Objective To evaluate the clinical,neuroimaging and electrophysiology features of 62 patients with multiple system atrophy(MSA).Methods Sixty-two cases with diagnosis of probable MSA were recruited in a retrospective studied.Clinical,neuroimage and external anal sphincter electromyography (EAS-EMG)data was retrospectively analyzed.Results In 62 cases(44 male and 18 female),the onset age was between 37 and 76.Among them,29 cases(46.8 % )were MSA-A,with orthostatie hypotension as the main clinical manifestation;24 cases(38.7 % )were MSA-C,with cerebellar ataxia ag the main chnical manifestation;9 cases(14.5 % )were MSA-P,with extrapyramidal symptoms as the main clinical manifestation.MRI showed that main lesion of MSA-A was in the cerebellum:that of MSA-C was in the cerebellum,pons and medulla;and that of MSA-P was in the putamen.Fifty-one cases did EAS-EMG and 46 cases showed neurogenie impairments.Nineteen cases were initially misdiagnosed with other diseases.Conclusions MSA is easy to be omitted or misdiagnosed at early stage.The diagnostie rate of MSA can be increased by the combination of clinical expressions,neuroimage,EAS-EMG and other necessary examinations.

Objective To investigate the clinical, electrophysiological and pathological features of critical illness polyneuropathy and myopathy (CIPNM). Methods The clinical outcomes, electromyogram Results as well as pathological features in nerves and muscles of 3 patients with CIPNM were investigated and analyzed. Results 3 patients were all provided with assisted respiration after tracheal intubation. 7～10 d after intubation, all the patients emerged muscle strength and tendon reflexes of extremities weakening; while 14 days after that, 2 patients appeared amyotrophy of extremities. Electromyogram showed that the conduction of many motor and sensory nerves for extremities decreased, while the amplitude of compound muscle action potential (CMAP) of part of motor nerves decreased. Biopsy for nerves revealed decreased medullated nerve fibers and regeneration phenomenon of auxiliary fibers; while that for muscles showed neuralgic damage and myopathy-like changes. Conclusion CIPNM can complicate after tracheal intubation. The electrophysiological and pathological examinations for nerves and muscles can be helpful for the diagnosis.

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Biopsy, Fine-Needle ; adverse effects ; methods ; Child ; Diagnosis, Differential ; Diagnostic Errors ; Female ; Hematoma ; etiology ; Humans ; Male ; Middle Aged ; Thyroid Neoplasms ; diagnosis ; pathology ; Thyroid Nodule ; pathology ; Young Adult

Objective To compare the clinical characteristics of multiple sclerosis ( MS) and neuromyelitis optica (NMO) for better diagnosis and differential diagnosis of them. Methods The characteristics of 40 MS and 38 NMO cases were retrospectively studied on clinic manifestations, electroneurophysiology,some laboratory indices, imaging characteristics and so on. Results The ratios of male to female were 1: 1. 35 and 1:4. 43 respectively in patients with MS and NMO, so patients with NMO were more likely to be female as compared with MS ( P < 0. 05 ). The mean onset age was ( 35. 5 ±13. 9 ) years in MS patients and (30. 6 ± 15. 6) years in NMO patients, but no significant difference was found (P>0. 05). The cases of visual acuity ≤0.1 in patients NMO was 13, which of MS was merely 1. The cases of visual acuity less than 0. 5 after treatment in NMO patients was 19, which in MS was only 1. The cases of cognitive impairment in NMO was 3, which of MS was 10. The cases of cerebrospinal fluid oligoclonal bands in MS was 16, which in NMO patients was 9. The lesions of spinal cord shown in MRI of MS patients were typically oval, peripheral and asymmetric, but those in NMO patients extended longitudinally and converged centrally. The mean number of involved vertebral segments in NMO patients was significantly greater than that in MS patients ( 6. 6 vs 2. 2, P < 0. 01). Furthermore, the number of spinal cord lesions in MS patients was alse remarkably greater than that in NMO patients (2. 0vs1.2, P <0. 01). Conclusions NMO may be a distinct clinical entity, which is likely to be differentiated from MS by its tendency to affect women, younger age at onset, and other features clinical manifestations, electroneurophysiology, laboratory parameters, neuroimaging show..

