and mental status are altered. Methods Totally 16 Alzheimer's disease(AD) patients and 16 age-matched controls were studied for two consecutive nights.All cases were video-monitored during the recordings of electroencephalography,electro-oculography,electrocardiography,electromyography in chin,and nasal airflow. Results (1) Compared with normal controls,duration of total sleep ( F =6.30, P =0.017 9),stage Ⅱ sleep ( F =16.03, P =0.000 4),and REM sleep ( F =9.84, P =0.002 1) were remarkably decreased,but waveform components of slow-wave (stage Ⅲ-IV) sleep ( F =11.50, P =0.004) were increased in AD patients.(2) Sleep spindle duration and density,and its relative and absolute power,and K complex density ( F =13.64～79.11, P

Objective To study clinical and electrophysiological features of multifocal motor neuropathy (MMN). Methods The clinical features and data of electrophysiological examination from 16 patients with MMN were analyzed retrospectively.Results In the present study, the onset age was 14 to 47 years with the mean age of (28.2?2.4) years. All the 16 patients presented with unsymmetrical weakness of extremities (Ⅱ～Ⅳ grade of muscle strength) and no hypoesthesia. More serious weakness was found in the upper limbs and in the right extremities. 4 cases were accompanied with mild amyotrophy. Electromyography showed segment conduct block (CB) and slow down of conduct velocity of motor nerves in all 16 patients. Slow conduct velocity of sensory nerve was found only in one case.Conclusions MMN is an unsymmetrical peripheral neuropathy, which affects mainly on motor nerve. Nerve electrophysiology plays an important role in diagnosis of MMN and CB is the characteristic manifestation of nerve electrophysiological change in MMN.

Churg-Strauss Syndrome

Objective To study the clinical and imaging characteristics of Chinese atopic myelitis (AM) patients.Methods Three diagnosed AM patients were retrospectively analyzed for the clinical data,serum IgE level,antigen specific IgE,cerebrospinal fluid,spinal MRI and therapeutic efficacy profiles.Results All the three patients were male and presented as subacute AM with the onset at 25,47 and 49 years old respectively.Two patients were allergic to pollen and other drugs,while another patient suffered from allergic rhinitis.Elevated serum total IgE and mite antigen specific IgE were found in all cases.Paraesthesia in limb extremities and positive Lhermitte sign were the main clinical features,while no optic,motor,urinary and defecation disturbance were found.Oligoclonal banding of cerebrospinal fluid and serum aquaporin 4 (AQP4) antibody were both negative in all cases.Spinal MRI showed lesions were hypointense on T1 and hyperintense on T2 at the posterior column of T2-3 segment with abnormal enhancement in case 1,hypointense on T1 and hyperintense on T2 at C2/3 segment with mild swelling in case 2 and hypointense on T1 and hyperintense on T2 at C3-5 segments with swelling and abnormal enhancement in case 3.Vitamin B were used in one patient,while the other two patients improved after the treatment with high-dose corticosteroids.Conclusions Subacute myelitis predominantly presents as paraesthesia in limb extremities with elevated serum total IgE and mite antigen specific IgE,while severe motor disorders are rare.Swelling and abnormal enhancement lesions at the posterior column of cervical cord are the common imaging features.Treatment with corticosteroids is recommended to be sustained for 3-6 months.

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Objective To study the role of human carcinoembryonic antigen-related cellular adhesion molecule 1 (hCEACAM1)in mediating the specific adhesion of N. gonorrhoeae to its human host cells.Methods A recombinant eukaryotic expression vector pCDPGICEA1 was constructed by putting hCEACAM1 cDNA behind both hCD46 promoter and rabbit β-globulin intron 2,and with which,the COS-1 cells were transfected. Following G418 selection, the COS-1 cells expressing hCEACAM1 were sorted out with flow cytometry. The adhesion of N. gonorrhoeae to the gene transfected COS-1 cells was analyzed with bacterial binding assay. Results hCEACAM1 cDNA could be expressed effectively under the direction of hCD46 promoter and rabbit β-globulin intron 2,and N. gonorrhoeae could adhere to COS-1 cells expressing hCEACAM1. Conclusion hCEACAM1 can mediate the adhesion of N. gonorrhoeae to animal originated COS-1 cells. thus its transgenic mice may be used as a novel animal model for studying N. gonorrhoeae infection.

