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 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.

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 summarize the clinical features, neuroimaging findings and pathological characteristics of 26 patients with tumor-like inflammatory demyelinating diseases (TIDD) confirmed by histopathology for better diagnosis and differential diagnosis. Methods The clinical features, neuroimaging findings and pathological characteristics of 26 patients (14 male, 12 female) with pathologically proven TIDD(24 brain-type and 2 spinal cord-type ) were retrospectively analysed. Results The mean onset age was 6-69 (36.7±13.8) years. Twenty-one patients had good prognosis with a median followed-up duration of 51.0 months. Two patients were died of post-operative complication and pulmonary infection respectively and the remaining 3 patients were lost to followed up. The TIDD patients almost showed monophasic clinical setting. Headache, indifference accompanied with hypomnesis were the commonest initial symptoms. The positive or abnormol rates of cerebrospinal fluid oligoclonal bands (OCB) and myelin basic protein (MBP)in TIDD patients were high. The involvements of bilateral and multi-lesions were commonest in TIDD (61.5%, 65.4% respectively). Twenty-two patients with CT unenhanced scanning showed hypodense lesions. Long T1 and long T2 signal intensity was showed on MRI and most cases apeared round-like lesion in shape. Acccording to the shape of enhancement of the 23 patients performed with contrast agents, 11 were shown with open-ring enhancement, 4 cases (including 2 accompanied with open-ring enhancement) with complete ring enhancement, 3 with asymmetrical dotted enhancement, 2 with diffused even enhancement,and no enhancement was seen in the other 6. Furthermore, 14 cases with DWI and 12 with FLAIR all appeared hyperdensity. The typical pathological changes were demyelinating, perivascular inflammatory cells infiltration and reactive gliosis. Occationally, the Creutzfeuldt cells were also found in brain tissue of some patients. Conclusions TIDD is a distinct demyelinating disease entity. In spite of being apt to be confused with the neoplasm in brain and spinal cord. TIDD has its own-features, for example, OCB is frequently positive in patients with TIDD and the level of MBP may be significantly increased. Furthermore, the involvements of bilateral and multi-lesions are the common in TIDD, and most cases showed open-ring enhancement or complete rim enhancement on MRI. In addition, all cases present hypodense lesions on unenhanced CT and patients with hyperdense seemed not to be considered as TIDD.

Objective To clone, express and purify Chlamydia trachomatis polymorphic membrane protein (Pmp G), and to identify its immunogenicity. Methods The Pmp G gene of C. trachomatis serotype E was amplified by PCR, cloned into prokaryotic expression vector PET30a (+). The positive recombinant was transformed into the bacterium E coli (BL-21), identified by enzyme digestion, PCR amplification and gene sequencing. Then, it was induced to express followed by the identification of expression product with SDS-PAGE and Western blotting. The purified protein was used to immunize BALB/C mice to test its immunogenicity. Results PCR produced a 1092 bp-sized DNA fragment, which had a sequence consistent with that of PmpG gene of C. trachomatis E type in the GenBank database. The molecular weight of expression product was 55 kD, which was proved to be the expected size, and Western Blotting confirmed it to be the specific protein. Moreover, special antibodies to PmpG were induced to be generated by mice immunized with the purified protein. Conclusions The constructed prokaryotic expression vector for PmpG is expressed successfully in E. coli, and the expression product shows immunogenicity.

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..

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.