Computed tomography (CT) is considered the most sensitive method for the detection of intraocular foreign bodies (IOFBs).The purpose of this study was to evaluate a new method of 3-dimentional (3D) localization of IOFBs that takes advantage of the anatomical structure of the optic nerve and to assess the clinical outcomes using this new method.Twenty-two trauma patients with IOFBs or suspected IOFBs admitted to our hospital were scanned with multislice CT (MSCT) between July and December 2003.All scanning was performed with a 16-row spiral CT in axial plane using a sequential scanning protocol.During the scanning,the eyeball of the patient was kept stable and was not allowed to rotate internally or externally.Section collimation was set at 16 mm × 0.75 mm.Table feed was 12 mm.Reconstruction index was 0.75 mm.After scanning,the reconstructed images were loaded into a workstation to create the multiplanar reconstruction images with the aid of the 3D software.We compared the localization results with the operative findings.Multiplanar reconstruction images showed IOFBs in all 22 patients.IOFBs occurred in the eyeball of 14 patients,in the wall of the eyeball of 5 patients and in the posterior orbits of 3 patients.Different surgical procedures were designed according to the localization by this new method and all IOFBs were successfully removed.All of these foreign bodies were metallic and the localization of IOFB using MSCT was consistent with that found by operative findings.It was suggested that MSCT is a simple and effective imaging modality for the localization of IOFBs.In our study,we localized the IOFBs more quickly and accurately by taking advantage of the fixed position of the intraocular segment of the optic nerve,and determined the necessary surgical parameters.

Objective To explore the value of brain CT scanning for distinguishing tumor-like inflammatory demyelinating diseases (TIDD) from glioma or primary central nervous system lymphoma.Methods The brain CT features in 20 patients with TIDD(10 female,10 male;mean age (35.6±14.0)years;range,6-51 years)and 32 gliomas(16 female,16 male;mean age(42.0±19.8)years;range,12-75 years)and 6 lymphomas(3 female,3 male;mean age(53.8±11.8)years;range,32-68 years)were retrospectively reviewed and compared between brain tumors and TIDD.Results (1)Among the 38 primary brain tumors,there were 19 cases(50%,14 gliomas,5 lymphomas)with hyperdense lesions,10 cases(26.3%,9 gliomas,1 lymphomas)with isodense lesions,and 9 glionms (23.7%)with hypodense lesions.In contrast,the brain unenhanced CT manifestation of 20 TIDD all showed with hypodense lesions.(2)On unenhanced CT the lesions of 6 lymphomas all were hyperdense or isodense,like 90% of 20 high grade gliomas(WHO grade Ⅲ and Ⅳ),but this rate for grade Ⅱ was only 41.7%.(3)According to the shape of hyperdense lesions of the 19 primary brain tumors with,7 cages(6 gliomas,1 lymphomas)manifested with asymmetric hyperdense small-patches,4 cases(1 gliomas,3 lymphomas)with symmetric hyperdense large-patches,4 cases(3 gliomas,1 lymphomas)with diffused hyperdensed lesions,and 4 cases(4 gliomas)with ring-shaped hyperdensed lesions.Furthermore,4 primary brain tumors(4 lymphomas)underwent CT enhanced scanning and all the cases showed strong enhancement(3 cases with hyperdense lesions and 1 with isodense lesions on unenhanced CT),but only 3 cases of 7 TIDD showed mild enhancement in contrast.(4)By Spearman's relevant analysis,hyperdense and isodense on unenhanced CT was proved to have significant positive correlation between the grade of gliomas(r=0.435,P=0.013).Therefore,the frequency of hyperdense and isodense lesions in lymphomas and WHO grade Ⅲ and Ⅳ astrocytoma was higher in contrast with low grade astrocytoma.Conclusions Brain CT as a simple,economical and practical examination method has significant meaning for differentiating TIDD from glioma or PCNSL and could be used as an adjuvant method for MRI and magnetic resonance spectroscopy.Patients with hyperdense or isodense on unenhanced CT or strong enhancement could be excluded from TIDD.