Intravenous thrombolysis is the only treatment that has the evidence of evidence-based medicine in acute ischemic stroke. However, the narrow time window has limited the treatment opportunities of many patients. Transcranial ultrasound thrombolysis is a very promising thrombolysis-assisted method, and transcranial ultrasound plus microbubble-assisted thrombolysis is the research hotspot in recent years. At present, more suitable ultrasonic instruments for thrombolysis and a number of ways of ultrasound-assisted thrombolysis are being developed.

Objective To investigate the early onset of learning and memory function of 4-month-old APP/PS1/Tau Alzheimer' s disease (3×Tg-AD) model mice and explore the pathogenesis of AD in early stage through evaluating neuron excitability and BKCa channel activity in cingulate cortex pyramidal cells.Methods Ten 4-month-old male 3×Tg-AD mice and matched ten wild type (WT) mice.Behavior was tested with the novel object recognition task to observe the ability of learning and memory.Whole-cell patch-clamp recordings were performed to assess the excitability of cingulate cortex pyramidal cells in terms of resting membrane potential and frequencies of spikes evoked by current injection.A train of five pulses of depolarizing currents were injected at 100 Hz to assess the spike width,which was used as an index for BKCa channel activity.Results Compared with the WT group (0.72±0.03),the novel object recognition index significantly decreased in 3 × Tg-AD group (0.55 ± 0.04) (P =0.004).Compared to the WT group((-66.03±0.43) mV),the resting membrane potential in cingulate cortex neurons of 3×Tg-AD group((-62.31±0.54)mV) was significantly depolarized(P=0.000).In contrast to WT group,the action potential firing frequencies evoked by depolarizing current injections were higher in neurons from 3×Tg-AD group(P=0.000),demonstrating that excitability of cingulate cortex neurons was elevated by intracellular Aβ.Spikes were broader in the 3×Tg-AD group than those in the WT group(P＜0.01).Suppression of BKCa channels in cingulate cortex neurons from the 3×Tg-AD group was confirmed on the basis of the spike half-width,since BKCa channels affect the descending phase of spikes.Conclusion Compared to WT mice,4-month-old 3×Tg-AD mice are impaired in learning and memory.The suppression of BKCa channels by intracellular Aβ leads to increase of excitability in cingulate cortex pyramidal cells.

Objective To summary the pathological and genetic features in nine Chinese limb girdle muscular dystrophy 2I (LGMD2I) patients.Methods Nine LGMD2I patients were recruited from Peking University First Hospital between 2011 and 2016, who came from nine unrelated and non-consanguineous families.The mean age of onset was (8.2±5.2) years (2 to 19 years), and the mean disease duration was (10.4±6.1) years (1 to 24 years).There were six males and three females, present with weakness in limb girdle muscles, four of whom accompanied with calf hypertrophy and three with scapular winging.Serum creatine kinase was 964-23 131 U/L (normal 25-190 U/L).Five of them who conducted electromyogram showed myogenic pattern.Muscle biopsy and next generation sequencing were performed in these patients, then sanger sequencing was performed to determine whether the variants co-segregated with the phenotype in these families.Results Muscle biopsy revealed myopathy features in six patients, dystrophic change in one, and only mild changes in two.Major histocompatibility complex-Ⅰ was positive in six cases, and rimmed vacuoles were found in two.There were seven mutations in fukutin-related protein (FKRP) gene.A reported mutation of c.545A>G (p.Y182C) appeared in eight cases, including three homozygotes and five compound heterozygotes.The mutation of c.1067T>C (p.Ile356Thr) was reported too.And c.1263C>A (p.Tyr421X), c.534G>T(p.Thr178Cys), c.1027G>C (p.Glu343Gln), c.1027G>T(p.Glu343X), c.1448A>G (p.Tyr483Cys) were found to be novel mutations.Conclusions LGMD2I showed large variation in myopathology.The missense mutation A545G(Y182C) is a hot spot of FKRP gene in our series.