Objective To investigate specific immune responses in mice induced by recombinant major outer membrane protein(rMOMP)of C.trachomatis serovaf E.Methods Thirty-six female BALB/cmice aged 3 to 4 weeks Were divided into three groups.i.e.,adjuvant group vaccinated、with purified rMOMP and Freund's adjutant,solitary group vaccinated with rMOMP only and control group vaccinated with phosphate buffered saline(PBS).All the mice were intramuscularly vaccinated on week 0,2 and 4.Blood samples and vaginal washes were obtained from these mice on week 6,then,mice were challenged with elementary body(EB)of C.trachomatis serovar E at the footpad followed by the observation of delayed hypersensitivity.On week 7.mice were genitally infected with C.trachomatis EB;one week later,blood samples and vaginal washes were obtained again;six weeks later,spleen lymphocytes were isolated from the mice and stimulated bv C.trachomatis or ConA followed by the detection of cell proliferation with MTT assay.In vitro neutralization assay was also performed.ELISA was used to determine the titers of Chlamydia-specific IgO antibody in sera and IgA antibody in vaginal washes,as well as the level of IFN-γ in culture supernatant of lymphocytes and sefa of mice.Vaginal swabs were collected after genital challenge and subjected to C.trachomatis culture.Results The absorbance at 405 ms of Chlamydia-specific IgG antibody and proliferation index of lymphocytes were 0.641±0.059 and 5.085±1.291.respectively,in mice immunized with rMOMP and Frennd's adjuvant.significantly higher than those in mice immunized with rMOMP only(0.424±0.015 and 3.123 ±0.840.both P<0.05).The thickness of right hind footpad increased by 0.324±0.054 mm and 0.272±0.064 mm,respectively,in solitary group and adjuvant group,respectively,with significant difference between the two groups(P<0.05).A significant increase was also observed in the adjuvant group compared with the control group in the above three parameters(all P<0.01).Conclusion The rMOMP of C.trachomatis could efficiently induce Chlamydia-specific humoml and cellular immune responses in mice.

Objective To report the clinical and pathological characteristics of one patient with glycogen storage disease Ⅳ (Anderson disease). Methods The patient was received detailed clinical examinations, ultrasound, electromyography, head MRI and muscle biopsy. Results The onset of the 22 years old male patient was 7yrs. The main symptoms were intolerance and fatigue in proximal limbs muscular movement, cardiopalmus by chance. Abdominal ultrasound examinations showed cirrhosis, portal hypertension, splenomegaly. Echocardiogram showed left ventricular myohypertrophia, mild mitral and tricuspid valve insufficiency. Electrophysiology study revealed widespread myogenic changes. Cranial MRI, MRA and MRS were normal. Muscle biopsy showed basophilic intracytoplasmic material in a lot of fibers deposits, which was intensively PAS-positive material and partially resistant to diastase digestion. In the electron microscope, the storage material consisted of filamentous and finely granular material. Conclusions There was the first case of glycogen storage disease Ⅳ reported in our country, mainly involved skeletal muscle, liver, spleen and cardiac muscle.

Objective To investigate the pathological changes and pathogenesis of the MELAS syndrome (mitochondrial encephalopathy lactic acidosis strok-like episodes ) by using the method of immunohistochemical staining in the brain biopsy specimens with anti-mitochondrial antibody (AMA).Methods We performed immunohistochemical staining in 3 confirmed MELAS patients' paraffin-imbed brain biopsy specimens.Results Small vessel proliferation and the uneven thickness of the wall were found in the 3 MELAS patients.A lot of brown deposits was shown in the wall of small vessels and also noted in neurons.Conclusions The main pathological change in the MELAS brain biopsy immunohistochemical staining with AMA was the small vessel proliferation,indicating that abnormal mitochondria accumulated in the vascular smooth muscle,endothelial cell and neurons of the lesion sites.This finding was consistent with the electron microscopic discovery and valuable for the diagnosis of MELAS.

Objective To investigate the special expression and diagnostic value of anti-mitochondrial antibody (AMA) in the muscle tissues of patients with mitochondria encephalopathy, lactic acidosis and stroke-like episodes (MELAS). Methods All cases including 10 cases of MELAS, which were diagnosed definitely by clinical, pathological and DNA analysis, and 2 cases of normal control and 3 cases of necrotic muscle fibers, underwent muscle biopsies in order to carry out AMA staining. At the same time the original MGT staining was observed. Results A large number of ragged brown fibers (RBF) were observed under light microscopy with AMA immunohistochemical staining in the muscle tissues of patients with MELAS. But no RBF were observed in control groups. Conclusion RBF might be the specific performance of paraplastic mitochondrial, which was similar to the ragged red fibers, so RBF might be a pathological index in evaluating abnormal mitochondrial function and in diagnosing mitochondriopathy.